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)

Four children had progressive degeneration of the cerebral cortex, with hepatic cirrhosis. They and four previously described ones, are representative of a distinct form of hepatocerebral degeneration. Onset of the neurological disorder is between ages 1 and 3 years, at times with mild developmental delay. Explosive onset of intractable convulsions, leaving the child in a stuporous and demented state, is characteristic. Generalized hypotonia or hemiparesis were observed in several affected children. Clinical evidences of hepatic disease, including ascites and jaundice, occurred late, if at all. The illness ended fatally within ten months of onset of convulsions. Pathological findings in the brain are neuronal loss and gliosis, in a pattern that is indistinguishable from that in degeneration of the cerebral gray matter in infancy (Alpers disease). The hepatic lesions consist of cirrhosis or of subacute hepatitis, with superimposed fatty infiltration of hepatocytes. The disorder is genetically determined, with recessive inheritance.
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PMID:Infantile diffuse cerebral degeneration with hepatic cirrhosis. 125 62

Galactosemia in newborns and infants is associated with the following symptoms: jaundice, hepatomegaly, failure to thrive, feeding difficulties, hypoglycemia, convulsions, lethargy, amino-aciduria, cataracts, hepatic cirrhosis, ascites, and mental retardation. If the preliminary evaluation indicates galactosemia, there is high risk for E. coli sepsis and death. Strong consideration should therefore be given for early antibiotic therapy in infants with suspected galactosemia in spite of the absence of clinical signs or symptoms of sepsis.
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PMID:Association of Escherichia coli sepsis and galactosemia in neonates. 156 28

Serum magnesium (Mg) was measured in 6,252 patients; in 1,246 (19.9%) the value was abnormal. Hypermagnesemia (serum Mg greater than or equal to 3.9 mg/dl) was observed in 51 patients (0.8%) and hypomagnesemia (Mg less than or equal to 1.5 mg/dl) in 165 (2.6%). Hypermagnesemia was found in patients with renal failure treated with Mg-containing antacids or cathartics, or with eclamptic convulsions treated with Mg sulfate. The most frequent clinical finding of hypermagnesemia was urinary disturbance, although various other neurological signs and symptoms were observed. Hypomagnesemia was seen in patients with various diseases such as cancer, hepatic cirrhosis, cerebrovascular disease, and generally poor condition. Abnormalities of electrolytes other than Mg were also frequently observed. The most common clinical findings of hypomagnesemia were personality changes and depression. The differentiation from psychiatric disease is important.
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PMID:An analysis of hypermagnesemia and hypomagnesemia. 227 20

Among the cattle admitted to the II. Medizinische Tierklinik of the University of Munich during the years 1982-86, 38 calves of up to 6 weeks of age had a dystrophy (n=5), pre-cirrhosis (n=21) or cirrhosis (n=12) of the liver. Similar hepatic lesions were found in three 1- to 7-day-old calves and in 1 foetus aborted in the 7th month of gestation which were submitted for necropsy. Of the 38 patients (74% male, 87% German Fleckvieh) 25 had been ill since the first seven days of life (mean age on admittance: x=14 days). These hepatopathies showed no significant preponderance in regard to breed, sex or season. The general condition of the calves was moderately to severely disturbed. Catarrhal enteritis resistant to treatment was the main clinical finding in 32 of the 38 patients. Nine calves developed central nervous symptoms (delayed reflexes, impaired coordination, convulsions, opisthotonus). Neurohistological examination of one of these calves revealed massive astrocytic oedema, shrinkage of individual nerve cells and formation of the so-called hepatic glia (hepatoencephalopathy). Even in the last days before death icterus of the sclera was noted in only 11 of the 38 patients; of these, nine also displayed icterus of the mucous membranes. The calves died between the third day to seventh week of life; mean age at death 18 days. Haematological examination: in 12 cases mild to marked anaemia (but without signs of haemolysis), marked granulocytosis with a shift to the left in 20 cases, sometimes additionally neutrocytes with nuclei resembling Pelger's nuclear anomaly (pseudo-Pelger), granulocytes with basophilic stippling as well as lymphoidocytes. The blood glucose levels were usually markedly reduced. 15 calves had a pronounced refractory hypoglycaemia with blood glucose levels under 1,66 mmol/l. Neither albumin concentration nor the specific serum globulin fractions showed significant differences when compared to an age matched control group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Liver cirrhosis in young calves]. 276 85

