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 58-year-old female was diagnosed as mitral regurgitation and tricuspid regurgitation, combined with liver cirrhosis (T. Bil 2.3 mg/dl, ICG-R 37%) and pancytopenia (Hb 9.4 g/dl, WBC 3,000, Plt 56,000). She underwent mitral and tricuspid annular plications. After extracorporeal circulation, the postoperative platelet count was maintained higher than preoperative one by transfusion of platelet-rich plasma. Administration of FOY was begun during operation. Levels of T. Bil. and BUN were highest at 15th postoperative day and decreased gradually. Postoperative pleural effusion was treated by administration of 25% albumin solution keeping the level of Alb. up 3.5 mg/dl.
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PMID:[A case of open heart surgery associated with liver cirrhosis and pancytopenia]. 194 90

Increase in the production of hepatic lymph is a compensatory mechanism known to occur in cirrhosis, neoplasia of the liver, biliary obstruction, and right-sided cardiac decompensation with elevated hepatic venous pressure. In hepatic lymphostasis, dilated hepatic lymphatics are reflected on CT as low attenuation rims--perivascular lucencies (PVLs)--surrounding the portal veins and the intrahepatic vena cava. In this study, CT scans of the livers of normal controls and patients in each of the four disease categories mentioned above were evaluated for the presence of PVLs; the presence/absence of PVLs was correlated with other CT findings and various biochemical and pathophysiologic parameters. The PVLs were seen with some frequency in patients in each of the four disease categories (12-52%) but in none of the normal controls. In patients with tricuspid incompetence, the presence of PVLs correlated perfectly with the presence of mottled enhancement of the hepatic parenchyma (evidence of sinusoidal stasis). The presence of PVLs was associated with elevation of certain biochemical parameters, but no common biochemical marker was observed across the disease categories. Perivascular lucencies on CT reflect altered hepatic lymphatic dynamics; in the absence of impaired efferent lymph drainage, they reflect increased lymphatic flow. However, the sensitivity of the finding--the relationship of the presence/absence of PVLs on CT to the degree of alteration of the lymphatic dynamics--has yet to be assessed.
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PMID:Increased lymphatic flow from the liver in different intra- and extrahepatic diseases demonstrated by CT. 206 65

A patient with cardiac cirrhosis due to severe tricuspid regurgitation associated with a mitral valve lesion successfully underwent mitral and tricuspid valve replacement. Preoperative rest cure with liver supporting therapy for 5 months was used to maximize hepatic and cardiac function. Postoperatively, jaundice developed despite improvements in cardiac function. Plasma exchange was performed nine times over a period of 10 days with the liver function improved. He was discharged with New York Heart Association class I symptoms and normal liver function 2 months following surgery.
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PMID:Successful surgical treatment of cardiac cirrhosis. Tricuspid surgery and plasma exchange. 869 69

Perioperative management of patients with liver cirrhosis undergoing open heart surgery (3 males and 1 female, mean age 57.3 years) was evaluated. All 4 patients had NYHA class III acquired valvular heart disease accompanied by severe tricuspid regurgitation, and the operation was a redo operation in 3 of them. Preoperatively, the total serum bilirubin was 1.7 mg/dl, ICGR15 24.3 +/- 1.7%, and blood platelet count was 83,000 +/- 19,000/m3. The postoperative course was uneventful in 2 patients who maintained good cardiac function and did not require homologous blood transfusion. The other two patients who underwent a re-redo operation, sustained a large volume of hemorrhage, and required massive transfusion showed severe postoperative complications (respiratory failure in 1, multiple organ failure in 1). The stage of the disease improved to NYHA class I in all 3 patients who survived the operation. Comprehensive measures including avoidance of massive hemorrhage, preservation of the hepatic blood flow, resolution of hepatic congestion, and prevention of complications such as digestive tract bleeding, infections, and renal failure are considered to be needed for prevention of postoperative liver failure. Especially, sufficient surgical hemostasis and autologous platelet rich plasma were useful for prevention of massive hemorrhage.
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PMID:[Perioperative management in patients with liver cirrhosis undergoing open heart surgery]. 891 65

The blood flow in the hepatic veins can normally be studied easily by Doppler ultrasound. The pattern of blood flow in normal individuals is described, and its relation to the cardiac cycle and changing pressure in the right atrium. The blood flow shows variations in healthy persons, and may change in cases of heart disease and hepatic disease. Conditions such as atrial fibrillation, tricuspid regurgitation, abnormal relaxation, restrictive cardiomyopathy, constrictive pericarditis and cardiac tamponade are reflected in the hepatic veins, and the pattern of blood flow may help in diagnosis, and in grading the pathology. In cirrhosis and portal hypertension the heart-synchronous variation in velocity is reduced. This is due to increased resistance to blood flow across the liver and the pressure gradient becoming larger than the variations in pressure in the right atrium.
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PMID:[Doppler ultrasound of blood flow in the hepatic veins]. 918 59

A 77-year-old woman was admitted to our hospital with congestive hepatic dysfunction and combined valvular disease. In addition to aortic stenosis, mitral stenosis, and tricuspid regurgitation, multi-organ dysfunction consisting of hepatic cirrhosis, pulmonary dysfunction and renal dysfunction were found. In consideration of the patient's anxiety for surgical treatment, we performed aortic valve replacement, open mitral commissurotomy and tricuspid annuloplasty. Although the patient required hemofiltration for 2 days and an assist of catecholamines for 1 month, she improved better and was discharged at 2 months after surgery. As preoperative assessment of preliminary function of other organs is difficult in the elderly, indications for surgery and optimal surgical procedures must be investigated in each case.
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PMID:[A surgical case report--combined valvular disease in a 77-year-old woman with multi-organ dysfunction]. 943 Sep 68

