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)

Biliary cystic disease, though uncommon, can present at a wide range of ages with a wide range of symptoms. Choledochal cysts are associated with the development of both cholangiocarcinoma and gallbladder cancer. Thus, most biliary cystic disease is best managed operatively. Many factors should be considered when performing surgery on patients with choledochal cysts, including age, presenting symptoms, cyst type, associated biliary stones, prior biliary surgery, intrahepatic strictures, hepatic atrophy/hypertrophy, biliary cirrhosis, portal hypertension, and associated biliary malignancy. When feasible, surgical treatment should consist of cholecystectomy and complete surgical excision of extrahepatic cysts with Roux-en-Y reconstruction. Because the risk of recurrent cholangitis is significant and additional symptoms and problems are common, the use of long-term soft Silastic biliary stents (Dow Corning Corp., Midland, MI) should be considered when complex intrahepatic and extrahepatic cystic disease is present. Alternatively, the Roux-en-Y jejunal limb can be marked at the fascia for future percutaneous access. Reconstruction via hepaticoduodenostomy and jejunal interposition has been associated with increased postoperative pain due to bile reflux gastritis. Thus, hepaticojejunostomy reconstruction is recommended. For choledochal cysts involving the distal bile duct, the bile duct should be excised at the intrapancreatic portion. Resection of the pancreatic head should be reserved for patients with established malignancies. Surgical excision of the intrahepatic portion of the bile duct should be individualized to include preservation of hepatic parenchyma when the liver is not cirrhotic. If cirrhosis is advanced, hepatic transplantation may be indicated, but this is rare. Oncologic principles should be followed in the presence of a malignancy. Lifelong follow-up is required because of the possibility of a "field" defect increasing susceptibility to cancer throughout the biliary tract epithelium.
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PMID:Biliary cystic disease. 1653 71

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 presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.
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PMID:Diagnosis and treatment of hepatic sarcoidosis. 1708 81

Hepatorenal syndrome is a severe, but not uncommon complication of decompensated liver cirrhosis. In particular, the rapidly progressive form of hepatorenal syndrome (type 1) is associated with a dismal prognosis. Established hepatorenal syndrome has a spontaneous reversibility below 5%. Hepatorenal syndrome is involved in more than 50% of cirrhosis-related mortality. Thus, any treatment capable of reversing hepatorenal syndrome would be expected to reduce morbidity and mortality from liver cirrhosis. A pathophysiological hallmark of hepatorenal syndrome is arterial underfilling due to an extreme splanchnic vasodilatation. Consequently, potent vasoconstrictors capable of reversing this vasodilatation have been investigated in hepatorenal syndrome. Several vasoconstrictors including the alpha-adrenergic agonists, midodrine and noradrenalin, and the vasopressor analogues, ornipressin and terlipressin, have all been associated with a significant improvement in renal function in 57 to 100% of cases and even reversal of hepatorenal syndrome in 42 to 100% of cases. The majority of recent studies are on terlipressin. A randomized, controlled trial showed a significant effect of terlipressin on reversal of hepatorenal syndrome. The contribution of volume expansion to the beneficial effects of vasoconstrictors on hepatorenal syndrome remains to be determined. In general, reversal of hepatorenal syndrome was associated with an improved survival. However, it remains to be determined if vasoconstrictor therapy should be used in hepatorenal syndrome in general, or if it should be reserved for potential candidates for liver transplantation. In conclusion, evidence for a beneficial effect of vasoconstrictor therapy for the treatment of hepatorenal syndrome is steadily accumulating. Confirmation of the preliminary data in larger randomized, controlled trials looking at long-term survival is required.
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PMID:Vasoconstrictor therapy for hepatorenal syndrome in liver cirrhosis. 1716 67

Because of the increased complications associated with cardiac surgery in patients with cirrhosis and portal hypertension, various preoperative preparations have been utilised. In order to reduce the bleeding risk by decompressing portosystemic collaterals and to correct the fluid shift, we performed transjugular intrahepatic portosystemic shunt (TIPS) in two patients with cirrhosis and portal hypertension prior to major cardiac surgery with cardiopulmonary bypass. Both patients had satisfactory surgical outcome with no bleeding complications. One patient developed hepatic encephalopathy which was managed medically. We believe that preoperative TIPS benefits the patient with cirrhosis and portal hypertension undergoing cardiac surgery by decreasing the major surgical complications through improvement of fluid imbalance and reduction of the bleeding risk. Because of the risks of TIPS, such as encephalopathy and liver failure, preoperative TIPS placement must be reserved for patients with fluid shift or high risk criteria of bleeding.
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PMID:Transjugular intrahepatic portosystemic shunt prior to cardiac surgery with cardiopulmonary bypass in patients with cirrhosis and portal hypertension. 1744 33

