A Study in Japanese Children With Short Bowel Syndrome Who Completed SHP633-302

A Prospective, Open-label, Long-term Safety and Efficacy Study of Teduglutide in Japanese Pediatric Subjects With Short Bowel Syndrome Who Completed SHP633-302

Status

Completed

Phases

Phase 3

Study type

Interventional

Source

ClinicalTrials.gov

Registry ID

NCT03268811

Enrollment

Registered

2017-08-31

Start date

2017-08-23

Completion date

2021-11-02

Last updated

2022-05-24

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Short Bowel Syndrome

Brief summary

The purpose of this clinical study is to evaluate the long-term safety and tolerability of teduglutide treatment in Japanese pediatric participants with short bowel syndrome (SBS) who completed Study SHP633-302 (NCT02980666).

Interventions

DRUGTeduglutide

Teduglutide 0.05 mg/kg SC injection will be administered once daily into 1 of the 4 quadrants of the abdomen or either thigh or arm.

Study design

Allocation

Intervention model

SINGLE_GROUP

Primary purpose

TREATMENT

Masking

NONE

Eligibility

Sex/Gender

ALL

Healthy volunteers

Inclusion criteria

* Informed consent by a parent or guardian prior to any study-related procedures. * When applicable, informed assent (as deemed appropriate by the Institutional Review Board \[IRB\]) by the participant prior to any study-related procedures. * Participant completed Study SHP633-302 (NCT02980666). * Participant (and/or parent/guardian) understands and is willing and able to fully adhere to study requirements as defined in this protocol.

