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Query: UMLS:C0000727 (acute abdomen)
 3,084 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a patient with von Recklinghausen's disease, who was admitted with a diagnosis of acute abdomen due to small bowel neurofibromatosis. The patient was submitted to an abdominal CT scan that showed a homogeneous round lesion, with a regular margin, probably belonging to the small bowel and with the appearance of a benign lesion that probably caused an intestinal intussusception. The patient was submitted to a surgical procedure that mainly consisted in multiple small bowel resections. The histopathological examination confirmed the benign nature of the lesions. About one third of patients affected by von Recklinghausen's disease present involvement of the bowel, but only 5% of them are symptomatic. The intestinal tumours are usually neurofibromas and are mainly localized in the jejunum. However, there have also been reports of stromal, nervous and endocrine tumours and even other tumours not belonging to these categories, including adenocarcinoma. The overall incidence of intestinal malignancy in patients with von Reckilnghausen's disease is about 10%. The surgical operation, as well as the histopathological and immunochemical examination of the intestinal lesions are of crucial importance for the treatment of the complications of intestinal neurofibromatosis and for the treatment and diagnosis of malignancy.
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PMID:[Von Recklinghausen's disease and intestinal neurofibromatosis: a case report]. 1274 3

Dysplasia of the renal and cervical arteries are well known, but dysplasia of the superior mesenteric artery (SMA) is less frequent and has specific presentation. There have been few reports on the different types of presentations. We report a series of 38 cases and present the characteristic features together with a comparison with data in the literature. These non-atheromatous non-inflammatory lesions of the arterial wall occurred either in a context of fibromuscular disease which cause is unknown (30/38 patients) or in patients with genetic disease such as neurofibromatosis (3/38) or Ehlers-Danlos disease (5/38). The fibromuscular disease presented three aspects with specific characteristics. a) stenosing lesions found predominantly in women with a different morphology than in the other localization (usually associated): irregular diffuse stenosis discovered in patients with hypertension, or ischemic digestive symptoms (6/14 patients). b) Aneurysms, also found predominantly in women, but less frequently associated with other dysplasias. These aneurysms were generally sacciform. Unlike data in the literature, our series only included rupture in 1/8 cases. The aneurysm was discovered during the exploration of abdominal pain or hypertension. c) The third type of fibromuscular disease concerned dissections which were observed in the male population, (except one case of segmental dysplasia), and presented with signs of digestive ischemia in the other seven patients, four in an emergency context requiring immediate treatment. Two genetic diseases were observed. a) Neurofibromatosis led to dysplasia analogous to fibromuscular disease, but with abnormal nerve formations in the wall. For the three cases observed, one involved only the visceral arteries and the two others thoraco-abdominal coarctation. b) We had five patients with Ehlers-Danlos syndrome, with dysplasia of the superior mesenteric artery. For three there was a fusiform dilatation and in one small aneurysms along the arterial trunk, which only required surveillance. There was only one aneurysm which required treatment by resection and venous graft. In these young patients, revascularization was generally achieved with autologous material and the prognosis was favorable. Embolization was successful in treating the sacciform aneurysm in one patient. In conclusion, dysplasia of the superior mesenteric artery has a specific presentation which must be recognized to enable diagnosis of this not uncommon condition in young subjects (even children) who often present with an acute abdomen requiring urgent treatment.
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PMID:[Thirty-eight cases of dysplasia of the superior mesenteric artery]. 1614 79

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

Spontaneous hemoperitoneum (SH) is a rare entity which can be life-threatening. Gastrointestinal neurofibromas are rare. Occasionally, such lesions may be the initial sign of NF1 in patients without any other clinical manifestations of the disease. The clinical presentations of isolated neurofibromatous lesions of the intestines are variable. In asymptomatic patient no treatment may be required and patient may be kept on follow up. Occasionally, they manifest with complications such as intestinal bleeding, obstruction or perforation. Surgery is the treatment of choice in symptomatic intestinal neurofibroma. We present the case of a 55 year-old male with acute abdomen due to rupture of isolated neurofibroma as one of the cause of SH. This case represents a rare presentation of isolated intestinal neurofibromatosis in a patient without systemic manifestations and highlights the need for high index of suspicion to exclude neurofibromatosis type 1 or multiple endocrine neoplasia type 2b.
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PMID:An unusual cause of hemoperitoneum: case report with review of literature. 2607 Jan 85