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Primary Aldosteronism Due to Adrenal Hyperplasia: Occurrence in a Boy Aged 10 Years

Primary Aldosteronism Due to Adrenal Hyperplasia: Occurrence in a Boy Aged 10 Years Abstract Since Conn's description of the clinical features associated with primary aldosteronism in 1955,1 several other cases have been reported in the literature.2-12 This syndrome, which is the result of hypersecretion by the adrenal gland of the mineralocorticoid hormone aldosterone, is usually due to a functioning adrenocortical adenoma. In the few cases without adenoma, hyperplasia chiefly involving the zona fasciculata has been found,9 and hyperaldosteronism due to adrenocortical carcinoma with hepatic metastases has been reported.4 All patients were adults except one girl 6 aged 13, and one boy 12 aged 9 years. The clinical syndrome originally described by Conn 1,13 consists essentially of polyuria and polydipsia, arterial hypertension, periodic weakness and "paralyses," paresthesiae, and intermittent tetany. These findings are associated with altered electrolyte metabo lism manifested by metabolic alkalosis with hypokalemia and hypernatremia, and elevated potassium content of sweat, saliva, and urine, with low sweat, salivary, and References 1. Conn, J. W.: Primary Aldosteronism, a New Clinical Syndrome , J. Lab. & Clin. Med. 45:3-17 ( (Jan.) ) 1955. 2. Chalmers, T. M.; Fitzgerald, M. G.; James, A. H., and Scarborough, H.: Conn's Syndrome with Severe Hypertension , Lancet 1:127-132 ( (Jan. 21) ) 1956. 3. Earle, D. P.; Sherry, S.; Eichna, L. W., and Conan, N. J.: Low Potassium Syndrome Due to Defective Renal Tubular Mechanisms for Handling Potassium , Am. J. Med. 11:283-301 ( (Sept.) ) 1951. 4. Foye, L. V., Jr., and Feichtmeir, T. V.: Adrenal Cortical Carcinoma Producing Solely Mineralocorticoid Effect , Am. J. Med. 19:966-975 ( (Dec.) ) 1955. 5. Campbell, C. H.; Nicholaides, N., and Steinbeck, A. W.: Adrenocortical Tumor with Hypokalaemia and Flaccid Muscle Paralysis , Lancet 2:553-555 ( (Sept. 15) ) 1956. 6. Holten, C., and Petersen, V. P.: Malignant Hypertension with Increased Secretion of Aldosterone and Depletion of Potassium , Lancet 2:918-922 ( (Nov. 3) ) 1956. 7. Mader, I. J., and Iseri, L. T.: Spontaneous Hypopotassemia, Hypomagnesemia, Alkalosis and Tetany Due to Hypersecretion of Corticosterone-like Mineralocorticoid , Am. J. Med. 19:976-988 ( (Dec.) ) 1955. 