Table 2.
Endocrine problems | Age of life | Screening and monitoring | Managements |
---|---|---|---|
Growth hormone deficiency | Early infancy | Monitoring of growth according to disease-specific growth curve | GHT start as diagnosis of PWS is made (as early as 3–6 months of age) |
Childhood and adolescence | Monitoring of growth, skeletal maturation, puberal development. | GHT start as soon as possible after PWS diagnosis or continue GHT with monitoring | |
If sleep apnea is developed, adenoid assessment and polysomnography are needed. | |||
Regular assessment of anthropometric measure, IGF-1, TFT, glucose metabolism, scoliosis | |||
Adulthood | Assessment of IGF-1, lipid and glucose metabolism, BMI, lean body mass | Considering restart GHT with confirmed GHD after achievement of final height | |
Hypothyroidism | Early infancy | TFT within first 3 months of age | Levothyroxine supplement at typical replacement dose |
After childhood | TFT annually, or every 6 months during GHT | ||
Central adrenal insufficiency | All age | Educate patients and their families on signs and symptoms of adrenal insufficiency | Hydrocortisone supplement at typical replacement dose |
Consider adrenal function test at presenting clinical features or prior to major surgery or anesthesia | |||
Hypogonadism | Newborn and early infancy | Examination for cryptorchidism in male | Orchiopexy in most case |
Childhood and adolescence | Monitoring of pubertal initiation and progression | If delayed or stalled puber ty, consider sex hormones replacement therapy | |
Assessment of LH, FHS, testosterone/estradiol, inhibin B | Consider sex hormones replacement therapy | ||
Adulthood | Assessment of LH, FHS, testosterone/estradiol, inhibin B | Counsel for contraception in female |
GHT, growth hormone treatment; IGF-1, insulin like growth factor-1; BMI, body mass index; TFT, thyroid function test.