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Endocrine function in thalassaemia

S Vannasaeng, S Ploybutr, P Visutkul

    Clinical Endocrinology
    |February 1, 1981
    PubMed
    Summary
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    This study on Thai adolescents with beta-thalassemia/hemoglobin E and hemoglobin H diseases found impaired growth and sexual development. Endocrine abnormalities, including reduced growth hormone secretion, were noted, with a lower diabetes incidence than in Western patients.

    Area of Science:

    • Pediatrics
    • Endocrinology
    • Hematology

    Background:

    • Beta-thalassemia/hemoglobin E and hemoglobin H diseases are common genetic blood disorders.
    • Endocrine complications are significant in thalassemia patients, particularly those receiving frequent transfusions.
    • Limited data exists on endocrine function in non-transfused or minimally transfused young patients.

    Purpose of the Study:

    • To investigate endocrine abnormalities in Thai adolescents with beta-thalassemia/hemoglobin E and hemoglobin H diseases.
    • To assess growth, sexual maturity, and hormonal secretion in this specific patient cohort.
    • To compare findings with previously reported data from hypertransfused populations.

    Main Methods:

    • Study included 21 Thai patients aged 8-20 years with beta-thalassemia/hemoglobin E and hemoglobin H diseases.

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  • Patients received none or minimal blood transfusions.
  • Evaluated clinical endocrine parameters, growth hormone (GH) secretion, oral glucose tolerance tests (GTT), insulin levels, thyroid function, and adrenal function (cortisol, 24-h urinary oxogenic steroids).

    • Main Results:

    • Significant growth retardation and sexual immaturity were observed.
    • Impaired GH secretion was present in most patients.
    • Chemical diabetes incidence (1/16) was lower than in Western hypertransfused patients.
    • Basal and post-glucose insulin levels were lower than in normal controls.
    • Thyroid and adrenal functions were normal in all patients.

    Conclusions:

    • Non-transfused or minimally transfused Thai adolescents with beta-thalassemia/hemoglobin E and hemoglobin H diseases exhibit distinct endocrine profiles.
    • Growth and sexual maturation are key concerns, alongside impaired GH secretion.
    • The lower incidence of chemical diabetes suggests transfusion status significantly impacts metabolic complications.