Physiological responses to increased dietary cholesterol: the case of the egg man.

Twenty of 320 patients with Wilson’sdisease initiallypresented with chemical and laboratory features ofchronic active hepatitis, confirmed histologicallyin 17. When h t seen, Cirrhosis was present in all 20and was complicated by ascites and/or jaundice in 11.Within 1 week to 8 years of the onset of overt liverdisease the diagnosis of Wilson’s disease was estab-lished, and treatment with D-peniciuamine was promptly initiated in 19 patients. One man refusedtreatment and died 4 months later. Treated patientsreceived D-penicillamine or trientine for a total of 264patient-years (median, 14 patient-years). Abnormalwater retention, for which salt restriction and di-uretics were added to penicillamine or trientine, dis-appeared in all but 1 of the patients so affected.Symptomatic improvement and virtually normallevels of serum albumin, bilirubin, aspartate ami-notransferase, and alanine aminotransferase followedwithin 1 year in the mqjority of subjects. One womandied after 9 months of treatment. Two patients, whobecame noncompliant with the therapeutic regimenafter 9 and 17 years of successful pharmacologicaltreatment, required liver transplants. These resultsindicate that the prognosis of specifically treatedWilsonian chronic active hepatitis is very good in spiteof the presence of cirrhosis.