Acute Interstitial Nephritis as the First Presenting Feature of Wilson's Disease: A case Report of Diagnostic Dilemma

Abstract

Ashmita Yadav, Ram Bahadur Nepali and A.M. Jahangir Alam

Introduction: Wilson's disease( also called as hepaticolenticular degeneration) is a rare genetic (autosomal recessive) disorder resulting from mutation in ATP7B gene presenting with features affecting various organs of the body namely( liver, brain, kidney, cornea) due to deposition of excess copper in these organs. It can lead to fatal consequences if not diagnosed and treated in time however prompt diagnosis and treatment can prevent further deterioration of symptoms.

Case Presentation: The authors hereby present a case of 11 years old male who presented with symptoms mimicking post streptococcal glomerulonephritis (PSGN) however after a thorough history , clinical examination findings and detailed investigations he was diagnosed as a case of decompensated chronic liver disease (CLD) due to Wilson’s disease with acute interstitial nephritis(AIN).

Discussion: He has been on chelating agent, zinc acetate and has been asked to avoid copper containing foods. The patient has been followed up regularly since the beginning of treatment.

Conclusion: The main objective of the authors is to highlight a rare case of wilson's disease whose first presentation was renal involvement which led to diagnostic dilemma; further evaluation and investigations ultimately established the diagnosis.

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