Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042961 (volvulus)
 4,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient's symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen's disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.
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PMID:Small bowel volvulus as a complication of von Recklinghausen's disease: a case report. 2497 35

Neurofibromatosis type 1(NF-1) with gastrointestinal stromal tumor (GIST) is a rare case in clinical practice. But it is even rarer that the GIST tumor in this case is so large that it cause small intestinal obstruction. Here we report such a male case of NF-1,with positive family history of NF-1 and nodules all over the patient's skin and abdominal cavity. The patient came to hospital with a sudden upper abdominal pain and nausea and vomiting over 5 d. Abdominal computerized tomography (CT) showed that there were numerous nodules of different size in his abdominal cavity. And the largest one is about 10 cm in diameter,which oppressed the small bowel and caused the small bowel mesenteric volvulus and obstruction of the bowel. Finally,the tumor was pathologically proved to be a GIST tumor but not NF nodule. The patient stays healthy until now after operation.
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PMID:[A case of neurofibromatosis complicated with small bowel obstruction]. 2765 Nov 96