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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The mucopolysaccharidoses are a group of clinically progressive, heritable, lysosomal storage disorders. Many organ systems are affected due to widespread accumulation of incompletely degraded mucopolysaccharide. The novel finding of hepatic fibrosis in each of six cases of mucopolysaccharidosis examined at autopsy (including examples of Hurler syndrome, Hunter syndrome, and Sanfilippo syndrome) is described. In each instance, the liver was diffusely involved by fibrosis that outlined the lobules, and there was extensive hepatocyte and Kupffer cell vacuolization. The pattern of hepatic fibrosis is not explained by either cardiac failure or drug toxicity. It is hypothesized that the hepatic fibrosis is due to the abnormal accumulation of a hepatotoxic metabolite. The frequency and severity of liver disease in the mucopolysaccharidoses deserve further study. In particular, with the advent of bone marrow transplantation as therapy for some of the mucopolysaccharidoses, the question of whether cirrhosis develops in these patients and, if so, what its rate of progression is, should be addressed.
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PMID:Hepatic fibrosis in the mucopolysaccharidoses. 309 34

The pathogenesis of hyponatremia remains debated; therefore, we determined the roles of plasma vasopressin, fluid intake and renal free water excretion in hyponatremic medical patients. We evaluated 100 consecutive hypo-osmolar hyponatremic patients (PNa = 127 +/- 0.7 mM l-1) in a prospective manner. We observed: hyponatremia was often found in association with advanced congestive cardiac failure (twenty-five of 100 patients), liver cirrhosis (16%) and primary volume contraction (29%). There was a 17% in-hospital mortality of hyponatremic patients. This was primarily related to the severity of underlying illnesses rather than to hyponatremia per se. The most consistently observed laboratory finding of hyponatremia was non-osmotic vasopressin stimulation; mean observed PADH was 4.7 +/- 0.7 pg ml-1 and vasopressin was detectable by radioimmunoassay (RIA) in 91% of all patients. In addition to vasopressin stimulation we also found evidence of advanced 'circulatory underfilling' in most hyponatremic patients. Mean urinary osmolality was hypertonic to plasma (441 +/- 17.4 m0sm kg H2O-1). This applied to patients with hyponatremic cardiac failure, liver cirrhosis and volume contraction. Almost all of these patients received high ceiling diuretics. (v) Spontaneous mean daily fluid intake was 2.4 +/- 0.2 l. In summary, our findings suggest that disturbances of vasopressin, fluid intake and renal free water excretion co-operate in the pathogenesis of hyponatremia. In clinical states of advanced circulatory underfilling the occurrence of hyponatremia indicates a poor prognosis of the patient.
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PMID:Pathogenesis of clinical hyponatremia: observations of vasopressin and fluid intake in 100 hyponatremic medical patients. 310 2

Of 511 cases of brucellosis studied between December 1983 and February 1986, four (0.8%) had sternoclavicular (STCL) arthritis. Two were male and two female, and only one was younger than 50 years old. All four cases had significantly high specific IgG antibody titres (1 of 1280), measured by the indirect immunofluorescent (IIF) test, and two had Brucella melitensis isolated from their blood. In two cases, STCL arthritis was the presenting problem, and it was associated in one with ankle arthritis, hepatitis, renal impairment, orogenital ulcers and a haematological picture of myelodysplasia; in the other it was a relapsing STCL arthritis. In the remaining two cases, STCL arthritis was part of an extensive osteoarticular disease, which was associated in one with cachexia, liver cirrhosis, heart failure and prostatitis with urine retention, and in the other with severe thrombocytopenia. Excellent results were obtained from six to eight weeks' therapy with streptomycin, rifampicin and cotrimoxazole or tetracycline.
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PMID:Brucellar sternoclavicular arthritis, the forgotten complication. 325 Mar 41

From January 1978 to August 1987, 21 patients received a peritoneovenous shunt using the Le Veen valve (LVV). The indications criteria were the long-term diuretic therapy failure (mean time = 24.4 months) or resistence to medical therapy during hospital internment. The 21 patients underwent 36 surgeries, being 4 valve position review and 11 changes of LVV. The mean age was 51.6 years. Fifteen patients had alcoholic cirrhosis, 3 postnecrotic cirrhosis, one Budd-Chiari syndrome, one mansoni Schistosomiasis, and one malignant ascites. Ten were Child B and 9 Child C patients. Eight patients with history of previous esophageal varices bleeding (EVB) underwent endoscopic sclerotherapy (EE) before LVV implantation. Seven patients died in the early postoperative period (3 Child B and 4 Child C patients). Three patients died due to EVB and the others as consequence of hepatic failure (one), cardiac insufficiency (one), sepsis (one), and bronchopneumonia (one). The mean follow-up was 19.9 months (1-61). Early LVV occlusion occurred in 4 patients and late valve occlusion in others 4 patients. The LVV changes were done at ambulatorial preceeding. Ten patients (47.6%) died in late follow-up and in these cases death was related to the main disease course. It is concluded that: 1) LVV is a useful therapy in patients with intractable ascites, since it is not the terminal manifestations of disease; 2) early mortality is related to liver function and late mortality to main disease course; 3) ascitic patients with EVB should undergo endoscopic sclerotherapy before LVV implantation.
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PMID:[Use of the Leveen shunt in the treatment of clinically intractable ascites]. 325 81

