ABSTRACT
Brazil has a young population and areas of endemic mansoni schistosomiasis where Wilson's disease might be easily misdiagnosed in patients erroneously classified as having either the hepatosplenic or the hepatointestinal form of the helminthiasis. Twenty five patients with the "hepatic form" of Wilson's disease (14 males and 11 females) were investigated in Belo Horizonte, MG; the mean age was 13.7 years (3 to 22). Nineteen had hepatomegaly (76%) and nine splenomegaly (36%). Twenty two (88%) had cirrhosis. The Kaiser-Fleisher ring was detected in fifteen (60%). Four (16%) had clear neurological abnormalities. Eleven (44%) had ascitis and/or jaundice. Ninety one point three per cent and 92% had low ceruloplasmin and copper serum levels respectively. Eighty four point two per cent showed an increased 24 hours urinary copper excretion; seven patients in whom hepatic copper was determined had increased values. Six out of nine had at least a ten fold increase in 24 hours urinary copper excretion following penicillamine use ("penicillamine test"). Three out of 19 patients (15.8%) had mansoni schistosoma ova in stools examination, a common prevalence in our population. Their biopsies showed inactive cirrhosis without schistosomiasis-associated alterations. At least fourteen patients (56%) could be misdiagnosed as having hepatointestinal or hepatosplenic schistosomisis when in fact they suffered from Wilson's disease with or without asymptomatic intestinal schistosomiasis, losing the chance of an early treatment. The follow-up time of 22 patients was 52 months (1 to 96); eight (36.3%) died, four from bleeding esphageal varices, three from terminal hepatic failure and one from fulminant liver failure. The majority of the patients, including those who died, had abandomned the use of penicillamine or had taken it irregularly, due mainly to its highly expensive cost. A 17 year old patient underwent a successful liver transplant in 1989.
Subject(s)
Hepatolenticular Degeneration/diagnosis , Liver Diseases/etiology , Schistosomiasis mansoni/epidemiology , Adolescent , Adult , Child , Child, Preschool , Copper/urine , Diagnosis, Differential , Female , Follow-Up Studies , Hepatolenticular Degeneration/etiology , Hepatolenticular Degeneration/therapy , Humans , Liver Cirrhosis/pathology , Liver Diseases/physiopathology , Male , Schistosomiasis mansoni/diagnosisABSTRACT
Alcohol has been shown to reduce serum calcium concentrations in several animal studies. In humans, using relatively low doses of alcohol, however, the results were inconclusive. In addition, the effect of alcohol consumption on serum magnesium concentrations is controversial. To elucidate the influence of alcohol ingestion on serum calcium and magnesium in humans 43 intoxicated patients and seven healthy volunteers who had not previously ingested alcohol were studied. There was an inversely related diminuition of serum calcium and magnesium concentrations with increasing serum alcohol. These effects of alcohol may play a role in the metabolic and clinical disorders observed in severely intoxicated people.
