Thalassemia, Endocrine Sequelae
| Autor: | A. Kyriakou, N. Skordis |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Delayed puberty
Pituitary gland medicine.medical_specialty Bone disease business.industry Absent pubertal growth spurt medicine.disease Bone remodeling medicine.anatomical_structure Endocrinology Hypogonadotropic hypogonadism Internal medicine medicine Endocrine system medicine.symptom business Endocrine gland |
| DOI: | 10.1016/b978-0-12-801238-3.04488-3 |
| Popis: | Multiple transfusions in patients with thalassemia major (TM) result in overload of iron, which accumulates in tissues with high levels of transferrin receptors such as the liver, heart, and endocrine glands. The anterior pituitary is particularly sensitive to iron overload causing hormonal secretion abnormalities. Growth failure in TM has been recognized for many years and has persisted despite major treatment advances. The child with TM has a particular growth pattern, which is relatively normal until age 9–10 years; after this age, a slowing down of growth velocity and a reduced or absent pubertal growth spurt are observed. The origin of growth failure is multifactorial, and the functional abnormalities of growth hormone/IGF-1 axis are accompanied by structural anomalies of the pituitary gland and its stalk. Iron deposition on gonadotropic cells of the pituitary leads to the disruption of gonadotropin production and consequently to delayed puberty and hypogonadotropic hypogonadism, which still remains the most common endocrinopathy in TM. Therapeutic approach of the patient with delayed or arrested puberty and hypogonadotropic hypogonadism is individualized and depends on factors such as age, severity of iron overload, damage of the hypothalamic–pituitary–gonadal axis, chronic liver disease, and the presence of psychological problems. The pathogenesis of bone disease, a major cause of skeletal complications in TM is multifactorial, complicated, and still unclear. Patients with TM display an unbalanced bone turnover with an increased resorption phase and decreased formation phase, resulting in severe bone loss. Endocrine dysregulation continues to be a significant challenge in TM patients due to interrelated factors, affecting their social adjustment and quality of life. Close follow-up, early recognition, and proper management are crucial for every patient. |
| Databáze: | OpenAIRE |
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