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Morbidity and mortality patterns
of thalassaemia major patients in Hong Kong: retrospective study
CK Li, CW Luk, SC Ling, KW Chik, HL Yuen, CK Li,
MMK Shing, KO Chang, PMP Yuen
Department of Paediatrics, The Chinese University of Hong Kong,
Prince of Wales Hospital, Shatin, Hong Kong
OBJECTIVES. To study the morbidity and mortality
patterns of transfusion-dependent thalassaemia major patients in
Hong Kong, and compare the outcomes of these patients according
to different periods of birth.
DESIGN. Retrospective study.
SETTING. Paediatric departments of three regional hospitals, Hong
Kong.
SUBJECTS AND METHODS. Medical records of thalassaemia major patients
were reviewed. Data gathered included demographic and survival data,
complications of iron overload, repeated transfusion, and bone marrow
transplantation; the probability of survival of three cohorts was
also estimated.
RESULTS. Two hundred and thirty-two patients were studied at a median
age of 15.5 years (range, 1.4-30.3 years). There were 60 patients
born before 1980 (cohort 1), 117 patients born between 1980 and
1989 (cohort 2), and 55 patients born after 1989 (cohort 3). The
median age of starting desferrioxamine was 8 years, 4 years, and
3 years for cohorts 1, 2, and 3, respectively. Cardiomyopathy, diabetes
mellitus, and hypothyroidism occurred in 15.1%, 8.6%, and 6.9% of
patients with thalassaemia major, respectively. The above complications
developed in 5% to 12% of cohort 2 patients. Delayed puberty was
present in 38.4% and hormonal replacement for gonadal failure was
required in 29.7% of evaluable patients. Short stature was common
and the median height standard deviation score was -1.63. Twenty
patients had died, and cardiomyopathy was the leading cause of death,
followed by complications of bone marrow transplantation. The probability
of survival beyond the age of 20 years was 87.6%.
CONCLUSION. Despite the use of iron chelation in the past two decades,
severe complications of iron overload still occurred even in those
who started chelation therapy early. Cardiomyopathy was the leading
cause of death, while endocrinopathies and short stature were common
complications especially in teenagers and adults.
Hong Kong Med J 2002;8:255-60
Key words: Beta-thalassemia; Morbidity; Mortality
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