(Table is included in full-text article.)PBC in the advanced stage, corresponding to PBC stage IV, was shown in the past to be associated with an increased incidence of hepatocellular carcinoma (HCC). There is currently a debate, about the increase in incidence of extrahepatic malignancies, as some, but not all studies reported these neoplasms to be more common, especially breast cancer, irrespective of the PBC disease stage. In this issue of the journal a case series is reported on the incidence of various malignancies in a cohort of 212 patients with PBC from Greece. Considering as reference the cancer registries of another Mediterranean Country, like Italy, we could suggest that the incidence of extrahepatic malignancy, breast included, is not increased in PBC patients. Indeed, a more accurate analysis of the literature, shows that higher incidence of breast cancer were reported only for PBC patients evaluated in the 1970s and early 1980s, for whom a contribution of immunosuppressive agents, largely under investigation at that time, could be speculated. PBC patients do not need, therefore, to be submitted to stricter surveillance programs for extrahepatic cancer than the general population. As far as the development of HCC is concerned, instead, PBC patients should undergo the usual surveillance reserved to other categories of cirrhotic patients, according to published guidelines for the management of HCC. Such surveillance should start only when PBC patients have reached disease stage IV (frank cirrhosis).
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PMID:Malignancies in primary biliary cirrhosis. 1809 Sep 82

Chronic viral hepatitis remains a major problem among patients with chronic renal failure. Hepatitis B and C viruses are frequent among dialysis patients and after renal transplantation and may significantly diminish the survival of both the patient and the graft. Hepatitis B and C viral infection in these patients is often characterized by normal transaminase levels despite viremia and progressive liver lesions. Liver biopsy remains essential for assessing the extent of liver disease. Cirrhosis is a contraindication to transplantation of only a kidney, because of elevated morbidity and mortality. A combined as liver-kidney transplantation may be considered. The best treatment of hepatitis infections is preventive: vaccination against the hepatitis B virus and attentive hygiene, especially to prevent nosocomial transmission. Among patients not awaiting transplant, antiviral treatment should be reserved for patients with active or even fibrotic liver disease. For hemodialysis patients awaiting kidney transplant: Alpha interferon is ineffective and poorly tolerated by dialysis patients. Lamivudine is effective and well tolerated, but its long-term efficacy and its optimal effective dose in dialysis patients remain unknown.
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PMID:[Chronic viral hepatitis and kidney failure]. 1829 15

Bile duct strictures are a common complication in patients with advanced chronic pancreatitis and have a variable clinical presentation ranging from an incidental finding to overt jaundice and cholangitis. The diagnosis is mostly made during investigations for abdominal pain but jaundice may be the initial clinical presentation. The jaundice is typically transient but may be recurrent with a small risk of secondary biliary cirrhosis in longstanding cases. The management of a bile duct stricture is conservative in patients in whom it is an incidental finding as the risk of secondary biliary cirrhosis is negligible. Initial conservative treatment is advised in patients who present with jaundice as most will resolve once the acute on chronic attack has subsided. A surgical biliary drainage is indicated when there is persistent jaundice for more than one month or if complicated by secondary gallstones or cholangitis. The biliary drainage procedure of choice is a choledocho-jejunostomy which may be combined with a pancreaticojejunostomy in patients who have associated pain. Since many patients with chronic pancreatitis have an inflammatory mass in the head of the pancreas, a Frey procedure is indicated but a resection should be performed when there is concern about a malignancy. Temporary endoscopic stenting is reserved for cholangitis while an expandable metal stent may be indicated in patients with severe co-morbid disease.
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PMID:Biliary tract obstruction in chronic pancreatitis. 1834 88

Gastric antral vascular ectasia (GAVE), though a rare disorder, causes up to 4% of non-variceal upper GI bleeding. This paper gives an overview of studies examining clinical presentation and pathophysiology, and reviews the current evidence for invasive and non-invasive treatments. GAVE is often associated with systemic illnesses, such as cirrhosis of the liver, autoimmune connective tissue disorders, bone marrow transplantation and chronic renal failure. The pathophysiological changes leading to GAVE have not been fully explained and remain controversial. Patient presentation varies from chronic iron-deficiency anaemia to heavy acute gastrointestinal bleeding. It is important to differentiate GAVE from portal hypertensive gastropathy as GAVE does not respond to measures reducing portal pressures. Endoscopic ablation (Nd:YAG-laser or argon plasma coagulation) is the first-line treatment of choice. As evidence for pharmacological therapy with oestrogen (and/or progesterone), tranexamic acid or thalidomide stems from case reports only, these should be used if endoscopic measures have failed to stop chronic blood loss. Surgical antrectomy should be reserved for unresponsive cases as it is associated with a high mortality. Ultimately, treatment of the underlying medical co-morbidities may lead to resolution of GAVE.
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PMID:Gastric antral vascular ectasia (GAVE): an update on clinical presentation, pathophysiology and treatment. 1839 91

Pregnancy in patients with portal hypertension is rare but worrying for the clinician. Although the effects of portal hypertension during pregnancy have not been fully elucidated, there is an evident increase in morbidity, especially associated with cirrhosis, which justifies the idea of at-risk pregnancy and requires management by a multidisciplinary team. The prevention and treatment of gastrointestinal haemorrhage is quite similar to that in nonpregnant patients. Investigation and management of portal hypertension before and at the beginning of pregnancy can reduce the risks of foetal loss, restricted intra-uterine growth, premature birth and maternal mortality, which are closely related to gastrointestinal haemorrhage. The risks related to the underlying disease, such as liver failure with cirrhosis and thromboembolic risk with vascular diseases associated with thrombophilia must be taken into consideration. Generally, vaginal delivery with early analgesics for the mother assisted by an extraction device should be preferred to caesarean section, which must be reserved for obstetrical indications.
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PMID:[Portal hypertension and pregnancy]. 1847 75


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