Clinical trial NCT02562911: Puberty in Girls Followed for Cystic Fibrosis
Official title: Epidemiological Study of the Progress of Puberty in Girls Followed for Cystic Fibrosis
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Cystic Fibrosis
Keywords: cystic fibrosis
menstrual cycle
puberty
Lead sponsor: University Hospital, Toulouse
Sponsor class: OTHER
First posted: 2015-09-29
Enrollment: 166
Sex: FEMALE
Minimum age (years): 8
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Girl aged 8 years of age, adolescents and young adults, had period or not, visit the Reference and Competence Center of Cystic Fibrosis.

Brief summary: 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.


Eligibility criteria: 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

Primary outcomes:
measure: The age of menarche in girls followed for cystic fibrosis in France
timeFrame: Day 0
Secondary outcomes:
Other outcomes:
Locations:
city: Besancon
country: France
facility: CHU Besançon St Jacques
city: Bordeaux
country: France
facility: Hôpital des Enfants-Pellegrin
city: Dijon
country: France
facility: Hôpital d'Enfants
city: Nancy
country: France
facility: Canton- Hôpitaux de Brabois
city: Reims
country: France
facility: American Memorial Hospital
city: Strasbourg
country: France
facility: Hôpital de Hautepierre
city: Toulouse
country: France
facility: Hôpital des enfants Purpan
Study officials:
affiliation: University Hospital, Toulouse
name: Catherine PIENKOWSKI, MD
role: PRINCIPAL_INVESTIGATOR
References: