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)

The description is given of Shwachman syndrome in a male of 17, with a long anamnesis of malabsorption syndrome, granulocytopenia, repeated bronchopneumonia, somatogenic subnanism and hypogonadism. The death resulted from acute cardiovascular insufficiency. Post-mortem examination revealed exocrine pancreas hypoplasia with lipomatosis, Wirsung duct atresia, fibrocholangiocytosis and fat hepatosis, visceral anemia, delay in the maturation of granulocytes in the bone marrow, somatogenic polyglandular endocrinopathy, metabolic myocardium necrosis, calcinosis of the small arteries wall, lymphoid tissue atrophy, focal pneumonia of the upper lobe of the left lung.
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PMID:[Congenital hypoplasia of the pancreas with lipomatosis and bone marrow dysfunction (Shwachman syndrome)]. 174 73

The authors describe a major pancreatic lipomatous infiltration, causing a "pseudo-hypertrophy" of the pancreas, in a 70-year-old man. This lesion was responsible of a hyperechogenic area at ultrasonography and of an empty pancreatic bed ("vanishing pancreas") at computed tomography, suggesting lipomatosis. This entity is a special modality of senescence of pancreatic tissue whose origin remains obscure. The progressive atrophy of the acinar lobules and the islets of Langerhans was finally responsible of a mild degree of malabsorption and of diabetes type II. The compression of the main bile duct by the enlarged lipomatous pancreas, demonstrated by percutaneous cholangiography, caused a cholestatic jaundice with abdominal pain, which was treated by a surgical derivation (hepatico-duodenostomy). This is the first description of a lipomatous pseudohypertrophy of the pancreas causing an obstruction of the common bile duct.
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PMID:[Cholestatic jaundice complicating lipomatous pseudo-hypertrophy of the pancreas]. 207 97

The case of a 57-year-old woman is described with a two months history of proximal muscle weakness and pain, marked hypotrophy and brisk reflexes. Clinical investigation demonstrated normal serum CK, myopathic EMG and osteomalacia. Muscle biopsy showed type II fibre atrophy and mitochondrial alterations without inclusions. Further examinations including a jejunal biopsy revealed malabsorption accounting for osteomalacia. At autopsy diffuse nodular lipomatosis of the small bowel was detected (Acta neurol. belg., 1982, 82, 65-71).
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PMID:Osteomalacic myopathy in a case of diffuse nodular lipomatosis of the small bowel. 709 Jul 30

Total pancreatic lipomatosis (PL) is an unusual entity of pathologic significance and speculative origin. It refers to complete replacement of pancreatic parenchyma by fat cells. Fat replacement may vary from mild fatty infiltration to massive replacement of the pancreas by adipose tissue, resulting in malabsorption syndrome due to pancreatic insufficiency. We present a case of a 60-year-old elderly woman with atypical abdominal complaints, diabetes mellitus, weight loss, and steatorrhea. Abdominal computed tomograms were diagnostic of PL. Magnetic resonance imaging verified this impression. The patient improved clinically after the 8-week trial of high-dose oral pancreatic enzyme replacement therapy. There is a marked reduction of steatorrhea and weight gain. This case report focuses on pathophysiology, diagnosis, and treatment guidelines of PL.
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PMID:A case report on total pancreatic lipomatosis: An unusual entity. 2908 72