ABSTRACT
A 15-year-old Caucasian female on human chorionic gonadotropin (HCG) diet presented
with fever, cholestasis, coagulopathy, hemolytic anemia, and acute renal dysfunction.
Imaging of the biliary system and liver were normal. She responded to intravenous
antibiotics, vitamin K and blood transfusions but experienced relapse upon discontinuation
of antibiotics. She had remission with reinstitution of antibiotics. Liver biopsy
revealed pronounced bile ductular reaction, bridging fibrosis, and hepatocytic anisocytosis
and anisonucleosis with degenerative enlarged eosinophilic hepatocytes, suggestive
of Wilson disease. Diagnosis of Wilson disease was further established based on the
low serum ceruloplasmin, increased urinary and hepatic copper and presence of Kayser-Fleischer
rings. The multisystem involvement of the liver, kidney, blood, and brain are consistent
with Wilson disease; however, the clinical presentation of cholangitis and reversible
coagulopathy is uncommon, and may result from concurrent acute cholangitis and/or
the HCG diet regimen the patient was on.
KEYWORDS
Wilson disease - cholestasis - cholangitis - biliary - human chorionic gonadotropin
diet
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Tamir MilohM.D.
1919 E. Thomas Road
Phoenix, AZ 85016
Email: tmiloh@phoenixchildrens.com
