Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.
Hypoglycemia is a common medical emergency. Insulinoma, a rare pancreatic endocrine tumour is the most common cause of hypoglycemia in patients without systemic illness. Most insulinomas are small, solitary and benign, which can be treated effectively by surgical removal of the tumour. Diagnosis is based on clinical features, laboratory tests and radiological investigation to localize the tumour. However, small size of this tumour leads to difficulty in localization of the disease, especially in occult insulinoma. Intra-arterial calcium stimulation with hepatic venous sampling can be utilised for preoperative localization of the tumour. We hereby present a case of a 33-year-old woman presenting with history of recurrent hypoglycemic symptoms and morbid obesity. A thorough history, clinical examination and laboratory investigation including prolonged fasting test were conducted and diagnosis of insulinoma was made. Intra-arterial calcium stimulation with hepatic venous sampling was carried out and revealed localization of insulinoma at pancreatic head. Bangladesh Journal of Medical Science Vol.20(1) 2021 p.216-219
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