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Analysis of renal impairment in children with Wilson's disease

Xiao-Hui Zhuang1, Ying Mo, Xiao-Yun Jiang

  • 1Department of Pediatrics, First Affiliated Hospital of Sun Yat-sen University, Guangzhou 510080, China.

World Journal of Pediatrics : WJP
|July 30, 2008
PubMed

Insights

Wilson's disease (hepatolenticular degeneration) often presents with varied renal impairment in children. Early kidney evaluation is crucial for diagnosis and monitoring, especially during D-penicillamine treatment.

Area of Science:

  • Pediatric Nephrology
  • Hepatology
  • Genetic Metabolic Disorders

Background:

  • Wilson's disease (hepatolenticular degeneration, HLD) can manifest with diverse renal complications, leading to potential misdiagnosis.
  • This study investigates the clinical characteristics of renal impairment in pediatric HLD patients.

Observation:

  • Of 85 pediatric HLD patients, 34 exhibited renal impairment.
  • Analysis of 25 patients revealed varied symptoms including edema, gross hematuria, acute renal failure, proteinuria, and hematuria.
  • Biochemical and urinalysis findings indicated tubular damage and glomerular involvement, with one case showing IgA nephropathy.

Findings:

  • Renal impairment in pediatric HLD is multifactorial, presenting with proteinuria, hematuria, and abnormal urine biomarkers.
  • Some patients showed signs of acute kidney injury and glomerular changes, highlighting the spectrum of renal involvement.
  • Corneal Kayser-Fleischer rings and decreased ceruloplasmin levels were consistent findings in affected children.

Implications:

  • HLD should be considered in the differential diagnosis of unexplained renal impairment in children.
  • Regular renal function and urinalysis monitoring are recommended for all HLD patients, particularly those undergoing D-penicillamine therapy.
  • Early detection and management of renal complications can improve outcomes for pediatric patients with Wilson's disease.
Abstract

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