Persistence of the enzymatic block in adolescent patients with salt-losing congenital adrenal hyperplasia

Chandra Edwin; Roberto Lanes; Claude J. Migeon; Peter Lee; Leslie P. Plotnick; A. Avinoam Kowarski

doi:10.1016/S0022-3476(79)80757-X

Persistence of the enzymatic block in adolescent patients with salt-losing congenital adrenal hyperplasia

Chandra Edwin, Roberto Lanes, Claude J. Migeon, Peter Lee, Leslie P. Plotnick, A. Avinoam Kowarski

Research output: Contribution to journal › Article › peer-review

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Abstract

The 24-hour integrated concentration of aldosterone, plasma renin activity, and 17-hydroxprogesteronewas measured simultaneously in 12 patients with congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency. Eight were salt-losers and four nonsalt-lowers. Seven normal children served as control subjects. All patients were receiving glucocorticoid replacement therapy (32 mg/m²/day). All but one of the SL-CVAH patients were also being treated with 9α-fluorocortisol. The mean (±SD) IC-PRA of the control subjects was 1.6±0.7 ng/ml/hour and in the NSL-CVAH patients was similar (1.6±0.7 and 2.2±1.0 ng/ml/hour). The IC-PRA of the SL-CVAH patients (21.1±28.8 ng/ml/hour) was significantly (P<0.001) higher than that of the normal subjects. The IC-ALDO/IC-PRA ratio of the control subjects and NSL-CVAH patients were 12.4±5.1 and 8.8±4.8, respectively. The ratio in SL-CVAH patients was significantly lower (2.0±1.4) than that of the control subjects (P<0.001). The lowered IC-AL DO/IC-PRA ratio in the SL-CVAH patients demonstrates the persistence of the 21-hydroxylase deficiency.

Original language	English (US)
Pages (from-to)	534-537
Number of pages	4
Journal	The Journal of Pediatrics
Volume	95
Issue number	4
DOIs	https://doi.org/10.1016/S0022-3476(79)80757-X
State	Published - Oct 1979

All Science Journal Classification (ASJC) codes

Pediatrics, Perinatology, and Child Health

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10.1016/S0022-3476(79)80757-X

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@article{ff622794c89847c9a7bc06407b1b66e3,

title = "Persistence of the enzymatic block in adolescent patients with salt-losing congenital adrenal hyperplasia",

abstract = "The 24-hour integrated concentration of aldosterone, plasma renin activity, and 17-hydroxprogesteronewas measured simultaneously in 12 patients with congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency. Eight were salt-losers and four nonsalt-lowers. Seven normal children served as control subjects. All patients were receiving glucocorticoid replacement therapy (32 mg/m2/day). All but one of the SL-CVAH patients were also being treated with 9α-fluorocortisol. The mean (±SD) IC-PRA of the control subjects was 1.6±0.7 ng/ml/hour and in the NSL-CVAH patients was similar (1.6±0.7 and 2.2±1.0 ng/ml/hour). The IC-PRA of the SL-CVAH patients (21.1±28.8 ng/ml/hour) was significantly (P<0.001) higher than that of the normal subjects. The IC-ALDO/IC-PRA ratio of the control subjects and NSL-CVAH patients were 12.4±5.1 and 8.8±4.8, respectively. The ratio in SL-CVAH patients was significantly lower (2.0±1.4) than that of the control subjects (P<0.001). The lowered IC-AL DO/IC-PRA ratio in the SL-CVAH patients demonstrates the persistence of the 21-hydroxylase deficiency.",

author = "Chandra Edwin and Roberto Lanes and Migeon, {Claude J.} and Peter Lee and Plotnick, {Leslie P.} and Kowarski, {A. Avinoam}",

note = "Funding Information: From the Division of Pediatric Endocrinology, Department of Pediatrics, The Johns Hopkins University School of Medicine 9 Supported by research grants RO1-HD-06284-07, AM-00180-22, and traineeship grant Tl-AM-5219 of the National Institutes of Health, United States Public Health Service; the patients were studied at the Clinical Research Center of Pediatrics supported by grant 5-MO1-RR-OO52 from the General Clinical Research Centers Program of the Division of Research. *Reprint address: CMSC 3-110, The Johns Hopkins Hospital Baltimore, MD 21205.",

