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Noonan syndrome: growth and clinical manifestations in 144 cases

  • Medical Genetics
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Abstract

We have analysed growth and the major clinical manifestations of 144 patients (89 males, 55 females) with Noonan syndrome from two West German centres. Size at birth was normal in both sexes. In both males and females, the mean height followed along the 3rd per centile until puberty, but decreased transiently due to an approximately 2 year delay in onset of puberty. Final height approaches the lower limits of normal at the end of the 2nd decade of life. The mean adult height was found to be (n=20) 162.5 cm in males and (n=13) 152.7 cm in females, respectively. Smoothed means and standard deviations for height were derived. These data may be used for the statistical evaluation of height of Noonan syndrome patients. Except for mental retardation and microcephaly, which are more frequent in males, the relative frequencies of minor anomalies and malformations were found to be similar in both sexes. The characteristic non-cyanotic heart defects in the Noonan Syndrome do not appear to have a major influence on growth. The auxological data were compared with those in the Ullrich-Turner syndrome.

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Abbreviations

UTS:

Ullrich-Turner syndrome

References

  1. Allanson JE (1987) Noonan syndrome. J Med Genet 24:9–13

    Google Scholar 

  2. Allanson JE, Hall JG, Hughes HE, Preus M, Witt RD (1985) Noonan syndrome: the changing phenotype. Am J Med Genet 21:507–514

    Google Scholar 

  3. Blieden LC, Schneeweis A, Shem-Tov A, Feigel A, Neufeld HN (1983) Unifying link between Noonan's and Leopard syndromes? Pediatr Cardiol 4:168–169

    Google Scholar 

  4. Brandt I (1979) Perzentilenkurven für die Gewichtsentwicklung. Bei Früh- und Reifgeborenen in den ersten fünf Jahren. Kinderarzt 10:713–718

    Google Scholar 

  5. Brandt I (1980) Perzentilenkurven für das Längenwachstum. Bei Früh- und Reifgeborenen in den ersten fünf Jahren. Kinderarzt 11:43–51

    Google Scholar 

  6. Char FC, Rodriguez-Fernandez HL, Scott C, Borgaonkar DS, Bell BB, Rowe RD (1972) The Noonan syndrome—a clinical study of forty five cases. Birth Defects 8:110–118

    Google Scholar 

  7. Collins E, Turner G (1973) The Noonan syndrome—a review of the clinical and genetic features of 27 cases. J Pediatr 83:941–950

    Google Scholar 

  8. Duncan WJ, Fowler RS, Farcas LG, Ross RB, Wright AW, Bloom KR, Huot DJ, Sondheimer HM, Rowe DR (1981) A comprehensive scoring system for evaluating Noonan syndrome. Am J Med Genet 10:37–50

    Google Scholar 

  9. Elders MY, Char F (1976) Possible etiologic mechanisms of the short stature in the Noonan syndrome. Birth Defects 12:127–133

    Google Scholar 

  10. Ferguson-Smith MA (1965) Karyotype-phenotype correlations in gonadal dysgenesis and their bearing on the pathogenesis of malformations. J Med Genet 2:142–156

    Google Scholar 

  11. Flavell G (1943) Webbing of neck with Turner's syndrome in the male. Br J Surg 31:150–153

    Google Scholar 

  12. Greulich W, Pyle S (1969) Radiographic atlas of skeletal development of the hand and wrist, 2nd edn. Stanford University Press, Stanford, Calif

    Google Scholar 

  13. Grumbach MM, Van Wyk JJ (1974) Disorders of sex differentiation. In: Williams RH (ed) Textbook of endocrinology. Saunders, Philadelphia, Pa, pp 423–501

    Google Scholar 

  14. Lippe BM (1982) Primary ovarian failure. In: Kaplan SA (ed) Clinical pediatric and adolescent endocrinology. Saunders, Philadelphia, Pa, pp 269–299

    Google Scholar 

  15. Majewski F, Bierich JR, Barz M, Haberland WF, Stockenius M (1974) On the frequency of malformations in the different forms of Turner's syndrome. Acta Endocrinol (Copenh) [Suppl] 184:48

    Google Scholar 

  16. Mendez HMM, Opitz JM (1985) Noonan syndrome: a review. Am J Med Genet 21:493–506

    Google Scholar 

  17. Money J, Kalus ME (1979) Noonan's syndrome IQ and specific disabilities. Am J Dis Child 133:846–850

    Google Scholar 

  18. Noonan JA (1968) Hypertelorism with Turner phenotype. Am J Dis Child 116:373–380

    Google Scholar 

  19. Noonan JA, Ehmke DA (1963) Associated noncardiac malformations in children with congenital heart disease. J Pediatr 63:468–470

    Google Scholar 

  20. Nora JJ, Sinha AK (1968) Direct familial transmission of the Turner phenotype. Am J Dis Child 116:343–350

    Google Scholar 

  21. Nora JJ, Nora AH, Sinha AK, Spangler RD, Lubs HA (1974) The Ullrich-Noonan syndrome (Turner phenotype). Am J Dis Child 127:48–55

    Google Scholar 

  22. Opitz JM, Pallister DP (1979) Brief historical note: the concept of “gonadal dysgenesis”. Am J Med Genet 4:333–334

    Google Scholar 

  23. Opitz JM, Summitt RL, Sarto GE (1965) Noonan's syndrome in girls: a genocopy of the Ullrich-Turner syndrome. J Pediatr 67: 968

    Google Scholar 

  24. Pearl W (1977) Cardiovascular anomalies in Noonan's syndrome. Chest 71:677–679

    Google Scholar 

  25. Prader A, Issler RH, Molinari L, Largo RH (1980) Physical growth in Swiss children from birth to 20 years of age. Helv Paediatr Acta [Suppl] 45:4–45

    Google Scholar 

  26. Ranke MB (1985) Spontanes Wachstum beim Turner Syndrom. Normalwerte und Somatogramme nach dem 2. Lebensjahr. Kinderarzt 16:1205–1208

    Google Scholar 

  27. Ranke MB, Pflüger H, Rosendahl W, Stubbe P, Enders H, Bierich JR, Majewski F (1983) Turner syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediatr 141:81–88

    Google Scholar 

  28. Rohn RD (1983) Short stature and dysmorphism in the child and adolescent female. Diagnostic dilemmas. Clin Pediatr 22:741–746

    Google Scholar 

  29. Summit RL (1969) Turner syndrome and Noonan's syndrome. J Pediatr 74:155–156

    Google Scholar 

  30. Tanner JM, Whitehouse RH, Takaishi M (1966) Standards from birth to maturity for height, weight velocity and weight velocity. British children. Arch Dis Child 41:454–471, 613–635

    Google Scholar 

  31. Turner HH (1938) A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology 23:566–574

    Google Scholar 

  32. Ullrich O (1930) Über typische Kombinationsbilder multipler Abartung. Z Kinderheilkd 49:271–276

    Google Scholar 

  33. Witt DR, Keena BA, Hall JG, Allanson JE (1986) Growth curves for height in Noonan syndrome. Clin Genet 30:150–153

    Google Scholar 

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Ranke, M.B., Heidemann, P., Knupfer, C. et al. Noonan syndrome: growth and clinical manifestations in 144 cases. Eur J Pediatr 148, 220–227 (1988). https://doi.org/10.1007/BF00441408

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