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Hypoglycaemia and Russell-Silver syndrome
1Great Ormond Street Hospital for Children, London, UK.
Journal of Pediatric Endocrinology & Metabolism : JPEM
|September 1, 2005
Summary
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.
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