Pulmonary hypertension is one of the major causes of morbidity and mortality of patients with sickle cell hemoglobinopathy (SCH). Although a clinically recognized complication of sickle cell disease (SCD), there are few published pathologic studies of pulmonary findings in these patients. The aim of this study was to define the pulmonary pathologic changes and to investigate correlation between the pathologic changes, the antemortem diagnosis of pulmonary hypertension, and the severity of SCH. Cases of SCH were identified from the autopsy database using Snomed codes. Clinical and echocardiograph data were collected for correlation with the pathologic data. A total of 20 adult patients (12 males and 8 females) were identified. Hemoglobin electrophoresis results were available for 16 patients, with hemoglobin S fraction percentages ranging from 23% to 97.8%. Eleven patients had SCD, 5 patients had sickle cell trait (SCT), and the remaining 4 patients without hemoglobin electrophoresis were included in the SCT group. The mean age of the SCT group was higher than that of the SCD group (P = 0.03). Histologically, all 20 patients demonstrated changes in pulmonary vasculature considered diagnostic of pulmonary hypertension grade I to grade IV, associated with plexiform lesions in 60% of patients. Medial hypertrophy and intimal hyperplasia/fibrosis, considered potentially reversible lesions, were seen in all patients. A weak association was found between SCD and plexiform lesions. Fibroelastic degeneration of small arteries, arterioles, and venules was identified in almost all (95%) cases. Clinically, tricuspid regurgitation was detected by echocardiogram in 10 of 20 (50%) patients; 6 of these 10 had significant regurgitation to allow estimation of systolic pressure. Sudden death occurred in 8 patients, with males having a significantly higher incidence. Cardiomegaly was present in 95% of patients, however, autosplenectomy and hepatic cirrhosis/hemochromatosis were observed almost exclusively in patients with SCD. Cirrhosis was found to have a strong positive association with SCD. This study demonstrates pulmonary hypertensive changes in all 20 autopsied patients who had SCH but died from various causes. We conclude that a high prevalence of pulmonary hypertension is associated with SCH with consequent high mortality. Therefore, patients with SCH would benefit from a regular periodic assessment for pulmonary hypertension regardless of age, sex, and severity of hemoglobinopathy.
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PMID:Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases. 1239 78

Doppler ultrasound is a well established method for assessment of the portal venous system to detect the direction of portal blood flow. It is helpful for non-invasive diagnosis of intra-abdominal portosystemic shunts, especially in patients with cirrhosis. Less attention has been paid to other influences on portal venous flow, e.g. tricuspid regurgitation, increased hepatic out-flow resistance, respiratory cycle. The aim of this pictorial review is to describe the spectrum of physiological and pathological Doppler ultrasound flow patterns in the portal venous system.
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PMID:Pictorial review: Colour Doppler ultrasound flow patterns in the portal venous system. 1246 60

Juvenile haemochromatosis is an autosomal, recessive inherited iron metabolism disorder. The rapid deterioration and malignant prognosis differentiate juvenile haemochromatosis from hereditary haemochromatosis. The authors summarize the history of a 25 year old man, who worked in Hungary as a guest worker living in Romania. No significant illness has occurred in his previous history. The abdominal pain was his first symptom and he was treated in different institutions, where cholecystitis, alcoholic hepatic disease, hepatic cirrhosis were considered as a cause of his symptoms. Some weeks later atrial tachycardia, and congestive heart failure were observed and he was sent to our Cardiology Department. The echocardiography revealed diffuse hypokinesis, serious systolic dysfunction (ejection fraction: 21%), grade II mitral and tricuspid insufficiency with pulmonary hypertension. Considering the rapid deterioration of his cardiac function, myocarditis was suspected. Myocardial biopsy and coronary arteriography were performed. Coronary arteries were normal. Ventricular fibrillation occurred during coronary arteriography. Myocardial biopsy revealed juvenile haemochromatosis. Special laboratory examinations (transferrin saturation) were made after biopsy, that also confirmed the diagnosis of juvenile haemochromatosis. Cardiac transplantation was planned. Some days after the diagnosis was made the patient died of cardiogenic shock and intractable heart failure. Autopsy revealed hypogonadism and serious haemochromatosis in different parenchymal organs. Juvenile haemochromatosis should be considered in every young patient with congestive heart failure of unknown etiology.
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PMID:[Juvenile haemochromatosis presenting as intractable congestive heart failure]. 1646 15

A 46-year-old woman presented with arthralgia. She had a history of fluctuating liver function impairment for 6 months. Laboratory investigations revealed elevated liver function test results, positive antinuclear antibodies and elevated serum IgG. The histological findings of a liver biopsy were interface hepatitis accompanied by plasmocytic infiltration with bridging fibrosis. There was no evidence of cirrhosis on pathological examination and no portal hypertension on endoscopic and radiographic studies. Autoimmune hepatitis was diagnosed, and treatment with prednisolone improved the liver dysfunction. After 6 months, she complained of dyspnea. Doppler echocardiography showed a dilated right ventricle, severe tricuspid insufficiency, and systolic pulmonary arterial pressure indicative of pulmonary arterial hypertension. We report this rare case of autoimmune hepatitis with pulmonary arterial hypertension.
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PMID:Autoimmune hepatitis associated with pulmonary arterial hypertension. 1901 10


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