Exclusion criteria

There are no

Design outcomes

Primary

Measure	Time frame	Description
Change From Baseline in BMI for Age Z-score at EOT of Cycle 9 During Teduglutide Treatment	Baseline, EOT of Cycle 9 (up to 196 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 9 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment	Baseline, EOT of Cycle 4 (up to 76 weeks)	Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Body Mass Index (BMI) for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment	Baseline, EOT of Cycle 1 (up to 24 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment	Baseline, EOT of Cycle 2 (up to 48 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment	Baseline, EOT of Cycle 3 (up to 72 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment	Baseline, EOT of Cycle 4 (up to 96 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 5 During Teduglutide Treatment	Baseline, EOT of Cycle 5 (up to 120 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 5 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 6 During Teduglutide Treatment	Baseline, EOT of Cycle 6 (up to 144 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 6 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 7 During Teduglutide Treatment	Baseline, EOT of Cycle 7 (up to 168 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 7 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in BMI for Age Z-score at EOT of Cycle 8 During Teduglutide Treatment	Baseline, EOT of Cycle 8 (up to 192 weeks)	BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 8 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants With Treatment-emergent Adverse Events (TEAEs)	From Baseline up to follow-up (up to 50 months)	An Adverse Event (AE) was any untoward medical occurrence in a clinical investigation participant administered a pharmaceutical product and that does not necessarily have a causal relationship with this treatment. TEAEs were defined as any AEs whose onset occurred, severity worsened or intensity increased after receiving the investigational product (IP) in the core study (SHP633-302 \[NCT02980666\]) or this extension study. Number of participants with TEAEs were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants With Clinically Significant Abnormalities in Vital Signs	From Baseline up to follow-up (up to 50 months)	Vital sign assessments included pulse rate, blood pressure (systolic and diastolic blood pressure) and body temperature. Number of participants with clinically significant abnormalities in vital signs which were deemed clinically significant by the investigator were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	From Baseline up to follow-up (up to 50 months)	Clinical laboratory parameters included biochemistry, hematology and urinalysis. Number of participants with clinically significant abnormalities in laboratory parameters which were deemed clinically significant by the investigator were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Average Total Urine Output at End of Treatment (EOT) of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Average total urine output was recorded over a 48-hour period of parental support (PS) stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The Average daily urine output milliliter per kilogram per day (mL/kg/day) was calculated as: (Total urine output over 48 hours / 2) / body weight (kilogram \[kg\]) where total urine output was calculated as the sum of the urine output in milliliter (mL) and the urine-only diaper weights in gram (g) (1g = 1mL) for the participant collected on the output diary form of electronic case report from (eCRF). Percent change from baseline in average total urine output at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Average Number of Stools Per Day at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized by the average number of stools per day. The average number of stools per day was calculated as (sum of the daily data in a 48-hour period/2). Percent change from baseline in average number of stools per day at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Average Stool/Mixed Stool Diaper Weight at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized by the average stool/mixed stool diaper weight (gram per kilogram per day \[g/kg/day\]). The body weight was used to calculate the daily stool/mixed stool diaper weight (g/kg/day). Percent change from baseline in average stool/mixed stool diaper weight at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Average Total Ostomy Output at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The body weight was used to calculate the average total ostomy output per day (mL/kg/day) using a formula analogous to that used to calculate the average daily urine output. Percent change from baseline in average total ostomy output at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Average Bristol Stool Form Score at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized the average typical stool form score using Bristol Stool Form Scale. The average typical stool form score was calculated as (sum of the daily data in a 48-hour period / 2). Typical Stool Form based on Bristol Stool Form Scale: 1 - Separate hard lumps, hard to pass, 2 - Sausage-shaped, but lumpy, 3 - Like a sausage but with cracks on the surface, 4- Like a sausage or snake, smooth and soft, 5- Soft blobs with clear-cut edges, 6- Fluffy pieces with ragged edges, a mushy stool, 7- Watery, no solid pieces. Entirely liquid. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]). A negative change from baseline indicates improvement.
Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Number of participants with ADAs (including NAbs) to teduglutide were used to summarize the presence of antibodies. The participants who tested positive only for ADAs (including NAbs) were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants With Clinically Significant Changes in Gastrointestinal (GI) Specific Testing	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	GI specific testing included colonoscopy or sigmoidoscopy, abdominal ultrasound, fecal occult blood testing, upper GI series with small bowel follow-through (UGI/SBFT). Number of participants with clinically significant changes findings in gastrointestinal specific testing were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Body Weight For Age Z-Score at EOT of Last Cycle During Teduglutide Treatment	Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Body weight was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in body weight for age Z-Score at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 1 During Teduglutide Treatment	Baseline, EOT of Cycle 1 (up to 24 Weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-Score at EOT of cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 2 During Teduglutide Treatment	Baseline, EOT of Cycle 2 (up to 48 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 3 During Teduglutide Treatment	Baseline, EOT of Cycle 3 (up to 72 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 4 During Teduglutide Treatment	Baseline, EOT of Cycle 4 (up to 96 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 5 During Teduglutide Treatment	Baseline, EOT of Cycle 5 (up to 120 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 5 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 6 During Teduglutide Treatment	Baseline, EOT of Cycle 6 (up to 144 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 6 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 7 During Teduglutide Treatment	Baseline, EOT of Cycle 7 (up to 168 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 7 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 8 During Teduglutide Treatment	Baseline, EOT of Cycle 8 (up to 192 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 8 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Height For Age Z-Score at EOT of Cycle 9 During Teduglutide Treatment	Baseline, EOT of Cycle 9 (up to 196 weeks)	Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 9 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment	Baseline, EOT of Cycle 1 (up to 24 weeks)	Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment	Baseline, EOT of Cycle 2 (up to 48 weeks)	Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment	Baseline, EOT of Cycle 3 (up to 72 weeks)	Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Secondary

Measure	Time frame	Description
Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent reduction in weight-normalized prescribed PS values from baseline was calculated using the formula: % reduction in PS value at the visit =(\[average daily value at the scheduled visit - average daily value at baseline of the core study (SHP633-302 \[NCT02980666\]) / average daily value at baseline of the core study (SHP633-302 \[NCT02980666\])) \*100. Number of participants who achieved at least 20% reduction from baseline in Investigator prescribed PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Change from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent change from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Change from baseline in investigator prescribed PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent change from baseline in investigator prescribed PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Enteral autonomy (completely weaned off PS) was defined as the first visit where there is no use of PS for the 7 days prior to the visit and there is no prescribed PS at that visit, and the participants remains off PS for the remainder of the treatment period of that cycle. Number of participants who achieved enteral autonomy off PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Days per week of diary PS was calculated as: Days per week of actual PS = (number of days with non-zero values for PS volume within the 7 days prior to the visit / number of days for which any PS intake data was recorded within the 7 days prior to the visit) \* 7. Change from baseline in days per week of diary PS usage at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	Change from baseline in days per week of prescribed PS usage at EOT of each cycle during teduglutide treatment was reported. Days per week of diary PS was calculated as: Days per week of actual PS = (number of days with non-zero values for PS volume within the 7 days prior to the visit / number of days for which any PS intake data was recorded within the 7 days prior to the visit) \* 7. Change from baseline in days Per week of prescribed PS usage at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).
Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)	PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent reduction in weight-normalized diary PS values from baseline was calculated using the formula: % reduction in PS value at the visit = (\[average daily value at the scheduled visit - average daily value at baseline of the core study (SHP633-302 \[NCT02980666\]) / average daily value at baseline of the core study (SHP633-302 \[NCT02980666\])) \*100. Number of participants who achieved at least 20% reduction from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Countries

