Abstract

Beta-Thalassemia is a genetic disorder which is associated with a lot of complications. Frequent blood transfusions result in increased iron deposition in various tissues leading to dysfunction of many vital organs. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients. Early diagnosis of hypoparathyroidism (HPT) could prevent other severe disorders such as Tetany, seizures, osteopenia, and osteoporosis. Growth retardation can occur as complication of thalassemia as early as the 1st or 2nd year of life but these abnormalities more prominent after the 6 – 8 years of life.The aim of this study was carried out to determine; 1. The prevalence of Hypoparathyroidism (HPT) and Growth retardation in patients with beta thalassemia and to correlate them with serum ferritin, calcium, phosphorus and alkaline phosphatase levels; 2. The relationships of growth failure with certain variable including age, serum ferritin, mean hemoglobin level and gender of the patients. This is a descriptive cross sectional research study which was conducted on 200 subjects (100 cases and 100 controls) in the age group of 10-25 years who had visited the OPD/IPD of Subharti Medical College & affiliated Hospitals, Meerut. The cases included were with confirmed diagnosis of beta thalassemia major, with regular blood transfusions and serum ferritin levels >2000 ng/ml irrespective of chelation therapy. Out of 100 patients, Hypoparathyroidism was diagnosed in 18% patients, Growth retardation/ Short stature 93% and Weight loss was found in 93% patients. The mean age at diagnosis was 12.6 years (range 11-16 years), mean serum calcium was 7.53 mg/dl (range7.58-9.04 mg/dl), mean serum ferritin was 5831.0 ng/ml (range 2000-8,064 ng/dl) and mean serum phosphate was 5.63 mg/dl (range 4.50-7.73 mg/dl). Serum parathyroid hormone (PTH) levels were low in most of the patients. Short stature was observed in most of the patients, while it was found normal in control subjects.Significant Hypoparathyroidism (HPT) observed along with growth retardation in beta thalassemia patients (p < 0.001). A significant decrease in serum calcium level was seen in cases when compared to controls, where as the levels of both serum phosphorus and alkaline phosphatase levels were found increased in cases as compared to control.

Highlights

  • Beta-Thalassemia is a genetic disorder which is associated with a lot of complications

  • We found there was a high prevalence of Growth retadrdation (93.2%) and weight loss (86%), but there were nonsignificant differences between single and combination therapy groups regarding frequency of delayed puberty or the frequency of short stature.Our results are similar to other studies done in other areas of world. 14,26 This indicate that thalassemic patient have a risk factors for growth failure as result from direct relation to iron toxicity especially endocrine gland, Intensive chelation therapy especially below 10 years of age 27 or may result from other factors like anemia, hypersplenism and Folate deficiency, Calcium and zinc deficiency.[28]

  • The research concluded Growth retardation (86%) and HPT(18%) are common endocrinopathies in TM patients who were on regular blood transfusion therapy

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Summary

Introduction

Beta-Thalassemia is a genetic disorder which is associated with a lot of complications. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients. Growth retardation can occur as complication of thalassemia as early as the 1st or 2nd year of life but these abnormalities more prominent after the 6 – 8 years of life. The prevalence of Hypoparathyroidism (HPT) and Growth retardation in patients with beta thalassemia and to correlate them with serum ferritin, calcium, phosphorus and alkaline phosphatase levels; 2. The relationships of growth failure with certain variable including age, serum ferritin, mean hemoglobin level and gender of the patients. The cases included were with confirmed diagnosis of beta thalassemia major, with regular blood transfusions and serum ferritin levels >2000 ng/ml irrespective of chelation therapy. Significant Hypoparathyroidism (HPT) observed along with growth retardation in beta thalassemia patients (p < 0.001). A significant decrease in serum calcium level was seen in cases when compared to controls, where as the levels of both serum phosphorus and alkaline phosphatase levels were found increased in cases as compared to control

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