The patient was a 59-year-old man who had been in hospital suffering from aplastic anemia with transfusion hemosiderosis. Sudden onset of weakness, shaking chills and headache was observed after his staying out overnight on July 25, 1981. His temperature was 39.3 degrees C and he complained of abdominal pain and abdominal distension. His blood pressure dropped to a dangerous level and tonic convulsions that had begun in the upper body gradually extended to the whole body and he died 23 hours after his return. V. vulnificus was isolated by the blood culture performed before death. During his stay away from the hospital, he had eaten raw cuttlefish, which was considered to be the source of infection. V. vulnificus is one of the halophilic marine vibrios and is isolated frequently in summertime from the sea foods and sea water near Japan. It has been disclosed that the presence of underlying diseases such as liver cirrhosis, hemochromatosis can predispose a person to fatal sepsis by V. vulnificus. In this case, besides leukocytopenia, the presence of hemosiderosis induced by many transfusions was considered to be a major cause leading to the fulminating course of the disease.
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PMID:[Fatal Vibrio vulnificus infection in a patient with aplastic anemia]. 667 24

Although elevated gamma globulin is known to produce hypoalbuminemia both experimentally and in disease, a low albumin concentration in chronic liver disease often is assumed to reflect impaired liver synthetic function. Albumin and gamma globulin measurements in a series of 200 patients with a variety of chronic diseases (including cirrhosis, connective tissue disease, chronic inflammation, and malignancy) associated with diffuse hypergammaglobulinemia were combined with similar measurements from a previous study (Am J Med 1959; 29:596-616). The mean serum albumin concentration correlated inversely with mean gamma globulin, irrespective of disease category. Double reciprocal plot analysis showed that the relationship fits a rectangular hyperbola (r = -0.915, P less than 0.001), with the mean albumin concentration approaching 2.31 g/dL at infinite gamma globulin. This suggests that serum albumin decreases to a similar extent in various chronic diseases and that hypoalbuminemia has no diagnostic implications, except to the extent that it reflects the severity of hypergammaglobulinemia.
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PMID:Hypoalbuminemia associated with diffuse hypergammaglobulinemia in chronic diseases: lack of diagnostic specificity. 670 48

Analyses of 23 fatal instances of hepatic injury in patients taking valproic acid reveals that all but three were less than 20 years old, and all but four had been taking the drugs for more than 1 month. Convulsions, facial edema, lassitude, and vomiting were prominent clinical features. Hypoglycemia was recorded in six patients. Rash and eosinophilia were not seen. Values for transaminases were modestly elevated in most patients. Most levels of SGOT were below 500 IU, and SGPT levels were below 200 IU. Livers showed microvesicular steatosis in most patients, usually accompanies by necrosis. Four patients had cirrhosis. Overt valproic acid-induced hepatic injury appears to be rare and hence, by definition, idiosyncratic. That it may be an idiosyncratic exaggeration of a much more frequent phenomenon is suggested by the higher incidence of seemingly trivial injury. The idiosyncrasy appears to be metabolic rather than immunologic, and the available information leads to the plausible hypothesis that a metabolite is responsible for the microvesicular steatosis seen in most fatal cases. The steatosis resembles that of Reye's syndrome and Jamaican vomiting sickness, and there is reason to believe that the metabolite responsible for the steatosis resembles the agent responsible for Jamaican vomiting sickness. A different metabolite is presumably responsible for the necrosis seen in many of the cases.
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PMID:Valproate-induced hepatic injury: analyses of 23 fatal cases. 681 94

