[Evaluation of hepatic blood supply in obesity].
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Biomedical subjects
Publications and source records attributed to M Hartleb.
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Wilson's disease presenting as fulminant hepatic failure is a rare and poorly recognized disorder. When ophthalmic and neurologic signs are missing and liver biopsy cannot be performed the patients may not be diagnosed and treated specifically. An acute hemolysis only occasionally occurs in Wilson's disease but is considered a distinctive feature in the form of fulminant hepatitis. Two homozygous sisters suffering fatal Wilsonian fulminant hepatitis with hemolytic episodes are presented. Failure or delay in diagnosis seems to be responsible for high mortality in this form of Wilson's disease. Diagnostic value of familial history and laboratory results is discussed.
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The medical diagnoses of 217 successively dead patients were compared with their postmortem findings. The rate of correct diagnoses was 67.8%, and that of false-negative diagnoses was 22.1%. In a corresponding earlier study (1964-75) carried out in the same department the rates were 35.1% and 55.6%, respectively, whereas the percentages of false-positive diagnoses remained the same. The considerable difference between older and younger age groups demonstrated in the previous studies was not found now. The improvement is believed to be due to the introduction of the new imaging, computerized tomography, ultrasonography, gammagraphy, and endoscopic retrograde cholangiopancreatography techniques.
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