Treatment of the Chronically Transfused Patient (3)

Endocrine Dysfunction
Endocrine and growth disturbances occur secondary to the disease, its treatment and iron overload. It is essential to monitor these patients for diabetes, gonadal failure, hypothyroidism, hypoparathyroidism, growth hormone deficiency and osteoporosis. These abnormalities are commonly secondary to iron induced organ damage. However, other factors such as Desferal toxicity and nutritional deficiency can occur. To monitor for these complications, children aged 10 years and older begin thyroid testing (TSH, free T4, T3), diabetes testing (oral glucose tolerance), pubertal development (GnRH dynamic testing-girls at 12 years and boys at 14 years), and biannual bone density evaluations commencing at 15 years. If a patient has an abnormal OGTT, aggressive chelation with counseling is initiated to prevent further endocrine and/or organ damage. Overt diabetes requires an aggressive diabetes program of education, nutrition counseling and parenteral deferrioxamine chelation. Hypoparathyroidism results in vitamin D replacement, and monitoring of serum calcium, magnesium and urinary calcium. Hypogonadism is screened for because of its implications on fertility, sexual relationships, osteoporosis, growth and sexual development. The risks and benefits of testosterone and estrogen are discussed. Significant abnormalities of bone density are reviewed by a physician at the Osteoporosis Center UCSF for consideration of chrondrate or calcitonin. In the young child, bone changes secondary to Desferal are screened for. <next>

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