congenital hyperinsulinism
Conditions
Interventions
After stabilizing the blood glucse by continuous high concentration glucose infusion, continuous subcutaneous octreotide injection is initiated at a dose of up to 25 micrograms/kg/day with continuous
Sponsors
Japan Agency for Medical Research and Development. Research group for the study on "Efficacy and safety of subcutaneous continuous octreotide infusion for congenital hyperinsulinism."
Japanese Society for Pediatric Endocrinology
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients who meet all criteria below. 1 Age between 2 weeks to 1 year and with congenital hyperinsulinemic hypoglycemia (insulin > 3 microunits/mL at blood glucose <45 mg/dL) 2 Requires continuous glucose infusion at > 6 mg/kg/min to maintain blood glucose > 60 mg/dL. 3 Inability to maintain blood glucose > 60 mg/dL with diazoxide at15 mg/kg/d. 4 Consent to participate in the study from the guardians. 5 Consent to undergo molecular testing for congenital hyperinsulinism.
Exclusion criteria
Exclusion criteria: 1) Hypoglycemic patients with causes other than hyperinsulinemia. 2) Patients with severe extrapancreatic comorbidities. a. intestinal dysfunction b. respiratory failure c. severe liver dysfunction with transaminases or bilirubin > 3x ULN for age. d. renal failure with urea nitrogen or creatinine > 3x ULN for age. e. patient with other comorbidities who would not be appropriate for enrollment.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Short term increase in blood glucose comparing the mean blood glucose before 24 hours and after 48 hours of the initiation of the treatment. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1) Long-term reduction in the amount of glucose infusion to maintain blood glucose at 1 week, 2 weeks, 4 weeks, and at every 3 month after 5 weeks. 2) Neurological outcome after > 1 year of the initiation of the treatment. 3) Number of ypoglycemic episodes < 45 mg/dL during the treatment, categorized as asymptomatic, symptomatic, or severe. | — |
Countries
Japan
Contacts
Public ContactTohru Yorifuji
Osaka City General Hospital Pediatric Endocrinology and Metabolism
Outcome results
None listed