1Zlatko
\uri},
2Vojislav
N. Peri{i},
1Gordana
Kosti},
1Emilija
Golubovi}
Abstract
Wilson
disease is one of the chronic liver diseases in which hemostasis can be impaired
by various
mechanisms. Gastrointestinal hemorrhages caused by ruptured esophageal varices
or as complica-
tion
of peptic ulcer can be relatively frequent in Wilson disease. We are presenting
two pediatric
patients with non-gastrointestinal bleeding as the leading sign of Wilson
disease.
Key
Words:
bleeding,
hemostasis,
Wilson disease.
Sa`etak
Wilson-ova bolest je jedna od hroni~nih bolesti jetre u kojoj hemostaza
mo`e biti poreme}ena
razli~itim mehanizmima. Krvarenja u gastrointestinalni trakt usled prskanja
variksa jednjaka ili kao
komplikacija ulkusne bolest su relativno ~esta u Wilson-ovoj bolesti.
Prikazujemo dva pedijatrijska
pacijenta u kojih je krvarenje van digestivnog trakta bilo vode}i znak Wilson-ove
bolesti.
Klju~ne re~i:
krvarenje,
hemostaza,
Wilson-ova bolest.
ExtraGI bleeding in Wilson�s disease
Address correspondence to:
Dr
Zlatko Djuric, M.D.
Children's Hospital
48 B.
Taskovica str., 18000 Ni�, Serbia
Phone. :381 18 334190, Fax. 381 18 331922
E-mail: astra@junis.ni.ac.yu
�
Gastroenterolo{ka sekcija SLD,
01753, 2003.
ARCH
GASTROENTEROHEPATOL 2003; 22 (No 3 - 4): 62 - 64
Non-gastrointestinal
bleeding as leading sign
of
impaired haemostasis
on
Wilson�s
disease
Krvarenje van gastrointestinalnog trakta kao
vode}i znak poreme}aja hemostaze u
Wilson-ovoj bolesti
(accepted December 15th, 2003)
Liver
and biliary tract
Jetra
i bilijarni trakt
1Children's
Hospital Ni�, Serbia,
2University
Children's Hospital, Belgrade, Serbia
INTRODUCTION
Wilson disease, an autosomal recessive disorder of
hepatic copper metabolism, occurs with an incidence of 1
in
30 000 persons worldwide. The gene responsible for the
expression of the Wilson disease is located on the chro-
mosome 13. As a result of the gene expression there is the
absence or the dysfunction of a copper transporting P-type
ATPase. The disease can be presented in different ages and
in a
variety of form. Paediatric patients usually present
with
liver disease (chronic hepatitis, cirrhosis or acute
liver
failure), renal, and hematological symptoms.
Neuropschiatric symptoms predominate in adolescents
and
young adults. It is well known that patients with the
chronic liver diseases are prone to various disturbances in
the
blood coagulation process. In this paper we are pre-
senting two of our patients in whom bleeding out of the
digestive tract was Wilson disease leading clinical sign.
CASE
REPORT 1
A 10-year-old girl had a three-year history of the pro-
longed nosebleeding frequent episodes. She was investi-
gated
at the ear-nose-throat Department where the diagno-
sis
of the nasal septum deviation was made. During a rou-
tine
preparation for the operation, a disturbance of the
coagulation screening was seen and girl was transferred to
our
hospital. Her history was unremarkable, except one
episode of febrile seizures seven years before the admis-
