Advertisement

European Journal of Pediatrics

, Volume 148, Issue 3, pp 220–227 | Cite as

Noonan syndrome: growth and clinical manifestations in 144 cases

  • M. B. Ranke
  • P. Heidemann
  • C. Knupfer
  • H. Enders
  • A. A. Schmaltz
  • J. R. Bierich
Medical Genetics

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.

Key words

Noonan syndrome Growth Ullrich-Turner syndrome 

Abbreviation

UTS

Ullrich-Turner syndrome

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Allanson JE (1987) Noonan syndrome. J Med Genet 24:9–13Google Scholar
  2. 2.
    Allanson JE, Hall JG, Hughes HE, Preus M, Witt RD (1985) Noonan syndrome: the changing phenotype. Am J Med Genet 21:507–514Google Scholar
  3. 3.
    Blieden LC, Schneeweis A, Shem-Tov A, Feigel A, Neufeld HN (1983) Unifying link between Noonan's and Leopard syndromes? Pediatr Cardiol 4:168–169Google Scholar
  4. 4.
    Brandt I (1979) Perzentilenkurven für die Gewichtsentwicklung. Bei Früh- und Reifgeborenen in den ersten fünf Jahren. Kinderarzt 10:713–718Google Scholar
  5. 5.
    Brandt I (1980) Perzentilenkurven für das Längenwachstum. Bei Früh- und Reifgeborenen in den ersten fünf Jahren. Kinderarzt 11:43–51Google Scholar
  6. 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 8:110–118Google Scholar
  7. 7.
    Collins E, Turner G (1973) The Noonan syndrome—a review of the clinical and genetic features of 27 cases. J Pediatr 83:941–950Google Scholar
  8. 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 10:37–50Google Scholar
  9. 9.
    Elders MY, Char F (1976) Possible etiologic mechanisms of the short stature in the Noonan syndrome. Birth Defects 12:127–133Google Scholar
  10. 10.
    Ferguson-Smith MA (1965) Karyotype-phenotype correlations in gonadal dysgenesis and their bearing on the pathogenesis of malformations. J Med Genet 2:142–156Google Scholar
  11. 11.
    Flavell G (1943) Webbing of neck with Turner's syndrome in the male. Br J Surg 31:150–153Google Scholar
  12. 12.
    Greulich W, Pyle S (1969) Radiographic atlas of skeletal development of the hand and wrist, 2nd edn. Stanford University Press, Stanford, CalifGoogle Scholar
  13. 13.
    Grumbach MM, Van Wyk JJ (1974) Disorders of sex differentiation. In: Williams RH (ed) Textbook of endocrinology. Saunders, Philadelphia, Pa, pp 423–501Google Scholar
  14. 14.
    Lippe BM (1982) Primary ovarian failure. In: Kaplan SA (ed) Clinical pediatric and adolescent endocrinology. Saunders, Philadelphia, Pa, pp 269–299Google Scholar
  15. 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] 184:48Google Scholar
  16. 16.
    Mendez HMM, Opitz JM (1985) Noonan syndrome: a review. Am J Med Genet 21:493–506Google Scholar
  17. 17.
    Money J, Kalus ME (1979) Noonan's syndrome IQ and specific disabilities. Am J Dis Child 133:846–850Google Scholar
  18. 18.
    Noonan JA (1968) Hypertelorism with Turner phenotype. Am J Dis Child 116:373–380Google Scholar
  19. 19.
    Noonan JA, Ehmke DA (1963) Associated noncardiac malformations in children with congenital heart disease. J Pediatr 63:468–470Google Scholar
  20. 20.
    Nora JJ, Sinha AK (1968) Direct familial transmission of the Turner phenotype. Am J Dis Child 116:343–350Google Scholar
  21. 21.
    Nora JJ, Nora AH, Sinha AK, Spangler RD, Lubs HA (1974) The Ullrich-Noonan syndrome (Turner phenotype). Am J Dis Child 127:48–55Google Scholar
  22. 22.
    Opitz JM, Pallister DP (1979) Brief historical note: the concept of “gonadal dysgenesis”. Am J Med Genet 4:333–334Google Scholar
  23. 23.
    Opitz JM, Summitt RL, Sarto GE (1965) Noonan's syndrome in girls: a genocopy of the Ullrich-Turner syndrome. J Pediatr 67: 968Google Scholar
  24. 24.
    Pearl W (1977) Cardiovascular anomalies in Noonan's syndrome. Chest 71:677–679Google Scholar
  25. 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] 45:4–45Google Scholar
  26. 26.
    Ranke MB (1985) Spontanes Wachstum beim Turner Syndrom. Normalwerte und Somatogramme nach dem 2. Lebensjahr. Kinderarzt 16:1205–1208Google Scholar
  27. 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 141:81–88Google Scholar
  28. 28.
    Rohn RD (1983) Short stature and dysmorphism in the child and adolescent female. Diagnostic dilemmas. Clin Pediatr 22:741–746Google Scholar
  29. 29.
    Summit RL (1969) Turner syndrome and Noonan's syndrome. J Pediatr 74:155–156Google Scholar
  30. 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, 613–635Google Scholar
  31. 31.
    Turner HH (1938) A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology 23:566–574Google Scholar
  32. 32.
    Ullrich O (1930) Über typische Kombinationsbilder multipler Abartung. Z Kinderheilkd 49:271–276Google Scholar
  33. 33.
    Witt DR, Keena BA, Hall JG, Allanson JE (1986) Growth curves for height in Noonan syndrome. Clin Genet 30:150–153Google Scholar

Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • M. B. Ranke
    • 1
  • P. Heidemann
    • 2
  • C. Knupfer
    • 1
  • H. Enders
    • 3
  • A. A. Schmaltz
    • 1
  • J. R. Bierich
    • 1
  1. 1.Universitäts-KinderklinikTübingenFederal Republic of Germany
  2. 2.Universitäts-KinderklinikGöttingenFederal Republic of Germany
  3. 3.Institut für Anthropologie und HumangenetikTübingenFederal Republic of Germany

Personalised recommendations