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)

In a case series of 56 patients with essential cryoglobulinemia, 35 were followed-up for 4-13 years (mean 7 years). A membranous proliferative glomerulonephritis, which in about half the cases showed a progression to renal insufficiency, was the commonest complication, observed in more than one third of the patients. In 2 patients hepatic cirrhosis became manifest after a completely asymptomatic period and in 2 others a lymphoproliferative disease appeared 2 and 8 years after the onset of purpura. In 51% of patients the intial clinical pattern did not change. In searching for a correlation between the development of nephropathy and cryoglobulin characteristics, none was demonstrated studying the cryoglobulin level, the presence of autoantibody and the complement components.
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PMID:A long-term follow-up study in essential cryoglobulinemia. 10 49

The female patient initially showed the acquired type of total lipoatrophy at about 8 years of age. At 12 years of age, the onset of diabetes mellitus was speculated from advanced pyodermia and dedentition. At 29 years of age, glucosuria was found, and she developed proteinuria, ascites, and pretibial edema. The physical examination revealed: hepatosplenomegaly, complete absence of subcutanous fat, cutaneous xanthomas, and emaciated facies with pronounced zygomatic arches. Diabetic retinopathy was revealed in the ophthalmological examination, and nephropathy was evident in renal biopsy specimens. She also had peripheral diabetic neuropathy. No adipose tissue was found in the mesenterium under peritoneoscopy. The hepatic biopsy specimen revealed advanced portal liver cirrhosis. Laboratory findings included: hyperlipidemia, elevation of BMR without evidence of hyperthyroidism, impaired renal function, and undetected anti-insulin antibodies and anti-insulin antibodies. Endocrinological examinations revealed normal value, except for an impaired hGH response in the arginine test. C-peptide immunoreactivity was high. Her condition was fairly well controlled by 140 units of insulin injection daily.
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PMID:Lipoatrophic diabetes. Report of a case. 15 92

An infant with alpha1-antitrypsin (alpha1-AT) deficiency PiSZ presented with liver cirrhosis and showed clinical and laboratory evidence of renal disease when hepatic decompensation developed, shortly before death at 12 months of age. Low serum levels of alpha1-AT were only demonstrated late in the disease. SZ phenotype was proved by starch gel electrophoresis. Post-mortem pathological studies revealed severe hepatic cirrhosis with intracytoplasmic inclusion of alpha1-AT and membranous glomerulonephritis with deposits of complement and immunoglobulins but without the presence of alpha1-AT. The present case suggests the importance of studying Pi phenotypes and serum levels of alpha1-AT in all cases of idiopathic cirrhosis or renal disease in infancy.
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PMID:Juvenile cirrhosis and membranous glomerulonephritis in a child with alpha1-antitrypsin deficiency PiSZ. 30 2

The postoperative courses of 26 patients admitted to an Intensive Care Unit after different types of surgery on their portal tree are studied. All were diagnosed as having portal hypertension secondary to chronic liver disease and had presented one or more episodes of bleeding. Those complications of greater risk with an important early mortality rate are: 1) recurrence of the gastrointestinal hemorrhage, independently of the type of lesion which originates it; 2) recurrence of ascites because these patients more often develop dehiscence of the abdominal wall, serious dilutional hyponatremia and severe functional renal insufficiency; 3) acute renal failure, both functional or caused by an organic tubulo-interstitial nephropathy; 4) peritonitis; 5) persistent hepatolytic episode; 6) hyperdynamic heart failure; and 7) re-operations in general, independently of the causes. The frequency of these complications and the greater or lesser seriousness of their development in the postoperative period are dependent on: 1) the age of the patient with a better prognosis for those under 50; 2) the histopathologic type of the hepatic lesion, with hepatic fibrosis having a more favourable evolution in comparison with cirrhosis; 3) the degree of decompensation of the hepatopathy immediately before the operation, evaluating signs of functional hepatic deficit, cytolysis and degree of portal hypertension. The greater the preoperative activity, the worse the postsurgical prognosis. 4) The elective or urgent character of the surgery. During the postoperative course of emergency surgery all types of complications may appear. The emergency operation which has effectively achieved the stopping of the esophageal bleeding has been the porto-azygos disconnection, which allows later a portosystemic shunt with a greater probability of success. 5) The type of anastomosis carried out. Radicular shunts were those which had a lower postoperative mortality rate and those which progressed better because of the small number of problems occurring in the early postoperative period.
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PMID:[Postoperative care in portal hypertension surgery (author's transl)]. 43 Nov 56

