Abstract

Background: 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.
 Methods: This is a descriptive cross sectional study carried out in 100 diagnosed patients of beta- thalassemia major who had visited the OPD/IPD of Subharti Medical College & affiliated Hospitals, Meerut for routine blood transfusion or for any other complication. Patients were clinically examined and investigated for presence of one or more endocrine disorders on their routine appointments.
 Results: Endocrine disorders were detected in a total of 82 patients. Diabetes mellitus was detected in 12% patients, hypothyroidism in 36% patients and delayed puberty was found in 72% patients. Mean serum ferritin level was found to be 5831.0±2860.5 ng/ml in beta-thalassemia Major patients, while it was in normal range in control subjects.
 Conclusion: Research concluded with finding of Delayed puberty (72%), Hypothyroidism (36%) and diabetes mellitus as (12%) in beta thalassemia patients who were on regular blood transfusion therapy. Iron overload as serum ferritin level was found to be highly raised in all study case. On the basis of our study we recommend that early detection and management protocols for these endocrinopathies may improve the life prospects of beta-thalassemia Major patients.
 Keywords: Endocrine disorders, Hypothyroidism, Delayed puberty, Diabetes Mellitus Serum ferritin, Thalassemia Major.

Highlights

  • Thalassemia major is a genetic disorder of haemoglobin synthesis with defect in production of one or more hemoglobin chains

  • Endocrine complications in thalassemia patients: Delayed puberty, diabetes mellitus, hypothyroidism and hypoadrenalism are some of the most common endocrine complications found in thalassaemia patients.[8]. Of all these complications delayed puberty due to hypogonadism is the most common occurring in upto 50-91% of patients.[8,9] Gonadal iron deposition resulting in primary gonadal failure is the most important cause of hypogonadism

  • The study focused to assess the prevalence of endocrine disorders in patients with β-thalassemia major who are regular blood transfusion leading to iron overload in Meerut district area

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Summary

Introduction

Thalassemia major is a genetic disorder of haemoglobin synthesis with defect in production of one or more hemoglobin chains. A number of these complications result from iron overload occurring due to repeated transfusions. Endocrine complications in thalassemia patients: Delayed puberty, diabetes mellitus, hypothyroidism and hypoadrenalism are some of the most common endocrine complications found in thalassaemia patients.[8] Of all these complications delayed puberty due to hypogonadism is the most common occurring in upto 50-91% of patients.[8,9] Gonadal iron deposition resulting in primary gonadal failure is the most important cause of hypogonadism.. The first mechanism involves decrease in insulin production either by direct impairment of insulin excretory function by chronic iron overload or immune system activation against pancreatic cells in β-thalassaemia patients. The study focused to assess the prevalence of endocrine disorders in patients with β-thalassemia major who are regular blood transfusion leading to iron overload in Meerut district area

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