Dexamethasone treatment of virilizing congenital adrenal hyperplasia: The ability to achieve normal growth

被引:90
作者
Rivkees, SA
Crawford, JD
机构
[1] Yale Univ, Sch Med, Dept Pediat, Sect Pediat Endocrinol, New Haven, CT 06510 USA
[2] Harvard Univ, Massachusetts Gen Hosp, Sch Med, Dept Pediat,Pediat Endocrine Unit, Boston, MA USA
关键词
congenital adrenal hyperplasia; dexamethasone; growth;
D O I
10.1542/peds.106.4.767
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Objective. To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes. Method. We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/- .01 mg/m(2)/day). Results. For individuals with comparable bone age (BA) and chronological age (CA) at the onset of dexamethasone therapy, males were 2.8 +/- .8 years (mean +/- standard error of the mean; n = 13) and females were 2.4 +/- 1.0 years (n = 6). Males were treated for 7.3 +/- 1.1 years (Delta CA) over which time the change in BA (Delta BA) was 7.0 +/- 1.3 years, and the change in height age (Delta HA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the Delta BA was 6.5 +/- 1.0 years, and the Delta HA was 6.3 +/- .8 years. During treatment 17 ketosteroid excretion rates were normal for age and 17-hydroxyprogesterone values were 69.6 +/- 18 ng/ dL. Testicular enlargement was first detected at 10.7 +/- .8 years and breast tissue at 9.9 +/- 1.2 years. Three boys and 1 girl had final heights of 171.8 +/- 6 cm and 161 cm, respectively, compared with midparental heights of 176.1 +/- 4.1 cm and 160 cm. Predicted adult heights for 6 other boys and 5 girls were 176.8 +/- 2.0 cm and 161.4 +/- 2.8 cm, respectively, compared with midparental heights of 174.6 +/- 1.4 cm and 158.2 +/- 2.0 cm. Statural outcomes were less favorable for 7 children started on dexamethasone when BAs were considerably advanced, although height predictions increased during therapy. Conclusions. These observations show that children treated with dexamethasone for CAH can achieve normal growth with the convenience of once-a-day dosing in most cases.
引用
收藏
页码:767 / 773
页数:7
相关论文
共 26 条
[11]   EFFECTS OF LONG-TERM DEXAMETHASONE TREATMENT IN ADULT PATIENTS WITH CONGENITAL ADRENAL-HYPERPLASIA [J].
HORROCKS, PM ;
LONDON, DR .
CLINICAL ENDOCRINOLOGY, 1987, 27 (06) :635-642
[12]   PLASMA PROGESTERONE, 17-HYDROXYPROGESTERONE, ANDROSTENEDIONE AND TESTOSTERONE IN PREPUBERTAL, PUBERTAL AND ADULT SUBJECTS WITH CONGENITAL VIRILIZING ADRENAL-HYPERPLASIA AS INDICATORS OF ADRENAL SUPPRESSION [J].
LEE, PA ;
GUTAI, UJP ;
MIGEON, CJ .
HORMONE RESEARCH, 1980, 13 (06) :347-357
[13]  
MIGEON CJ, 1995, PEDIAT ENDOCRINOLOGY, P333
[14]  
New M I, 1997, Curr Ther Endocrinol Metab, V6, P179
[15]  
New M I, 1988, Acta Paediatr Jpn, V30 Suppl, P79
[16]   17-HYDROXYPROGESTERONE RHYTHMS AND GROWTH VELOCITY IN CONGENITAL ADRENAL-HYPERPLASIA [J].
PINCUS, DR ;
KELNAR, CJH ;
WALLACE, AM .
JOURNAL OF PAEDIATRICS AND CHILD HEALTH, 1993, 29 (04) :302-304
[17]   Adrenal cortical hyperplasia with virilism diagnosis, course and treatment [J].
Talbot, NB ;
Butler, AM ;
Berman, RA .
JOURNAL OF CLINICAL INVESTIGATION, 1942, 21 (05) :559-570
[18]  
Tanner J, 1986, HUMAN GROWTH COMPREH, P95
[19]   CLINICAL LONGITUDINAL STANDARDS FOR HEIGHT AND HEIGHT VELOCITY FOR NORTH-AMERICAN CHILDREN [J].
TANNER, JM ;
DAVIES, PSW .
JOURNAL OF PEDIATRICS, 1985, 107 (03) :317-329
[20]  
WILKINS L, 1950, B JOHNS HOPKINS HOSP, V86, P249