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Published online March 23, 2007
Diabetes Care 30:1590-1592, 2007
DOI: 10.2337/dc06-2327
© 2007 by the American Diabetes Association
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Clinical Care/Education/Nutrition
Brief Report

Coexistence in the Same Family of Both Focal and Diffuse Forms of Hyperinsulinism

Vassili Valayannopoulos, MD1, Martine Vaxillaire, PHD2, Yves Aigrain, MD, PHD3, Francis Jaubert, MD, PHD4, Christine Bellanné-Chantelot, MD5, Maria-Joao Ribeiro, MD, PHD6, Francis Brunelle, MD, PHD7, Philippe Froguel, MD, PHD2,8, Jean-Jacques Robert, MD, PHD9, Michel Polak, MD, PHD9, Claire Nihoul-Fékété, MD, PHD3 and Pascale de Lonlay, MD, PHD1

1 Department of Metabolic Disorders, Hôpital Necker-Enfants Malades, Paris, France
2 Lille Institute of Biology, Lille, France
3 Department of Pediatric Surgery, Hôpital Necker-Enfants Malades, Paris, France
4 Department of Pathology, Hôpital Necker-Enfants Malades, Paris, France
5 Department of Biology, Hôpital Saint-Antoine, Paris, France
6 Service Hospitalier Frédéric Joliot, Orsay, France
7 Department of Radiology, Hôpital Necker-Enfants Malades, Paris, France
8 Department of Genomic Medicine, Hammersmith Hospital, Imperial College, London, U.K
9 Department of Endocrinology, Hôpital Necker-Enfants Malades, Paris, France

Address correspondence and reprint requests to Vassili Valayannopoulos, Department of Metabolic Disorders, Hôpital Necker-Enfants Malades, 149, Rue des Sèvres, 75015 Paris, France. E-mail: vassili.valaya@nck.aphp.fr

The first 20% of the full text of this article appears below.


   INTRODUCTION
 
Neonatal hyperinsulinism is the most important cause of hypoglycemia in infancy (1,2). The inappropriate oversecretion of insulin is responsible for profound hypoglycemia, requiring aggressive treatment to prevent brain damage (1–3). Neonatal hyperinsulinism is often resistant to medical therapy (1–4), and pancreatectomy is required for many sufferers (1,5–6). The histopathological lesions associated with neonatal hyperinsulinism may be described as diffuse or focal (7–8). Focal adenomatous islet cell hyperplasia is sporadic and has been demonstrated to arise in individuals who have a germline mutation in the paternal allele of the sulfonylurea receptor 1 ABCC8 gene (9,10) or the inward-rectifying potassium channel Kir6.2 (KCNJ11) (10) in addition to a somatic loss of the maternally derived chromosome region 11p15 in adenomatous pancreatic ß-cells (9–11). Diffuse hyperinsulinism may be familial and arises from the autosomal recessive inheritance of mutations in both ABCC8 (12) and KCNJ11 (13–14) genes. The therapeutic outcome for the patients is heavily dependent on distinguishing between the two histopathological lesions. Diffuse hyperinsulinisms, which are unresponsive to medical treatment, require extensive pancreatectomy, with a high risk of diabetes (5,15–16). Conversely, focal hyperinsulinism can be cured by limited pancreatectomy (6,17). Genetic counseling is dramatically different, as focal hyperinsulinism is considered a . . . [Full Text of this Article]


   RESEARCH DESIGN AND METHODS—
 

   RESULTS—
 

   CONCLUSIONS—
 

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Copyright © 2007 by the American Diabetes Association.