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

To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes. We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day). For individuals with com...

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Veröffentlicht in:Pediatrics (Evanston) 2000-10, Vol.106 (4), p.767-773
Hauptverfasser: RIVKEES, Scott A, CRAWFORD, John D
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description To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes. We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day). 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 (DeltaCA) over which time the change in BA (DeltaBA) was 7.0 +/- 1.3 years, and the change in height age (DeltaHA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the DeltaBA was 6.5 +/- 1.0 years, and the DeltaHA 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. 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.congenital adrenal hyperplasia, dexamethasone, growth.
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We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day). 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 (DeltaCA) over which time the change in BA (DeltaBA) was 7.0 +/- 1.3 years, and the change in height age (DeltaHA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the DeltaBA was 6.5 +/- 1.0 years, and the DeltaHA 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. 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Renin-angiotensin system (diseases) ; Adrenocorticotropic Hormone - blood ; Adrenogenital syndrome ; Age Determination by Skeleton ; Biological and medical sciences ; Bone Development - drug effects ; Child, Preschool ; Children ; Congenital adrenal hyperplasia ; Dexamethasone ; Dexamethasone - pharmacology ; Dexamethasone - therapeutic use ; Drug therapy ; Endocrinopathies ; Female ; Glucocorticoids - pharmacology ; Glucocorticoids - therapeutic use ; Growth ; Growth - drug effects ; Health aspects ; Humans ; Male ; Medical disorders ; Medical sciences ; Non tumoral diseases. Target tissue resistance. 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We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day). 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 (DeltaCA) over which time the change in BA (DeltaBA) was 7.0 +/- 1.3 years, and the change in height age (DeltaHA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the DeltaBA was 6.5 +/- 1.0 years, and the DeltaHA 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. 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.congenital adrenal hyperplasia, dexamethasone, growth.</description><subject>17-alpha-Hydroxyprogesterone - blood</subject><subject>17-Ketosteroids - urine</subject><subject>Adrenal glands</subject><subject>Adrenal Hyperplasia, Congenital - drug therapy</subject><subject>Adrenal Hyperplasia, Congenital - metabolism</subject><subject>Adrenal Hyperplasia, Congenital - physiopathology</subject><subject>Adrenals. Adrenal axis. Renin-angiotensin system (diseases)</subject><subject>Adrenocorticotropic Hormone - blood</subject><subject>Adrenogenital syndrome</subject><subject>Age Determination by Skeleton</subject><subject>Biological and medical sciences</subject><subject>Bone Development - drug effects</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Congenital adrenal hyperplasia</subject><subject>Dexamethasone</subject><subject>Dexamethasone - pharmacology</subject><subject>Dexamethasone - therapeutic use</subject><subject>Drug therapy</subject><subject>Endocrinopathies</subject><subject>Female</subject><subject>Glucocorticoids - pharmacology</subject><subject>Glucocorticoids - therapeutic use</subject><subject>Growth</subject><subject>Growth - drug effects</subject><subject>Health aspects</subject><subject>Humans</subject><subject>Male</subject><subject>Medical disorders</subject><subject>Medical sciences</subject><subject>Non tumoral diseases. Target tissue resistance. 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Benign neoplasms</topic><topic>Pediatrics</topic><topic>Physical growth</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>RIVKEES, Scott A</creatorcontrib><creatorcontrib>CRAWFORD, John D</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: High School</collection><collection>Physical Education Index</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Nursing &amp; Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatrics (Evanston)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>RIVKEES, Scott A</au><au>CRAWFORD, John D</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Dexamethasone treatment of virilizing congenital adrenal hyperplasia : The ability to achieve normal growth</atitle><jtitle>Pediatrics (Evanston)</jtitle><addtitle>Pediatrics</addtitle><date>2000-10-01</date><risdate>2000</risdate><volume>106</volume><issue>4</issue><spage>767</spage><epage>773</epage><pages>767-773</pages><issn>0031-4005</issn><eissn>1098-4275</eissn><coden>PEDIAU</coden><abstract>To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes. We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day). 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 (DeltaCA) over which time the change in BA (DeltaBA) was 7.0 +/- 1.3 years, and the change in height age (DeltaHA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the DeltaBA was 6.5 +/- 1.0 years, and the DeltaHA 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. 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.congenital adrenal hyperplasia, dexamethasone, growth.</abstract><cop>Elk Grove Village, IL</cop><pub>American Academy of Pediatrics</pub><pmid>11015521</pmid><doi>10.1542/peds.106.4.767</doi><tpages>7</tpages></addata></record>
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subjects 17-alpha-Hydroxyprogesterone - blood
17-Ketosteroids - urine
Adrenal glands
Adrenal Hyperplasia, Congenital - drug therapy
Adrenal Hyperplasia, Congenital - metabolism
Adrenal Hyperplasia, Congenital - physiopathology
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Adrenocorticotropic Hormone - blood
Adrenogenital syndrome
Age Determination by Skeleton
Biological and medical sciences
Bone Development - drug effects
Child, Preschool
Children
Congenital adrenal hyperplasia
Dexamethasone
Dexamethasone - pharmacology
Dexamethasone - therapeutic use
Drug therapy
Endocrinopathies
Female
Glucocorticoids - pharmacology
Glucocorticoids - therapeutic use
Growth
Growth - drug effects
Health aspects
Humans
Male
Medical disorders
Medical sciences
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Pediatrics
Physical growth
title Dexamethasone treatment of virilizing congenital adrenal hyperplasia : The ability to achieve normal growth
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