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September 1960, Vol 100, No. 3 >
Article | September 1960

Congenital Virilizing Adrenal Hyperplasia FREE

HARVEY D. KLEVIT, M.D.
[+] Author Affiliations

Submitted for publication March 16, 1960.

4200 E. Ninth Ave. (20).

Chief Resident in Pediatrics.


Am J Dis Child. 1960;100(3):415-423. doi:10.1001/archpedi.1960.04020040417016.
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In the past several years, great strides have been made in the elucidation of certain inborn defects in steroid metabolism. This has led to a better understanding of the pathophysiology of congenital virilizing adrenal hyperplasia and has given us a more rational approach to the treatment of this disorder. The purpose of this discussion is to review some of the newer knowledge of steroidal biogenesis, to point out the different enzymatic deficiencies that have been found in various forms of the adrenogenital syndrome, to summarize the salient clinical features, and to discuss the rationale of therapy.

Physiological Considerations  Adrenal Physiology in the Newborn.—It is helpful to be familiar with the physiology of the adrenal gland in the newborn infant in order to understand the changes that take place in congenital adrenal hyperplasia. At birth the newly born baby is endowed with an extremely large adrenal gland which is

REFERENCES

1 +
Gardner, L. I.:  Symposium on Endocrinology: Adrenal Function in the Premature and Newborn , Pediatrics 17:414, 1956;.
2 +
Gardner, L. I.: Adrenal Function in Infants and Children: A Symposium , New York, Grune & Stratton, Inc., 1956;.
3 +
Gardner, L. I.:  Adrenocortical Metabolism of the Fetus, Infant and Child , Pediatrics 17:897, 1956;.
4 +
Klein, R.:  Symposium on Clinical Advances: Neonatal Adrenal Physiology , Pediat. Clin. North America 1:321, 1954;.
5 +
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , Ed. 2, Springfield, Ill., Charles C Thomas, Publisher, 1957;,  Chap. 14 , 15, and 16.
6 +
Bongiovanni, A. M.; Eberlein, W. R., and Cara, J.:  Studies on the Metabolism of Adrenal Steroids in the Adrenogenital Syndrome , J. Clin. Endocrinol. 14:409, 1954;.
7 +
Bongiovanni, A. M., and Eberlein, W. R.:  Clinical and Metabolic Variations in the Adrenogenital Syndrome , Pediatrics 16:628, 1955;.
8 +
Bongiovanni, A. M., and Eberlein, W. R.:  Defective Steroidal Biogenisis in Congenital Adrenal Hyperplasia , Pediatrics 21:661, 1958;.
9 +
Eberlein, W. R., and Bongiovanni, A. M.:  Congenital Adrenal Hyperplasia with Hypertension: Unusual Steroid Pattern in Blood and in Urine , J. Clin. Endocrinol. 15:1531, 1955;.
10 +
Blizzard, R. M.; Liddle, G. W.; Migeon, C. J., and Wilkins, L.:  Aldosterone Excretion in Virilizing Adrenal Hyperplasia , J. Clin. Invest. 38: 1442, 1959;.
11 +
Childs, B.; Grumbach, M. M., and Van Wyk, J. J.:  Virilizing Adrenal Hyperplasia: A Genetic and Hormonal Study , J. Clin. Invest. 35: 213, 1956;.
12 +
Wilkins, L.; Jones, H. W., Jr.; Holman, G. H., and Stempfel, R. S., Jr.:  Masculinization of Female Fetus Associated with Administration of Oral and Intramuscular Progestins During Gestation: Nonadrenal Female Pseudohermaphroditism , J. Clin. Endocrinol. 18:559, 1958;.
13 +
Bongiovanni, A. M.; DiGeorge, A. M., and Grumbach, M. M.:  Masculinization of Female Infant Associated with the Use of Stilbesterol During Pregnancy , A.M.A.J. Dis. Child. 98:476, 1959;.
14 +
Silver, H. K.; Kempe, C. H., and Bruyn, H. B.: Handbook of Pediatrics , Ed. 3, Los Altos, Calif., Lange Medical Publications, University Medical Publishers, 1959;, p. 571.
15 +
Iversen, T.:  Congenital Adrenalcortical Hyperplasia with Disturbed Electrolyte Regulation: Dysadrenocorticism , Pediatrics 16:875, 1955;.
16 +
Deamer, W. C., and Silver, H. K.:  Abnormalities in the Secretions of the Adrenal Cortex During Early Life , J. Pediat. 37:490, 1950;.
17 +
