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Pubertal development in cystic fibrosis: an overview

Teresa Arrigo1, I Rulli, C Sferlazzas

  • 1Department of Medical and Surgical Pediatrics, University of Messina, Italy. tarrigo@unime.it

Journal of Pediatric Endocrinology & Metabolism : JPEM
|May 6, 2003
PubMed

Insights

Cystic fibrosis (CF) patients experience delayed puberty and growth, impacting reproductive health and hormone levels. This maturational lag affects insulin-like growth factor-I and gonadotropins, despite improved treatments.

Area of Science:

  • Pediatric Endocrinology
  • Genetics and Inherited Diseases
  • Pulmonology

Background:

  • Cystic fibrosis (CF) is an autosomal recessive disorder causing respiratory and intestinal issues.
  • Patients with CF often exhibit delayed growth, puberty, and menarche.
  • Infertility and menstrual dysfunction are prevalent in CF patients.

Purpose of the Study:

  • To investigate the maturational lag in cystic fibrosis patients.
  • To examine the correlation between growth failure and pulmonary disease severity.
  • To assess pubertal hormone levels in CF patients.

Main Methods:

  • Review of existing literature on CF growth and development.
  • Analysis of correlations between malnutrition, pulmonary disease, and growth.
  • Assessment of pubertal hormone levels including IGF-I, LH, FSH, and sex steroids.

Main Results:

  • CF patients show delayed puberty and growth spurts, even with enhanced nutrition and treatment.
  • A significant delay in achieving pubertal levels of IGF-I, LH, FSH, and sex steroids was observed.
  • Growth failure in CF is linked to malnutrition and the severity of lung disease.

Conclusions:

  • Cystic fibrosis leads to a distinct maturational lag affecting pubertal development.
  • Hormonal imbalances, including delayed gonadotropin and sex steroid levels, characterize this lag.
  • Intensive treatment and nutrition do not fully overcome the pubertal delay in CF.

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