PRENATAL TREATMENT OF CONGENITAL ADRENAL-HYPERPLASIA - REPORT OF A NEW CASE

被引:9
作者
LOEUILLE, GA
DAVID, M
FOREST, MG
机构
[1] HOP CLAUDE HURIEZ,SERV GENET & MALAD HEREDITAIRES METAB ENFANT,F-59037 LILLE,FRANCE
[2] HOP DEBROUSSE,SERV PEDIAT,F-69322 LYONS 05,FRANCE
[3] HOP DEBROUSSE,INSERM,U34,F-69322 LYONS 05,FRANCE
关键词
21-Hydroxylase deficiency; Congenital adrenal hyperplasia; Dexamethasone; Prenatal diagnosis and treatment;
D O I
10.1007/BF02106280
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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 karyotype was 46,XX, and because of a possible intra HLA recombination, treatment was continued to term. The newborn had a modest virilization and hormonal studies confirmed the diagnosis of congenital adrenal hyperplasia (CAH). This observation and review of the literature suggest that efficient prenatal treatment of CAH requires a higher and more frequent dosage of dexamethasone. © 1990 Springer-Verlag.
引用
收藏
页码:237 / 240
页数:4
相关论文
共 21 条
[1]   A POSSIBLE NEW HLA-DR ALLELE [J].
BETUEL, H ;
GEBUHRER, L ;
LAMBERT, J ;
FREIDEL, AC ;
FARRE, A .
HUMAN IMMUNOLOGY, 1983, 8 (03) :227-237
[2]   PRENATAL TREATMENT OF CONGENITAL ADRENAL-HYPERPLASIA RESULTING FROM 21-HYDROXYLASE DEFICIENCY [J].
DAVID, M ;
FOREST, MG .
JOURNAL OF PEDIATRICS, 1984, 105 (05) :799-803
[3]  
DORR HG, 1986, PEDIATR RES, V20, P1182
[4]  
DORR HG, 1988, 70TH ANN M END SOC N
[5]   PHARMACOLOGIC SUPPRESSION OF THE FETAL ADRENAL-GLAND INUTERO - ATTEMPTED PREVENTION OF ABNORMAL EXTERNAL GENITAL MASCULINIZATION IN SUSPECTED CONGENITAL ADRENAL-HYPERPLASIA [J].
EVANS, MI ;
CHROUSOS, GP ;
MANN, DW ;
LARSEN, JW ;
GREEN, I ;
MCCLUSKEY, J ;
LORIAUX, DL ;
FLETCHER, JC ;
KOONS, G ;
OVERPECK, J ;
SCHULMAN, JD .
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION, 1985, 253 (07) :1015-1020
[6]   ONTOGENIC STUDY OF PLASMA 17-ALPHA-HYDROX-PROGESTERONE IN HUMAN .1. POSTNATAL-PERIOD - EVIDENCE FOR A TRANSIENT OVARIAN ACTIVITY IN INFANCY [J].
FOREST, MG ;
CATHIARD, AM .
PEDIATRIC RESEARCH, 1978, 12 (01) :6-11
[7]   HYPOPHYSIO-GONADAL FUNCTION IN HUMANS DURING FIRST YEAR OF LIFE .1. EVIDENCE FOR TESTICULAR ACTIVITY IN EARLY INFANCY [J].
FOREST, MG ;
SIZONENKO, PC ;
CATHIARD, AM ;
BERTRAND, J .
JOURNAL OF CLINICAL INVESTIGATION, 1974, 53 (03) :819-828
[9]   CONCENTRATION OF 14 STEROID-HORMONES IN HUMAN AMNIOTIC-FLUID OF MID-PREGNANCY [J].
FOREST, MG ;
DEPERETTI, E ;
LECOQ, A ;
CADILLON, E ;
ZABOT, MT ;
THOULON, JM .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1980, 51 (04) :816-822