Puberty in Girls Followed for Cystic Fibrosis

NCT ID: NCT02562911

Last Updated: 2017-03-14

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 166 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2013-01-31
• Study Completion Date: 2015-12-31

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.

Conditions

Cystic Fibrosis

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Interventions

Data collection

• Clinical examination
• Complete pelvic ultrasound abdominal ultrasound
• Bone age: X-ray
• biological examination
• Lung function assessed by the Maximum Volume Expired
• Brasfield radiological score (if available)

Intervention Type: PROCEDURE

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

• Minimum Eligible Age: 8 Years
• Eligible Sex: FEMALE
• Accepts Healthy Volunteers: No

Sponsors

University Hospital, Toulouse

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Catherine PIENKOWSKI, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital, Toulouse

Locations

CHU Besançon St Jacques

Besançon, , France

Hôpital des Enfants-Pellegrin

Bordeaux, , France

Hôpital d'Enfants

Dijon, , France

Canton- Hôpitaux de Brabois

Nancy, , France

American Memorial Hospital

Reims, , France

Hôpital de Hautepierre

Strasbourg, , France

Hôpital des enfants Purpan

Toulouse, , France

Countries

France

Other Identifiers

12 485 03

Identifier Type: -

Identifier Source: org_study_id