Status:

UNKNOWN

Safety, Efficacy Evaluation of Empagliflozin Administration for Neutropenia in Glycogenosis Type 1b and G6PC3 Deficiency

Lead Sponsor:

Cliniques universitaires Saint-Luc- Université Catholique de Louvain

Conditions:

Glycogen Storage Disease Type I

Glucose 6 Phosphatase Deficiency

Eligibility:

All Genders

1-18 years

Phase:

PHASE2

Brief Summary

Treatment of neutropenia of G6PC3 and Glycogenosis type 1b patients with empagliflozin

Detailed Description

Ubiquitous glucose-6-phosphatase deficiency (G6PC3) and glucose-6-phosphate transporter deficiency (G6PT/SLC37A4) both cause neutropenia. Studies on a G6PC3 deficient mouse model by Dr Veiga-da-Cunha ...

Eligibility Criteria

Inclusion

  • Glycogenosis type 1b confirmed by biochemical analyzes and / or genetic analysis. These patients with Glycogenosis must have had a liver transplant
  • Alternatively, G6PC3 deficiency confirmed by genetic analysis
  • Age 1 to 18 years old female or male
  • Informed consent signed by the recipient and / or parents / assigns.
  • Information and agreement of the referring medical team.
  • A Negative Blood Pregnancy Test at the time of screening and a negative urinary pregnancy test at Day 1 of the protocol are required for female with child bearing potential.
  • Sexually active patients should use an effective method of contraception throughout the duration of the study and up to 7 days after the last dose of Empaglifozine. (The combination of a hormonal method and a barrier method; Two barrier methods, the male condom being one of these two methods;Use intrauterine device or tubal ligation;-A total sex abstinence.)

Exclusion

  • Presence of advanced fibrosis (Metavir F4) or cirrhosis.
  • Impossibility of long-term and / or non-compliance monitoring.
  • Other medical problems which, in the opinion of the physicians in charge of the patient, would constitute a contraindication to the procedure.
  • Sexually active patients who do not consent to use effective contraception during the study.

Key Trial Info

Start Date :

June 20 2019

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

June 30 2020

Estimated Enrollment :

5 Patients enrolled

Trial Details

Trial ID

NCT04138251

Start Date

June 20 2019

End Date

June 30 2020

Last Update

October 28 2019

Active Locations (1)

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1

Cliniques universitaires Saint-Luc

Brussels, Belgium, 1200