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Hepatic encephalopathy in Wilson's disease
*For correspondence: vgajanan@rediffmail.com
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 17 yr old male child†, a known case of Wilson's disease, presented to the outpatient department of the Kasturba Hospital, Chinckpokli, Mumbai, India, in October 2019, with fever (38.6°C) for two days, yellowish discolouration of the sclera (Figure A and C), skin (Figure B, D, and E). On examination, the patient was not oriented to time, place and person. Pallor and icterus were noted along with mild splenomegaly (Figure B). Bilateral flapping tremors were present (Video). Bilateral Kayser–Fleischer ring was seen (Figure A and C arrows). His haemoglobin level was 8.6 gm%, total and direct bilirubin were 32.7 mg% and 14.9 mg% respectively. Liver enzymes were elevated (SGOT: 617 U/l, SGPT: 607 U/l, alkaline phosphate 93IU/L,) prothrombin time was 26.2 sec, international normalization ratio was 2.54 (Nazer score was 12), blood urea nitrogen 11 mg% and creatinine was 0.6 mg%. Leptospirosis, dengue fever and malaria were ruled out. Viral markers were also negative (for HBV, HCV, HAV, HEV). The serum ceruloplasmin level was 0.03 O.D. and urinary copper excretion was 210 μg/24 h. He was referred to KEM Hospital, Mumbai for further management. He was treated with a combination of D-penicillamine (250 mg), injection (Inj.) cefotaxime (1 g) (BD), Inj. metronidazole (100 mg) (TDS), Inj. thiamine (100 mg) (BD), Tab. zinc (50 mg) (TDS), Inj. vitamin K (10 mg) (BD), rifaximin (550 mg) (BD). lactulose (30 ml) (TDS). He improved symptomatically and followed up on a monthly basis to keep track of his liver function tests and overall well-being. His Nazer score was still on the higher side, and he was, therefore, advised liver transplantation.
- (A and C) show yellowish discolouration of sclera. (B, D and E) Yellowish discolouration of the skin. (B) Mild splenomegaly. (A and C) (arrows) Bilateral Kayser–Fleischer ring: brownish ring rimming the cornea.
Video available at ijmr.org.in.
Conflicts of Interest: None.
