Endocrinol Metab.  2012 Dec;27(4):329-333. 10.3803/EnM.2012.27.4.329.

Two Case of Primary Aldosteronism Induced by Aldosterone Producing Adrenal Adenoma in a Family

• Jang YR
• Kim SH
• Eom YS
• Lee KY

• KMID: 1973799
• DOI: http://doi.org/10.3803/EnM.2012.27.4.329

Abstract

Primary aldosteronism, is defined as a group of disorders characterized by the excess of aldosteron, with suppressed rennin activity, resulting in hypertension and hypokalemia. In most cases, primary aldosteronism is sporadic due to a unilateral adrenal adenoma or bilateral adrenal hyperplasia. Familial hyperaldosteronism is a rare cause of primary aldosteronism and its prevalence has not been established well. We describe two cases of primary aldosteronism in a family involving a sister and brother due to an aldosterone producing adenoma in the left adrenal gland. Their hypokalemia and hypertension were cured by complete resection of the adrenal adenoma. Genetic analyses could not be done because of patients' rejection.

Keyword

Adrenocortical adenoma; Hyperaldosteronism

MeSH Terms

Adenoma
Adrenal Glands
Adrenocortical Adenoma
Aldosterone
Chymosin
Humans
Hyperaldosteronism
Hyperplasia
Hypertension
Hypokalemia
Prevalence
Rejection (Psychology)
Siblings
Aldosterone
Chymosin

Figure

• 

  Fig. 1 Computed tomography (CT) finding in case 1 and 2. (A) Case 1. An enhanced mass measured 1.5 cm in left adrenal gland. (B) Case 2. A nodular mass measured about 1 cm in medial rim of left adrenal gland.

• 

  Fig. 2 Histology of adrenal adenoma in case 1 and 2. (A) The cells form sheets and nests exhibit uniform nuclei and clear cytoplasm due to their high lipid content (H&E stain, × 200). (B) The cells arrange in nests and groups and have abundant lipid vacuoles (H&E stain, × 200).

Reference

1. Young WF Jr. Primary aldosteronism: a common and curable form of hypertension. Cardiol Rev. 1999. 7:207–214.

2. Geller DS, Zhang J, Wisgerhof MV, Shackleton C, Kashgarian M, Lifton RP. A novel form of human mendelian hypertension featuring nonglucocorticoid-remediable aldosteronism. J Clin Endocrinol Metab. 2008. 93:3117–3123.

3. Gordon RD, Stowasser M, Tunny TJ, Klemm SA, Rutherford JC. High incidence of primary aldosteronism in 199 patients referred with hypertension. Clin Exp Pharmacol Physiol. 1994. 21:315–318.

4. Lim PO, Rodgers P, Cardale K, Watson AD, MacDonald TM. Potentially high prevalence of primary aldosteronism in a primary-care population. Lancet. 1999. 353:40.

5. Olivieri O, Ciacciarelli A, Signorelli D, Pizzolo F, Guarini P, Pavan C, Corgnati A, Falcone S, Corrocher R, Micchi A, Cressoni C, Blengio G. Aldosterone to Renin ratio in a primary care setting: the Bussolengo study. J Clin Endocrinol Metab. 2004. 89:4221–4226.

6. Kwon SH, Cho YM, Park HK, Park DJ, Shin CS, Park KS, Kim SY, Cho BY, Lee HK. Hypertensive complications in patients with primary aldosteronism. J Korean Soc Endocrinol. 2002. 17:95–103.

7. Cho SC, Park YS, Park HG, Lee SH, Kim SG, Choi WH, Ahn YH, Jung PJ, Kim TW. A clinical observation on twelve cases of primary aldosteronism. J Korean Soc Endocrinol. 2004. 19:194–202.

8. Lee IS, Kim SY, Jang HW, Kim MK, Lee JH, Lee YH, Jo YS. Genetic analyses of the chimeric CYP11B1/CYP11B2 gene in a Korean family with glucocorticoid-remediable aldosteronism. J Korean Med Sci. 2010. 25:1379–1383.

9. Rich GM, Ulick S, Cook S, Wang JZ, Lifton RP, Dluhy RG. Glucocorticoid-remediable aldosteronism in a large kindred: clinical spectrum and diagnosis using a characteristic biochemical phenotype. Ann Intern Med. 1992. 116:813–820.

10. Litchfield WR, Anderson BF, Weiss RJ, Lifton RP, Dluhy RG. Intracranial aneurysm and hemorrhagic stroke in glucocorticoid-remediable aldosteronism. Hypertension. 1998. 31(1 Pt 2):445–450.

11. Mulatero P. A new form of hereditary primary aldosteronism: familial hyperaldosteronism type III. J Clin Endocrinol Metab. 2008. 93:2972–2974.

12. Stowasser M, Huggard PR, Rossetti TR, Bachmann AW, Gordon RD. Biochemical evidence of aldosterone overproduction and abnormal regulation in normotensive individuals with familial hyperaldosteronism type I. J Clin Endocrinol Metab. 1999. 84:4031–4036.

13. Stowasser M, Bachmann AW, Huggard PR, Rossetti TR, Gordon RD. Severity of hypertension in familial hyperaldosteronism type I: relationship to gender and degree of biochemical disturbance. J Clin Endocrinol Metab. 2000. 85:2160–2166.

14. Stowasser M, Bachmann AW, Huggard PR, Rossetti TR, Gordon RD. Treatment of familial hyperaldosteronism type I: only partial suppression of adrenocorticotropin required to correct hypertension. J Clin Endocrinol Metab. 2000. 85:3313–3318.

15. Sukor N, Mulatero P, Gordon RD, So A, Duffy D, Bertello C, Kelemen L, Jeske Y, Veglio F, Stowasser M. Further evidence for linkage of familial hyperaldosteronism type II at chromosome 7p22 in Italian as well as Australian and South American families. J Hypertens. 2008. 26:1577–1582.

16. Funder JW, Carey RM, Fardella C, Gomez-Sanchez CE, Mantero F, Stowasser M, Young WF Jr, Montori VM. Endocrine Society. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008. 93:3266–3281.

17. Nanba K, Tamanaha T, Nakao K, Kawashima ST, Usui T, Tagami T, Okuno H, Shimatsu A, Suzuki T, Naruse M. Confirmatory testing in primary aldosteronism. J Clin Endocrinol Metab. 2012. 97:1688–1694.

18. Weinberger MH, Fineberg NS. The diagnosis of primary aldosteronism and separation of two major subtypes. Arch Intern Med. 1993. 153:2125–2129.