Puberty in cystic fibrosis

Bracha Goldsweig; Beth Kaminski; Aniket Sidhaye; Scott M Blackman; Andrea Kelly

doi:10.1016/j.jcf.2019.08.013

Puberty in cystic fibrosis

J Cyst Fibros. 2019 Oct:18 Suppl 2:S88-S94. doi: 10.1016/j.jcf.2019.08.013.

Authors

Bracha Goldsweig¹, Beth Kaminski², Aniket Sidhaye³, Scott M Blackman⁴, Andrea Kelly⁵

Affiliations

¹ Division of Pediatric Endocrinology, Children's Hospital and Medical Center, 8200 Dodge Street, Omaha, NE 68114, USA. Electronic address: bgoldsweig@childrensomaha.org.
² Department of Pediatrics, Division of Endocrinology, University Hospitals Rainbow Babies and Children's Hospital, 11100 Euclid Avenue, Cleveland, OH 44106, USA. Electronic address: beth.kaminski@uhhospitals.org.
³ Division of Endocrinology, Diabetes and Metabolism, Johns Hopkins University School of Medicine, 1830 E. Monument Street, Suite 333, Baltimore, MD 21287, USA. Electronic address: asidhay1@jhmi.edu.
⁴ Division of Pediatric Endocrinology, Johns Hopkins University School of Medicine, 200 N Wolfe Street, Baltimore, MD 21287, USA. Electronic address: sblackman@jhmi.edu.
⁵ Division of Pediatric Endocrinology and Diabetes, Children's Hospital of Philadelphia, University of Pennsylvania, Perelman School of Medicine, 2716 South Street, Room 14363, Philadelphia, PA 19146, USA. Electronic address: kellya@email.chop.edu.

PMID: 31679734
DOI: 10.1016/j.jcf.2019.08.013

Abstract

Historically, delayed puberty was considered a common clinical feature of cystic fibrosis (CF). More recent reports have documented normal pubertal progression in the majority of individuals with CF. However, youth with more severe disease are still at risk for delayed puberty. Careful evaluation of pubertal development in children and adolescents with CF is important as pubertal timing impacts linear growth, bone mineral accrual, body image and psychosocial wellbeing, all of which can also be impacted directly by CF. This article reviews the physiology of puberty, timing of puberty in CF, evaluation of pubertal development, and the differential diagnosis, evaluation, and management of delayed and precocious puberty in people with CF.

Keywords: Cystic fibrosis; Delayed puberty; Growth; Puberty.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Adolescent
Child
Cystic Fibrosis / complications*
Cystic Fibrosis / physiopathology*
Diagnosis, Differential
Female
Humans
Male
Puberty / physiology*
Puberty, Delayed / diagnosis
Puberty, Delayed / etiology*
Puberty, Delayed / therapy