Japan

Participant flow

Recruitment details

The study was conducted at 6 centers in Japan from 23 August 2017 (first participant first visit) and 02 November 2021 (last participant last visit).

Pre-assignment details

A total of 9 Japanese pediatric participants who completed Study SHP633-302 (NCT02980666) were enrolled into the extension study based on age of participants i.e., 7 children (aged 1 through 15 years of age) and 2 infants (aged 4 months through \< 12 months of corrected gestational age).

Participants by arm

Arm	Count
Total Children (Aged: 1 to 15 Years) Participants aged from 1 through 15 years who participated in the core study (SHP633-302 \[NCT02980666\]) were enrolled into this extension study and received teduglutide 0.05 mg/kg SC injection once daily for 24 weeks in each treatment cycle depending on the disease course.	7
Infants (Corrected Gestational Age: 4 to < 12 Months) Participants (Infants) from 4 through \< 12 months of corrected gestational age who participated in the core study (SHP633-302 \[NCT02980666\]) were enrolled into this extension study and received teduglutide 0.05 mg/kg SC injection once daily for 24 weeks in each treatment cycle depending on the disease course.	2
Total	9

Withdrawals & dropouts

Period	Reason	FG000	FG001
Overall Study	Adverse Event	0	1

Baseline characteristics

Characteristic	Total Children (Aged: 1 to 15 Years)	Infants (Corrected Gestational Age: 4 to < 12 Months)	Total
Age, Customized Aged: 1 to 15 Years	7 Participants	0 Participants	7 Participants
Age, Customized Aged: 4 to < 12 Months	0 Participants	2 Participants	2 Participants
Ethnicity (NIH/OMB) Hispanic or Latino	0 Participants	0 Participants	0 Participants
Ethnicity (NIH/OMB) Not Hispanic or Latino	7 Participants	2 Participants	9 Participants
Ethnicity (NIH/OMB) Unknown or Not Reported	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) American Indian or Alaska Native	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) Asian	7 Participants	2 Participants	9 Participants
Race (NIH/OMB) Black or African American	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) More than one race	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) Native Hawaiian or Other Pacific Islander	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) Unknown or Not Reported	0 Participants	0 Participants	0 Participants
Race (NIH/OMB) White	0 Participants	0 Participants	0 Participants
Sex: Female, Male Female	1 Participants	1 Participants	2 Participants
Sex: Female, Male Male	6 Participants	1 Participants	7 Participants

Adverse events

Event type	EG000 affected / at risk	EG001 affected / at risk
deaths Total, all-cause mortality	0 / 7	0 / 2
other Total, other adverse events	7 / 7	2 / 2
serious Total, serious adverse events	7 / 7	2 / 2

Outcome results

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 2 (up to 48 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, BMI was calculated only for participants \>= 2 years of age.

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment	0.489 Z-score	Standard Deviation 0.8474

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 3 (up to 72 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment	0.409 Z-score	Standard Deviation 1.1688

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 4 (up to 96 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment	0.118 Z-score	Standard Deviation 0.8827

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 5 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 5 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 5 (up to 120 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 5 During Teduglutide Treatment	0.070 Z-score	Standard Deviation 0.977

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 6 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 6 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 6 (up to 144 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 6 During Teduglutide Treatment	0.925 Z-score	Standard Deviation 0.2556

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 7 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 7 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 7 (up to 168 weeks)

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 7 During Teduglutide Treatment	0.839 Z-score

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 8 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 8 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 8 (up to 192 weeks)

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 8 During Teduglutide Treatment	0.015 Z-score

Primary

Change From Baseline in BMI for Age Z-score at EOT of Cycle 9 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 9 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 9 (up to 196 weeks)