8. Mader, I. J., and Iseri, L. T.: Spontaneous Hypopotassemia, Hypomagnesemia, Alkalosis, and Tetany: Metabolic and Physiologic Studies , J. Lab. & Clin. Med. 44:895-896 ( (Dec.) ) 1954. 9. Van Buchem, F. S.; Doorenbos, H., and Elings, H. S.: Primary Aldosteronism Due to Adrenocortical Hyperplasia , Lancet 2:335-337 ( (Aug. 18) ) 1956. 10. Fine, D.; Meiselas, L. E.; Colsky, J., and Oxenhorn, S.: Primary Aldosteronism; Report of a Case and Discussion of the Pathogenesis , New England J. Med. 254:147-152 ( (Jan. 24) ) 1957. 11. Crane, M. G.; Vogel, P. J., and Richland, K. J.: Observations on a Presumptive Case of Primary Aldosteronism , J. Lab. & Clin. Med. 48:1, 1956. 12. Kretchmer, N.; Dickinson, A., and Karl, R.: Aldosteronism in a 9-Year-Old Child , A. M. A. J. Dis. Child. 94:452 ( (Oct.) ) 1957. 13. Conn, J. W., and Louis, L. H.: Primary Aldosteronism, A New Clinical Entity , Ann. Int. Med. 44:1-15 ( (Jan.) ) 1956. 14. Smith, R. W., Jr.; Mellinger, R. C., and Patti, A. A.: Modifications of the Reddy Procedure for 17-Hydroxycorticoids in Urine , J. Clin. Endocrinol. 14:336-338 ( (March) ) 1954. 15. Klendshoj, N. C.; Feldstein, M., and Sprague, A.: Determination of 17-Ketosteroids in Urine , J. Clin. Endocrinol. 13:922-927 ( (Aug.) ) 1953. 16. Neher, R., and Wettstein, A.: Physicochemical Estimation of Aldosterone in Urine , J. Clin. Invest. 35:800-805 ( (July) ) 1956. 17. Nowaczynski, W.; Koiw, E., and Genest, J.: Chemical Method for the Determination of Urinary Aldosterone , Canad. J. Biochem. & Physiol. 35: 425-443 ( (June) ) 1957. 18. Dunstan, H. P.; Corcoran, A. C., and Farrell, G. L.: Primary Aldosteronism , Lancet 2:1120-1121 ( (Nov. 26) ) 1955. 19. Luetscher, J. A., Jr., and Curtis, R. H.: Relationship of Aldosterone in Urine to Sodium Balance and to Some Other Endocrine Functions , J. Clin. Invest. 34:951 ( (June) ) 1955. 20. Aldosterone , Lancet 1:141-2 ( (Jan. 21) ) 1956. 21. Genest, J.: Clinical States Associated with Abnormal Aldosterone Excretion , Canad. M. A. J. 77:780-785 ( (Oct. 15) ) 1957. 22. Giroud, C. J. P.; Stachenko, J., and Piletta, P.: In Vitro Studies of the Functional Zonation of the Adrenal Cortex and the Production of Aldosterone , in International Symposium on Aldosterone , edited by A. F. Muller and C. M. O'Connor, Boston, Little, Brown & Company, 1958, pp. 56-72. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Primary Aldosteronism Due to Adrenal Hyperplasia: Occurrence in a Boy Aged 10 Years

