Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Alimentary tract manifestations were found in all of 17 patients with multiple endocrine neoplasia, type 2b. The manifestations are important because (1) they were chronic, (2) they were severe and led to abdominal operation in 5 patients, (3) they antedated detection of the endocrine neoplasms in the syndrome in 16 patients (94%), and (4) they provided clinical clues that stimulated search for thyroidal C-cell and adrenal medullary disease in 6 patients. The alimentary tract manifestations were diverse: symptoms included constipation, diarrhea, difficulty with feeding, projectile vomiting, crampy abdominal pain, and loud borborygmi; findings included thickened lips, nodules on the anterior third of the tongue, abdominal distention, visible peristaltic waves, and roentgenographic evidence of megacolon or diverticulosis of the colon or of dilatation of the small intestine and stomach. Initial misinterpretation or failure to realize the significance of one or more of these alimentary tract manifestations led to suspicion of aganglionic megacolon (three patients), malabsorption syndrome (two patients), and tracheal ring (one patient).
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PMID:Alimentary tract manifestations of multiple endocrine neoplasia, type 2b. 89 96

Nineteen children underwent subtotal pancreatectomy for hyperinsulinemic hypoglycemia. Of the four who were older than 10 years of age at onset of symptoms, three had islet adenomas, and one had endocrine cell dysplasia (ECD). The two patients with multiple adenomas had a family history of multiple endocrine neoplasia, type 1 (MEN 1). Of the 15 who were younger than 1 year of age at onset of symptoms, 12 had ECD alone, and three had ECD with adenomatosis. Four children required a second surgical procedure and near-total pancreatectomy because of persistent hypoglycemia. All 19 patients' conditions improved, with no postsurgical complications. After near-total pancreatectomy, all four patients were treated for fat malabsorption, but only two required insulin because of secondary diabetes mellitus. We concluded that subtotal pancreatectomy is a safe, effective adjunct to the treatment of children with hyperinsulinemic hypoglycemia.
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PMID:Surgical management of hyperinsulinemic hypoglycemia in children. 614 50

Zollinger-Ellison syndrome (ZES) should be suspected if a patient has severe peptic ulceration, ulcers and kidney stones, a family history of ulcers or endocrine diseases, watery diarrhoea or malabsorption with or without ulcers, or if hypergastrinaemia is found. Any patient in whom ZES is suspected, and certainly if diagnosed, should be given large doses of antisecretory medication immediately. This should never be stopped except under controlled conditions or unless acid outputs have been reduced surgically. Patients cannot be managed safely without measuring acid outputs. These should be lowered to < 10 mmol/h, or < 5 mmol/h in patients with a previous gastric resection or severe oesophageal disease. Acid secretion can be controlled acutely in 70% of patients with an infusion of ranitidine 1 mg/kg/h, while 4 mg/kg/h will control acid in all. The initial oral dosage of omeprazole or lansoprazole should be 60 mg/day. Doses should then be adjusted daily on the basis of acid outputs. Proton pump inhibitors in a dosage of 60 mg/day will control acid output in most patients and 60 mg every 12 hours will control acid output in all. Doses can then often be slowly and progressively reduced. A parietal cell vagotomy reduces acid secretion and reduces, but does not abolish, the need for antisecretory medication. In patients with multiple endocrine neoplasia type 1 and hyperparathyroidism, a parathyroidectomy that results in normocalcaemia will reduce acid secretion and drug requirements. A total gastrectomy is rarely if ever needed nowadays. Given the high degree of safety of gastric antisecretory medications versus the risks of acid hypersecretion in patients with ZES, the mistakes in management of acid hypersecretion that must be avoided are those of giving insufficient medication and not measuring acid secretory rates.
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PMID:Zollinger-Ellison syndrome. Recognition and management of acid hypersecretion. 879 83

The parathyroid glands are the main regulator of plasma calcium and have a direct influence on the digestive tract. Parathyroid disturbances often result in unknown long-standing symptoms. The main manifestation of hypoparathyroidism is steatorrhea due to a deficit in exocrine pancreas secretion. The association with celiac sprue may contribute to malabsorption. Hyperparathyroidism causes smooth-muscle atony, with upper and lower gastrointestinal symptoms such as nausea, heartburn and constipation. Hyperparathyroidism and peptic ulcer were strongly linked before the advent of proton pump inhibitors. Nowadays, this association remains likely only in the particular context of multiple endocrine neoplasia type 1/Zollinger-Ellison syndrome. In contrast to chronic pancreatitis, acute pancreatitis due to primary hyperparathyroidism is one of the most studied topics. The causative effect of high calcium level is confirmed and the distinction from secondary hyperparathyroidism is mandatory. The digestive manifestations of parathyroid malfunction are often overlooked and serum calcium level must be included in the routine workup for abdominal symptoms.
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PMID:Digestive manifestations of parathyroid disorders. 2203 19