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 onset of spontaneous hemolytic jaundice in a young subject should lead to the search for Wilson's disease when clinical examination reveals cirrhosis. This hemolysis may evolve in the form of severe jaundice to a stage where the cirrhosis remains usually latent or well tolerated. The intervention of a toxic, allergic of infective factor liable to produce a hepatic lesion which frees a dose of copper sufficient to trigger off hemolysis, is discussed. The mechanism of the latter, that of the coagulation disorders observed, liver cell failure and widespread intravascular coagulation, are analysed in this paper and compared with data in the literature. The dramatic character of the case indicates that it is necessary to treat as a routine with penicillamine all homozygous forms of Wilson's disease.
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PMID:[Severe hemolytic jaundice and Wilson's disease]. 19 22

Hepatic symptoms are usually the first in Wilson's disease of children and adolescents, while neurologic symptoms and the corneal ring are still missing. Liver lesions due to copper accumulation may develop throughout years without clinical symptoms or biochemical abnormalities. Hemolytic jaundice or gastrointestinal bleeding are the presenting symptoms in some cases. In spite of being a rare syndrom Wilson's disease ought to be considered after hepatitis B or autoimmune liver disease have been excluded as causes of juvenile cirrhosis of the liver. If life-long treatment with D-penicillamin is started in an early stage of Wilson's disease, prognosis is rather good.
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PMID:[Wilson's liver disease in children and adolescents (author's transl)]. 52 94

A discussion is made of the basic peculiarties and differences in the clinico-laboratory profile in various forms of pre- and postmicrosome jaundice. The importance of direct to total bilirubin ratio, and of quantitative bilirubin and urobilinogen bodies' determination in the urine is stressed. Bile pigments conjugation by the hepatic cells and free bilirubin conversion to glucuronose may be also assayed by resorting to some additional tests. The test with N-acetyl-paraaminophenol (NAPA) provides for an indirect assessment of the liver's glucuronidase function and has a good informative value. It is optimally characterized by the percentage of free (non-conjugated) NAPA with respect to the total. The latter indicator is normal in chronic hepatitis and in Dubin-Johnson's syndrome, and is at the uppermost normal limit in hepatic cirrhosis and cholestatic jaundice. It is strongly increased (in the average three times with respect to normal values) in Gilbert's disease and posthepatitis hyperbilirubinemia (PHHB). It affords some information on the severity of the defect in transport of bile pigments in the mentioned affections. In hemolytic jaundice a normal percentage of free NAPA is usually found. Glucuronose conversion of bilirubin hardly plays an essential role in the pathogenesis of hyperbilirubinemia i the listed above diseases. This is also confirmed by the NAPA test, performed subsequent to novobiocin loading. The percentare of free NAPA under the conditions just outlined is furthermore increased in Gilbert's disease and PHHB, while in hemolytic jaundice it remains within normal limits. In Gibert's disease and PHHB, a strongly pronounced delay in the excretion of substances is noted. Not infrequently, a similar disorder is also observed in hepatic cirrhosis. It is interpreted as an expression of an overall disturbance in hepatic blood flow and function of the heavily affected hepatic parenchyma.
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PMID:[Study of liver glucuronidase function in indirect hyperbilirubinemia and liver cirrhosis]. 123 97

The total activity of lactatedehydrogenase (Merck tests) and its isoenzymes (electrophoretic fractionation by Helm) were studied in the blood sera of 202 patients with various hepato-biliary diseases (viral hepatitis, chronic persisting and chronic active hepatitis, cirrhosis of liver, chronic cholecystitis, benign cholestasis, malignant formations with and without cholestasis, Gilbert's syndrome and hemolytic jaundice). The referent limits are determined in the sera of 43 clinically healthy subjects (22 females and 21 males). The anaerobic fifth fraction of lactatedehydrogenase (LDH) was most increased in viral hepatitis (about 15 times), in the group with malignant formations with cholestasis (about 2 times) and in benign cholestasis (about 5 times). The determination of isoenzymes of LDH, is presumed, to be obligatorily included in the spectrum of modern enzyme constellation for hepatobiliary diseases.
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PMID:[Lactate dehydrogenase isoenzymes in the diagnosis of hepatobiliary diseases]. 653 74