Original Articles
Endocrine abnormalities in children with Beta thalassaemia major
Authors:
Rajesh Joshi ,
Department of Paediatrics, B. J. Wadia Hospital for Children, Parel, Mumbai, India, IN
About Rajesh
Associate Professor, Department of Paediatrics, B. J. Wadia Hospital for Children, Parel, Mumbai, India
Ankur Phatarpekar
Department of Paediatrics, B. J. Wadia Hospital for Children, Parel, Mumbai, India, IN
About Ankur
Registrar, Department of Paediatrics, B. J. Wadia Hospital for Children, Parel, Mumbai, India
Abstract
Background: Endocrine complications in ?-thalassaemia major patients in developing countries are likely to occur at younger ages due to suboptimal iron chelation.
Objective: To assess the prevalence of endocrine abnormalities and correlate serum ferritin, degree of anaemia and liver dysfunction with endocrine dysfunction
Method: A cross sectional study was carried out in B. J. Wadia Hospital, over a period of 2 years, on all children with ?-thalassemia major over the age of 4 years receiving regular blood transfusions. Patients with transfusion dependent anaemia other than ?-thalassemia major were excluded.
Results: The total number of children over 4 years of age with ?-thalassaemia major receiving regular blood transfusions during the study period was 135. Mean haemoglobin was 7.8 ± 0.6g/dl and the mean serum ferritin level 5295 ± 2736ng/ml. The most common endocrine abnormality was delayed puberty (68%). Seventy one (52.5%) patients had short stature with the height Z-score 2.8±0.8. Hypocalcaemia was observed in 40 (38%) patients. Ten (9.4%) patients had hypoparathyroidism while in 7 (6.6%) it was suspected based on hypocalcaemia, high phosphorus and normal alkaline phosphatase. Rickets was seen in 25 (23.5%) patients. Hypothyroidism was present in 22 (22%) patients of whom 14 had compensated hypothyroidism. Insulin resistance, impaired fasting glucose/impaired glucose tolerance (IFG/IGT) and diabetes mellitus were seen in 7.6%, 4.4% and 2.6% patients respectively. Mean age of patients with delayed puberty was 15.8±1, short stature 10.3±3.4, hypoparathyroidism 10.4±4.2, hypothyroidism 10.4±3, IFG/IGT 10.4±2 and diabetes mellitus 14.2±1.2 years. Bone mineral density was done in 48 patients older than 10 years.
Eighteen (37.5%) patients had spinal osteoporosis. Five patients had more than one endocrine abnormality.
Conclusions: There were no statistically significant differences between the patients with and without endocrine abnormalities with respect to serum ferritin, mean pre-transfusion haemoglobin and liver dysfunction except for ferritin in patients with hypoparathyroidism and diabetes mellitus.
(Key words: ?-thalassaemia; serum ferritin; endocrine; haemoglobin; transaminase)
http://dx.doi.org/10.4038/sljch.v42i2.5628
How to Cite:
Joshi, R. and Phatarpekar, A., 2013. Endocrine abnormalities in children with Beta thalassaemia major. Sri Lanka Journal of Child Health, 42(2), pp.81–86. DOI: http://doi.org/10.4038/sljch.v42i2.5628
Published on
01 Jun 2013.
Peer Reviewed
Downloads