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)

Watermelon stomach (WMS), or gastric antral vascular ectasia, is an uncommon but clinically important cause of chronic occult or overt gastrointestinal (GI) blood loss. Patients typically present with symptomatic anemia and hemoccult positive stools. Although the diagnosis is based primarily on the typical endoscopic appearance, the lesion may be overlooked on initial evaluation or interpreted as "gastritis." Gastric biopsy may be helpful in confirming the diagnosis by showing vascular ectasia, typically without inflammation. WMS is idiopathic but is often associated with autoimmune diseases or cirrhosis of the liver. The majority of patients with classic WMS are elderly and female. In contrast, there is no gender predominance with the diffuse pattern associated with cirrhosis. General supportive care includes transfusion of blood products as necessary to control symptomatic anemia and coagulopathy, iron replacement therapy by oral or parenteral routes, identification and treatment of iatrogenic (eg, warfarin) or hereditary (eg, von Willebrand's disease) coagulopathy, and avoidance of substances that might cause gastric mucosal damage and/or bleeding (eg, aspirin, NSAIDs, alcohol). The goals of therapy are to eliminate or decrease the need for blood transfusions, hospitalization, office visits, and endoscopic therapy sessions aimed at cessation of GI blood loss and resolution of symptomatic anemia. Multipolar electrocoagulation is our preferred technique for endoscopic ablation of WMS. A 10-Fr probe (3.2 mm in diameter) is used at a generator setting of 12 to 16 W. Pulse duration can be as short as 1 to 2 seconds if a pinpoint coagulation technique is used, or continuous if a paint-stroke technique is used to coagulate all vascular stripes in the classic WMS pattern or as many small lesions as possible in the diffuse type. Other techniques we currently use are argon plasma coagulation (APC) or heater probe. In the past, lasers (neodymium: yttrium-aluminum-garnet , KTP, or argon) were successfully used for such treatments. An initial treatment interval of 4 to 8 weeks should allow for interim healing of iatrogenic ulcers. Patients are routinely given standard doses of available proton-pump inhibitors (PPIs) to facilitate healing of iatrogenic ulcers and to prevent secondary bleeding. The treatment interval can be gradually lengthened as the long-term goals of obliteration of angiomata and resolution of anemia are reached. Side effects may include iatrogenic ulceration at the site of treatment, bleeding, and transient abdominal pain. Antral scarring (after APC or Nd:YAG laser) and hyperplastic polyps have also been reported after endoscopic treatment of classic WMS. Surgical antrectomy is very effective in the prevention of bleeding but has substantial (5% to 10%) mortality in older patients with comorbidity and is now reserved for endoscopic failures.
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PMID:Treatment of watermelon stomach. 1653 78

The glycosylation profile of von Willebrand factor (VWF) is known to strongly influence its plasma levels. VWF contains several carbohydrate structures, including O-linked glycans that primarily consist of sialylated T antigen (NeuAc(alpha2-3)Gal-(beta1-3)-[NeuAc(alpha2-6)]GalNAc). It is not yet known whether O-linked carbohydrates affect VWF levels. We developed an immunosorbent assay based on neuraminidase incubation allowing subsequent binding of peanut agglutinin (PNA) to desialylated O-linked T antigen on VWF. An inverse relation was found between PNA binding and VWF antigen levels in healthy individuals (n = 111; Pearson rank = -0.43; P < .001). A similar inverse association was observed in randomly selected plasma samples from our diagnostic laboratory: 252% +/- 125% for VWF levels less than 0.5 U/mL (n = 15); 131% +/- 36% for VWF levels between 0.5 and 1.5 U/mL (n = 32); and 92% +/- 40% for VWF levels more than 1.5 U/mL (n = 19). Reduced or increased PNA binding was also observed in patients with increased (liver cirrhosis) or reduced (von Willebrand disease [VWD] type 1) VWF antigen levels, respectively. VWD type 1 patients further displayed increased ratios of propeptide over mature VWF antigen levels (0.38 +/- 0.18 versus 0.17 +/- 0.03 for patients and controls, respectively; P < .001), which is indicative of reduced VWF survival in these patients. Of interest, a linear relation between PNA binding and propeptide/VWF ratio was observed (Spearman rank = 0.47), suggesting a potential association between O-linked glycosylation and VWF survival. Finally, we detected a marked decrease in PNA binding in post-DDAVP (1-deamino-8-D-arginine vasopressin) samples from various patients, indicating that the O-linked glycosylation profile of VWF stored in endothelial storage organelles may differ from circulating VWF.
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PMID:Variations in glycosylation of von Willebrand factor with O-linked sialylated T antigen are associated with its plasma levels. 1709 Jun 49

Stimulation with the vasopressin analogue desmopressin (DDAVP) of extrarenal arginine vasopressin (AVP) V2-receptors in endothelial cells and possible in platelets increases the circulating levels of coagulation factor VIII (FVIII), von Willebrand factor (VWF) and tissue plasminogen activator (t-PA). The purpose of this paper is to provide an updated review of current information on the efficacy and safety of DDAVP in the treatment of haemophilia, von Willebrand disease (VWD), uremia, liver cirrhosis, and in congenital or drug-induced platelet dysfunction--under surgical or non-surgical conditions. In summary, desmopressin is an effective haemostatic drug that when administered i.v., s.c. or intranasally increases plasma levels of FVIII and VWF 2-6 times and improves platelet function. It has a proven haemostatic efficacy in mild haemophilia A and VWD as well as in uremia, liver cirrhosis and in congenital and acquired, drug induced platelet dysfunction. Desmopressin has few side effects but observation is advised in small children and elderly.
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PMID:Desmopressin in treatment of haematological disorders and in prevention of surgical bleeding. 2470 70

Liver diseases are associated with complex changes in the hemostatic system and elevated levels of the platelet-adhesive protein Von Willebrand factor (VWF) are reported in patients with acute and chronic liver damage. Although elevated levels of VWF are associated with fibrosis in the general population, the role of VWF in acute and chronic liver injury has not been examined in depth in experimental settings. We tested the hypothesis that VWF deficiency inhibits experimental liver injury and fibrosis. Wild-type (WT) and VWF-deficient mice were challenged with carbon tetrachloride (CCl4) and the impact of VWF deficiency on acute liver injury and chronic liver fibrosis was determined. VWF deficiency did not significantly affect acute CCl4-induced hepatocellular necrosis in mice. Chronic CCl4 challenge, twice weekly for 6weeks, significantly increased hepatic stellate cell activation and collagen deposition in livers of WT mice. Interestingly, hepatic induction of several profibrogenic and stellate cell activation genes was attenuated in VWF-deficient mice. Moreover, birefringent sirius red staining (indicating type I and III collagens) and type I collagen immunofluorescence indicated a reduction in hepatic collagen deposition in CCl4-exposed VWF-deficient mice compared to CCl4-exposed WT mice. The results indicate that VWF deficiency attenuates chronic CCl4-induced liver fibrosis without affecting acute hepatocellular necrosis. The results are the first to demonstrate that VWF deficiency reduces the progression of liver fibrosis, suggesting a mechanistic role of elevated plasma VWF levels in cirrhosis.
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PMID:Von Willebrand factor deficiency reduces liver fibrosis in mice. 2852 13


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