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References (23)

Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1959.02070020092012
Publisher site
See Article on Publisher Site

Abstract

Abstract Since Conn's description of the clinical features associated with primary aldosteronism in 1955,1 several other cases have been reported in the literature.2-12 This syndrome, which is the result of hypersecretion by the adrenal gland of the mineralocorticoid hormone aldosterone, is usually due to a functioning adrenocortical adenoma. In the few cases without adenoma, hyperplasia chiefly involving the zona fasciculata has been found,9 and hyperaldosteronism due to adrenocortical carcinoma with hepatic metastases has been reported.4 All patients were adults except one girl 6 aged 13, and one boy 12 aged 9 years. The clinical syndrome originally described by Conn 1,13 consists essentially of polyuria and polydipsia, arterial hypertension, periodic weakness and "paralyses," paresthesiae, and intermittent tetany. These findings are associated with altered electrolyte metabo lism manifested by metabolic alkalosis with hypokalemia and hypernatremia, and elevated potassium content of sweat, saliva, and urine, with low sweat, salivary, and References 1. Conn, J. W.: Primary Aldosteronism, a New Clinical Syndrome , J. Lab. & Clin. Med. 45:3-17 ( (Jan.) ) 1955. 2. Chalmers, T. M.; Fitzgerald, M. G.; James, A. H., and Scarborough, H.: Conn's Syndrome with Severe Hypertension , Lancet 1:127-132 ( (Jan. 21) ) 1956. 3. Earle, D. P.; Sherry, S.; Eichna, L. W., and Conan, N. J.: Low Potassium Syndrome Due to Defective Renal Tubular Mechanisms for Handling Potassium , Am. J. Med. 11:283-301 ( (Sept.) ) 1951. 4. Foye, L. V., Jr., and Feichtmeir, T. V.: Adrenal Cortical Carcinoma Producing Solely Mineralocorticoid Effect , Am. J. Med. 19:966-975 ( (Dec.) ) 1955. 5. Campbell, C. H.; Nicholaides, N., and Steinbeck, A. W.: Adrenocortical Tumor with Hypokalaemia and Flaccid Muscle Paralysis , Lancet 2:553-555 ( (Sept. 15) ) 1956. 6. Holten, C., and Petersen, V. P.: Malignant Hypertension with Increased Secretion of Aldosterone and Depletion of Potassium , Lancet 2:918-922 ( (Nov. 3) ) 1956. 7. Mader, I. J., and Iseri, L. T.: Spontaneous Hypopotassemia, Hypomagnesemia, Alkalosis and Tetany Due to Hypersecretion of Corticosterone-like Mineralocorticoid , Am. J. Med. 19:976-988 ( (Dec.) ) 1955. 8. Mader, I. J., and Iseri, L. T.: Spontaneous Hypopotassemia, Hypomagnesemia, Alkalosis, and Tetany: Metabolic and Physiologic Studies , J. Lab. & Clin. Med. 44:895-896 ( (Dec.) ) 1954. 9. Van Buchem, F. S.; Doorenbos, H., and Elings, H. S.: Primary Aldosteronism Due to Adrenocortical Hyperplasia , Lancet 2:335-337 ( (Aug. 18) ) 1956. 10. Fine, D.; Meiselas, L. E.; Colsky, J., and Oxenhorn, S.: Primary Aldosteronism; Report of a Case and Discussion of the Pathogenesis , New England J. Med. 254:147-152 ( (Jan. 24) ) 1957. 11. Crane, M. G.; Vogel, P. J., and Richland, K. J.: Observations on a Presumptive Case of Primary Aldosteronism , J. Lab. & Clin. Med. 48:1, 1956. 12. Kretchmer, N.; Dickinson, A., and Karl, R.: Aldosteronism in a 9-Year-Old Child , A. M. A. J. Dis. Child. 94:452 ( (Oct.) ) 1957. 13. Conn, J. W., and Louis, L. H.: Primary Aldosteronism, A New Clinical Entity , Ann. Int. Med. 44:1-15 ( (Jan.) ) 1956. 14. Smith, R. W., Jr.; Mellinger, R. C., and Patti, A. A.: Modifications of the Reddy Procedure for 17-Hydroxycorticoids in Urine , J. Clin. Endocrinol. 14:336-338 ( (March) ) 1954. 15. Klendshoj, N. C.; Feldstein, M., and Sprague, A.: Determination of 17-Ketosteroids in Urine , J. Clin. Endocrinol. 13:922-927 ( (Aug.) ) 1953. 16. Neher, R., and Wettstein, A.: Physicochemical Estimation of Aldosterone in Urine , J. Clin. Invest. 35:800-805 ( (July) ) 1956. 17. Nowaczynski, W.; Koiw, E., and Genest, J.: Chemical Method for the Determination of Urinary Aldosterone , Canad. J. Biochem. & Physiol. 35: 425-443 ( (June) ) 1957. 18. Dunstan, H. P.; Corcoran, A. C., and Farrell, G. L.: Primary Aldosteronism , Lancet 2:1120-1121 ( (Nov. 26) ) 1955. 19. Luetscher, J. A., Jr., and Curtis, R. H.: Relationship of Aldosterone in Urine to Sodium Balance and to Some Other Endocrine Functions , J. Clin. Invest. 34:951 ( (June) ) 1955. 20. Aldosterone , Lancet 1:141-2 ( (Jan. 21) ) 1956. 21. Genest, J.: Clinical States Associated with Abnormal Aldosterone Excretion , Canad. M. A. J. 77:780-785 ( (Oct. 15) ) 1957. 22. Giroud, C. J. P.; Stachenko, J., and Piletta, P.: In Vitro Studies of the Functional Zonation of the Adrenal Cortex and the Production of Aldosterone , in International Symposium on Aldosterone , edited by A. F. Muller and C. M. O'Connor, Boston, Little, Brown & Company, 1958, pp. 56-72.

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: Jul 1, 1959

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