Renal prostaglandins have several potential functions in renal physiology. Perhaps their best documented role is the maintenance of renal blood flow during renal ischemia, although they are apparently not essential to blood flow autoregulation in the absence of ischemia. Alterations in sodium excretion parallel the hemodynamic changes induced by prostaglandin infusions and prostaglandin inhibition with indomethacin. A direct action on sodium balance is unproven. Numerous studies, in vivo and in vitro, have convincingly demonstrated that prostaglandins or their precursors stimulate renin release and prostaglandin inhibition blunts renin release independent of hemodynamic and electrolyte balance. These functions of prostaglandins have implicated them in the manifestations of Bartter's syndrome, the nephropathy of liver cirrhosis, renovascular hypertension, and other nephropathies.
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PMID:Prostaglandins: renin release and renal function. 72 86

Nafcillin, a semisynthetic penicillin effective against penicillinase-producing staphylococci, is eliminated largely in man via the liver. This study assessed the effect of cirrhosis and extrahepatic biliary obstruction in man on the pharmacokinetics of nafcillin. The plasma clearance of nafcillin controls was 583 +/- 144.2 ml per min (mean +/- SD) and fell strikingly to 291 +/- 147.6 and 163 +/- 56.3 ml per min in patients with cirrhosis and extrahepatic obstruction, respectively (P less than 0.001). In the latter two groups nafcillin excreted in urine increased from about 30 to 50% of administered dose (P less than 0.02), suggesting that renal disease superimposed on hepatic disease would further decrease over-all nafcillin clearance. The depression of nafcillin clearance with hepatobiliary disease did not correlate with any conventional liver laboratory test. The initial volume of distribution of nafcillin (V1) was unaltered but at steady state (Vd()) there was a significant reduction in the distribution volume in the patients with liver disease. Accordingly, the impairment in drug elimination, as assessed by its clearance from plasma, was underestimated by the prolongation of the nafcillin elimination half-life (t1/2(beta)) which was 1.02 +/- 0.20 hr in controls, and 1.23 +/- 0.31 (P greater than 0.05) and 1.73 +/- 0.44 hr (P less than 0.03), respectively, in patients with cirrhosis and extrahepatic obstruction.
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PMID:Disposition of nafcillin in patients with cirrhosis and extrahepatic biliary obstruction. 91 79

Plasma carcinoembryonic antigen (CEA) levels have been determined by the zirconyl phosphate gel (Z-gel) method, using materials provided by Hoffman-LaRoche Inc., on 512 samples from 425 hospital patients, and on single samples from 124 normal controls (98 blood donors and 26 healthy staff members). Of the controls, 98% had CEA levels less than 5 ng/ml. Forty-six hospital patients had CEA levels above 20 ng/ml; 45 (98%) had known present or past cancer. Nineteen patients had levels between 10 and 20 ng/ml; 11 (58%) had present or past cancer. Sixty-seven patients had levels between 5 and 10 ng/ml, and most of these had non-malignant diseases; only 34% had present or past cancer. Cigarette smoking was associated with elevated CEA levels among patients with non-neoplastic diseases, notably those with cirrhosis of the liver and chronic renal disease. There was a gradation of increasing specificity for cancer with increasing levels of CEA from 5 to over 20 ng/ml; but, on the other hand, higher levels were associated with more disseminated cancer, which would be less amenable to cure.
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PMID:Plasma carcinoembryonic antigen in an Australian hospital population. 97 58