The long-term follow-up of 80 heart transplant patients (70 men, 10 women) from January 1982 to July 1985 who had received cyclosporine (CsA) showed a high incidence of mild to severe liver dysfunction. Fifty patients (62.5%) had long-lasting postoperative biological disturbances (alanine amino transferase greater than 2N and/or alkaline phosphatase greater than 1.5N for 3 months or more). Most patients were asymptomatic; eight were icteric, and one had arthralgia. The most common biological feature consisted of isolated elevation of ALAT (27 cases). Assessment of causes led to a definite etiology in 42 patients: 7 cardiac failure, 13 HBsAg-positive liver disease (26%) (chronic persistent hepatitis 8, chronic active hepatitis 2, subacute necrosis 2). Fourteen patients (28%) sustained non-A, non-B (NANB) hepatitis (chronic persistent hepatitis 5, chronic active hepatitis 1, cirrhosis 1), and 7 (14%) sustained a drug-related hepatitis. Liver biopsy and complete virus screening was contributive to the diagnosis in nearly all patients. Additionally, prolonged impairment of liver function tests occurred in 62% of heart transplant recipients, mostly during the first 6 postoperative months. Hepatitis B virus (HBV) and NANB hepatitis accounted for 26% and 28% of the cases of liver dysfunction, respectively; drug-induced hepatitis may have been involved in 14% of the cases. Complete hepatitis virus screening should be performed before heart transplant and in any case of abnormal liver function posttransplantation. HBV vaccination prior to heart transplant is recommended in HBsAg- and HBcAb-negative candidates for heart replacement. Long-term follow-up of these patients is mandatory to assess the severity of these liver dysfunctions.
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PMID:Prevalence and causes of long-lasting hepatic dysfunction after heart transplantation: a series of 80 patients. 329 31

Fifty-seven cirrhotic patients with intractable ascites had a portosystemic shunt. In 35 patients, a peritoneovenous shunt had previously failed. Forty-six patients were in Pugh's class B and 11 were in class C. There were three operative deaths (5.3%). Fifty-three (98.2%) of the 54 survivors were cleared of ascites. In one patient, ascites persisted because of postshunt heart failure that resulted in a marked increase of caval pressure. Twenty-seven patients (50%) had late encephalopathy, which was severe and disabling in 12 (22%). One- and three-year survival rates were 72% and 36%, respectively. These results suggest that although portosystemic shunts are remarkably effective in dealing with ascites, the high rate of postoperative encephalopathy is a strong argument against their routine use in the management of intractable ascites in cirrhosis.
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PMID:Should portosystemic shunt be reconsidered in the treatment of intractable ascites in cirrhosis? 339 43

To assess the relative importance of cardiac versus peripheral vascular failure in patients dying of septic shock, a series of 42 patients with documented septic shock was retrospectively evaluated. Patients were included in the study if serial hemodynamic and metabolic studies had been performed: the first one within 12 hours after onset of septic shock and the last one within 12 hours (median 2 hours; range 0.1 to 12 hours) before death in nonsurvivors. Nonsurvivors were included only if they died in shock. From the patient records the first, highest, and last measured cardiac indexes (CI) (t = 1, t = 2, and t = 3) with concomitant hemodynamic and metabolic variables were obtained. Group I (n = 21) consisted of survivors and group II (n = 21) of nonsurvivors. Group II was divided into three subgroups: group IIa (n = 4) consisted of nonsurvivors with liver cirrhosis, group IIb (n = 9) patients with final CI less than 4 1 X min-1 X m-2, and group IIc (n = 8) patients with final CI greater than 4 1 X min-1 X m-2. At t = 1 no significant differences in hemodynamic variables were found between groups I and II, and all patients, whether surviving or not, were able to increase CI to similar levels. At t = 3 group II showed a marked decrease in mean arterial pressure and systemic vascular resistance index compared with group I (p less than 0.001), whereas CI did not differ significantly. The nonsurvivors showed progressive lactic acidemia. Even group IIb patients showed persistent vasodilation despite a decrease in CI. Our data suggest that many patients in septic shock die as a result of peripheral vascular rather than cardiac failure, since persistent vasodilation, irrespective of CI, was a major hemodynamic determinant in nonsurvivors, of whom 57% maintained a high CI until shortly before death.
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PMID:Hemodynamic determinants of mortality in human septic shock. 351 60

