Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors presents the clinical case of autoimmune overlap syndrome: autoimmune hepatitis and primary biliary cirrhosis with outcomes in the hepatic cirrhosis complicated fatal intestinal bleeding.
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PMID:[Syndrome of overlap of autoimmune hepatitis and primary biliary cirrhosis with the outcome in cirrhosis of the liver, complicated by fatal bleeding in the small intestine]. 2394 68

Watermelon stomach or gastric antral vascular ectasia (GAVE) syndrome is an uncommon cause of sometimes severe upper gastro-intestinal bleeding. Essentially based on a pathognomonic endoscopic appearance, its diagnosis may be unrecognised because mistaken with portal hypertensive gastropathy, while treatment of these two entities is different. Its etiopathogeny remains still unclear, even if it is frequently associated with different systemic illnesses as hepatic cirrhosis, autoimmune disorders and chronic renal failure. The mechanism inducing these vascular ectasia may be linked with mechanical stress on submucosal vessels due to antropyloric peristaltic motility dysfunction modulated by neurohormonal vasoactive alterations. Because medical therapies are not very satisfactory, among the endoscopic modalities, argon plasma coagulation seems to be actually the first-line treatment because the most effective and safe. However, surgical antrectomy may be sometimes necessary. Recently GAVE syndrome appeared as a new adverse reaction of imatinib mesylate, one of the tyrosine kinase inhibitors used in chronic myeloid leukemia, and we report here the observation of such a pathology in one patient treated at the same time by haemodialysis and by imatinib mesylate for chronic myeloid leukemia.
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PMID:[Watermelon stomach: Chronic renal failure and/or imatinib?]. 2616 99

We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.
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PMID:Diverse multi-organ histopathologic changes in a failed Fontan patient. 2761 84

A 60-year-old women with a history of congenital pulmonary valve stenosis developed right heart failure, cardiac cirrhosis and end-stage renal disease requiring renal replacement therapy. Cirrhosis was complicated by portal hypertension, resulting in intractable gastro-intestinal bleedings despite optimal treatment with beta-blockers and endoscopic band ligation. Because of fears for worsening right heart failure, a decision for placement of a transjugular intrahepatic portosystemic shunt (TIPS) was initially turned down. However, as intractable bleeding problems persisted and caused heavy transfusion needs, TIPS was ultimately performed as a rescue procedure. Although TIPS successfully reduced the hepatic venous pressure gradient from 16 mmHg to 4 mmHg, portal pressure remained high at 14 mmHg because of persisting right heart failure with elevated central venous pressure. Hepatic encephalopathy soon developed after TIPS placement and culminated in multi-organ failure after another episode of gastro-intestinal bleeding. At this point, the family of the patient decided to withdraw care and the patient died subsequently. This case illustrates how important it is to diagnose and optimally treat right heart failure before cardiac cirrhosis with its impending complications emerges. Although TIPS may effectively treat complications of portal hypertension in the context of cirrhosis, persisting right heart failure may abrogate its beneficial effects.
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PMID:Unsuccessful transjugular intrahepatic portosystemic shunt for a patient with right heart failure and portal hypertension. 2936


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