Disorders of growth

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CHAPTER 25

Disorders of growth

1. Summarize normal growth velocity for children until the pubertal growth spurt.

2. Summarize growth velocity during the pubertal growth spurt.

3. How is height measured accurately?

4. What technique is used for infants up to 2 years of age?

5. Describe the technique for children 2 years of age and older.

6. How is height recorded?

7. List the common errors in plotting growth charts.

8. What is meant by “appropriate growth chart”?

9. How do age and position affect growth measurements?

10. What historic information is necessary for interpreting a growth chart?

11. What physical examination findings help interpret a growth chart?

image Signs of chronic illness

image Stigmata of a syndrome

image Specific signs of hormonal abnormality (thyroid deficiency, growth hormone [GH] deficiency, glucocorticoid excess)

12. How does radiologic imaging help interpret a growth chart?

13. Explain the significance of parental target height or “midparental height.”

14. What is the most important factor in identifying an abnormal growth curve?

15. What causes abnormal growth in children?

16. Which syndromes are associated with abnormal growth?

17. List nonendocrine diseases and treatments that may be associated with poor growth.

18. Using the tools of growth curve, bone age, and height, how does one distinguish between familial (genetic) short stature and other causes?

19. Give an example of distinguishing familial short stature from other causes of short stature.

A 5-year-old whose height is below the third percentile, whose growth has traced a line parallel to the third percentile, whose height projects within the parental target range, and whose bone age is also 5 years is likely to have familial short stature. However, if the growth velocity is abnormal or projected height falls below the predicted range, other factors may be involved in the short stature (Figs. 25-1 and 25-2).

20. Other than familial short stature, what is the most common cause of short stature?

Constitutional delay of growth (constitutional short stature), which affects up to 2% of children, is characterized by short stature and delayed bone age and represents a normal growth pattern simply shifted to a later age. Affected children typically have a period of subnormal growth between 18 and 30 months of age, followed by normal growth velocity throughout the remainder of childhood. In accord with the delayed developmental pattern, bone age is delayed. The continuing growth delay also results in a delay in pubertal development and physical maturity. Such children (often boys) generally have a family history of a similar growth pattern and may have a more dramatic deceleration of growth velocity before they enter puberty than normal children. They complete their growth at a later age, reaching an adult height within the expected genetic potential (Fig. 25-3).

21. How is the diagnosis of constitutional delay of growth made?

22. What is the effect of testosterone therapy on boys with constitutional delay of growth?

23. List the endocrine causes for short stature in children, in order of prevalence.

24. What laboratory measurements should be considered in evaluating a patient for short stature?

25. Which laboratory tests help exclude undiagnosed chronic illness?

26. Which laboratory tests help exclude gastrointestinal disorders associated with poor growth?

27. List the laboratory tests for genetic disorders associated with poor growth.

28. Which hormonal disorders should be excluded by laboratory results?

29. Describe the causes of GH deficiency.

30. How is GH deficiency diagnosed?

31. List the components of the laboratory evaluation for GH deficiency.

32. Why is the serum level of IGF-1 important?

33. Does a normal level of IGF-1 exclude GH deficiency?

34. Does a low serum level of IGF-1 confirm the diagnosis of GH deficiency?

35. How is GH testing done?

36. How are the results of GH testing interpreted?

37. How is idiopathic GH deficiency diagnosed?

38. How is GH deficiency treated?

39. What is the prognosis for adult height in treated children with GH deficiency?

40. When is GH therapy discontinued?

41. What other syndromes are considered indications for GH therapy?

42. What is the prognosis for girls with Turner’s syndrome treated with GH?

43. What are the potential risks of GH therapy?

44. List the common but clinically unimportant side effects of GH therapy.

45. List the uncommon side effects with potential clinical importance.

46. What rare or theoretical side effects may be associated with GH therapy?

47. Should children with idiopathic short stature (without GH deficiency) be treated with GH?

The FDA has approved the use of GH in children with idiopathic short stature with a predicted adult height less than 63 inches for boys and less than 59 inches for girls. However, the use of GH in children in whom no hormonal abnormality can be demonstrated continues to be intensely controversial among pediatric endocrinologists. Short-term studies involving small cohorts have demonstrated a consistent increase in growth velocity with GH therapy in such children. Several studies that monitored children to final height disagreed about the overall effectiveness of therapy. However, most studies agree that the increase in final adult height is limited and can be obtained only at significant financial cost. The decision to use GH in such children should be carefully considered and requires a thoughtful dialogue among child, family, and an experienced pediatric endocrinologist who knows the child well.

48. How does the pattern of growth in children with excessive glucocorticoids differ from the pattern in children with exogenous obesity?

49. What conditions are associated with excessive growth in childhood?

50. Explain constitutional advanced growth.

51. List the hormonal causes of excessive growth.

52. Summarize the characteristics of GH excess in childhood.

53. With what findings is androgen excess associated?

54. With what findings is estrogen excess associated?

55. List the genetic syndromes associated with excessive growth.

image Klinefelter’s syndrome (47,XXY): tall stature, small testes, delay of puberty

image Connective tissue disorders

image Marfan’s syndrome: tall stature, arachnodactyly, joint laxity, lens displacement

image Stickler’s syndrome

image Soto’s syndrome (cerebral gigantism): macrocephaly, progressive macrosomia, dilated ventricles, retardation, advanced bone age

image Beckwith-Wiedemann syndrome: macroglossia, umbilical hernia, hypoglycemia, macrosomia in infancy

image Homocystinuria: arachnodactyly, retardation, homocystine in urine

Bibliography

Carel, JC, Management of short stature with GnRH agonist and co-treatment with growth hormone. a controversial issue. Mol Cell Endocrinol 2006;254:226–233.

Clayton, PE, Cianfarani, S, Czernichow, P, et al, Management of the child born small for gestational age through to adulthood. a consensus statement of the International Societies of Pediatric Endocrinology and the Growth Hormone Research Society. J Clin Endocrinol Metab 2007;92:804–810.

Cytrynbaum, CS, Smith, AC, Rubin, T, et al, Advances in overgrowth syndromes. clinical classification to molecular delineation in Sotos syndrome and Beckwith-Wiedemann syndrome. Curr Opin Pediatr 2005;17:740–746.

Davenport, ML. Evidence for early initiation of growth hormone and transdermal estradiol therapies in girls with Turner syndrome. Growth Horm IGF Res. 2006;16:591–597.

Lee, MM. Clinical practice. Idiopathic short stature. N Engl J Med. 2006;354:2576–2582.

Myers, SE, Carrel, AL, Whitman, BY, et al. Sustained benefit after 2 years of growth hormone on body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome. J Pediatr. 2000;137:42–49.

Quigley, CA. Growth hormone treatment of non-growth hormone deficient disorders. Endocrinol Metab Clin North Am. 2007;36:131–186.

Rosenbloom, AL, Connor, EL. Hypopituitarism and other disorders of the growth hormone-insulin like growth factor-1 axis. In: Lifshitz, F, eds. Pediatric endocrinology, vol. 2. New York: Informa Healthcare; 2007:65–100.

Zeitler, PS, Meacham, LR, Allen, DB, et al, Principles and Practice of Pediatric Endocrinology. Springfield (IL): Charles C Thomas; 2005;857–910.