Three alpha1-antitrypsin (alpha1AT) deficient, protease inhibitor type ZZ children who died from cirrhosis and its complications had membranoproliferative glomerulonephritis at postmortem examination. During life, all three had clinical and laboratory evidence of renal disease which became apparent when hepatic decompensation developed. Immunofluorescence studies and electron microscopy performed in one patient revealed subendothelial deposits of alpha1AT, complement, and immune globulins along the glomerular basement membrane. The pathogenesis of these renal lesions is speculative. Glomerular lesions were not observed in kidney sections of 16 children who died from cirrhosis but who were not alpha1AT-deficient. The present study suggests that renal involvement may be yet another manifestation of disease associated with alpha1AT deficiency.
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PMID:Membranoproliferative glomerulonephritis in childhood cirrhosis associated with alpha1-antitrypsin deficiency. 108 72

This is the definitive review of the cardiovascular effects of liver disease. Physiologic and pathologic studies accomplished primarily in the 1950s and 1960s have been applied to a case of a 16-year-old boy with cirrhosis of the liver who presented with weakness, dyspnea, cyanosis, and clubbing. The probable mechanisms for his signs and symptoms are discussed in detail. The cause for the hyperkinetic circulation is unknown. By exclusion, the cyanosis is attributed to intrapulmonary shunting. Portapulmonary shunts are not quantitatively important. The suitability of the terms micronodular and macronodular cirrhosis is highlighted. Indications for various types of surgical portal shunts are discussed. The value of preoperative hemodynamic measurements of the portal circulation to the individual patient is debated. A liver transplant is considered in this case with no promising medical therapy and a very poor prognosis. The renal disease manifested by red cell casts in the urine is thought to be caused by an immunologic reaction in the kidney somehow related to his liver disease. This discussion is led by Dr. Jack Myers, but his opinions are correlated with those of a pathologist, radiologist, surgeon, and gastroenterologist. It is a clinical tour de force, although not all the predictions are confirmed by laboratory studies such as this patient's hepatic wedge pressure, pulmonary artery pressure, and cardiac fluoroscopy.
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PMID:Multidisciplinary Conferences in Gastroenterology. Cardiovascular effects of severe liver disease. 127 11

The long-term clinical course of patients with primary Type II essential mixed cryoglobulinaemia is unclear as many reports fail to separate this group from patients with Type III disease. We have reviewed 13 patients with Type II essential mixed cryoglobulinaemia who presented to the Hammersmith Hospital between 1976 and 1990. All patients had a cryoglobulin level greater than 0.1 mg/ml (range 0.27-6.50 mg/ml), and characterization of the cryoglobulin in all cases revealed the presence of a monoclonal IgM kappa component with rheumatoid factor activity together with polyclonal IgG. All patients had evidence of activation of the classical pathway of complement with greatly reduced levels of C4, while C3 levels were moderately reduced in three patients. All patients had skin disease and joint symptoms were reported by nine patients, with erosive arthritis in one. Eight patients had peripheral sensorimotor neuropathy. Renal disease was observed in 10 patients, manifesting as raised creatinine level, proteinuria or haematuria. Renal tissue was examined in eight patients: in six the appearances were those of a mesangiocapillary glomerulonephritis Type I while in the other two patients there was a mesangioproliferative glomerulonephritis, in one diffuse and in the other focal and segmental. Glomerular capillary 'hyaline thrombi' were found in six biopsies, extracapillary proliferation was found in three and evidence of vasculitis was found in all eight. Liver biopsy showed macronodular cirrhosis in one patient, while a second with recurrent episodes of jaundice showed only chronic inflammatory changes. No patient was positive for hepatitis B surface antigen; however one patient had low titre anti-hepatitis B surface antibody. Normochromic normocytic anaemia was present in nine patients. Bone marrow examination was carried out in 13 patients at presentation to our unit: 10 showed no evidence of a lymphoproliferative disorder, while three suggested the presence of a non-Hodgkin's lymphoma (some years after original presentation in all three). Unusual clinical features included one patient with retinal vasculitis and one patient with severe pulmonary haemorrhage.
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PMID:Type II essential mixed cryoglobulinaemia: presentation, treatment and outcome in 13 patients. 162 Aug 12


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