year = "1979",

month = oct,

doi = "10.1016/S0022-3476(79)80757-X",

language = "English (US)",

volume = "95",

pages = "534--537",

journal = "Journal of Pediatrics",

issn = "0022-3476",

publisher = "Mosby Inc.",

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TY - JOUR

T1 - Persistence of the enzymatic block in adolescent patients with salt-losing congenital adrenal hyperplasia

AU - Edwin, Chandra

AU - Lanes, Roberto

AU - Migeon, Claude J.

AU - Lee, Peter

AU - Plotnick, Leslie P.

AU - Kowarski, A. Avinoam

N1 - Funding Information: From the Division of Pediatric Endocrinology, Department of Pediatrics, The Johns Hopkins University School of Medicine 9 Supported by research grants RO1-HD-06284-07, AM-00180-22, and traineeship grant Tl-AM-5219 of the National Institutes of Health, United States Public Health Service; the patients were studied at the Clinical Research Center of Pediatrics supported by grant 5-MO1-RR-OO52 from the General Clinical Research Centers Program of the Division of Research. *Reprint address: CMSC 3-110, The Johns Hopkins Hospital Baltimore, MD 21205.

PY - 1979/10

Y1 - 1979/10

N2 - The 24-hour integrated concentration of aldosterone, plasma renin activity, and 17-hydroxprogesteronewas measured simultaneously in 12 patients with congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency. Eight were salt-losers and four nonsalt-lowers. Seven normal children served as control subjects. All patients were receiving glucocorticoid replacement therapy (32 mg/m2/day). All but one of the SL-CVAH patients were also being treated with 9α-fluorocortisol. The mean (±SD) IC-PRA of the control subjects was 1.6±0.7 ng/ml/hour and in the NSL-CVAH patients was similar (1.6±0.7 and 2.2±1.0 ng/ml/hour). The IC-PRA of the SL-CVAH patients (21.1±28.8 ng/ml/hour) was significantly (P<0.001) higher than that of the normal subjects. The IC-ALDO/IC-PRA ratio of the control subjects and NSL-CVAH patients were 12.4±5.1 and 8.8±4.8, respectively. The ratio in SL-CVAH patients was significantly lower (2.0±1.4) than that of the control subjects (P<0.001). The lowered IC-AL DO/IC-PRA ratio in the SL-CVAH patients demonstrates the persistence of the 21-hydroxylase deficiency.

AB - The 24-hour integrated concentration of aldosterone, plasma renin activity, and 17-hydroxprogesteronewas measured simultaneously in 12 patients with congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency. Eight were salt-losers and four nonsalt-lowers. Seven normal children served as control subjects. All patients were receiving glucocorticoid replacement therapy (32 mg/m2/day). All but one of the SL-CVAH patients were also being treated with 9α-fluorocortisol. The mean (±SD) IC-PRA of the control subjects was 1.6±0.7 ng/ml/hour and in the NSL-CVAH patients was similar (1.6±0.7 and 2.2±1.0 ng/ml/hour). The IC-PRA of the SL-CVAH patients (21.1±28.8 ng/ml/hour) was significantly (P<0.001) higher than that of the normal subjects. The IC-ALDO/IC-PRA ratio of the control subjects and NSL-CVAH patients were 12.4±5.1 and 8.8±4.8, respectively. The ratio in SL-CVAH patients was significantly lower (2.0±1.4) than that of the control subjects (P<0.001). The lowered IC-AL DO/IC-PRA ratio in the SL-CVAH patients demonstrates the persistence of the 21-hydroxylase deficiency.

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DO - 10.1016/S0022-3476(79)80757-X

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AN - SCOPUS:0018292673

VL - 95

SP - 534

EP - 537

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 4

ER -

Persistence of the enzymatic block in adolescent patients with salt-losing congenital adrenal hyperplasia

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