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)

A 54-year-old man with end-stage cirrhosis of the liver presented for evaluation of dyspnea. Intrapulmonary arteriovenous shunting was suspected. Transthoracic echocardiography with agitated saline contrast injection from the right antecubital vein was performed. Late arrival of saline contrast bubbles in the left atrium by pulmonary veins leading to complete left heart opacification confirmed the diagnosis of significant intrapulmonary shunting. This case and images illustrate the use of agitated saline contrast in combination with transthoracic echocardiography for the diagnosis of intrapulmonary arteriovenous fistula.
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PMID:Intrapulmonary arteriovenous shunt: diagnosis by saline contrast bubbles in the pulmonary veins. 1222 24

Hepatocellular carcinoma is the seventh most common cancer in men and ninth in women. The authors describe a case of an 83-year old man with a history of cryptogenic cirrhosis and a remote history of tuberculosis who presented with sudden onset of dyspnea. Screening ultrasound of the abdomen and alpha-fetoprotein levels three months before this acute presentation were not suggestive of hepatocellular carcinoma. Chest roentgenogram showed a miliary pattern of pulmonary nodules and the patient was admitted with the diagnosis of miliary tuberculosis. Biopsy established the diagnosis of hepatocellular carcinoma with pulmonary metastases.
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PMID:Unusual presentation of hepatocellular carcinoma: a case report. 1240 55

We report a case of congenital biliary atresia and liver cirrhosis who developed hepatopulmonary syndrome thirteen years after Kasai operation. This 15-yea-old girl suffered from hyperbilirubinemia and acholic stool since birth. Congenital biliary atresia was diagnosed at 6 weeks of age and she received Kasai operation when liver cirrhosis was also found during the surgery. Dyspnea and cyanosis insidiously occurred since the age of 13 and she had been admitted to our hospital due to episodes of esophageal varices bleeding where esophageal varices ligation was performed. Serial examinations showed PaO2: 71 mmHg despite of 100% oxygen supplement. Pulmonary catheterization showed multiple arteriovenous shunts and macroaggregated albumin test revealed significant shunting. Contrast-enhanced echocardiogram showed microbubbles in the left heart. Thus, hepatopulmonary syndrome type II was diagnosed. Treatment is a dilemma although liver transplantation with concomitant lung transplantation is a possible choice.
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PMID:Hepatopulmonary syndrome in a child. 1271 13

The majority of cirrhotic patients present dyspnea, but most of the time this is a consequence of ascites, hepatic hydrothorax or cardiopulmonary disease. Very few of these patients undergo the hepatopulmonary syndrome, an infrequent complication of chronic liver disease, especially when associated with portal hypertension and cirrhosis. Hepatopulmonary syndrome is characterised by dyspnea and, consequently, arterial hypoxemia, due to intrapulmonary vasodilation and shunting. The real pathogenesis of this vasodilation is still unknown, although many hypotheses have been suggested. Furthermore, no medical therapy appears to be really useful in the treatment of hepatopulmonary syndrome. At the present time, the only treatment that is efficacious for the resolution, or for the improvement of blood gas oxygenation abnormalities, is orthotopic liver transplantation.
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PMID:Hepatopulmonary syndrome. A review of the literature. 1285 33

A 59-year-old woman was admitted to hospital 10 months after receiving a liver transplant (LT) for hepatitis C virus (HCV) cirrhosis because of fever, dyspnea and basal patchy peripheral infiltrates. Microscopic examinations and blood, sputum and BAL cultures were negative. Empirical anti-infective therapy was ineffective. Thoracoscopic lung biopsy was performed, and histology showed a pattern suggesting bronchiolitis obliterans organizing pneumonia (BOOP). Prednisone led to rapid clinical and radiologic improvement. BOOP has been anecdotally reported in LT cases, and this case was unrelated to any infectious agent. BOOP should be taken into account in the differential diagnosis of pneumonia in LT.
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PMID:A 59-year-old liver-transplanted woman with fever, dyspnea and pulmonary infiltrates. 1461 53

