Growth Retardation in β-Thalassemia Major

Abstract

ABSTRACT: BACKGROUND: Thalassemia is an inherited autosomal recessive blood disease caused by genetic defects result in reduced rate of synthesis of one of the globin chains that make up hemoglobin. The combination of transfusion and chelating therapy has dramatically extended the life expectancy of thalassemic patients. OBJECTIVE: The objective of this study is to determine the factors associated with short stature in thalassemic patients and main endocrine complications. PATIENTS AND METHODS: A case-control study was performed prospectively in Ibn-Al Balady hospital in (Al- Sader city-Baghdad- Rasafa) for the period extended from the 1st of January to 31st of May (2013). Data were collected from 181 thalassemic patients, their ages were 10 to 20 years old being attended the hospital for regular follow up and blood transfusion. The control in this research work was one of the patients' relatives with the same age after thalassemia was ruled out in the control. Data collected in this study included: age, sex, height and weight were assessed by the National Center of Health statistics (NCHS) growth curves , history of splenectomy, times of blood transfusion , hepatitis infection, type of chelating agent. Serum ferritin, hormones level, fasting and random blood sugar and serum calcium were estimated. RESULT: It was founded that 79% of the β-thalassemic patients had short stature (their height equal or less than5rd percentile) with significant relation with high serum ferritin (P-value=0.006). Delayed puberty was the commonest endocrine complication in thalassemic patients (83.9%). CONCLUSION: High serum ferritin is associated with growth retardation and many endocrine complications . Aggressive iron-chelating therapy and regular measurement of hormones concentration are necessary for thalassemic patients mainly during puberty to avoid growth retardation.