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 legs are preferentially affected by systemic edematous states. Pathogenetic factors are: increased hydrostatic pressure, increased capillary permeability (leak), reduced colloid-oncotic pressure, reduced lymph drainage and miscellaneous rare conditions. The most frequent cause of increased hydrostatic pressure is right heart failure, which can be treated with diuretic drugs. Increased capillary leak is seen in septic-toxic events, where the underlying disease must be treated. Reduced oncotic pressure is caused by hypoalbuminemia, either due to reduced production, e.g., in liver cirrhosis, or to increased albumin loss, e.g., through the kidney in nephrotic syndrome or the gut in protein-loosing enteropathy. Lymphedema is classified either as primary or secondary and is treated by lymph drainage. Idiopathic edemas, primarily seen in females, often lead to chronic abuse of diuretics, which in turn can cause diuretic-induced edemas.
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PMID:[Leg edema]. 982 97

The hypothesis tested that mechanisms other than retrograde transsinusoidal fluid wave transfer reported in patients with right heart failure are responsible for the ultrasonographic sign of advanced portal vein pulsatility (APP). Within a time-period of 3 years we have seen 13 patients with APP, defined as temporary portal flow reversal in the face of a normal echocardiogram. Nine of these patients had biopsy-proven liver cirrhosis and four with liver disease were without cirrhosis or cardiac pathology. A randomly selected control group of 18 healthy subjects was studied. Doppler ultrasound evaluation of the hepatic veins as well as the intra and extrahepatic portal vein territories was performed in both groups. Hepatopetal portal flow with APP reversed to hepatofugal flow in follow up studies in two patients. In another two hepatopetal flow with APP in the main portal vein and hepatofugal flow in the intrahepatic portal radicles was recorded during the same examination. The remaining group displayed APP in the intra and extrahepatic portal vein territories. None of the normal subjects presented with APP. Hepatic venous outflow obstruction associated with excessive arterioportal shunting is likely to account for APP of all of our patients. Based on a causal link between angiographic 'to-and-fro' flow pattern and the sonographic APP sign in patients with sinusoidal outflow obstruction we suggest, that APP expresses a short, transitional period of portal hypertension just before the occurence of flow reversal.
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PMID:Doppler ultrasound evaluation of advanced portal vein pulsatility in patients with normal echocardiograms. 984 93

Chronic right heart failure determines cardiac liver sclerosis as a consequence of the hepatic venous congestion that is easily detectable with pulsed Doppler sonography measuring hepatic venous pulsatility. These Doppler parameters profoundly change on the basis of the causative agent of the liver sclerosis. In fact, on pulsed Doppler examination, we detected a flat waveform in hepatic veins of subjects with viral liver cirrhosis whereas we only observed biphasic waveforms in subjects with cardiac liver sclerosis. On the contrary, a pulsed Doppler sonography pattern in subjects with viral cirrhosis and associated chronic right heart failure is unknown. We suppose that splanchnic pulsed Doppler sonography has an important role both in differentiating cardiac liver sclerosis from viral cirrhosis with chronic right heart failure and in the follow-up of the seriousness of the right ventricular failure.
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PMID:Viral cirrhosis with chronic right heart failure and cardiac liver sclerosis: a hypothesis on the differentiation between the two diseases through pulsed Doppler sonography examination. 1237 85

Chylous ascites and chylothorax are rare clinical entities and usually caused by neoplasms, particularly lymphomas, liver cirrhosis, superior vena cava thrombosis, nephrotic syndrome, and some cardiac events such as dilated cardiomyopathy or right heart failure. Constrictive pericarditis is an extremely rare cause of this clinical state. We report a 41-year-old male patient undergoing haemodialysis who presented with chylous ascites and chylothorax. Echocardiography and heart catheterisation revealed constrictive pericarditis. He underwent pericardiectomy and after the operation the ascites and pleural effusion resolved rapidly. We suggest that constrictive pericarditis should be considered in the differential diagnosis of chylous ascites and chylothorax.
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PMID:Chylous ascites and chylothorax due to constrictive pericarditis in a patient undergoing haemodialysis. 1512 33

