D-Penicillamine improved laparoscopic and histological findings of the liver in a patient with Wilson's disease: 3-year follow-up after diagnosis of Coombs-negative hemolytic anemia of Wilson's disease.
We report a 13-year-old girl who presented with hepatic failure and hemolytic anemia. Laboratory findings showed a normal serum copper level (104 microg/dl), high urinary copper level (2370 microg/dl), and low serum ceruloplasmin level (14.3 microg/dl). Slit-lamp examination revealed Kayser-Fleischer rings on her cornea, and she was diagnosed with Wilson's disease. Plasma exchange and continuous hemodiafiltration relieved the serious condition, after that laparoscopic examination was performed. Administration of D-penicillamine and restriction of dietary copper (<1 mg/day) were started, leading to a normalized serum alanine amino transferase (ALT) level. After 3 years, she again underwent laparoscopic examination, and the laparoscopic and histological findings of her liver were obviously improved. Management of the copper level can reverse severe liver fibrosis in Wilson's disease.
Sakaida
Isao
I
Department of Gastroenterology and Hepatology, School of Medicine, Yamaguchi University, 1-1-1 Minami-Kogushi, Ube, 755-8505, Japan.
Kawaguchi
Kotaro
K
Kimura
Teruaki
T
Tamura
Fusako
F
Okita
Kiwamu
K
D000293:Adolescent; D000743:Anemia, Hemolytic / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D000931:Antidotes / Q000627:therapeutic use*; D001706:Biopsy; D003298:Coombs Test; D003300:Copper / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D017583:Hemodiafiltration; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D010535:Laparoscopy; D008099:Liver / Q000378:metabolism / Q000473:pathology; D017093:Liver Failure / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D010396:Penicillamine / Q000627:therapeutic use*; D010784:Photomicrography; D013997:Time Factors
D000931:Antidotes; D003300:Copper; D010396:Penicillamine