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
Managing endocrinopathiesGrowth and developmentNormal growth and development can be achieved in the firstdecade by maintaining near-normal pretransfusion Hb levels of9-10 g/dL.33 However, iron-induced damage to the hypothalamicpituitary axis can cause delayed pubertal growth and sexualdevelopment despite timely initiation of iron chelation in earlychildhood. Therefore, annual endocrine evaluations are recommended,including measures of pancreatic, thyroid, parathyroid,gonadal function, and bone health with nutritional counseling.34Tanner staging should be performed every 6 months in theprepubescent child. Annual bone age films are performed to assessskeletal maturation. We begin annual monitoring between 8 and10years of age for luteinizing hormone, follicular stimulating hormone,insulin-like growth factor, and insulin-like growth factorbinding protein-3. Tests measuring these factors are required tomake early diagnoses of growth hormone deficiency, which can bemanaged successfully with hormone replacement before the completionof puberty. If pubertal changes have not developed by 13 yearsof age in females, or 16 years of age in males, the use ofgonadotropin releasing hormone and gonadal steroids may benecessary.35 Starting at 8-10 years of age, annual glucose tolerancetesting for the early detection of insulin resistance is recommendedto identify prediabetic or diabetic states caused by pancreaticdestruction, which might benefit from metformin administration orindicate the need for insulin therapy.35
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