Rhinocerebral phycomycosis is an uncommon opportunistic infection with ubiquitous fungi of the class Phycomycetes, starting in the nose and extending to the paranasal sinuses and then intracranially. The condition is often characterized by poor prognosis because of occlusion of the internal carotid artery. This disease is commonly associated with predispositions such as uncontrolled diabetes mellitus, which is the most common, immunosuppressive states and metabolic bankruptcy including leukemia, lymphoma, myeloma, malnutrition, uremic or diarrheal acidosis, severe burns, anemia, carcinoma, radiotherapy, liver cirrhosis, hemochromatosis, tuberculosis, septicemia, long-term medication of steroid, antibiotics and antimetabolite, drug addiction, cytotoxic drug administration and AIDS. Cases with unknown predisposition, however, have been infrequently reported in the literature. The authors report a case of rhinocerebral phycomycosis in which concurrence of Candida species instead of the above-mentioned common predispositions was considered a potential predisposition. To our knowledge, only 1 report in which Candida species are referred to as a potential predisposition for this disease has been previously issued. A 85-year-old man was admitted to our hospital on March 2, 1994 because of generalized convulsion. He had received a total extirpation of an ascending colon cancer in July 1993. On admission, physical inspection showed no abnormalities and neurological examination revealed obtunded consciousness without other abnormalities. He had no diabetes mellitus. Hematological and blood chemistry values were normal except for CA19-9 of 45 U/ml.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of rhinocerebral phycomycosis]. 760 36

A new non-linear mathematical model was constructed in order to perform in vivo quantification of the RES phagocytic function. This method is based on the same technical facilities as used for the routine liver-spleen scintigraphy with radiocolloids [1, 2]. But kinetic modeling of dynamic Tc-99m-sulfur colloid data produced estimations of the functional RE-parameters: the clearance rate of the colloidal particles, the rate of phagocytosis, and the RES functional volume, which can not be obtained by classical approaches. This non-linear model was designed on the basis of the principal characteristics of particulate material interaction with macrophages (attachment, phagocytosis, digestion) [3, 4, 5]. The theoretically examined behavior of this in vivo mathematical model corresponds with the experimental behavior of the RES. The mathematical expression of the dynamics is the system of non-linear differential equations with constant coefficients that have no analytical solution. Fitting of the normalized heart blood time-activity curve was obtained to identify the unknown model parameters via non-linear regression. For this purpose general interactive PASCAL procedure IDPAR for a PDP-11/34 computer was used (an IBM PC version is also available). Two to three iterations were needed to estimate the set of unknown parameters for any patient study (1-1.5 min). A very good fitting was obtained between experimental and model curves in every case of different pathologies (error of the approximation is about 2-3%). Studies were performed using an in vivo bolus injection of 3.6 mg/80 kg commercially available colloid KOREN labeled with 3m-Ci 99m-Tc (analog of TCK-1). Our method was used to determine the RES functional parameters for patient groups with different levels of the RES dysfunction. Obtained results illustrate the possibilities of our technique to quantitatively estimate not only great pathology (portal cirrhosis), but also small changes of the RE-function (case of hyperlipidemia and ulcer gaster). In all patient groups marked changes of Tc-99m-sulfur colloid turnover were observed. In general, tracer clearance from the circulation was decreased, and the rate of phagocytosis and the RES volume were diminished compared with controls. The effect of a reduction of phagocytosis increases when the RES dysfunction becomes stronger. It can be shown that a non-parametric Wilcoxon-Mann-Whitney test gives a significant difference (P95%) for these patient groups. Further, we represent the possibility of using the model for monitoring changes of the RES-function parameters during and after therapy. The quantitative test of the RES function can significantly enhance the diagnosis and management of different diseases. Serial colloidal studies may document changes in the RES-function for the tumors, cirrhosis, hyperlipidemia, reticulosis, hepatitis, thrombosis, infection, AIDS, burn injury, shock and trauma patients. The technique may be useful for the different RES investigations with laboratory animals. Created computer software can be used as a tool for kinetic models, simulation, and unknown parameters identification.
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PMID:A non-linear mathematical model for the in vivo evaluation of the RES phagocytic function. 859 76

A 15-year-old boy presented with biochemical and histological hepatic abnormalities consistent with chronic aggressive hepatitis and concomitant hyperthyroidism. After treatment with antithyroid therapy not only the hyperthyroidism subsided but also the hepatic function normalized. Two years later, the hyperthyroidism and liver function disorders relapsed in combination with a striking vitiligo and once again the liver function normalized after treating the hyperthyroidism. The liver biopsy sample taken in remission showed a normal picture without signs of hepatitis, fibrosis or cirrhosis. The diagnosis of a polyglandular syndrome was made, of which three types have been described. At this moment the patient fits best in type 3, which is a rather heterogenic group. Recovery of chronic hepatitis by treatment of concomitant hyperthyroidism has never been described before.
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PMID:Recovery of chronic hepatitis by treatment of concomitant hyperthyroidism. 1169 69


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