The following is true about Nesidioblastosis except?
Correct Answer: More common in adults than in children
Description: Nesidioblastosis
In 1938, Laidlow coined the term nesidioblastosis to describe the neodifferentiation of islets of Langerhans from pancreatic ductal epithelium.
Severe recurrent hypoglycemia associated with an inappropriate elevation of serum insulin, C-peptide and proinsulin defines this disorder.
The disorder later was called persistent hyperinsulinemic hypoglycemia of infancy (PHHI); currently many authors prefer the term congenital hyperinsulinism (CI).
PHHI is the most common cause of hyperinsulinemic hypoglycemia in neonates.
usually affects the infants and children. Adults can also be affected but at a much lower incidence.
Histopathology
Microscopically, pheochromocytomas are composed of polygonal to spindle-shaped chromaffin cells and their supporting cells, compartmentalized into small nests or "Zellballen," by a rich vascular network.
The cytoplasm of the neoplastic cells often has a finely granular appearance, highlighted by a variety of silver stains, because of the presence of granules containing catecholamines. Electron microscopy reveals variable numbers of membrane-bound, electron-dense granules, representing catecholamines and sometimes other peptides. The nuclei of the neoplastic cells are often quite pleomorphic.
Presentation
Most patients with PHHI present shortly after birth with symptoms of hypoglycemia (e.g. hunger, jitteriness, lethargy, apnea, seizures). Older children, in addition to above symptoms, may also show diaphoresis, confusion, or unusual mood or behaviour changes.
The symptoms may be exacerbated by fasting and may improve after eating.
Treatment
Immediate treatment of hypoglycemia is essential. Patients may require continuous IV glucose infusion. Glucagon may also be administered.
Other drugs used are diazoxide, octeride and nifedipine.
Surgical treatment is indicated if medical therapy does not maintain normoglycemia.
Category:
Pediatrics
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