Severe portopulmonary hypertension (PPHTN) is a rare complication of liver cirrhosis and carries a poor prognosis. In the last years, intravenous (IV) epoprostenol has been suggested to be the optimal medical treatment for PPHTN, and recently oral bosentan has been shown to be efficacious and safe in selected patients with PPHTN. We report a case of PPHTN suffering from recurrent right heart failure while on treatment with IV iloprost, which was successfully managed by combination therapy with IV iloprost plus oral bosentan, providing sustained cardiopulmonary stabilization for at least two years. This report documents the first case of a patient with PPHTN successfully treated with the combination of IV iloprost and oral bosentan over an extended period. Thus, combination therapy with IV iloprost and oral bosentan might be a promising new option for selected patients suffering from PPHTN and recurrent right heart failure.
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PMID:Combination therapy for portopulmonary hypertension with intravenous iloprost and oral bosentan. 1616 Sep 26

Novel treatments, such as prostanoids or endothelin receptor antagonists, have been introduced for various forms of pulmonary arterial hypertension, but the long-term effects of these treatments on portopulmonary hypertension (PPHT) are unknown. In a retrospective analysis, the present authors assessed the safety and efficacy of inhaled iloprost, a prostacyclin analogue, and bosentan, an endothelin receptor antagonist, in patients with PPHT. In total, 31 consecutive patients with Child class A or B cirrhosis and severe PPHT were treated for up to 3 yrs with either inhaled iloprost (n = 13) or bosentan (n = 18), and the effects on exercise capacity, haemodynamics and survival were evaluated. In the iloprost group, the survival rates at 1, 2 and 3 yrs were 77, 62 and 46%, respectively. In the bosentan group, the respective survival rates were 94, 89 and 89%. Event-free survival rates, i.e. survival without transplantation, right heart failure or clinical worsening requiring the introduction of a new treatment for pulmonary hypertension, was also significantly better in the bosentan group. Bosentan had significantly better effects than inhaled iloprost on exercise capacity, as determined by the 6-min walk test, as well as on haemodynamics. Both treatments proved to be safe, especially in regards of liver function. In the present series of patients with well-preserved liver function and severe portopulmonary hypertension, treatment with both inhaled iloprost and bosentan appeared to be safe. Patients treated with bosentan had higher survival rates, but prospective controlled studies are required to confirm these findings.
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PMID:Experience with inhaled iloprost and bosentan in portopulmonary hypertension. 1805 1

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

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
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PMID:Gastrointestinal and hepatic manifestations of sarcoidosis. 1936 62

Hepatocellular carcinoma has been commonly associated with multiple etiologic factors including hepatitis B and C, alcoholic liver disease, and more rarely congenital metabolic liver diseases. 'Cardiac cirrhosis' is the cirrhosis resulting from prolonged passive liver venous congestion secondary to right-sided congestive heart failure; hepatocellular carcinoma is a rarely reported outcome. In this study we present two female patients with congenital heart defects treated with the Fontan procedure who survived into their third decade, and developed hepatocellular carcinoma in the setting of cardiac cirrhosis. The Fontan procedure diverts blood from the inferior vena cava and superior vena cava to the pulmonary arteries, thereby increasing survival in infants born with a single effective ventricle. As such patients live longer, however elevated pulmonary and right-sided heart pressures cause chronic passive liver congestion and eventual cardiac cirrhosis. The two patients in this study had no risk factors for hepatocellular carcinoma other than cardiac cirrhosis secondary to their prolonged survival after their Fontan procedure. In conclusion, we suggest that cardiac cirrhosis may be a risk factor for developing hepatocellular carcinoma and recommend close follow-up and hepatocellular carcinoma screening for patients with known right heart failure and passive hepatic congestion.
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PMID:Hepatocellular carcinoma in two patients with cardiac cirrhosis. 1955 Mar 46

Atrial septal defects constitute the second most common cardiac congenital abnormality. These defects when undiagnosed during childhood, present in adulthood with symptoms of right heart failure. We describe a case of uncorrected ostium primum atrial septal defect that presented as liver failure in a 43-year-old woman. The patient was found to have liver cirrhosis and a complete work-up of hepatic causes for cirrhosis was negative. Further examination revealed an atrial primum septal defect. Liver cirrhosis in this patient was thought to be secondary to chronic congestion from the right heart failure resulting from the atrial septal defect. Although right heart failure is a known cause of cirrhosis, liver cirrhosis resulting specifically from an atrial septal defect is uncommon. In fact, an ostium primum atrial septal defect presenting as liver cirrhosis has not been well described. We present such a case to highlight this manifestation of atrial septal defect and also to reiterate that cardiac causes should always be considered in the differential diagnosis whenever a patient presents with liver cirrhosis.
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PMID:Cardiac cirrhosis: a rare manifestation of an uncorrected primum atrial septal defect. 1996 72


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