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)

On the basis of limited experimental and clinical studies, increased activity of the vasodilatory nitric oxide-cyclic guanosine monophosphate pathway is considered to play a key role in the pathogenesis of hepatopulmonary syndrome. We report a 46-year-old woman with Child-Pugh class C cirrhosis and progressive dyspnoea for 12 months. Investigations revealed elevated circulating concentrations of nitric oxide metabolites and exhaled nitric oxide levels, an hyperdynamic circulation with low systemic vascular resistance and mean arterial pressure, a large right to left intrapulmonary shunt fraction on radiolabelled macroaggregated albumin perfusion scanning, positive contrast-enhanced echocardiography, reduced diffusion capacity of carbon monoxide, hypoxaemia and orthodeoxyia, all in keeping with severe hepatopulmonary syndrome. Sequential inhibition of the nitric oxide-cyclic guanosine monophosphate pathway using curcumin (diferuloylmethane), terlipressin and methylene blue was associated with substantial improvements in vascular tone and the hyperdynamic circulation. No improvement, however, in the intrapulmonary shunt was demonstrated. Both hypoxaemia and orthodeoxia were substantially, reproducibly and reversibly worsened with all three treatments. Our findings argue against the contention that intrapulmonary shunting and impairment in arterial oxygenation in hepatopulmonary syndrome are necessarily the consequence of on-going, nitric oxide-cyclic guanosine monophosphate-mediated vasodilatation, at least in the chronic stage, and, given the possibility of substantial worsening of pulmonary oxygen exchange, suggest that inhibition of the nitric oxide-cyclic guanosine monophosphate pathway should be avoided in this setting.
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PMID:Deleterious effect of nitric oxide inhibition in chronic hepatopulmonary syndrome. 1735 1

Pneumococcal peritonitis is rare in adults without cirrhosis, ascites or nephrotic syndrome. Women comprise the majority of patients: an ascending genital infection is thought to be the major portal of entry. This is a case study of severe pneumococcal peritonitis in a young woman with no predisposing features. During laparoscopic laparotomy, abundant pus was found in the peritoneal cavity. Cultures grew Streptococcus pneumoniae. The post-operative course was complicated by acute respiratory distress. Dyspnea, elevated body temperature and elevated inflammatory markers, continued for more than two weeks, despite appropriate antibiotic therapy. We traced two additional cases of pneumococcal peritonitis at our hospital during the last decade. Pneumococci should be considered in the differential diagnosis of any young woman who develops peritonitis without an obvious cause.
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PMID:[Pneumococcal peritonitis--why women?]. 1747 30

A 63-year-old female had been treated for liver cirrhosis and hepatocellular carcinoma (HCC) since 3 years before. She developed symptoms of dyspnea and echocardiography revealed a large tumor in the right atrium. The stalk of tumor was attached to the intraatrial septum just above the tricuspid valve and the tumor was floating between the right atrium and the right ventricle according to cardiac rhythm. The size of tumor was 30 x 15 mm in diameter. She was admitted to our hospital for emergency operation. She was operated for cardiac tumor under cardiopulmonary bypass successfully and the postoperative course was uneventful. But the pathology reported direct intracavitary metastasis of HCC without intravascular involvement. She was discharged on the 7th postoperative day for the treatment of HCC.
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PMID:[Isolated intracavitary metastatic hepatocellular carcinoma of the right atrium without inferior vena cava involvement; report of a case]. 1756 70

Hepatopulmonary syndrome (HPS) is the one of the complication of liver cirrhosis with portal hypertension, irrespective of etiology, age and sex. It has also been observed in non cirrhotic portal hypertension and in acute hepatic conditions. Presence of hypoxemia or abnormal alveolar arterial oxygen tension with intrapulmonary vasodilation in liver cirrhosis is termed as HPS. Contrast echocardiogram is the better screening tool to demonstrate intrapulmonary shunt. Clinicians should be aware of other common chronic pulmonary and cardiac comorbid conditions, in particular COPD, tuberculosis, bronchial asthma and idiopathic pulmonary fibrosis, etc. which may coexist with HPS. There is no specific clinical finding to diagnose but digital clubbing, cyanosis, dyspnoea, platypnoea, and spider naevi are more common among cirrhosis with HPS. The presence of HPS independently worsens prognosis of cirrhosis. Even though number of mechanisms have been proposed to explain arterial hypoxemia in HPS, role of nitric oxide is the major one along with cytokines. Liver transplantation is the choice of treatment though mortality is comparatively high. There is no still effective recommended medical therapy to reverse this condition and anti cytokine/ nitric oxide inhibitors, etc are under preliminary stage.
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PMID:Hepatopulmonary syndrome - past to present. 1778 38

