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. 2021 Dec 31;26(4):227–236. doi: 10.6065/apem.2142164.082

Table 2.

Recommendations for endocrinologic problems in Prader-Willi syndrome (PWS)

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.