EPIDEMIOLOGY ADN PHYSIOPATHOLOGY: Hereditary haemochromatosis is the most common genetic disease in France. Its frequency is on average 1 out of 300 French individuals. It is due to excessive dietary iron absorption, leading to accumulation of iron in the body. Mutations of the HFE1 gene are responsible for the majority of the case of haemochromatosis. FROM A CLINICAL POINT OF VIEW: The first clinical manifestations (weakness, sexual dysfunction, arthralgia, cardiac symptoms, dyspnoea on effort) can occur after the age of 30 years in men and 35 years in women (protected for longer by menstruation, pregnancy and delivery). In the absence of diagnosis, severe complications can develop during the 5th decade: nervous breakdown, arthropathy, heart failure, diabetes mellitus, cirrhosis with risk of progression towards carcinoma, responsible for handicaps and premature death. DIAGNOSTIC ELEMENTS: The diagnosis is evoked in the case of an increase in transferrine saturation (>45%), associated or not with excessive ferritin plasma levels. It is confirmed by the genetic test, showing homozygotes for the C282Y mutation or compound heterozygotes for the C282Y and H63D mutations on the HFE1 gene. RMI quantifies hepatic iron loading and generally avoids the need for a liver biopsy. The differential diagnosis must exclude secondary iron overload due to chronic transfusions in congenital or acquired blood diseases, a polymetabolic syndrome, chronic viral or alcoholic hepatic diseases and porphyria cutanea tarda. EFFICIENT TREATMENT: Today, haemochromatosis is still treated by phlebotomy. This consists in withdrawing 400 to 500ml of blood every week at the initial depletion stage and subsequently a maintenance therapy in order to maintain ferritin levels below 50 ng/ml. Paradoxically and through ignorance, hereditary haemochromatosis remains a serious disease, although its diagnosis is easy and the treatment simple and effective.
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PMID:[Hereditary haemochromatosis]. 1579 37

Pleural effusion in patients with liver cirrhosis and intractable ascites is well known, but hepatic hydrothorax in the absence of ascites is a rare complication. We present the case of a 43-year old male, with a medical history of liver cirrhosis due to hepatitis C virus, who was admitted to the Pneumology Clinic for dyspnoea, worsening of general status and chronic asthenia. The pleural effusion, revealed on physical and laboratory examinations, persisted despite the therapy with diuretics and the frequent thoracocentesis. The thoracostomy followed by pleurodesis also failed. The pecularity of this case was the presence of refractory hydrothorax in the absence of ascites.
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PMID:Hydrothorax without ascites in liver cirrhosis. 1472 78

Hepatocellular carcinoma is a tumor that rarely invades the inferior vena cava and right heart. We describe the case of a 63-year-old man with jaundice and a history of cryptogenic hepatic cirrhosis with diagnosis of hepatocellular carcinoma. He was referred to the echocardiography laboratory for sudden dyspnea and suggestion of pulmonary thromboembolism. Echocardiographic study reported a mass in the right atrium proceeding from the inferior vena cava.
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PMID:Hepatocellular carcinoma with invasion into right cardiac cavities: report of a case and literature review. 1475 97

Hepatocellular carcinoma (HCC) is one of the leading causes of cancer death in Taiwan. In order to delineate the unique demographic features and clinical profile of terminal HCC, we conducted a retrospective study in a hospital-based hospice in Taiwan. Of a total of 991 terminally ill cancer patients (654 men and 337 women, mean age 66.1 years) admitted to our palliative care unit during a three-year period, 110 patients (11.1%) were diagnosed as having HCC (93 men and 17 women, mean age 60.5 years). The most common metastatic sites were bone and lung. Eighty-five HCC patients (77.3%) also had associated liver cirrhosis. The most common symptoms of HCC patients upon admission to the hospice ward were pain, fatigue or weakness, anorexia/vomiting, peripheral edema, cachexia, and ascites. Hypoalbuminemia, anemia, hyponatremia and jaundice were common laboratory abnormalities. Eighty-four patients (76.4%) required opiates for pain management. Upper gastrointestinal bleeding or varices bleeding developed in 76 patients (69.1%). Ninety-four patients (85.5%) died at the hospital, and the overall median survival time at hospice ward was 12 days. Because of more severe underlying portal hypertension and deteriorated liver function, terminal HCC patients with decompensated liver cirrhosis (Child-Pugh class C) had a significantly higher prevalence of peripheral edema, ascites, dyspnea, jaundice, thrombocytopenia, and stage III-IV hepatic encephalopathy than noncirrhotic or Child-Pugh class A and B terminal HCC patients. Symptoms and signs resulting from these portal hypertensions frequently complicated the symptomatic management of terminal HCC patients in the hospice ward. The treatment of these complications is mostly empirical in hospice ward, where intensive laboratory or diagnostic tests are usually not performed. In conclusion, symptoms and signs of terminally ill HCC patients in hospice are unique and should be managed appropriately.
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PMID:Hospice palliative care for patients with hepatocellular carcinoma in Taiwan. 1504 5

The hepatopulmonary syndrom occurs when pulmonary microvacular dilatation causes hypoxemia in cirrhosis. It is found in between 15-20% of patients with chronic liver diseases and should be considered in the differential diagnosis of dyspnea or abnormal arterial oxygenation in this group. The presence of HPS appears to significantly increase mortality in affected patients with cirrhosis. The mediators of intrapulmonary vasodilatation and HPS are not fully characterized although pulmonary nitric oxide overproduction appears to be a key event in human and experimental models. Contrast echocardiography is the best screening test for pulmonary vasodilatation. Currently there are no effective medical therapies for HPS, although liver transplantation results in reversal of HPS in most cases. However, mortality is higher in patients with HPS undergoing transplantation relative to those without HPS.
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PMID:Hepatopulmonary syndrome. 1548 33


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