HYPOGONADISM IN MULTIPLY TRANSFUSED β- THALASSEMIA SYNDROME PATIENTS
Abstract
Background: Beta thalassemia syndrome represents a wide spectrum and clinically diverse group of recessively inherited dyserythropoietic anaemias including beta thal-assemia major, beta thalassemia intermedia and beta- thalassemia compound hetero-zygotes. The group is signified by defective synthesis of one or more globin subunits of the hemoglobin tetramer requiring blood transfusions. Human body does not have an internal mechanism to eliminate the surplus iron, thus iron overload and its se-quelae follow, effecting complexion, growth, vital organs, sexual maturity, calcium metabolism and vulnerable endocrine functions.
Objectives: To determine the frequency of hypogonadism in multi-transfused beta thalassemia syndrome patients in Khyber Pakhtunkhwa province of Pakistan.
Materials and Methods: This cross sectional descriptive study included 97 patients, 55 males and 42 females aged 15 to 32 years, regularly transfused at Fatimid foun-dation Hayatabad Peshawar. Patients’ data was recorded in questionnaires. Clini-cal characteristics relevant to growth and puberty were recorded. Serum Ferritin, FSH, LH, Testosterone and Estradiol assays were performed using Chemilumines-ence (CLIA) technique. Data was analyzed by SPSS version 20. Means, standard deviation and frequencies were calculated for numerical variables.
Results: Hypogonadism was seen in 40.2% of the whole population (56.4% in males and 19.04% in females). Amongst 42 females, 32 were above 16 years. 43.7% had primary amenorrhoea while 12.5% had secondary amenorrhoea, with an average age of menarche 16±2 years. Delayed puberty was observed in 54.7% of females and 83.6% of males
Conclusion: Delayed puberty and hypogonadism are obvious endocrinopathies showing higher frequencies in iron overloaded thalassemic patients of Khyber Pakhtunkhwa. In this study, frequency of hypogonadism was found more in males as compared to females. Regular endocrine evaluation and timely in-tervention can ensure sexual maturity and improved quality of life for these patients.
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