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Hypoglycaemia and Russell-Silver syndrome

C Azcona1, R Stanhope

  • 1Great Ormond Street Hospital for Children, London, UK.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|September 1, 2005
PubMed

Insights

Children with Russell-Silver syndrome (RSS) often experience hypoglycemia, particularly if feeding is irregular. Growth hormone (GH) insufficiency is a likely cause, necessitating specific feeding guidelines for affected children.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders

Background:

  • Russell-Silver syndrome (RSS) is a rare genetic disorder characterized by intrauterine and postnatal growth retardation.
  • Hypoglycemia is a potential complication in children with RSS, impacting their health and development.

Purpose of the Study:

  • To investigate the incidence and causes of chronic hypoglycemia in symptomatic children with Russell-Silver syndrome (RSS) within their first four years of life.

Main Methods:

  • A cohort of 24 children with RSS (under 4 years) underwent a 48-hour hospital admission for glucose and cortisol profiling.
  • Diagnostic fasting and dietary assessments were conducted. Growth hormone (GH) levels were evaluated using a glucagon test.

Main Results:

  • Ten of 24 children had documented hypoglycemia. Seven of 12 patients exhibited GH insufficiency. All patients were described as poor, picky eaters, with seven requiring nasogastric tube feeding.
  • The mean spontaneous energy intake was low (56 kcal/kg/day). Nocturnal sweating was the most frequent symptom (23.96%). Hypoglycemia was observed in seven children's glucose profiles, though only four were symptomatic.

Conclusions:

  • Children with RSS are susceptible to spontaneous hypoglycemia, especially with irregular feeding patterns.
  • Accelerated starvation and/or GH insufficiency are the probable causes.
  • Guidelines are proposed to minimize hypoglycemia risk in children with RSS.
Abstract

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