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Biochemical Titration of Glycogen In vitro
07:16

Biochemical Titration of Glycogen In vitro

Published on: November 24, 2013

Improved growth with growth hormone therapy in a child with glycogen storage disease Ib

R A Noto1, V Vijayaraghavan, A Timoshin

  • 1Division of Pediatric Endocrinology, Metabolism and Nutrition, New York Medical College, Valhalla, New York 10595, USA.

Acta Paediatrica (Oslo, Norway : 1992)
|September 2, 2003
PubMed

Insights

Growth hormone (GH) therapy can significantly improve growth velocity in children with glycogen storage disease (GSD) type Ib. Careful monitoring for hyperlipidemia is recommended during treatment for this rare metabolic disorder.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Genetics and Genomics

Background:

  • Type Ib glycogen storage disease (GSD) frequently presents with growth impairment and short stature in affected children.
  • Limited data exists on the efficacy and safety of growth hormone (GH) therapy in pediatric GSD patients.

Observation:

  • A case study of a poorly growing adolescent female with GSD type Ib was evaluated.
  • Substitutive GH treatment was administered to address the patient's growth deficiency.

Findings:

  • The patient exhibited a substantial increase in growth velocity, rising from 2.5 cm/year to an average of 8.7 cm/year during GH therapy.
  • GH treatment did not induce metabolic decompensation but was associated with elevated cholesterol and triglyceride levels.

Implications:

  • GH therapy may offer a viable therapeutic option for improving growth outcomes in pediatric patients with GSD.
  • Pre-existing or treatment-induced hyperlipidemia in GSD patients requires management to ensure safe GH therapy administration.
Abstract

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