Paper Information

Journal:   INTERNATIONAL JOURNAL OF PEDIATRICS   SEPTEMBER 2014 , Volume 2 , Number 3-3 (SUPP 5); Page(s) 25 To 29.
 
Paper: 

THE CLINICAL MANIFESTATIONS, TREATMENT EFFICACY AND ADVERSE DRUG REACTIONS IN 62 IRANIAN CHILDREN WITH WILSON DISEASE

 
 
Author(s):  NAJAFI MEHRI, ALIMADADI HOSEIN, ARASTOO LEILA*, MOTAMED FARZANEH, KHODADAD AHMAD, FALLAHI GHOLAMHOSEIN, FARAHMAND FATEMEH, DOROUDIAN RANA
 
* TEHRAN UNIVERSITY OF MEDICAL SCIENCES, TEHRAN, IRAN
 
Abstract: 

Introduction: The Wilson disease is an autosomal recessive disease in which the liver, central nervous system, eyes, blood and other parts of the body involved. Timely diagnosis and appropriate treatment of the disease requires awareness of the clinical presentations of this disease in children.
Materials and Methods: This case series study included 62 patients with Wilson disease who admitted to children's Medical Center, Tehran-Iran in 2003-2012 years.
Results: 56% of patients were male. The average age of diagnosis was 9.73
±2.35 years old (5-17 years) and this was higher in patients with early neurologic symptoms (P=0.85). 64.5% of the patients had the hepatic symptoms at the time of diagnosis and the most common type of hepatic involvement was cirrhosis (39.3%) and hepatitis (17.5%) respectively. 17.7% of the patients also had early neurological symptoms. A positive family history for the Wilson disease were found in 27.4% of patients. 74.2% of patients had Kayser–Fleischer rings (KF ring) and the frequency of these symptom was higher in patients with early neurological involvement. 83.9% of patients were treated successfully with D-penicillamine and in 30% of patients, adverse drug reactions were seen.
Conclusion: Children with unknown liver disease should be evaluated for Wilson disease and the first-degree relatives of patients should be screened. D-penicillamine have important side effects, but due to the low cost and the availability is an appropriate drug to treat the Wilson disease.

 
Keyword(s): D-PENICILLAMINE, HEPATIC INVOLVEMENT, KF RING, NEUROLOGIC INVOLVEMENT, WILSON DISEASE
 
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