[Male pseudo-hermaphroditism. Prenatal factors].
The authors report on nine cases of male pseudo-hermaphroditism (MPH) diagnosed at birth. They had a karyotype 46 XY and their external genital organs were ambiguous. Analysing the conditions under which the pregnancies occurred we found that there was primary infertility before the pregnancy in 4 out of 9 cases. There was raised maternal blood pressure in 3 out of 9 cases and one of them led to eclampsia. Three babies were preterm and 7 showed intra-uterine growth retardation (below the 10th centile). In 3 cases there were other birth defects and difficulties in the neonatal stage in 6 (2 with respiratory distress, 1 with birth asphyxia, 1 with infection and 2 with jaundice). The diagnosis was based on cytogenetic analysis, x-ray of the urinary tract, x-ray of the genital organs, plasma testosterone levels and the binding capacity of dihydrotestosterone receptors on fibroblasts of the sexual skin areas (fentomoles/mg DNA). The partial androgen insensitivity syndrome was proven in two cases. These mothers had taken drugs that could potentially cause external genital ambiguity (demegestone and chlorpromazine). In 7 cases the diagnosis could not be arrived at. It is known to be very difficult to find an aetiology for MPH but the abnormalities of pregnancy and the frequent occurrence of intra-uterine growth retardation suggest that there are metabolic and endocrine upsets so that fetal malnutrition could play a role in poor external genitalia development. It should be possible to set up a large multicentric study in order to acquire more data and work out the factors that lead to MPH and their relationship to one another.
Aguilar-Martinez
P
P
Service de néonatologie, Hôpital Saint-Charles, Montpellier.
Montoya
F
F
Montoya
P
P
Laffargue
F
F
Viala
J L
JL
D000328:Adult; D000013:Congenital Abnormalities; D012734:Disorders of Sex Development / Q000209:etiology* / Q000235:genetics; D005260:Female; D005315:Fetal Diseases; D005317:Fetal Growth Retardation / Q000150:complications; D006801:Humans; D007231:Infant, Newborn; D007247:Infertility, Female / Q000150:complications; D007621:Karyotyping; D008297:Male; D011225:Pre-Eclampsia / Q000150:complications; D011247:Pregnancy; D011248:Pregnancy Complications; D012189:Retrospective Studies
Pseudo-hermaphrodismes masculins. Facteurs anténatals.