Medical Journal of the Islamic Republic Of Iran
مجله پزشکی جمهوری اسلامی ایران
Med J Islam Repub Iran
Medical Sciences
http://mjiri.iums.ac.ir
2
journal2
1016-1430
2251-6840
8
10.18869/mjiri
14
8888
13
en
jalali
1384
8
1
gregorian
2005
11
1
18
4
online
1
fulltext
en
EVALUATION OF LABORATORY FINDINGS IN CHILDREN WITH WILSON'S DISEASE IN EMA M KHOMEINI HOSPITAL DURING 1994-2003
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.
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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.