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Pediatrics Education and Research

Volume  5, Issue 2, May-Aug 2017, Pages 27-40
 

Original Article

The Endocrine Dysfunction in Multitransfused Thalassemic Patients

Pankaj Gupta*, Ashok Gupta**

*Consultant, Department of Pediatrics, Jammu and Kashmir Health Department, Jammu, India. **Formerly, HOD, Department of peadiatrics, Govt Medical College, Jammu, India.

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DOI: https://dx.doi.org/10.21088/per.2321.1644.5217.6

Abstract

Background: Every year 10,000 children with thalassemia major are born in India, which constitutes 10% of the total numbers in the world. The current management of thalassemia major (TM) includes regular transfusion programs and chelation therapy. The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassaemic patients but is complicated by citrate toxicity and subsequent iron overload. Excessive iron is deposited in most tissues primarily in the liver, heart and the endocrine glands Disorders of growth, sexual development & fertility, abnormal bone mineralisation, diabetes mellitus, hypothyroidism and hypoadrenalism are the main endocrine complications found in thalassaemic patients. Aims And Objectives: To determine the incidence of thyroid dysfunction by estimating T3, T4 and TSH, hypoparathyroidism by estimating serum calcium, phosphorus and alkaline phosphatase levels,diabetes mellitus by estimating fasting and postprandial blood suger, to see for pubertal delay in these patients according to sexual maturity rating and finally to determine if there is any correlation between increased serum ferritin and these abnormalities. Material and Methods: This study was conducted in the department of Paediatrics, Thalassemia Unit, Government Medical College, and Jammu. All thalassemic children below the age of 19 years who had received more than 20 transfusions and whose ferritin levels were more than 1000mg/ng and were coming regularly for transfusion in the thalassemia unit of department of paediatrics were included in the study. Results: The height and weight of these children were significantly less (>2.5 SD) as compared to normal children. Hypothyroidism was detected in 12.82% of the patients,hypocalcemia was detected in 20 cases, hypoparathyroidism in 8 patients, impaired glucose tolerance was detected in 25.7%, Conclusion: Increasing awareness of endocrinological problems in thalassemic patients is essential not only because such patients are living longer now, but also because much of the morbidity and mortality from these complications can be reduced with regular surveillance, early treatment and followup.

Keywords: Thalassemia Major; Blood Transfusion; Serum Ferritin; Delayed Growth; Puberty; Hyopothyroidism; Hypoparathyroidism; Diabetes. 


Corresponding Author : Pankaj Gupta, Consultant, Department of Pediatrics, Jammu and Kashmir Health Department, Jammu, India