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Article
ADRENARCHE IN PATIENTS WITH CONGENITAL ADRENAL HYPERPLASIA (CAH) DUE TO 21-HYDROXYLASE DEFICIENCY
In order to evaluate adrenarche in female patients with CAH, plasma concentrations of dehydroepiandrosterone sulfate (DHEAS) were determined (by a specific RIA) longitudinally from 4 to 11 years of age in 16 C...
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Article
PRENATAL TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA (CAH) DUE TO 21-HYDROXYLASE DEFICIENCY: EUROPEAN EXPERIENCE IN 223 PREGNANCIES AT RISK
In the aim to prevent the in utero virilization of CAH affected females a prenatal treatment (Rx) has first been attempted by David and Forest in 1979; later these authors reported their first results and propose...
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Article
PRENATAL DIAGNOSIS OF CONGENITAL ADRENAL HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY
Prenatal diagnosis of the classic forms of the congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency has progressed at the same time as the advances made in the field of both endocrinology and ...
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Article
Prenatal treatment of congenital adrenal hyperplasia: Report of a new case
A mother at risk for 21-hydroxylase deficiency was treated with oral dexamethasone (0.5 mg 12 hourly) from early pregnancy, in an attempt to prevent in utero virilization in case of a female fetus. Fetal karyo...
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Article
124 IN VIVO AND IN VITRO STUDIES OF A JUVENILE GRANULOSA CELL TUMOR
A 16 month-old girl was seen for precocious puberty (breast, pubic hair). Plasma levels (ng/dl) of progesterone (P) (574-1830), 17OH-progesterone (OHP) (181-195) and estradiol (E2) (23-40) were in the range of an...
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Article
9 CRYPTORCHIDISM: COMPARISON OF 2 PROTOCOLS OF TREATMENT BY HUMAN CHORIONIC CONADOTROPIN (hCG)
It is Deneved that the positive action of hCG on cryptorchidism is mediated by the rise in endogenous testosterone (T). Previous studies have shown that plasma T rises steadily for 4-5 days (d.) after a single...
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Article
SEX STEROID BINDING PROTEIN (SBP) IN PREMATURE INFANTS
We have recently reported that SBP is measurable in amniotic fuid: low levels with no sex difference, but showing a significant (p=0.02) correlation (r=0.587) with gestational age (GA). This prompted us to exa...
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Article
DEVELOPMENTAL PATTERN OF PLASMA SEX STEROID BINDING PROTEIN (SBP) IN THE ANDROGEN INSENSITIVITY SYNDROME (AIS): PUBERTAL CHANGES INDEPENDENT OF SEX STEROIDS ?
SBP was measured by a solid phase method in 80 plasma samples from 24 AIS subjects 1 mo-17 yr old. Results (μg/l) were compared to those found in 375 normal males matched for age. Weight was normal for height ...
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Article
SEX HORMONE BINDING PROTEIN (SBP) AS A MARKEh OF ANDROGEN SENSITIVITY IN INFANTS AND CHILDREN
Establishing a reliable test of androgen sensitivity is still prerequisite for etiological diagnosis in infants suspected of androgen insensitivity syndrome (AIS). Since androgens are known to decrease SBP lev...
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Article
BIOACTIVE LUTEOTROPIN (LH) IN URINE: NYCTHEMERAL VARIATION IN BIOLOGICAL POTENCY IN MID-PUBERTY
A previous work (Arch.Fr.Pediatr. 1985,42, 579) has shown the usefulness of immunoassayabled (I) urinary LH levels for assessing gonadotropic function in children and adolescents. To further investigate the physi...
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Article
123 LATE ONSET 21-OH-DEFICIENCY (21-OHD) CAN BE MISDIAGNOSED AS “TYPICAL PREMATURE PUBARCHE” (PP) IN CHILDHOOD
The isolated and early growth of pubic hair (PH) is often associated with slight advance in bone age (BA) and statural age (SA). Diagnosis of PP is classically made on high levels of dehydroepiandrosterone sul...
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Article
124 PRENATAL DIAGNOSIS OF 17-KETOSTEROID REDUCTASE DEFICIENCY
In the index case, first child of the family, diagnosis of 17-ketosteroid reductase deficiency(17-KSRD) was made at 3 months of age on the response of testosterone(T) and Δ4-androstenedone(Δ4) after an hCG test :...
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Article
103 LONG-TERM FOLLOW UP OF 2 CASES OF MIXED GONADAL DYSGENESIS(MGD): TRAGIC CONSEQUENCES OF REARING IN MALE GENDER
Two boys, now 20 yr old, were seen at birth for ambigous genitalia(hypospadias stade III); only 1 gonad was palpated in an inguinal hernia; karyotypes were 46,XO/XY. At laparotomy, small vagina, uterus; one sm...
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Article
82 PRENATAL TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA (CAH): FURTHER STUDIES IN MOTHERS AND CAH UNAFFECTED INFANTS
In an attempt to prevent in utero virilization of female fetuses with CAH due to 21-hydroxylase deficiency, we have proposed the following protocol of treatment(Rx)(J.Ped 1984, 105, 799. This protocol is based on...
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Article
HLA and congenital 21-hydroxylase (21-OHD) deficiency : further evidence for a 21-OH locus between DR and GLO
Congenital adrenal hyperplasia (CAH) due to 21-OHD has been found to be closely linked to the major histocompatibility complex (MHC) and more specifically to HLA-B. A two gene model, one on either side of HLA-...
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Article
Δ4−Androstenedione (Δ4, a biochimical marker of 11β-ydroxylas deficiency (11β-OHD)
Two female newborns (N° 1 & 2), a 13-yr girl (N° 3) (stages II-IV) and a 2-yr boy (N°4) were studied. 11β-OHD was ascertained on elevated levels of plasma 11-deoxycortisol (S) or urinary THS. Plasma levels (in...
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Article
Pittfalls in the etiological diagnosis of congenital adrenal hyperplasias (CAH) in the neonatal period
We have studied 9 males (0-34 d.) and 9 females (0-7 d.) affected with either 21-hydroxylase (21OH), 11-hydroxylase (11OH) and 3β-ol dehydrogenase (3β-ol) defects. Plasma levels of 8 steroids (specific RIAs) w...
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Article
Comparative study of persistant mullerian structures in 5 boys with ambiguous genitalia and in 2 true hermaphrodites (TH): implication for sex orientation
The 7 subjects, considered male at birth,were studied at.3-17yrs. Blood karyotypes were 46 XY/47 XXY (n°1),45XO/46XY (n°2),45XO/46 XY (n°3) in the 3 cases of mixed gonadal dysgenesis,46XX (n°4), 47XXY/46XY/46X...
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Article
Misleading prediction from fetal HLA ty** in the prenatal diagnosis of 21 hydroxylase deficiency adrenal hyperplasia (21 OH DAH
Fetal HLA ty** was performed in a 13 weeks pregnant woman for the prenatal diagnosis of 21 OH DAH. She was the mother of an 11 year old girl with late-onset 21 OH DAH (first clinical manifestations appeared ...
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Article
Effects of oestradiol benzoate (E2B), tamoxifen (tamox.) and 1,4,6-androstatriene-3,17-dione (ATD) on rat testicular function.
The effects of 2 anti-oestrogens - ATD, an aromatase inhibitor, and tamoxifen - as well as E2B itself were studied in adult male rats. E2B and tamox were both given in a single I.M. dose of 100 μg either 12, 2...