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Insights in Congenital Hyperinsulinism
2007Congenital hyperinsulinism is characterized by the unregulated secretion of insulin from pancreatic Beta-cells. The inappropriate insulin secretion causes severe and persistent hypoglycaemia, which is a potent cause of brain damage if inappropriately managed.
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Surgery for Congenital Hyperinsulinism
2018Transient hypoglycemia in the newborn period is common and generally associated either with immaturity of the glucose regulatory pathways (which responds to frequent feeds and resolves spontaneously within hours), or with stress-associated hyperinsulinism (which responds well to hyperglycemic drugs and resolves spontaneously within the first few weeks ...
Pablo Laje, N. Scott Adzick
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Rapid Genetic Analysis in Congenital Hyperinsulinism
Hormone Research in Paediatrics, 2006<i>Backgound:</i> In severe, medically unresponsive congenital hyperinsulinism (CHI), the histological differentiation of focal versus diffuse disease is vital, since the surgical management is completely different. Genetic analysis may help in the differential diagnosis, as focal CHI is associated with a paternal germline <i>ABCC8<
Christesen, Henrik Thybo+9 more
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Congenital Hyperinsulinism of Infancy
2019Hyperinsulinism as a cause of persistent hypoglycaemia in infancy and childhood is very uncommon (1:50,000). The importance of preventing hypoglycaemia (and reducing the likelihood of neurologic damage) by the administration of adequate quantities of carbohydrate cannot be overstressed.
Lewis Spitz+3 more
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Congenital Hyperinsulinism (CHI)
2019The aim of this chapter is to highlight a rare endocrine condition (Congenital Hyperinsulinism, CHI), which can cause low blood glucose levels leading to permanent brain injury. Many Paediatric Nurses are unfamiliar with this condition. CHI is caused by unregulated insulin secretion from the pancreas and typically presents in the newborn period, but it
Kate Morgan+3 more
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Diazoxide-Unresponsive Forms of Congenital Hyperinsulinism
2019Congenital hyperinsulinism (HI) is defined as being “diazoxide-unresponsive” if the hypoglycemia persists despite maximum doses of diazoxide for at least five days . Inactivating mutations in the genes encoding the two subunits of the beta-cell ATP-sensitive potassium (KATP) channel are the most frequent cause of diazoxide-unresponsive hyperinsulinism.
Rayannavar, Arpana+2 more
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Histopathology of the Pancreas in Congenital Hyperinsulinism
2019The underlying genetic alterations in congenital hyperinsulinism (HI) are reflected in characteristic histopathologic changes in the pancreas of affected patients. The majority of cases with inactivating KATP mutations can be segregated into two morphologic forms, diffuse and focal HI, with corresponding alterations in islet cell nuclear size and ...
Tricia R. Bhatti, Eduardo Ruchelli
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Lanreotide therapy for congenital hyperinsulinism
Endocrine Abstracts, 2014Senthil Senniappan+3 more
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