Wilson disease chronic hepatitis

UGT activity is modulated by various hormones. Excess thyroid hormone and ethinyl oestradiol (but not other oral contraceptives) inhibit bilirubin glucuronidation. In contrast, the combination of progestational and oestrogenic steroids results in increased enzyme activity. Bihrubin glucuronidation can also be inhibited by certain antibiotics (e.g. novobiocin or gentamicin, at serum concentrations exceeding therapeutic levels) and by chronic hepatitis, advanced cirrhosis and Wilson s disease. 

Chronic Hepatitis B Chronic Hepatitis C Autoimmune Hepatitis Wilson s Disease... 

Similarly, in various liver diseases, thought should be given to the presence of HE if neuropsychiatric disturbances occur. This is true for acute liver diseases (severe acute viral hepatitis, acute liver failure) and for severe (particularly alcohol-related) fatty liver, Wilson s disease, severe chronic hepatitis, severe infectious or parasitic liver diseases such as schistosomiasis, metastatic liver, nodular regenerative hyperplasia, and liver cirrhosis. (1,16,17, 22, 24,28,29, 67,76,78,95,104) The diagnosis of HE can prove difficult if the liver disease is (still) unknown. 

Fig. 31.20 Chronic hepatitis in Wilson s disease. Pronounced simian cleft with barely recognizable hepar succenturatum (s. p. 15)...

Ceruloplasmin. This protein is decreased in Wilson s disease, cirrhosis, and many causes of chronic hepatitis, but may be increased by inflammation, cholestasis, hemochromatosis, pregnancy, and estrogen therapy. It is discussed in more detail under Wilson s disease. 

Inherited liver diseases that present as chronic hepatitis include hemochromatosis, Wilson s disease, and alphaj-antitrypsin (AAT) deficiency. 

The early hepatic s3unptoms and signs are those of acute or chronic hepatitis, which then develops into a postnecrotic type of cirrhosis. There is nothing to distinguish the symptoms referable to involvement of the hver in the course of Wilson s disease from those of postnecrotic cirrhosis... 

The incidence of Wilson disease was estimated to be at least 1 30,000-50,000 with a gene frequency of 1 90-1 150. Among selected groups of patients Wilson disease is certainly more frequent. About 3-6% of patients transplanted for fulminant hepatic failure and 16% of young adults with chronic active hepatitis of... 

Most patients with Wilson disease, whatever their clinical presentation, have some degree of liver disease. Chronic liver disease (if undiagnosed and untreated) may precede manifestation of neurological symptoms for more than 10 years. Patients can present with liver disease at any age. The most common age of hepatic manifestation is between 8 and 18 years, but cirrhosis may already be present in children below the age of 5. On the other hand, Wilson disease is diagnosed also in patients presenting with advanced chronic liver disease in their 50s or 60s, without neurological symptoms and without Kayser-Fleischer rings. 

Hepatic copper content exceeding 250 pg g dry weight (normal up to 50) is increased in 82°% of patients with Wilson disease. In the absence of other tests suggestive for abnormal copper metabolism, diagnosis of Wilson disease cannot be made based on an inaeased hepatic copper content alone. Patients with chronic cholostatic... 

In cirrhosis, reduced bone density of the vertebral bodies could be observed in 16% and of the antebrachial bones in 23% of patients (compared to 7% and 5% respectively, in the control group). Fractures of the vertebral bodies were detected in 16% and of the antebrachial bones in 21% of cases (compared to 8% and 8% respectively, in the control group). Hepatic osteopathy usually occurs independently of the cause and type of the chronic liver disease. However, alcoholism, haemochromatosis and Wilson s disease result in osteoblastic hypofunction, so that osteopathy is more likely to occur in these patients, even prior to the development of cirrhosis. The occurrence of osteopathy is closely correlated with the severity of the liver disease and with hypogonadism. In liver cirrhosis, the prevalence of osteopenia is about 30%. (49,73)... 

Symptoms in patients with Wilson s disease usually begin in the second or third decade of life, but may be earlier or later. However, mutations that completely destroy gene function may be associated with onset of liver disease as early as 3 years of age. The initial clinical presentation may be hepatic, with presentation similar to acute hepatitis or to chronic active hepatitis neurological (e.g., clumsiness, dysarthria, ataxia, and tremors) renal (renal tubular acidosis with aminoaciduria) or, less commonly, hematological, with hemolysis secondary to acute release of free copper from tissue and subsequent oxidation of erythrocyte membranes. The hepatic, and possibly CNS, damage may also be secondary to copper-induced oxidative damage to mitochondrial membranes. Hepatic levels of Cp messenger ribonucleic acid (mRNA) are reduced in patients with Wilson s disease, probably secondary to inhibition of transcription by increased intracellular levels of apoCp. ... 

The classic clinical finding of increased copper deposition in the eye is the Kayser-Fleischer ring, caused by deposition of copper in Descemet membrane at limbus of the cornea. Although found in about 95% of patients with neurological or psychiatric manifestations, it is present in only about half of patients with hepatic forms of Wilson s disease" and is rarely present in children. As mentioned earlier, hemolytic anemia and renal failure commonly accompany acute forms of Wilson s disease hemolytic anemia may be episodic even in chronic forms of Wilson s disease. ... 

Scott J, Goilan J, Samourian S, Sherlock S. Wilson s disease, presenting as chronic active hepatitis. Gastroenterology 1978 74 645-51. 

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