Literature DB >> 16291362

Growth-reductive therapy in children with marfan syndrome.

L Rozendaal1, S le Cessie, J M Wit, R C M Hennekam.   

Abstract

OBJECTIVES: To determine the accuracy and precision of 2 height-prediction methods in Marfan syndrome and to assess the growth-reductive effect and side effects of sex hormone treatment. STUDY
DESIGN: In a retrospective study in 31 untreated (17 boys) and 43 treated patients (21 boys) with Marfan syndrome, we assessed bone age and predicted adult height by 2 methods. The accuracy of the methods was assessed in the untreated group. The effect of therapy was corrected for outcome in the untreated group and other confounding variables with multivariate analysis.
RESULTS: Accuracy strongly varied with sex, chronological age, and prediction method. Overall precision was low. Treatment was started at a mean age of 12.8 +/- 1.4 years (boys) and 11.4 +/- 1.2 years (girls). With multiple regression analysis, a statistically significant effect was observed only in boys using a pharmacologic dosage (5.5 cm, 95% CI 0.96-10.1 cm; P = .02). Side effects were worsening of acne and weight and muscle gain.
CONCLUSIONS: In adolescents with Marfan syndrome, the accuracy and precision of 2 height-prediction methods were limited. The apparent growth-reductive effect of sex hormone treatment appears similar to earlier reports on adolescents with constitutional tall stature. There were no clinically important short-term side effects.

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Year:  2005        PMID: 16291362     DOI: 10.1016/j.jpeds.2005.06.011

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  3 in total

1.  Appropriate Age for Height Control Treatment in Patients With Marfan Syndrome.

Authors:  Sung Eun Kim; Dong-Yun Lee; Min-Sun Kim; Sung Yoon Cho; Dong-Kyu Jin; DooSeok Choi
Journal:  Front Endocrinol (Lausanne)       Date:  2021-10-21       Impact factor: 5.555

2.  Estrogen-mediated Height Control in Girls with Marfan Syndrome.

Authors:  Dong-Yun Lee; Hye Sun Hyun; Rimm Huh; Dong-Kyu Jin; Duk-Kyung Kim; Byung-Koo Yoon; DooSeok Choi
Journal:  J Korean Med Sci       Date:  2016-01-26       Impact factor: 2.153

Review 3.  A scoping review presenting a wide variety of research on paediatric and adolescent patients with Marfan syndrome.

Authors:  Ingeborg Beate Lidal; Trine Bathen; Heidi Johansen; Gry Velvin
Journal:  Acta Paediatr       Date:  2020-02-17       Impact factor: 2.299

  3 in total

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