Wilson's Disease; Clinical Presentations among Patients Attending Gastroenterology Clinic/ Baghdad Teaching Hospital
Keywords:
wilson, copper, kayser,, Presentation, Iraq.Abstract
Background:Wilson’s disease (WD) is an inherited
disorder of copper metabolism that is characterized
by tremendous variation in the clinical presentation.
Objective: To assess demographic distribution,
clinical presentations, diagnostic evaluation, and any
association between clinical presentations and other
studied variables of a sample of Iraqi patients with
WD.
Methods: A descriptive cross sectional study with
analytic elements was conducted during 2011, from
the 1st of February till the 10th of June. The sampling
method was a convenient non-random one, carried
out through consecutive pooling of registered WD
patients. A questionnaire-form paper had been
developed for the process of data collection.
Results: The study had enrolled 29 patients, with a
male to female ratio of (1.07:1), their mean age was
27.12±12.18 years. 82.8% of them lived in urban
area. 48.3% were singles. Only 20.7% of patients had
a positive family history of WD. 69% of patients had
consanguineous parents. The main initial clinical
presentations were; hepato-neurologic (31%), pure
hepatic (27.6%), neuro-psychiatric (13.8%) and other
presentations (27.6%). Hepatic manifestations were
seen in (82.8%) of patients; jaundice was the most
frequent (89.7%). Ophthalmologic manifestations in
(55.1%) of patients including; Kayser-Fleischer rings
(51.7%), diplopia (6.9%) and cataracts (3.4%).
Neurologic manifestations existed in 44.8% of
patients; tremors were the most frequent (41.4%).
Psychiatric manifestations existed in 31% of patients;
depression was the commonest (27.6%). Joints
manifestations existed in 20.7% of patients. The
diagnosis delay was 11.26±8.2 months.
Conclusion: The higher percentage of patients were
of hepato-neurologic and pure hepatic presentations.
Patients with hepato-neurologic type are diagnosed in
older age, while those with neuro-psychiatric type are
diagnosed in younger age and with longer diagnosis
delay.