Hepatopulmonary syndrome is characterized by advanced liver disease, hypoxemia, and intrapulmonary shunting. The only reported curative option is orthotopic liver transplantation. We describe here a beneficial effect of inhaled prostacyclin including a decrease in respiratory symptoms and improved oxygenation in this clinical situation, with no approved pharmacological long-term therapy. The prostanoid iloprost, approved for pulmonary and portopulmonary hypertension, caused an increase in oxygenation, relief of dyspnea, and increased exercise tolerance in a patient suffering from liver-cirrhosis-associated hepatopulmonary syndrome. After liver transplantation, restitution of hepatopulmonary syndrome did not occur immediately. Inhaling iloprost resulted in improved physical condition and better clinical rehabilitation potential until hypoxemia finally resolved 3 months after transplantation. Therefore, iloprost could improve quality of life in patients with hepatopulmonary syndrome waiting for liver transplantation and post surgery until the resolution of the hypoxemia.
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PMID:Inhaled iloprost for hepatopulmonary syndrome: improvement of hypoxemia. 1799 41

We report two rare cases of hepatocellular carcinoma with invasion into the right atrium. In both our cases, the patients had liver cirrhosis and were admitted to our department due to symptoms such as worsening of the jaundice, ascites and edema of the lower extremities. The diagnosis of the HCC was established when we found high levels of alpha-fetoprotein, and the patients underwent MRI and CT-scan that were indicative of HCC. The clinical suspicion of invasion of the RA by the tumor emerged from the presence of heart-related symptoms, such as dyspnea and chest discomfort. The patients underwent two-dimensional transthoracic echocardiogram, which revealed from the sub-costal view a mobile atrial mass protruding from the inferior vena cava. In conclusion, when a patient with a history of chronic hepatic disease presents with symptoms of right heart failure one must be cautious and should bear in mind that right heart involvement from a malignant tumor may be present.
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PMID:Hepatocellular carcinoma with invasion into the right atrium. Report of two cases and review of the literature. 1825 Nov 69

We report a case of pseudocirrhosis arising in the setting of regression of liver metastases from pancreatic cancer. A 55-year-old asymptomatic woman presented to our clinic with newly diagnosed metastatic pancreatic cancer with extensive liver metastases. She underwent systemic chemotherapy with gemcitabine and oxaliplatin (GEMOX). After 8 cycles of therapy, she had a remarkable response to the therapy evidenced by decline of carcinoembryonic antigen (CEA) and CA19 by > 50% and nearly complete resolution of hepatic metastases in computed tomography (CT) scan. Shortly after, she developed increasing bilateral ankle edema and ascites, associated with dyspnea, progressive weight gain, and declining performance status. Gemcitabine and oxaliplatin were discontinued as other causes of her symptoms such as congestive heart disease or venous thrombosis were ruled out. CT scan 6 mo after the initiation of GEMOX revealed worsening ascites with a stable pancreatic mass. However, it also revealed a lobular hepatic contour, segmental atrophy, and capsular retraction mimicking the appearance of cirrhosis. She was managed with aggressive diuresis and albumin infusions which eventually resulted in a resolution of the above-mentioned symptoms as well as complete resolution of pseudocirrhotic appearance of the liver and ascites in CT scan. This case demonstrates that pancreatic cancer patients can develop pseudocirrhosis. Clinicians and radiologist should be well aware of this entity as early recognition and management can lead to a near complete recovery of liver function and much improved quality of life as illustrated in this case.
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PMID:Pseudocirrhosis in a pancreatic cancer patient with liver metastases: a case report of complete resolution of pseudocirrhosis with an early recognition and management. 1833 Sep 59

