Hepatolenticular Degeneration Presented with Nephrotic Syndrome in Children
CAI Yong
MO Ying
JIANG Xiao-yun
ZHENG Ju-ying
HUANG Sui
Abstract：Objective To summarize the clinical manifestations of hepatolenticular degeneration(HLD) in children presenting with nephrotic syndrome in order to improve the level of diagnosis and treatment. Methods Retrospective analysis on clinical data of one case of HLD presenting with nephrotic syndrome was made. Results The patient complained edema, oliguria, tea like urine for one and a half months. The patient had a family history of acute hepatitis and nephritis. He was misdiagnosed as having nephrotic syndrome and treated with Hormones. But Hormone effect was poor. In our hospital after fur-ther inspection, all kinds of hepatitis virus markers were negative. K-F ring was found. Serum copper blue protein was de-creased. 24 h urine copper was high. HLD was confirmed. The patient was treated with Penicillamine and Zinc, and was dis-charged after the symptoms were relieved remarkably. Conclusion For the patient presenting with nephrotic syndrome with positive family history and multiple system damage, we should pay attention to HLD, and choose the appropriate medical ex-amination as soon as possible in order to reduce misdiagnosis and mistherapy rates.
Keywords：Hepatolenticular degenerationNephrotic syndromeChildMisdiagnosis
Publication Date：2016-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：3( 46-48 )