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PubMed · 12069542

Persistent hyperinsulinaemic hypoglycaemia.

Pascale de Lonlay·Guy Touati·Jean-Jacques Robert·Jean-Marie Saudubray
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Abstract

Congenital hyperinsulinism (CI) is the most important cause of hypoglycaemia in early infancy. The inappropriate oversecretion of insulin is responsible for profound hypoglycaemias which require aggressive treatment to prevent severe and irreversible brain damage. Hypoglycaemia have a neonatal or infancy onset. Medical treatment with diazoxide is first used to treat CI, but patients who are medically resistant (mostly of neonatal-onset) require pancreatectomy. CI is a heterogeneous disorder with two histopathological lesions, diffuse and focal which are clinically indistinguishable. Only diazoxide-sensitive neonates should be orientated to transient hyperinsulinism or hyperinsulinism-hyperammonemia syndrome. Focal CI is characterized by a sporadic somatic islet-cell hyperplasia. Diffuse CI corresponds to a functional abnormality of insulin secretion in the whole pancreas and involves several genes with different transmissions. The knowledge of both focal and diffuse lesions is very important. Focal lesions are effectively treated by limited pancreatic resection while diffuse lesions which are unresponsive to drug or dietary treatment require extensive pancreatectomy with high risk of diabetes mellitus.

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DiazoxideDiureticsHumansHyperinsulinismHypoglycemiaInfant, NewbornPancreatectomyPrognosisSodium Chloride Symporter InhibitorsJournal ArticleReview

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BibTeXRIS

Pascale de Lonlay, Guy Touati, Jean-Jacques Robert, Jean-Marie Saudubray. 2002. Persistent hyperinsulinaemic hypoglycaemia.. https://doi.org/10.1053/siny.2001.0090

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PubMed1998

Long-term treatment of persistent hyperinsulinaemic hypoglycaemia of infancy with diazoxide: a retrospective review of 77 cases and analysis of efficacy-predicting criteria.

UNLABELLED: Primary persistent hyperinsulinaemic hypoglycaemia of infancy is rare. Diazoxide treatment remains the mainstay of medical therapy in long-term management. We reviewed 77 cases of primary persistent hyperinsulinism in neonates and infants who were treated with diazoxide and studied criteria predictive of therapeutic efficacy. The only criterion identified was age at manifestation. All but 1 of the 31 neonatal cases were unresponsive to diazoxide. Responsiveness increased with age: 12 of 39 early-infantile cases, and all seven late-infantile cases were diazoxide-responsive. In responders, a diazoxide dose of 10-15 mg/kg per day was always effective, suggesting an "all or none" response. Diazoxide-resistant hyperinsulinism is characterized by its severity with higher plasma insulin levels. The analysis of 46 surgically treated patients showed that the efficacy of diazoxide is not related to the aetiology of the pancreatic lesions. In six cases, after many years of management, diazoxide treatment was stopped without recurrence of hypoglycaemia. CONCLUSION: Diazoxide is an efficient treatment in the long-term management of most persistent hyperinsulinaemic hypoglycaemia of infancy revealed in infants and children but is usually ineffective in neonatal forms. Drug efficacy does not correlate with anatomical lesions. Medical treatment can sometimes be stopped after many years of management without recurrence of disease manifestations.

Diazoxide↗