Puberty in Girls Followed for Cystic Fibrosis
Puberty in Girls Followed for Cystic Fibrosis
In the general population, age of puberty, and age of menarche in girls, are closely
 correlated with those of their mother. The severity of chronic disease has an impact on the
 stature, the weight gain and the onset of puberty: age of menarche is even later that chronic
 illness is serious. Cystic fibrosis is one of the major chronic diseases of children.

 Neonatal screening is organized since 2002, most girls with cystic fibrosis therefore
 currently have a very specialized and early care. Older patients were diagnosed during
 childhood. The optimization of the nutritional and respiratory management has allowed over
 the past two decades a significant improvement in the survival of these patients. Most young
 people reach adulthood, puberty and reproductive desire are a crucial issue in the care of
 these young adults.

 In literature, it is often noted an age of menarche delayed an average of 2 years compared to
 the general population, but correlated with the age of menstruation from the mothers.

 The factors involved in the conduct of cycles in the girl followed for cystic fibrosis are
 poorly known and there are few data on the characteristics of cycles. The menstrual
 irregularity and amenorrhoea episodes concern nearly half of women.

 On the pathophysiological level, studies in animals show that there is a direct effect of
 CFTR (Cystic Fibrosis Transmembrane Regulator) on the hypothalamic pituitary gonadal and
 reproductive capacity in mice.

 The literature data on the pubertal development, fertility and hormonal profile of these
 girls are for studies with low numbers and are very old. There is to date no French cohort
 study on this topic.

 The study is a multicenter cross-sectional descriptive study for pubertal and gynecological
 characteristics in girls followed annually in the reference centers Cystic Fibrosis two
 inter-region.
Inclusion Criteria:

 -  Patient diagnosed with cystic fibrosis

 -  Girl aged 8 years of age, adolescents and young adults, had period or not,

 -  visit the Reference and Competence Center of Cystic Fibrosis.

 -  Affiliated to a social security scheme

 Exclusion Criteria:

 -  Lack of consent of the legal representative or the relevant patient.

 -  Patient majoring in legal disability or minor patient whose legal representative is
 legal disability