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in BMI for Age Z-score at EOT of Cycle 9 During Teduglutide Treatment	0.480 Z-score

Primary

Change From Baseline in Body Mass Index (BMI) for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment

BMI Z-score was calculated by using the height and weight Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in BMI for Age Z-score at EOT of Cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 1 (up to 24 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Body Mass Index (BMI) for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment	0.090 Z-score	Standard Deviation 0.6642

Primary

Change From Baseline in Body Weight For Age Z-Score at EOT of Last Cycle During Teduglutide Treatment

Body weight was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in body weight for age Z-Score at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Body Weight For Age Z-Score at EOT of Last Cycle During Teduglutide Treatment	0.229 Z-score	Standard Deviation 0.5636
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Body Weight For Age Z-Score at EOT of Last Cycle During Teduglutide Treatment	3.982 Z-score	Standard Deviation 3.6363

Primary

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment

Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 1 (up to 24 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, head circumference was collected only for participants with less than (\<) 12 months of age.

Arm	Measure	Value (MEAN)	Dispersion
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment	2.400 Z-score	Standard Deviation 0.6472

Primary

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment

Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 2 (up to 48 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, head circumference was collected only for participants with \< 12 months of age.

Arm	Measure	Value (MEAN)
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment	1.431 Z-score

Primary

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment

Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 3 (up to 72 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, head circumference was collected only for participants with \< 12 months of age.

Arm	Measure	Value (MEAN)
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment	2.080 Z-score

Primary

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment

Head circumference was measured using Age Z-score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in head circumference for Age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 4 (up to 76 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, head circumference was collected only for participants with \< 12 months of age.

Arm	Measure	Value (MEAN)
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment	2.055 Z-score

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 1 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-Score at EOT of cycle 1 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 1 (up to 24 Weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 1 During Teduglutide Treatment	0.002 Z-score	Standard Deviation 0.6049
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 1 During Teduglutide Treatment	1.376 Z-score	Standard Deviation 0.3363

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 2 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 2 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 2 (up to 48 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 2 During Teduglutide Treatment	0.152 Z-score	Standard Deviation 0.5181
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 2 During Teduglutide Treatment	3.114 Z-score	—

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 3 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 3 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 3 (up to 72 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 3 During Teduglutide Treatment	0.098 Z-score	Standard Deviation 0.3467
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 3 During Teduglutide Treatment	3.554 Z-score	—

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 4 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 4 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 4 (up to 96 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 4 During Teduglutide Treatment	0.126 Z-Score	Standard Deviation 0.2622
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 4 During Teduglutide Treatment	3.542 Z-Score	—

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 5 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 5 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 5 (up to 120 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here,0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 5 During Teduglutide Treatment	0.342 Z-score	Standard Deviation 0.1012

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 6 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 6 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 6 (up to 144 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here,0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 6 During Teduglutide Treatment	0.276 Z-score	Standard Deviation 0.2593

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 7 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 7 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 7 (up to 168 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here,0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 7 During Teduglutide Treatment	0.570 Z-score

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 8 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 8 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 8 (up to 192 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here,0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 8 During Teduglutide Treatment	0.851 Z-score

Primary

Change From Baseline in Height For Age Z-Score at EOT of Cycle 9 During Teduglutide Treatment

Height was measured using Age Z-Score. A Z-score was the deviation of the value for an individual from the mean value of the reference population divided by the standard deviation for the reference population. Centers for Disease Control and Prevention (age \>= 2 years old) and World Health Organization (age \< 2 years old) Z-score calculation charts were used for calculation. A negative Z-score indicates values lower than the mean while a positive Z-score indicates values higher than the mean. Change from baseline in height for age Z-score at EOT of Cycle 9 during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of Cycle 9 (up to 196 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here,0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Height For Age Z-Score at EOT of Cycle 9 During Teduglutide Treatment	0.477 Z-score

Primary

Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment

Number of participants with ADAs (including NAbs) to teduglutide were used to summarize the presence of antibodies. The participants who tested positive only for ADAs (including NAbs) were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed refer to the participants evaluable for this outcome measure and number analyzed refer to participants evaluable for this outcome at given categories. 0 in number analyzed signifies that no participant was evaluable for specified category for the specified group.

