Cystic Fibrosis Clinical Trial
Official title:
Epidemiological Study of the Progress of 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.
n/a
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