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Frequency of Delayed Puberty in Boys with Contemporary Management of Duchenne Muscular Dystrophy.
McCarrison, Sarah; Denker, Melissa; Dunne, Jennifer; Horrocks, Iain; McNeilly, Jane; Joseph, Shuko; Wong, Sze Choong.
Afiliación
  • McCarrison S; Department of Paediatric Endocrinology, Royal Hospital for Children, Glasgow.
  • Denker M; Department of Paediatric Endocrinology, Royal Hospital for Children, Glasgow.
  • Dunne J; Department of Paediatric Neurology, Royal Hospital for Children, Glasgow.
  • Horrocks I; Department of Paediatric Neurology, Royal Hospital for Children, Glasgow.
  • McNeilly J; Department of Biochemistry, Queen Elizabeth University Hospital, Glasgow, United Kingdom.
  • Joseph S; Department of Paediatric Neurology, Royal Hospital for Children, Glasgow.
  • Wong SC; Department of Paediatric Endocrinology, Royal Hospital for Children, Glasgow.
J Clin Res Pediatr Endocrinol ; 2024 Jun 25.
Article en En | MEDLINE | ID: mdl-38915199
ABSTRACT

Background:

Delayed puberty is thought to be common in boys with Duchenne muscular dystrophy (DMD) treated with long term oral glucocorticoid. This study aims to report the frequency of delayed puberty in DMD from examination by a paediatric endocrinologist alongside detailed endocrine investigations.

Methods:

All boys with DMD aged at least 14 years in January 2022 known to the paediatric neuromuscular service (2016-2022) were included in this study. Delayed puberty was defined based on testicular volume and genital staging in comparison to published puberty nomogram.

Results:

Twenty-four out of 37 boys (65%) had evidence of delayed puberty, 23/24 (96%) of those with delayed puberty were on glucocorticoid therapy all of whom were on daily glucocorticoid. On the other hand, 7/13 (54%) of those with normal timing of puberty were on glucocorticoid; 2/7 (29%) were on the intermittent regimen. Of those who were on daily glucocorticoid therapy at the time of assessment of puberty, 23/28 (82%) had evidence of delayed puberty. In boys with delayed puberty, endocrine investigations showed low luteinizing hormone (LH) with undetectable testosterone levels, a pre-pubertal response with lutenizing hormone releasing hormone test and sub-optimal testosterone levels with prolonged human chorionic gonadotropin stimulation.

Conclusion:

The frequency of delayed puberty in boys with DMD was 65%. Eighty-two percent of adolescent boys with DMD on daily glucocorticoid had evidence of delayed puberty. Biochemical investigations point to functional central hypogonadism in these adolescents. Our data supports the routine monitoring of puberty in boys with DMD.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Clin Res Pediatr Endocrinol Año: 2024 Tipo del documento: Article Pais de publicación: Turquía
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Clin Res Pediatr Endocrinol Año: 2024 Tipo del documento: Article Pais de publicación: Turquía