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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1787861 | PMC |
http://dx.doi.org/10.1136/bmj.2.5805.112-c | DOI Listing |
Hum Pathol
March 2007
Department of Pediatrics, Hospital Necker-Enfants Malades, Paris 75743, France.
Neonatal hyperinsulinism is a life-threatening disease that, when treated by total pancreatectomy, leads to diabetes and pancreatic insufficiency. A more conservative approach is now possible since the separation of the disease into a nonrecurring focal form, which is cured by partial surgery, and a diffuse form, which necessitates total pancreas removal only in cases of medical treatment failure. The pathogenesis of the disease is now divided into K-channel disease (hyperinsulinemic hypoglycemia, familial [HHF] 1 and 2), which can mandate surgery, and other metabolic causes, HHF 3 to 6, which are treated medically in most patients.
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