Literature DB >> 30939478

Treatment with Growth Hormone in Noonan Syndrome Observed during 25 Years of KIGS: Near Adult Height and Outcome Prediction.

Michael B Ranke1, Anders Lindberg2, Martin Carlsson3, Cecilia Camacho-Hübner3, Raoul Rooman4.   

Abstract

BACKGROUND/AIMS: There is little information how rhGH treatment affects height in NS. This study aims to analyze data from the NS patients assembled in KIGS over 25 years. PATIENTS/
METHODS: Of 613 (389 m/224 f) NS patients documented, 476 (302 m/174 f) were treated for 1 year, 237 (160 m/77 f) of which served to develop a 1st year height velocity (HV) prediction algorithm. One-hundred and forty (74 m/66 f) had reached near adult height (NAH). Factors affecting NAH on rhGH were determined.
RESULTS: At the start of rhGH, the NAH groups were (median, m, f) 11.0 and 10.3 years, with a height SDS of -3.2 and -3.8 SDS (Prader), respectively. The total gain after 6.3 and 5.6 years on rhGH (0.27 and 0.30 mg/kg/week) was 1.2 and 1.3 SDS. Age at the start of rhGH (negative), height at the start of rhGH, rhGH dose, number of rhGH injections/wk and birth weight (all positive) explained 36% of the variability of 1st year HV. Height at the start of rhGH, 1st year growth on rhGH, birth weight, and gender explained 74% of the variability of NAH. Causes for rhGH treatment discontinuation and adverse events were also analyzed.
CONCLUSION: rhGH treatment increases NAH in NS. Prediction algorithms may optimize treatment in the future.
© 2019 S. Karger AG, Basel.

Entities:  

Keywords:  1st year growth prediction; GH treatment; Near adult height (NAH); Noonan syndrome

Mesh:

Substances:

Year:  2019        PMID: 30939478     DOI: 10.1159/000498859

Source DB:  PubMed          Journal:  Horm Res Paediatr        ISSN: 1663-2818            Impact factor:   2.852


  6 in total

Review 1.  A PTPN11 mutation in a woman with Noonan syndrome and protein-losing enteropathy.

Authors:  Na Wang; Wen Shi; Yang Jiao
Journal:  BMC Gastroenterol       Date:  2020-02-13       Impact factor: 3.067

2.  Long-Term Safety of Growth Hormone Treatment in Childhood: Two Large Observational Studies: NordiNet IOS and ANSWER.

Authors:  Lars Sävendahl; Michel Polak; Philippe Backeljauw; Joanne C Blair; Bradley S Miller; Tilman R Rohrer; Anita Hokken-Koelega; Alberto Pietropoli; Nicky Kelepouris; Judith Ross
Journal:  J Clin Endocrinol Metab       Date:  2021-05-13       Impact factor: 5.958

3.  Growth in Children With Noonan Syndrome and Effects of Growth Hormone Treatment on Adult Height.

Authors:  Annachiara Libraro; Vito D'Ascanio; Marco Cappa; Mariangela Chiarito; Maria Cristina Digilio; Silvia Einaudi; Anna Grandone; Mohamad Maghnie; Laura Mazzanti; Alessandro Mussa; Giuseppa Patti; Emanuela Scarano; Antonietta Spinuzza; Silvia Vannelli; Malgorzata Gabriela Wasniewska; Giovanni Battista Ferrero; Maria Felicia Faienza
Journal:  Front Endocrinol (Lausanne)       Date:  2021-12-22       Impact factor: 5.555

4.  Cardiovascular safety of growth hormone treatment in Noonan syndrome: real-world evidence.

Authors:  Alicia Romano; Juan Pablo Kaski; Jovanna Dahlgren; Nicky Kelepouris; Alberto Pietropoli; Tilman R Rohrer; Michel Polak
Journal:  Endocr Connect       Date:  2022-01-31       Impact factor: 3.335

Review 5.  Inside the Noonan "universe": Literature review on growth, GH/IGF axis and rhGH treatment: Facts and concerns.

Authors:  Stefano Stagi; Vittorio Ferrari; Marta Ferrari; Manuela Priolo; Marco Tartaglia
Journal:  Front Endocrinol (Lausanne)       Date:  2022-08-18       Impact factor: 6.055

Review 6.  Etiology and Treatment of Growth Delay in Noonan Syndrome.

Authors:  Fernando Rodríguez; Ximena Gaete; Fernando Cassorla
Journal:  Front Endocrinol (Lausanne)       Date:  2021-06-04       Impact factor: 5.555

  6 in total

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