en Pediatric Pediatric Original Research: Clinical Science Original Research: Clinical Science Wilson's disease is a rare but treatable condition with variable clinical presentations. Its diagnosis depends on a combination of clinical and laboratory findings. We evaluated the clinical and laboratory findings in children with Wilson's disease (WD). Twenty -seven children (4-14 years, 59.2 % male, 40.7% female) with confirmed WD were evaluated between 1994 and 2003 at Imam Khomeini Hospital. Seventeen patients (64%) presented with liver abnormalities, 3( 11 %) with neurological features, 3(11 %) with fulminant hepatic failure and 4(14%) were asymptomatic siblings of patients with WD. The presence of Kayser Fleischer rings, high urine copper excretion, low ceruloplasmin level, and elevated liver copper concentration were detected in 51.8%, 77.7%, 92.5% and 72.7% of patients respectively. We emphasize on clinical findings together with one or more laboratory findings as a diagnostic guide in WD and also recommend evaluation of serum ceruloplasmin level and 24 hour urine copper excretion particularly when liver biopsy may not be obtained. Wilson\'s disease, laboratory findings, children. 327 330 http://mjiri.iums.ac.ir/browse.php?a_code=A-10-298-74&slc_lang=en&sid=1 SHAHLA BAHREMAND 20031947532846003510 20031947532846003510 Yes the Pediatric Gastroenterology Ward, Emam Khomeini Hospital, Tehran University of Medical Sciences YAHYA AGHIGHI 20031947532846003511 20031947532846003511 No the Pediatric Rheumatology Ward, Emam Khomeini Hospital, Tehran University of Medical Sciences ZOHREH OLOOMI YAZDI 20031947532846003512 20031947532846003512 No thePediatric Hematology Ward, Emam Khomieni Hospital, Tehran University of Medical Sciences HESHMAT MOAYERI 20031947532846003513 20031947532846003513 No the Pediatric Endocrinology Ward, Emam Khomeini Hospital, Tehran University of Medical Sciences LALEH RAZAVI 20031947532846003514 20031947532846003514 No the Pediatric Ward, Emam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. MANSOUR NATEGHI 20031947532846003515 20031947532846003515 No the Pediatric Ward, Emam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.