Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten children (4.6%) among a cohort of 219 with Kawasaki disease (KD) had their onset with severe abdominal complaints. Incomplete KD presentation at the time of acute abdomen was present in nine of 10 patients. Acute abdominal pain and distension, vomiting, hepatomegaly, and jaundice were the most common symptoms at onset. Hematemesis was present in one; toxic shock syndrome requiring care in the intensive care unit occurred in four. Five patients had laparotomy, three had percutaneous transhepatic biliary drainage, and one had a gastrointestinal endoscopy. Postoperative diagnosis was gallbladder hydrops with cholestasis in five, paralytic ileus in three, appendicular vasculitis in one, and hemorrhagic duodenitis in one. All patients completely recovered, but 50% developed coronary aneurysms despite early intravenous gammaglobulin treatment. Acute surgical abdomen can be the presenting manifestation of KD. In older children with fever, rash, and acute abdominal pain or hematemesis, KD should be considered in the differential diagnosis.
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PMID:Acute surgical abdomen as presenting manifestation of Kawasaki disease. 1283 7

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.
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PMID:Etiology and consequences of thrombosis in abdominal vessels. 1653 66

Although infants with meconium ileus usually present with apparent symptoms shortly after birth, the diagnosis of meconium ileus and cystic fibrosis (CF) may be delayed, awaiting newborn screening (NBS) results. We present the case of an 11-day-old term girl with delayed passage of meconium at 48 hours who had 2 subsequent small meconium stools over the following week. There was a normal feeding history and no signs of abdominal distension or distress. She then presented with an acute abdomen, decompensated shock, bowel perforation, and peritonitis, requiring multiple intestinal surgeries. Her NBS for CF was positive, and CF was ultimately confirmed with mutation analysis. Her course was complicated by prolonged parenteral feedings and mechanical ventilation via tracheostomy. The infant was managed with soy oil, medium chain triglycerides, olive oil, fish oil lipids and experienced only transaminitis without cholestasis and no chronic liver sequelae, with subsequent normalization of her transaminases without treatment. Because her only symptom was decreased stool output and NBS results were unavailable, the CF diagnosis was delayed until she presented in extremis. Delayed meconium passage and decreased stool output during the first week of life should lead to suspicion and additional evaluation for CF while awaiting NBS results. Careful monitoring is indicated to prevent serious, life-threatening complications. The use of soy oil, medium chain triglycerides, olive oil, fish oil lipids for infants requiring prolonged parenteral nutrition may also be considered proactively to prevent cholestasis, particularly for high risk groups.
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PMID:Delayed Presentation of Meconium Ileus in an Infant With Cystic Fibrosis. 3297 95