Arm	Measure	Group	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment	Positive ADA at EOT of the Last Cycle	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment	Positive NAbs at EOT of the Last Cycle	4 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment	Positive ADA at EOT of the Last Cycle	0 Participants

Primary

Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters

Clinical laboratory parameters included biochemistry, hematology and urinalysis. Number of participants with clinically significant abnormalities in laboratory parameters which were deemed clinically significant by the investigator were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: From Baseline up to follow-up (up to 50 months)

Arm	Measure	Group	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Biochemistry	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Hematology	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Urinalysis.	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Biochemistry	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Hematology	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters	Urinalysis.	0 Participants

Primary

Number of Participants With Clinically Significant Abnormalities in Vital Signs

Vital sign assessments included pulse rate, blood pressure (systolic and diastolic blood pressure) and body temperature. Number of participants with clinically significant abnormalities in vital signs which were deemed clinically significant by the investigator were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: From Baseline up to follow-up (up to 50 months)

Arm	Measure	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants With Clinically Significant Abnormalities in Vital Signs	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Clinically Significant Abnormalities in Vital Signs	0 Participants

Primary

Number of Participants With Clinically Significant Changes in Gastrointestinal (GI) Specific Testing

GI specific testing included colonoscopy or sigmoidoscopy, abdominal ultrasound, fecal occult blood testing, upper GI series with small bowel follow-through (UGI/SBFT). Number of participants with clinically significant changes findings in gastrointestinal specific testing were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants With Clinically Significant Changes in Gastrointestinal (GI) Specific Testing	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Clinically Significant Changes in Gastrointestinal (GI) Specific Testing	0 Participants

Primary

Number of Participants With Treatment-emergent Adverse Events (TEAEs)

An Adverse Event (AE) was any untoward medical occurrence in a clinical investigation participant administered a pharmaceutical product and that does not necessarily have a causal relationship with this treatment. TEAEs were defined as any AEs whose onset occurred, severity worsened or intensity increased after receiving the investigational product (IP) in the core study (SHP633-302 \[NCT02980666\]) or this extension study. Number of participants with TEAEs were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: From Baseline up to follow-up (up to 50 months)

Arm	Measure	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants With Treatment-emergent Adverse Events (TEAEs)	7 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants With Treatment-emergent Adverse Events (TEAEs)	2 Participants

Primary

Percent Change From Baseline in Average Bristol Stool Form Score at EOT of Last Cycle During Teduglutide Treatment

Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized the average typical stool form score using Bristol Stool Form Scale. The average typical stool form score was calculated as (sum of the daily data in a 48-hour period / 2). Typical Stool Form based on Bristol Stool Form Scale: 1 - Separate hard lumps, hard to pass, 2 - Sausage-shaped, but lumpy, 3 - Like a sausage but with cracks on the surface, 4- Like a sausage or snake, smooth and soft, 5- Soft blobs with clear-cut edges, 6- Fluffy pieces with ragged edges, a mushy stool, 7- Watery, no solid pieces. Entirely liquid. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]). A negative change from baseline indicates improvement.

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Average Bristol Stool Form Score at EOT of Last Cycle During Teduglutide Treatment	-7.51 Percent change	Standard Deviation 16.758
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Average Bristol Stool Form Score at EOT of Last Cycle During Teduglutide Treatment	0.00 Percent change	Standard Deviation 0

Primary

Percent Change From Baseline in Average Number of Stools Per Day at EOT of Last Cycle During Teduglutide Treatment

Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized by the average number of stools per day. The average number of stools per day was calculated as (sum of the daily data in a 48-hour period/2). Percent change from baseline in average number of stools per day at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Average Number of Stools Per Day at EOT of Last Cycle During Teduglutide Treatment	24.37 Percent change	Standard Deviation 63.623
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Average Number of Stools Per Day at EOT of Last Cycle During Teduglutide Treatment	50.00 Percent change	Standard Deviation 0

Primary

Percent Change From Baseline in Average Stool/Mixed Stool Diaper Weight at EOT of Last Cycle During Teduglutide Treatment

Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The average daily fecal output was summarized by the average stool/mixed stool diaper weight (gram per kilogram per day \[g/kg/day\]). The body weight was used to calculate the daily stool/mixed stool diaper weight (g/kg/day). Percent change from baseline in average stool/mixed stool diaper weight at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Average Stool/Mixed Stool Diaper Weight at EOT of Last Cycle During Teduglutide Treatment	-12.678 Percent change	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Average Stool/Mixed Stool Diaper Weight at EOT of Last Cycle During Teduglutide Treatment	206.017 Percent change	Standard Deviation 332.458

Primary

Percent Change From Baseline in Average Total Ostomy Output at EOT of Last Cycle During Teduglutide Treatment

Fecal output was recorded over a 48-hour period of PS stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The body weight was used to calculate the average total ostomy output per day (mL/kg/day) using a formula analogous to that used to calculate the average daily urine output. Percent change from baseline in average total ostomy output at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, Overall number of participants analyzed signifies those participants who were evaluable for this outcome measure. Here, 0 in Overall number of participants analyzed signifies that no participant was evaluable at specified time point for the specified group.

Arm	Measure	Value (MEAN)	Dispersion
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Average Total Ostomy Output at EOT of Last Cycle During Teduglutide Treatment	3.143 Percent change	Standard Deviation 145.8659

Primary

Percent Change From Baseline in Average Total Urine Output at End of Treatment (EOT) of Last Cycle During Teduglutide Treatment

Average total urine output was recorded over a 48-hour period of parental support (PS) stability before every scheduled site visit and within 1 week of implementing any PS adjustment. The Average daily urine output milliliter per kilogram per day (mL/kg/day) was calculated as: (Total urine output over 48 hours / 2) / body weight (kilogram \[kg\]) where total urine output was calculated as the sum of the urine output in milliliter (mL) and the urine-only diaper weights in gram (g) (1g = 1mL) for the participant collected on the output diary form of electronic case report from (eCRF). Percent change from baseline in average total urine output at EOT of last cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of last cycle (up to Month 45) (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Arm	Measure	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Average Total Urine Output at End of Treatment (EOT) of Last Cycle During Teduglutide Treatment	-37.228 Percent change	Standard Deviation 42.9396
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Average Total Urine Output at End of Treatment (EOT) of Last Cycle During Teduglutide Treatment	146.881 Percent change	Standard Deviation 295.8061

Secondary

Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment

Days per week of diary PS was calculated as: Days per week of actual PS = (number of days with non-zero values for PS volume within the 7 days prior to the visit / number of days for which any PS intake data was recorded within the 7 days prior to the visit) \* 7. Change from baseline in days per week of diary PS usage at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 9	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 6	0.0 Days/week	Standard Deviation 0
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 7	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 8	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-1.2 Days/week	Standard Deviation 2.86
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-1.2 Days/week	Standard Deviation 2.86
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-1.2 Days/week	Standard Deviation 2.86
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-1.4 Days/week	Standard Deviation 3.13
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 5	0.0 Days/week	Standard Deviation 0
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	0.0 Days/week	Standard Deviation 0
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	0.0 Days/week	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	0.0 Days/week	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-7.0 Days/week	—

Secondary

Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment

Change from baseline in days per week of prescribed PS usage at EOT of each cycle during teduglutide treatment was reported. Days per week of diary PS was calculated as: Days per week of actual PS = (number of days with non-zero values for PS volume within the 7 days prior to the visit / number of days for which any PS intake data was recorded within the 7 days prior to the visit) \* 7. Change from baseline in days Per week of prescribed PS usage at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 9	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 6	0.0 Days/week	Standard Deviation 0
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 7	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 8	0.0 Days/week	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-1.2 Days/week	Standard Deviation 2.86
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-1.2 Days/week	Standard Deviation 2.86
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-16.7 Days/week	Standard Deviation 40.82
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-20.0 Days/week	Standard Deviation 44.72
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 5	0.0 Days/week	Standard Deviation 0
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	0.0 Days/week	Standard Deviation 0
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	0.0 Days/week	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	0.0 Days/week	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-100.0 Days/week	—

Secondary

Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Change from baseline in investigator prescribed PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 9	-77.9 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 6	-63.6 mL/kg/day	Standard Deviation 32.1
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 7	-91.8 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 8	-77.3 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-20.9 mL/kg/day	Standard Deviation 11.06
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-28.0 mL/kg/day	Standard Deviation 13.51
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-34.8 mL/kg/day	Standard Deviation 21.13
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-36.3 mL/kg/day	Standard Deviation 23.52
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 5	-53.3 mL/kg/day	Standard Deviation 26.24
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-31.3 mL/kg/day	Standard Deviation 26.77
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-67.5 mL/kg/day	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-60.6 mL/kg/day	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-98.7 mL/kg/day	—