Grumbach, M. M., and Wilkins, L.:  Symposium on Endocrinology: Pathogenesis and Treatment of Virilizing Adrenal Hyperplasia , Pediatrics 17:418, 1956;.
18 +
Bongiovanni, A. M.:  Fluid Therapy in Adrenocortical Failure , Pediat. Clin. North America 6:209, 1959;.
19 +
Wilkins, L., and Cara, J.:  Further Studies on the Treatment of Congenital Adrenal Hyperplasia with Cortisone: V. Effects of Cortisone Therapy on Testicular Development , J. Clin. Endocrinol. 12:1015, 1952;.
20 +
Money, J.; Hampson, J. C., and Hampson, J. L.:  Hermaphroditism: Recommendations Concerning Assignment of Sex, Change of Sex and Psychologic Management , Bull. Johns Hopkins Hosp. 97:284, 1955;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Gardner, L. I.:  Symposium on Endocrinology: Adrenal Function in the Premature and Newborn , Pediatrics 17:414, 1956;.
2 +
Gardner, L. I.: Adrenal Function in Infants and Children: A Symposium , New York, Grune & Stratton, Inc., 1956;.
3 +
Gardner, L. I.:  Adrenocortical Metabolism of the Fetus, Infant and Child , Pediatrics 17:897, 1956;.
4 +
Klein, R.:  Symposium on Clinical Advances: Neonatal Adrenal Physiology , Pediat. Clin. North America 1:321, 1954;.
5 +
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , Ed. 2, Springfield, Ill., Charles C Thomas, Publisher, 1957;,  Chap. 14 , 15, and 16.
6 +
Bongiovanni, A. M.; Eberlein, W. R., and Cara, J.:  Studies on the Metabolism of Adrenal Steroids in the Adrenogenital Syndrome , J. Clin. Endocrinol. 14:409, 1954;.
7 +
Bongiovanni, A. M., and Eberlein, W. R.:  Clinical and Metabolic Variations in the Adrenogenital Syndrome , Pediatrics 16:628, 1955;.
8 +
Bongiovanni, A. M., and Eberlein, W. R.:  Defective Steroidal Biogenisis in Congenital Adrenal Hyperplasia , Pediatrics 21:661, 1958;.
9 +
Eberlein, W. R., and Bongiovanni, A. M.:  Congenital Adrenal Hyperplasia with Hypertension: Unusual Steroid Pattern in Blood and in Urine , J. Clin. Endocrinol. 15:1531, 1955;.
10 +
Blizzard, R. M.; Liddle, G. W.; Migeon, C. J., and Wilkins, L.:  Aldosterone Excretion in Virilizing Adrenal Hyperplasia , J. Clin. Invest. 38: 1442, 1959;.
11 +
Childs, B.; Grumbach, M. M., and Van Wyk, J. J.:  Virilizing Adrenal Hyperplasia: A Genetic and Hormonal Study , J. Clin. Invest. 35: 213, 1956;.
12 +
Wilkins, L.; Jones, H. W., Jr.; Holman, G. H., and Stempfel, R. S., Jr.:  Masculinization of Female Fetus Associated with Administration of Oral and Intramuscular Progestins During Gestation: Nonadrenal Female Pseudohermaphroditism , J. Clin. Endocrinol. 18:559, 1958;.
13 +
Bongiovanni, A. M.; DiGeorge, A. M., and Grumbach, M. M.:  Masculinization of Female Infant Associated with the Use of Stilbesterol During Pregnancy , A.M.A.J. Dis. Child. 98:476, 1959;.
14 +
Silver, H. K.; Kempe, C. H., and Bruyn, H. B.: Handbook of Pediatrics , Ed. 3, Los Altos, Calif., Lange Medical Publications, University Medical Publishers, 1959;, p. 571.
15 +
Iversen, T.:  Congenital Adrenalcortical Hyperplasia with Disturbed Electrolyte Regulation: Dysadrenocorticism , Pediatrics 16:875, 1955;.
16 +
Deamer, W. C., and Silver, H. K.:  Abnormalities in the Secretions of the Adrenal Cortex During Early Life , J. Pediat. 37:490, 1950;.
17 +
Grumbach, M. M., and Wilkins, L.:  Symposium on Endocrinology: Pathogenesis and Treatment of Virilizing Adrenal Hyperplasia , Pediatrics 17:418, 1956;.
18 +
Bongiovanni, A. M.:  Fluid Therapy in Adrenocortical Failure , Pediat. Clin. North America 6:209, 1959;.
19 +
Wilkins, L., and Cara, J.:  Further Studies on the Treatment of Congenital Adrenal Hyperplasia with Cortisone: V. Effects of Cortisone Therapy on Testicular Development , J. Clin. Endocrinol. 12:1015, 1952;.
20 +
Money, J.; Hampson, J. C., and Hampson, J. L.:  Hermaphroditism: Recommendations Concerning Assignment of Sex, Change of Sex and Psychologic Management , Bull. Johns Hopkins Hosp. 97:284, 1955;.

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