Since 2004, pegylated interferon (P-IFN) in combination with ribavirin has become the optimal choice of therapy for chronic hepatitis C virus (HCV) infection. IFN a-2b suppresses HCV replication and restores elevated serum aminotransferase levels, leading to improvements in the histological changes in the livers of patients with chronic hepatitis C. Unfortunately, P-IFN has several adverse effects, including pneumonitis. This complication has been reported in the treatment of malignant diseases and CHC. We report a patient with interstitial pneumonitis thought to be caused by an IFN-based treatment in an unusual scenario of a patient with HCV-related Child-Pugh stage A cirrhosis, who experienced dyspnea, fever, and cough after 12 months of treatment with P-IFN a-2b. Her lung injury and pulmonary symptoms did not disappear despite discontinuation of IFN and the administration of corticosteroid. We concluded that the patient developed a fatal interstitial pneumonitis associated with P-INF a-2b therapy.
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PMID:Interstitial pneumonitis associated with pegylated interferon alpha-2b therapy for chronic hepatitis C: case report. 1837 74

Alpha-1-antitrypsin deficiency (AAT) is one of the three most common lethal genetic diseases in the caucasian population (together with cystic fibrosis and Down syndrome). Its primary manifestation is early-onset panacinar emphysema. Slowly progressive dyspnea is the primary symptom, although some patients initially have symptoms of cough, sputum production, or wheezing. A minority of patients develops hepatic cirrhosis. We present a case of a 40 year-old male, light smoker, with chronic obstructive lung disease with predominance of panacinar emphysema, with AAT deficiency (72 mg/dl; normal values = 200-300 mg/dl) complicated with cor pulmonale and chronic respiratory failure. The main clinical consequence of AAT deficiency is the early onset of panacinar emphysema, typically more severe at the lung bases. Smoking plays an important part in the natural history of the disease, both increasing the severity and decreasing the age at onset of emphysema.
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PMID:A rare case of pulmonary emphysema. 1838 23

Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder that manifests as pulmonary emphysema, liver cirrhosis and, rarely, as the skin disease panniculitis, and is characterized by low serum levels of AAT, the main protease inhibitor (PI) in human serum. The prevalence in Western Europe and in the USA is estimated at approximately 1 in 2,500 and 1 : 5,000 newborns, and is highly dependent on the Scandinavian descent within the population. The most common deficiency alleles in North Europe are PI Z and PI S, and the majority of individuals with severe AATD are PI type ZZ. The clinical manifestations may widely vary between patients, ranging from asymptomatic in some to fatal liver or lung disease in others. Type ZZ and SZ AATD are risk factors for the development of respiratory symptoms (dyspnoea, coughing), early onset emphysema, and airflow obstruction early in adult life. Environmental factors such as cigarette smoking, and dust exposure are additional risk factors and have been linked to an accelerated progression of this condition. Type ZZ AATD may also lead to the development of acute or chronic liver disease in childhood or adulthood: prolonged jaundice after birth with conjugated hyperbilirubinemia and abnormal liver enzymes are characteristic clinical signs. Cirrhotic liver failure may occur around age 50. In very rare cases, necrotizing panniculitis and secondary vasculitis may occur. AATD is caused by mutations in the SERPINA1 gene encoding AAT, and is inherited as an autosomal recessive trait. The diagnosis can be established by detection of low serum levels of AAT and isoelectric focusing. Differential diagnoses should exclude bleeding disorders or jaundice, viral infection, hemochromatosis, Wilson's disease and autoimmune hepatitis. For treatment of lung disease, intravenous alpha-1-antitrypsin augmentation therapy, annual flu vaccination and a pneumococcal vaccine every 5 years are recommended. Relief of breathlessness may be obtained with long-acting bronchodilators and inhaled corticosteroids. The end-stage liver and lung disease can be treated by organ transplantation. In AATD patients with cirrhosis, prognosis is generally grave.
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PMID:Hereditary alpha-1-antitrypsin deficiency and its clinical consequences. 1856 11


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