glycogen storage disease type Ib
Conditions
Interventions
Experimental group:engliazin treatment
Sponsors
Peking Union Medical College Hospital, Chinese Academy of Medical Sciences
Eligibility
Sex/Gender
All
Age
2 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. Patients diagnosed with GSD type Ib; 2. Gender is not limited, the age is more than 2 years old 3. Patients with neutropenia (<1.5*10^9/L); 4. Voluntarily participate in the trial, and sign the informed consent form by himself or his legal guardian.
Exclusion criteria
Exclusion criteria: 1. Allergic to empagliflozin; 2 Any disease or condition that may affect the conduct of the study or the interpretation of the results; 3. Oral raw corn starch is 1-1.5g/kg/time in early childhood, every 4-6 hours, and 1.5-2g/kg/time in childhood, every 4-6 hours, fasting blood sugar cannot be maintained at 3.9-6.1mmol/L; 4. Exclude neutropenia caused by other factors (such as certain infections, drug effects, etc.). 5. GFR is less than 45ml/ml/min/1.73m2 6. Alcohol, drug or drug (eg laxative) abusers; 7. Patients who participated in or participated in another clinical trial within 1 month that may have an impact on the purpose of this research; 8. The researchers believe that other reasons are not suitable for clinical trials.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Blood routine;Neutrophil function; | — |
Secondary
| Measure | Time frame |
|---|---|
| Blood biochemical;lactic acid;Urinary microglobulin;Stool Routine;Abdominal ultrasound; | — |
Countries
China
Contacts
Public ContactQiu Zhengqing
Peking Union Medical College Hospital, Chinese Academy of Medical Sciences
Outcome results
None listed