6533b7d4fe1ef96bd1262a5c

RESEARCH PRODUCT

D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis.

K. H. HoltermüllerW. SonntagVolker F. EckardtW. PrellwitzW. VielhauerB. SchulteJ. B. Lüth

subject

medicine.medical_specialtyPathologyCirrhosisAdolescentPhysiologyDiseaseGastroenterologyHemolysisFulminant hepatic failureHepatolenticular DegenerationInternal medicinemedicineHumansGlucocorticoidsbusiness.industryLiver DiseasesPenicillaminePenicillamineGastroenterologyLiver failureCeruloplasminHepatologyMiddle Agedmedicine.diseaseHemolysisWilson's diseaseAcute DiseaseFemalebusinessCoppermedicine.drug

description

Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediate D-penicillamine and high-dose steroid therapy did not influence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.

10.1007/bf01391452https://pubmed.ncbi.nlm.nih.gov/7172963