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Genetics of Congenital Hyperinsulinism
Endocrine Pathology, 2004Congenital hyperinsulinism (CHI) is a clinically and genetically heterogeneous entity and causes severe hypoglycemia in neonates and infants. The clinical heterogeneity is manifested by severity ranging from extremely severe, life-threatening disease to very mild clinical symptoms, which may even be difficult to identify.
Claudine Junien, Jean-Christophe Fournet
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New England Journal of Medicine, 1971
IN attempting to evolve a unitary concept to explain the metabolic and clinical aberrations of diabetes mellitus, one is hard put to find a single defect that will satisfy all the known facts.
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IN attempting to evolve a unitary concept to explain the metabolic and clinical aberrations of diabetes mellitus, one is hard put to find a single defect that will satisfy all the known facts.
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Fetal hyperinsulinism in anencephaly
American Journal of Obstetrics and Gynecology, 1989Insulin was measured in the blood collected in utero from three midtrimester anencephalic fetuses. The hyperinsulinism found could be due to an underutilization of glucose in the absence of most of the brain and could be responsible for the relatively normal growth in anencephaly despite the absence of the hypothalamohypophysial axis.
Hubinont, C +3 more
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Current Opinion in Pediatrics, 2013
Congenital hyperinsulinism (CHI) is a multifaceted disease and continues to be the most common cause of persistent hypoglycemia in infants. The purpose of the review is to highlight important recent developments regarding CHI.Several recent studies have highlighted the advances in medical genetics, imaging techniques, histological variety, and surgical
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Congenital hyperinsulinism (CHI) is a multifaceted disease and continues to be the most common cause of persistent hypoglycemia in infants. The purpose of the review is to highlight important recent developments regarding CHI.Several recent studies have highlighted the advances in medical genetics, imaging techniques, histological variety, and surgical
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Hyperinsulinism in syndromal disorders
Acta Paediatrica, 2001Analysis of a German database comprising a total of 54 patients with neonatal manifestations of persistent hyperinsulinism revealed 5 patients in whom hyperinsulinism was associated with additional clinical symptoms, suggesting an underlying syndromal disorder.
René Santer +5 more
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Seminars in Fetal and Neonatal Medicine, 2005
Congenital hyperinsulinism is a cause of persistent hypoglycaemia in the neonatal period. It is a heterogeneous disease with respect to clinical presentation, molecular biology, genetic aetiology and response to medical therapy. The clinical heterogeneity may range from severe life-threatening disease to very mild clinical symptoms.
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Congenital hyperinsulinism is a cause of persistent hypoglycaemia in the neonatal period. It is a heterogeneous disease with respect to clinical presentation, molecular biology, genetic aetiology and response to medical therapy. The clinical heterogeneity may range from severe life-threatening disease to very mild clinical symptoms.
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CLINICAL ASPECTS OF HYPERINSULINISM
Journal of the American Medical Association, 1956• Hyperinsulinism in forms satisfying rigid diagnostic criteria was found in 91 cases. The attacks must be associated with exercise or fasting, the blood sugar level during an attack must be less than 50 mg. per 100 cc., and the attack must be relieved by the administration of sugar.
Edward H. Rynearson +2 more
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Hyperinsulinism and Cerebral Microangiopathy
Stroke, 1996Background and Purpose High insulin levels are a recognized risk factor for atherosclerosis. Microvascular endothelium is more susceptible to metabolic and mitogenic effects of insulin than large-vessel endothelium. Besides their atherogenic effect, high insulin levels impair fibrinolysis by enhancing plasminogen activator ...
Darius G. Nabavi +6 more
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Rate of Serious Adverse Events Associated with Diazoxide Treatment of Patients with Hyperinsulinism
Hormone Research in Paediatrics, 2019Introduction: Diazoxide is the first line and only Federal Drug Agency approved pharmacological agent for the treatment of hyperinsulinism. Its use has increased over the years to include patients with various genetic forms of hyperinsulinism, perinatal ...
P. Thornton +4 more
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Hyperinsulinism in Infancy and Childhood
1984Hypoglycaemia is the most common metabolic abnormality in childhood. In addition to the high prevalence of the disorder, hypoglycaemia is of particular importance for paediatricians since it is a potent cause of severe neurological damage when it is persistent or recurrent.
Albert Aynsley-Green, G. Soltész
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