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INAUGURAL ISSUE : Volume One - JUNE 2002 -Number ONE

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PREVALENCE OF PUBERTAL DELAY IN MULTIPLY TRANSFUSED PATIENTS WITH THALASSAEMIA: RESULTS FROM A THALASSAEMIA DAYCARE CENTRE IN MALAYSIA

Rahman Jamal, MRCP (Ire), PhD (London)
Marina Nor, MD, MMed
Idris Nor*, MD, MPH
Syed Zulkifli Zakaria, MD, MMed
Rahmah Rasat, MBBS, MMed

Department of Paediatrics, *Department of Community Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, 56000 Cheras, Kuala Lumpur, Malaysia.

Abstract

Objective: The aim of the study were to investigate the problem of pubertal delay among transfusion-dependent thalassaemics and the associated factors that might contribute to this problem.
Materials and Methods: A cross-sectional study on 52 patients with transfusion- dependent thalassaemia aged 14 years attending the thalassaemia Day Care Centre at the Hospital Universiti Kebangsaan Malysia. Pubertal staging was performed using standard methods.
Results: All patients had significant iron overload with a mean serum ferritin level of 7343 g/L. Delayed puberty was present in eleven patients (21.2%). There were no significant differences between the sexes, transfusion groups (<8-weekly versus >8-weekly), and type of thalassaemia (beta-thalassaemia major versus HbE-beta thalassaemia). The mean age of menarche was 16.67  1.53 years in those receiving hormonal replacement therapy and 13.64  1.36 years in those who attained spontaneous menarche. The mean serum ferritin level was higher in those with pubertal delay compared to those without pubertal delay (P=0.057).
Conclusions: These findings highlight the high incidence of pubertal delay among our thalassaemia patients and the importance of iron chelation in preventing complications of iron overload.

Keywords: beta-thalassaemia, HbE-beta thalassaemia, puberty, iron overload, ferritin

Correspondence: Professor Dr. Rahman Jamal, Department of Paediatrics, Faculty of Medicine UKM, Hospital Universiti Kebangsaan Malaysia, Jalan Yaacob Latiff, Bandar Tun Razak 56000 Cheras, Kuala Lumpur, Malaysia.

Updated 15th August 2005