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idopathic short stature

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idopathic short stature

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idopathic short stature

  1. 1. Dr.Yassin M Al-Saleh
  2. 2. • Their Prophet said to them: 'Allah has raised Saul to be your king. ' But they replied: 'Should he be given the kingship over us, when we are more deserving of it than he and he has not been given abundant wealth? ' He said: 'Allah has chosen him over you and increased him with amplitude in knowledge and body. Allah gives His kingship to whom He will. Allah is the Embracer, the Knower.
  3. 3. Case senario • 12 year old boy healthy • his height is far below 3ed centile • aslo far from mid parentral height. • No FH of constitutional delay. • GV 4 cm/year , on exam : non dysmorphic • bone age 1yaer delay . • GH stimulation test normal. IGF-1, IGFBP3 normal.
  4. 4. • It is common condition. • The term has been in use since at least 1975. • It is a clinical description rather than a disease. Idiopathic short stature
  5. 5. introduction • Definition: stature that is 2 standard deviations (SD) or more below the mean for age (approximately the 2nd percentile) and for whom no endocrine, metabolic, or other diagnosis can be made .
  6. 6. • Children with ISS fall into two main groups: • Familial short stature (in which bone age is not delayed and the child is growing within the parental target range) • Constitutional delay of growth (in which bone age is delayed) • Also combination of these two condition. introduction
  7. 7. presentation • usually Children with ISS have normal growth velocity • no biochemical or other evidence for a specific growth-retarding condition. • normal growth hormone (GH) responses to pharmacologic agents • some children with ISS may have low serum concentrations of IGF-I
  8. 8. treatment • Several studies have demonstrated that growth hormone therapy generally increases height velocity acutely and may increase adult height in children with ISS. GROWTH HORMONE
  9. 9. • was approved by the United States Food and Drug Administration (FDA) in 2003. • The indication is for children with current height below -2.25 SD of the mean, in whom the epiphyses are not closed, and whose expected adult height is less than 63 inches (160 cm) for boys and 59 inches (150 cm) for girls GROWTH HORMONE
  10. 10. • the use of growth hormone for ISS remains controversial. • A majority of children with short stature will experience some catch-up growth during puberty without GH treatment • There is little evidence that short stature has a detrimental effect on an individual's psychosocial or physical functioning. GROWTH HORMONE
  11. 11. • The available evidence suggests only modest efficacy for GH treatment in children and adolescents with ISS. • treated individuals remain relatively short compared with their peers GROWTH HORMONE
  12. 12. • The decision to treat children and adolescents with ISS using growth hormone requires complex psychosocial considerations • GH treatment should be considered only if the short stature represents a disability to the child and is not amenable to counseling and reassurance • GH treatment could have adverse psychosocial consequences due to the extreme focus on a child's stature conferred by GH treatment. • Most patients with ISS have normal psychosocial functioning. GROWTH HORMONE Psychosocial considerations
  13. 13. • Growth hormone treatment at standard doses appears to have minimal physiological adverse effects. • High-dose GH treatment (71 mcg/kg/day) was reported to accelerate the onset of puberty and epiphyseal closure in children with ISS but lower doses (34 or 53mcg/kg/day) did not GROWTH HORMONE Safety
  14. 14. • For children with ISS the optimal dosing range is well established. • In prepubertal children with ISS, GH routinely is used in the range of 25 to 50 mcg/kg/day. • Higher doses lead to modest increases in short-term growth velocity and adult height . GROWTH HORMONE Dosing
  15. 15. For how long • continue treatment only if the height velocity increases by 50 percent or at least 2.5 cm/year above the baseline height velocity. • If the initial growth response is significant . treatment is continued until linear growth decreases to less than 2.0 to 2.5 cm (about 1 inch)/year. • If bone age >14 for girls, >16 for boys. GROWTH HORMONE
  16. 16. Dose adjustment • evidence supports the idea of adjusting GH dose based on IGF-I levels when treating children with ISS. • GH dosing be adjusted to maintain IGF-I within a normal range. GROWTH HORMONE
  17. 17. • IGF-I levels approximately four weeks after beginning therapy or changing the GH dose, and approximately every 6 to 12 months thereafter, similar to the approach we use for patients with GH deficiency GROWTH HORMONE Dose adjustment
  18. 18. OTHER TREATMENTS • IGF-1. • GnRH analogs. • Testosterone. • Aromatase inhibitors.
  19. 19. Recombinant human IGF-I • Recombinant IGF-I (rhIGF-I) has been effectively used for treatment of children with "severe primary IGF-I deficiency. • A preparation of rhIGF-I is approved by the FDA for this use.
  20. 20. • Patients with ISS may have degrees of growth hormone insensitivity, with normal or elevated circulating serum GH levels but low levels of insulin-like growth factor-I (IGF-I) and IGF binding protein 3 (IGFBP-3). • For these children, direct replacement of IGF-I may be more effective than GH treatment. Recombinant human IGF-I
  21. 21. GnRH analogs • An alternative approach to attempt to delay pubertal development and epiphyseal fusion. • The range of the effect is limited to approximately 0 to 4 cm.
  22. 22. Testosterone • Boys with mild to moderate short stature whose puberty and bone age are delayed • treatment with testosterone during adolescence may be helpful to promote puberty and accelerate linear growth.
  23. 23. Aromatase inhibitors • in adolescent males it will facilitate growth by delaying epiphyseal closure. • Preliminary studies suggest that treatment with aromatase inhibitors, with or without concomitant growth hormone, increases predicted adult height. • long-term safety and efficacy results are not available • In girls, aromatase inhibitors would be expected to slow growth because they inhibit estrogen production.
  24. 24. conclusion • Treatment of children with idiopathic short stature (ISS) with growth hormone is controversial . • the decision to treat children with ISS using growth hormone depends on individual considerations. • Growth hormone treatment is likely to yield only modest gains in height compared with no treatment
  25. 25. IGF-1

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