All of the following statements about Nesidioblastosis are true, Except:
Correct Answer: Occurs in adults more than children
Description: Answer is B (Occurs in adults more than children) Nesidioblastosis is primarily a disease of infants & children. This condition is extremely rare in adults. Nesidioblastosis * Nesidioblastosis is essentially a non-insulinoma pancreatogenous hypoglycemic syndrome associated with islet cell hyperplasia and 13 cell hyper function * It is characterized by persistant or recurrent hyper insulinemic hypoglycemia (resulting from hyperplasia of the (3 cells of the pancreatic islet) The term `Nesidioblastosis' originates from the GretlIllf Wesidion meaning' islet. Age of Presentation * Nesidioblastosis is primarily a disease of infants and children * Nesidioblastosis is rare in adults Adult individuals with symptomatic hypoglycemia due to islet cell hyperophy (Nesidioblastosis) without insulinoma have been repoed but are rare Pathology * Islet hyperplasia with prominent hyperophic bizarre beta cells * Islet cell originating from pancreatic duct epithelium and then proliferating to form Islets of Langerhans Presentation * Symptomatic Hypoglycemia with endogenous hyperinsulinemia Treatment * Medical * Surgical Treatment with diazoxide and /or chlohiazide or with long acting somatostatin should be tried In infants with hyperinsulinism due to nesidioblastosis and adults with islet hyperplasia (nesidioblastosis) treatment should be instituted with diazoxide and chlohiazide or with long acting somatostatin - Clinical Endocrinology treatment should be considered only if above medical treatment if above medical treatment fails Subtotal Pancreatectomy (80% removal of pancreases) should be tried initially Total Pancreatectomy may be required in severe cases
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