Secondary

Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Change from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 9	-79.1 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 6	-63.8 mL/kg/day	Standard Deviation 31.57
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 7	-93.8 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 8	-80.3 mL/kg/day	—
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-21.5 mL/kg/day	Standard Deviation 11.83
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-25.5 mL/kg/day	Standard Deviation 14.38
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-32.1 mL/kg/day	Standard Deviation 18.19
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-37.2 mL/kg/day	Standard Deviation 24.1
Total Children (Aged: 1 to 15 Years)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 5	-54.8 mL/kg/day	Standard Deviation 26.58
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 1	-31.9 mL/kg/day	Standard Deviation 16.24
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 3	-62.8 mL/kg/day	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 2	-54.2 mL/kg/day	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Change at EOT of Cycle 4	-95.8 mL/kg/day	—

Secondary

Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent reduction in weight-normalized prescribed PS values from baseline was calculated using the formula: % reduction in PS value at the visit =(\[average daily value at the scheduled visit - average daily value at baseline of the core study (SHP633-302 \[NCT02980666\]) / average daily value at baseline of the core study (SHP633-302 \[NCT02980666\])) \*100. Number of participants who achieved at least 20% reduction from baseline in Investigator prescribed PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 9	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 6	2 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 7	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 8	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 1	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 2	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 3	6 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 4	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 5	3 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 1	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 3	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 2	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 4	1 Participants

Secondary

Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent reduction in weight-normalized diary PS values from baseline was calculated using the formula: % reduction in PS value at the visit = (\[average daily value at the scheduled visit - average daily value at baseline of the core study (SHP633-302 \[NCT02980666\]) / average daily value at baseline of the core study (SHP633-302 \[NCT02980666\])) \*100. Number of participants who achieved at least 20% reduction from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 9	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 6	2 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 7	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 8	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 1	4 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 2	4 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 3	6 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 4	5 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 5	3 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 1	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 3	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 2	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment	At least 20% Reduction: At EOT of Cycle 4	1 Participants

Secondary

Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment

Enteral autonomy (completely weaned off PS) was defined as the first visit where there is no use of PS for the 7 days prior to the visit and there is no prescribed PS at that visit, and the participants remains off PS for the remainder of the treatment period of that cycle. Number of participants who achieved enteral autonomy off PS volume at EOT of each cycle during teduglutide treatment were reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (COUNT_OF_PARTICIPANTS)
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 4	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 2	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 3	1 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 8	0 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 9	0 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 5	0 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 6	0 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 7	0 Participants
Total Children (Aged: 1 to 15 Years)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 1	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 4	1 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 2	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 1	0 Participants
Infants (Corrected Gestational Age: 4 to < 12 Months)	Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment	At EOT of Cycle 3	0 Participants

Secondary

Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent change from baseline in investigator prescribed PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 9	-70.5 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 6	-76.3 Percent change	Standard Deviation 2.41
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 7	-83.0 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 8	-69.9 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 1	-38.8 Percent change	Standard Deviation 35.19
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 2	-46.9 Percent change	Standard Deviation 30.42
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 3	-55.4 Percent change	Standard Deviation 27.78
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 4	-62.5 Percent change	Standard Deviation 26.45
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 5	-73.3 Percent change	Standard Deviation 5.51
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 1	-32.1 Percent change	Standard Deviation 26.51
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 3	-68.3 Percent change	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 2	-61.4 Percent change	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 4	-100.0 Percent change	—

Secondary

Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment

PS (parenteral nutrition or intravenous fluids) was considered for managing nutritional support in terms of volume during the treatment period. Percent change from baseline in participants diary PS volume at EOT of each cycle during teduglutide treatment was reported. Baseline refers to the baseline value of the core study (SHP633-302 \[NCT02980666\]).

Time frame: Baseline, EOT of each cycle 1, 2, 3, 4, 5, 6, 7, 8 and 9 (Each Cycle 1 to 8 = 24 weeks, and Cycle 9 = 4 weeks)

Population: The safety population included all enrolled participants in the study and who received at least one dose of teduglutide (in study SHP633-302 \[NCT02980666\] or SHP633-305 \[NCT03268811\]). Here, overall number of participants analyzed signifies to the participants evaluable for this outcome measure and number analyzed signifies to participants evaluable for this outcome at given timepoints.

