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- W2246560265 abstract "Several genetic abnormalities are known to cause hyperinsulinism. The formerly held concept that congenital hyperinsulinism results from abnormal pancreatic development, termed ‘nesidioblastosis', is no longer accepted. The pathology of pancreases resected from individuals with inactivating mutations in the ATP-sensitive potassium channel is best studied and classified into diffuse and focal forms with their respective genetic alterations. A few variations within this group have been observed. The knowledge of the different histologic features aids surgical management by intraoperative frozen section evaluation along with preoperative imaging studies. Surgical intervention is less frequently applied to patients with other genetic abnormalities, including glucokinase and glutamate dehydrogenase abnormalities; therefore, systematic analyses are sparse. The pancreases of various genetic forms of hyperinsulinism may show different histologies from one another. Published reports along with our experience on this topic are summarized. Histology of the developing pancreas in young infants differs from that of normal adult pancreas. These features are the source of confusion in the earlier observations. Careful examination of the resected pancreases considering the age of the patients is necessary to describe and categorize the morphologic features, as well as and identify unrecognized patterns." @default.
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- W2246560265 date "2012-01-01" @default.
- W2246560265 modified "2023-09-25" @default.
- W2246560265 title "Pancreatic Histopathology of Hyperinsulinism" @default.
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- W2246560265 doi "https://doi.org/10.1159/000334493" @default.
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