Levels of carcinoembryonic antigen(CEA)in the serum and pleural effusion in malignancies (65) and benign (25) of lung were determined. There are 20 cases of adenocarcinoma, 16 undifferentiated carcinoma, 7 squamous cell carcinoma, 4 alveolar carcinoma, 12 unclassified carcinoma, 1 polymorphous adenoma, 1 mesothelioma, 1 thymoma, 1 metastatic cancer from kidney and 2 metastatic breast cancer. In the benign lesions, there are 20 tuberculosis, 2 heart failure, 1 pneumonia, 1 empyema and 1 cirrhosis. The mean of the CEA level in the serum of lung cancer group was 12.63 ng/ml as compared with that of the tuberculosis group, 3.01 ng/ml (P less than 0.01). The level of CEA in pleural fluid in the lung cancer group was 57.30 ng/ml as compared with that of tuberculosis group, 5.55 ng/ml (P less than 0.01). The content of CEA in the serum and pleural fluid in lung cancer group was remarkably different (P less than 0.01). CEA level in the serum of adenocarcinoma is the highest (mean 15.51 ng/ml). If we set 5 ng/ml as the margin of normal CEA level in serum, the positive rate for cancer would be 54.2%. It is suggested that the margin of CEA normal value be set at 10 ng/ml for the pleural fluid. Higher readings may imply cancer.
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PMID:[Carcinoembryonic antigen assay in serum and pleural effusion of pulmonary malignancies and benign lesions]. 358 9

Emphysematous pyelonephritis is a severe infection characterized by the presence of gas within the renal parenchyma and perirenal tissues. Two cases of this disease are presented. They were the 16th and 17th case reported in Japan. Case 1 was a 63-year-old woman with diabetes mellitus and liver cirrhosis. She was hospitalized for abdominal pain and anuria. Renal X-rays showed a gas shadow in and around the left kidney, but no evidence of upper urinary tract obstruction. Although hemodialysis was done, she died of heart failure. Case 2 was a 54-year-old man with diabetes mellitus was admitted with the complaint of fever and left abdominal pain. Renal X-rays showed a gas shadow in and around the left kidney but no evidence of upper urinary tract obstruction. He was treated with intensive antibiotic therapy, control of blood sugar, intravenous drips and percutaneous drainage. Clinical features improved, but deteriorated after 40 days of therapy. The gas shadow remained unchanged on CT scanning, and aortography showed the occlusion of the left renal artery. Nephrectomy was done after 50 days. Seventeen cases of emphysematous pyelonephritis in the literature including our cases are reviewed, especially the choice of the treatment is discussed.
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PMID:[Two cases of emphysematous pyelonephritis--considerations on the choice of treatment]. 359 89

Nine patients, six men and three women, 40 to 73 years of age, were included in the study. All of the patients had severe heart failure refractory to aggressive therapy including digitalis, diuretics, and vasodilators. Eight patients underwent one treatment of peritoneal dialysis while the remaining patient received two dialyses. The urine output was measured by an indwelling catheter; glomerular filtration rate (GFR) was determined by creatinine and inulin clearance, and renal blood flow (RBF) was determined by sodium paraamino hippurate (PAH) clearance. Following one peritoneal dialysis, the mean fluid loss/patient was 3,995 ml (range 3,200 to 5,100 ml). Dialysis was generally well tolerated. One patient, who had underlying hepatic cirrhosis and underwent two dialyses, developed hepatic failure and died 10 days after the second dialysis. At postmortem, peritonitis was discovered. All of the patients showed a marked subjective and objective clinical improvement. The mean plasma urea decreased from 154 to 71 mg/dl (P less than 0.005), and mean plasma creatinine decreased from 1.83 to 1.13 mg/dl (P less than 0.005). Blood pH was 7.30 before dialysis and increased to 7.37 (P less than 0.0125) after treatment. Mean urine output predialysis was 955 ml and increased to 1,472 ml post dialysis (P less than 0.0005). Creatinine clearance increased from 35 to 73 ml/min (P less than 0.0005). The mean inulin clearance increased from 33 ml/min predialysis to 69 ml/min post dialysis (P less than 0.0005), and mean PAH clearance increased from 96.7 to 362.5 ml/min (P less than 0.0005). Acute peritoneal dialysis is a safe and effective means for removing large quantities of excess fluid from patients with intractable heart failure.
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PMID:Improved renal function following acute peritoneal dialysis in patients with intractable congestive heart failure. 369 51


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