Arm	Measure	Group	Value (MEAN)	Dispersion
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 9	-69.8 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 6	-75.7 Percent change	Standard Deviation 0.54
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 7	-82.9 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 8	-70.9 Percent change	—
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 1	-39.6 Percent change	Standard Deviation 34.86
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 2	-44.3 Percent change	Standard Deviation 33.11
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 3	-52.8 Percent change	Standard Deviation 28.54
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 4	-62.9 Percent change	Standard Deviation 26.18
Total Children (Aged: 1 to 15 Years)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 5	-73.9 Percent change	Standard Deviation 5.3
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 1	-32.5 Percent change	Standard Deviation 18.1
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 3	-65.6 Percent change	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 2	-56.6 Percent change	—
Infants (Corrected Gestational Age: 4 to < 12 Months)	Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment	Percent change at EOT of Cycle 4	-100.0 Percent change	—

Source: ClinicalTrials.gov · Data processed: Feb 9, 2026

A Study in Japanese Children With Short Bowel Syndrome Who Completed SHP633-302

Conditions

Brief summary

Related papers

Interventions

Sponsors

Study design

Eligibility

Inclusion criteria

Exclusion criteria

Design outcomes

Primary

Secondary

Countries

Participant flow

Recruitment details

Pre-assignment details

Participants by arm

Withdrawals & dropouts

Baseline characteristics

Adverse events

Outcome results

Change From Baseline in BMI for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 5 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 6 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 7 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 8 During Teduglutide Treatment

Change From Baseline in BMI for Age Z-score at EOT of Cycle 9 During Teduglutide Treatment

Change From Baseline in Body Mass Index (BMI) for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment

Change From Baseline in Body Weight For Age Z-Score at EOT of Last Cycle During Teduglutide Treatment

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 1 During Teduglutide Treatment

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 2 During Teduglutide Treatment

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 3 During Teduglutide Treatment

Change From Baseline in Head Circumference for Age Z-score at EOT of Cycle 4 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 1 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 2 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 3 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 4 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 5 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 6 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 7 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 8 During Teduglutide Treatment

Change From Baseline in Height For Age Z-Score at EOT of Cycle 9 During Teduglutide Treatment

Number of Participants With Anti-drug Antibodies (ADAs) (Including Neutralizing Antibodies) at EOT of Last Cycle During Teduglutide Treatment

Number of Participants With Clinically Significant Abnormalities in Laboratory Parameters

Number of Participants With Clinically Significant Abnormalities in Vital Signs

Number of Participants With Clinically Significant Changes in Gastrointestinal (GI) Specific Testing

Number of Participants With Treatment-emergent Adverse Events (TEAEs)

Percent Change From Baseline in Average Bristol Stool Form Score at EOT of Last Cycle During Teduglutide Treatment

Percent Change From Baseline in Average Number of Stools Per Day at EOT of Last Cycle During Teduglutide Treatment

Percent Change From Baseline in Average Stool/Mixed Stool Diaper Weight at EOT of Last Cycle During Teduglutide Treatment

Percent Change From Baseline in Average Total Ostomy Output at EOT of Last Cycle During Teduglutide Treatment

Percent Change From Baseline in Average Total Urine Output at End of Treatment (EOT) of Last Cycle During Teduglutide Treatment

Change From Baseline in Days Per Week of Diary PS Usage at EOT of Each Cycle During Teduglutide Treatment

Change From Baseline in Days Per Week of Prescribed PS Usage at EOT of Each Cycle During Teduglutide Treatment

Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment

Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

Number of Participants Who Achieved At Least 20 Percent (%) Reduction From Baseline in Participants Diary Parenteral Support (PS) Volume at EOT of Each Cycle During Teduglutide Treatment

Number of Participants Who Achieved Enteral Autonomy PS Volume at EOT of Each Cycle During Teduglutide Treatment

Percent Change From Baseline in Investigator Prescribed PS Volume at EOT of Each Cycle During Teduglutide Treatment

Percent Change From Baseline in Participant Diary PS Volume at EOT of Each Cycle During Teduglutide Treatment