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Published on: November 16, 2011
[Persistent hyperglycemia and hyperinsulinemia in a newborn infant]
L Tapia Ceballos1, J Romero Sánchez, B Picazo Angelin
1Area de Pediatría, Hospital Costa del Sol. Marbella, Málaga. leotapiaceb@hotmail.com
Insights
Neonatal hyperglycemia management is complex, often caused by iatrogeny. This case highlights Donohue syndrome (leprechaunism) as a rare cause of severe insulin resistance in newborns requiring insulin therapy.
Area of Science:
- Neonatology
- Endocrinology
- Genetics
Background:
- Neonatal hyperglycemia presents complex management challenges, particularly in preterm infants.
- Iatrogenic causes are frequent, but rare conditions like neonatal diabetes mellitus also occur.
- High insulin requirements ( > 2 IU/kg/day) suggest insulin resistance syndrome.
Observation:
- A case of a newborn infant with Donohue syndrome (leprechaunism) is presented.
- Donohue syndrome is a rare, genetically determined disorder.
- The infant exhibited significant insulin resistance.
Findings:
- Donohue syndrome leads to profound insulin resistance in neonates.
- Management requires careful monitoring of insulin needs.
- This genetic condition underscores the diverse etiologies of neonatal hyperglycemia.
Implications:
- Recognizing rare genetic syndromes is crucial for diagnosing neonatal hyperglycemia.
- Early identification of insulin resistance syndromes can guide appropriate treatment.
- Understanding Donohue syndrome improves clinical approaches to complex neonatal metabolic disorders.
Abstract:
Management of neonatal hyperglycemia is complex, especially in immature newborns. Etiology is diverse, the most frequent cause being iatrogeny. Neonatal diabetes mellitus is a rare cause of hyperglycemia during the first days of life. Insulin treatment is sometimes required to control the condition. When insulin requirements exceed 2I U/kg/day insulin resistance syndrome should be suspected. We present the case of a newborn infant with Donohue syndrome, or leprechaunism, which is a rare, genetically determined dysmorphic syndrome leading to significant insulin resistance.
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