Managing endocrinopathies
Growth and development
Normal growth and development can be achieved in the first
decade by maintaining near-normal pretransfusion Hb levels of
9-10 g/dL.33 However, iron-induced damage to the hypothalamic
pituitary axis can cause delayed pubertal growth and sexual
development despite timely initiation of iron chelation in early
childhood. Therefore, annual endocrine evaluations are recommended,
including measures of pancreatic, thyroid, parathyroid,
gonadal function, and bone health with nutritional counseling.34
Tanner staging should be performed every 6 months in the
prepubescent child. Annual bone age films are performed to assess
skeletal maturation. We begin annual monitoring between 8 and10
years of age for luteinizing hormone, follicular stimulating hormone,
insulin-like growth factor, and insulin-like growth factor
binding protein-3. Tests measuring these factors are required to
make early diagnoses of growth hormone deficiency, which can be
managed successfully with hormone replacement before the completion
of puberty. If pubertal changes have not developed by 13 years
of age in females, or 16 years of age in males, the use of
gonadotropin releasing hormone and gonadal steroids may be
necessary.35 Starting at 8-10 years of age, annual glucose tolerance
testing for the early detection of insulin resistance is recommended
to identify prediabetic or diabetic states caused by pancreatic
destruction, which might benefit from metformin administration or
indicate the need for insulin therapy.35