Growth hormone treatment in Noonan syndrome increases growth velocity significantly during the first 2 years of treatment and, to some extent, until puberty. This increase is more pronounced if treatment is started at an early age. Treatment before the age of 5 years is not recommended due to an increased risk of malignancies. In contrast to other growth hormone-treated patients, a slight but significant further increase in height gain can be expected during pubertal growth (at least in boys). Final height improvement varies between 1 and 2 SDS in different studies. Cardiac function does not seem to be impaired during treatment. No significant adverse events have been reported.

1.
Noonan JA, Ehmke DA: Associated noncardiac malformations in children with congenital heart disease. J Pediatr 1963;63:468–469.
2.
Allanson JE: Noonan syndrome. J Med Genet 1987;24:9–13.
3.
Ranke MB, Heidemann P, Knupfer C, Enders H, Schmaltz AA, Bierich JR: Noonan syndrome: growth and clinical manifestations in 144 cases. Eur J Pediatr 1988;148:220–227.
4.
Romano AA, Blethen SL, Dana K, Noto RA: Growth hormone treatment in Noonan syndrome: the National Cooperative Growth Study experience. J Pediatr 1996;128:18–21.
5.
MacFarlane CE, Brown CD, Johnston LB, Patton MA, Dunger DB, et al: Growth hormone therapy and growth in children with Noonan’s syndrome: results of 3 years’ follow up. J Clin Endocrinol Metab 2001;86:1953–1956.
6.
Kirk JM, Betts PR, Butler GE, Donaldson MD, Dunger DB, et al: Short stature in Noonan syndrome: response to growth hormone therapy. Arch Dis Child 2001;84:440–443.
7.
Noordam C, Van der Burgt I, Sengers RC, Delemarre-van de Waal HA, Otten BJ: Growth hormone treatment in children with Noonan’s syndrome: four year results of a partly controlled trial. Acta Paediatr 2001;90:889–894.
8.
Osio D, Dahlgren J, Wikland KA, Westphal O: Improved final height with long-term growth hormone treatment in Noonan syndrome. Acta Paediatr 2005;94:1232–1237.
9.
Raaijmakers R, Noordam C, Karagiannis G, Gregory JW, Hertel NT, et al: Response to growth hormone treatment and final height in Noonan syndrome in a large cohort of patients in the KIGS database. J Pediatr Endocrinol Metab 2008;21:267–273.
10.
Noordam C, Peer PG, Francois I, De Schepper J, van der Burgt I, Otten BJ: Long-term GH treatment improves adult height in children with Noonan syndrome with and without mutations in protein tyrosine phosphatase, non-receptor-type 11. Eur J Endocrinol 2008;159:203–208.
11.
Tartaglia M, Mehler EL, Goldberg R, Zampino G, Brunner HG, et al: Mutations in PTPN11 encoding the protein tyrosine phosphatase SHP-2, cause Noonan syndrome. Nat Genet 2001;29:465–468.
12.
Schubbert S, Zenker M, Rowe SL, Boll S, Klein C, Bollag I, et al: Germline KRAS mutation causes Noonan syndrome. Nat Genet 2006;38:331–336.
13.
Roberts AE, Araki T, Swanson KD, Montgomery KT, Schiripo TA, et al: Germline gain-of-function mutations in SOS1 cause Noonan syndrome. Nat Genet 2007;39:70–74.
14.
Razzaque MA, Nishizawa T, Komoike Y, Yagi H, Furutani M, et al: Germline gain-of-function mutation in RAF1 cause Noonan syndrome. Nat Genet 2007;39:1013–1017.
15.
Nava C, Hanna N, Michot C, Pereira S, Pouvreau N, Niihort T, et al: Cardio-facio-cutaneous and Noonan syndromes due to mutations in the RAS/MAPK signalling pathway: genotype-phenotype relationships and overlap with Costello syndrome. J Med Genet 2007;44:763–771.
16.
Binder G, Neuer K, Ranke MB, Wittekindt NE: PTPN11 mutations are associated with mild GH resistance in individuals with Noonan syndrome. J Clin Endocrinol Metab 2005;90:5377–5381.
17.
Ferreira LV, Souza SA, Arnhold IJ, Mendonca BB, Jorge AA: PTPN11 (protein tyrosine phosphatise nonreceptor type 11) mutations and response to growth hormone therapy in children with Noonan syndrome. J Clin Endocrinol Metab 2005;90:5156–5160.
18.
Limal JM, Parfait B, Cabrol S, Bonnet D, Leheup B, Lyonnet S, et al: Noonan syndrome: relationships between genotype, growth and growth factors. J Clin Endocrinol Metab 2006;91:300–306.
19.
van der Burgt I, Berends E, Lommen E, van Beersum S, Hamel B, et al: Clinical and molecular studies in a large Dutch family with Noonan syndrome. Am J Med Genet 1994;53:187–191.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.