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Arch Dis Child. 1982 January; 57(1): 13–17.
PMCID: PMC2863270

Growth and pubertal development in five boys with Noonan's syndrome.

Abstract

Five boys, each with at least 4 features characteristic of Noonan's syndrome, were studied throughout childhood. In each the height was on or below the 3rd centile. Height velocity was essentially normal during the prepubertal period whereas bone age and pubertal development were delayed. Four boys had cryptorchidism and in 2 no testicular enlargement or spontaneous puberty occurred, necessitating testosterone replacement. Plasma concentrations of luteinising hormone and follicle-stimulating hormone before and after stimulation with luteinising hormone-releasing hormone were increased in 4 of the boys. These findings are consistent with primary gonadal failure associated with impaired spermatogenesis.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • HELLER RH. THE TURNER PHENOTYPE IN THE MALE. J Pediatr. 1965 Jan;66:48–63. [PubMed]
  • Nora JJ, Nora AH, Sinha AK, Spangler RD, Lubs HA. The Ullrich-Noonan syndrome (Turner phenotype). Am J Dis Child. 1974 Jan;127(1):48–55. [PubMed]
  • Bolton MR, Pugh DM, Mattioli LF, Dunn MI, Schimke RN. The Noonan syndrome: a family study. Ann Intern Med. 1974 May;80(5):626–629. [PubMed]
  • Saez JM, Morera AM, Bertrand J. Testicular endocrine function in males with Nooman's syndrome. Lancet. 1969 Nov 15;2(7629):1078–1079. [PubMed]
  • Miller M, Motulsky AC. Noonan syndrome in an adult family presenting with chronic lymphedema. Am J Med. 1978 Aug;65(2):379–383. [PubMed]
  • Grant DB, Laurance BM, Atherden SM, Ryness J. HCG stimulation test in children with abnormal sexual development. Arch Dis Child. 1976 Aug;51(8):596–601. [PMC free article] [PubMed]
  • Savage MO, Preece MA, Cameron N, Jones J, Theintz G, Penfold JL, Tanner JM. Gonadotrophin response to LH-RH in boys with delayed growth and adolescence. Arch Dis Child. 1981 Jul;56(7):552–556. [PMC free article] [PubMed]
  • Tanner JM, Whitehouse RH. Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty. Arch Dis Child. 1976 Mar;51(3):170–179. [PMC free article] [PubMed]
  • Wilroy RS, Jr, Summitt RL, Tipton RE, Primm PA, Martens PR. Phenotypic heterogeneity in the Noonan syndrome. Birth Defects Orig Artic Ser. 1979;15(5B):305–311. [PubMed]
  • SCHOEN EJ. DIMINISHED TESTICULAR FUNCTION IN "MALE TURNER'S SYNDROME". J Clin Endocrinol Metab. 1965 Jan;25:101–113. [PubMed]
  • Kauschansky A, Eilam N, Elian E. LH-RH and HCG studies in a Turner phenotype male (Noonan's syndrome). A case report. Helv Paediatr Acta. 1977 Sep;32(3):237–240. [PubMed]
  • Ross JL, Shenkman L. Noonan's syndrome and hypopituitarism. Am J Med Sci. 1980 Jan-Feb;279(1):47–52. [PubMed]
  • Smals AG, Kloppenborg PW, Benraad TJ. The effect of short and long term human chorionic gonadotrophin (HCG) administration on plasma testosterone levels in Klinefelter's syndrome. Acta Endocrinol (Copenh) 1974 Dec;77(4):753–764. [PubMed]

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