Hepatorenal dysfunction

Other relevant history, including preexisting medical conditions (e.g.. allergies, race, pregnancy, smoking and alcohol use, hepatorenal dysfunction, etc. ... 

Cytosolic tyrosine aminotransferase (EC 2.6.1A). Elevated tyrosine in blood and cerebrospinal fluid. Increased urinary tyrosine, 4-hydroxyphenylpyruvate, 4-hydroxyphenyllactate and 4-hydroxyphenyiaeetate. Slight to moderate mental retar tion. Blistering and hyperkeratosis of palms and soles of feet. Photophobia. No hepatorenal dysfunction (cf. Hereditary tyrosinemia type I). Controlled with diet low in phenylalanine and tyrosine. 

Hereditary tyrosinemia type I, or Tyrosinosis, or Hereditary hepatorenal dysfunction (see Fig. 2). 

Tyrosyluria (the excretion of 4-hydroxyphenylpyruvate, 4-hydroxyphenyl-lactate and 4-hydroxyphenylacetate with hypertyrosinaemia) occurs in some normal newborn babies, particularly premature babies, scurvy (ascorbate or vitamin C deficiency), tyrosinosis (Medes, 1932), hepatic cytosol tyrosine transaminase deficiency (oculo-cutaneous tyrosinaemia) (Kennaway and Buist, 1971), in hereditary tyrosinaemia ( inborn hepatorenal dysfunction with tryosyluria ) (LaDu and Gjessing, 1978) and as a secondary phenomenon to a number of liver diseases, including several inherited metabolic diseases unassociated directly with tyrosine metabolism (for example, propionic acidaemia) or involving disorders of carbohydrate metabolism (for example, galactosaemia and fructosaemia). 

This was first reported by Buist and his colleagues (Buist et aL, 1969) in a 5-year-old male patient with tyrosinaemia and tyrosyluria who presented with multiple congenital abnormalities and severe mental retardation. The patient did not have any hepatorenal dysfunction and was similar to the original patient... 

Fig. 16.16 Inherited metabolic diseases associated with hepatorenal dysfunction and tyrosyluria and close similarities to hereditary tyrosinaemia type I. (From Chalmers, 1974)...

Frequent monitoring for signs of hepatorenal syndrome, pulmonary insufficiency, and endocrine dysfunction is necessary. 

Hepatic function impairment In patients with preexisting severe liver disease, hepatic encephalopathy (manifested by tremors, confusion, and coma, and increased jaundice) may occur. Because amiloride is not metabolized by the liver, drug accumulation is not anticipated in patients with hepatic dysfunction, but accumulation can occur if hepatorenal syndrome develops. 

Increased risk of renal dysfunction and hepatorenal syndrome... 

Platt, J.F., liUis, J.H., Rnbin, J.M., Merion, R.M., Lucey, M.R. Renal duplex Doppler ultrasonography a noninvasive predictor of kidney dysfunction and hepatorenal failure in liver disease. Hepatology 1994 20 362-369... 

Complications such as variceal bleeding, hepatic encephalopathy, ascites and infections as well as reduced renal function also influence the mortality rate of liver cirrhosis (in Germany some 25,000/year). The main causes of death are hepatic coma or liver failure (25-40%), bleeding (20-30%), infections (about 10%) and HCC (about 5%). Spontaneous bacterial peritonitis is fatal in 50-70%, and with liver dysfunction even in 90% of cases. Occurrence of the hepatorenal syndrome is almost invariably fatal. 

Cirrhosis and the pathophysiologic abnormalities that cause it result in the commonly encountered problems of ascites, portal hypertension and esophageal varices, hepatic encephalopathy, and coagulation disorders. Other less commonly seen problems in patients with cirrhosis include hepatorenal syndrome, hepatopulmonary syndrome, and endocrine dysfunction, and these are discussed in the section dealing with management of complications. 

In addition to the more common complications of chronic liver disease discussed above, a number of other complications can occm, including hepatorenal syndrome, hepatopulmonary syndrome, coagulation disorders, and endocrine dysfunction. 

Physical examination to exclude, particularly, hepatorenal, cardiorespiratory, neurological, and visual dysfunction. Where there is reason to suspect adverse effects, appropriate special investigations may need... 

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