Abstract: FR-PO605
Adrenal Mass in Bartter: Incidentaloma or Adenoma?
Session Information
- Genetic Diseases: Tubulopathies
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Genetic Diseases of the Kidneys
- 1202 Genetic Diseases of the Kidneys: Non-Cystic
Authors
- Rajasekaran, Rhoshini, The University of Alabama at Birmingham, Birmingham, Alabama, United States
- Chandramohan, Deepak, The University of Alabama at Birmingham, Birmingham, Alabama, United States
- Deotare, Apoorv, The University of Alabama at Birmingham, Birmingham, Alabama, United States
Introduction
Bartter’s syndrome is rare, characterized by salt wasting caused by impaired absorption of sodium in the thick ascending limb of the loop of Henle. Young patients present with hypokalemia, metabolic alkalosis, and hyperaldosteronism. Our case presents a patient with Bartter’s syndrome with an incidental adrenal mass.
Case Description
A 39 yo F with hx of Bartter’s syndrome type III (heterozygous for CLCKNB) and Sjogren’s syndrome presents for an office visit for evaluation of incidental right adrenal complex mass 1.5cm X 1.7cm with a question of whether the hyperaldosteronism was because of Bartter’s syndrome or a hypersecretory adrenal nodule. She denies any h/o Hypertension. She was diagnosed at the age of 5 months with Bartter’s syndrome, hypokalemia, and hypomagnesemia. Vitals - temp 97.3, pulse 80, BP 120/81. The physical exam was unremarkable. Her daily medications include Losartan 50 mg, Amiloride 10 mg BID, and Potassium and Magnesium supplements. Labs: BMP: Na 138, K 4.9, Cl 103, bicarb 23, BUN 10, Cr 0.8, Mg 1.7, Hb 12.6, WBC 9.2, Plt 419. Renin 48ng/mL/h, aldosterone 1100ng/dL.
Discussion
Bartter syndrome presents as hypochloremic, hypokalemic metabolic alkalosis. It is diagnosed in young individuals with an incidence of 1/1000000. Hyperaldosteronism and high rennin levels occur secondary to compensation mechanisms that cause macula densa hypertrophy. An adrenal mass occurring in Bartter syndrome has not been reported in the literature. This patient was normotensive with an elevated renin and aldosterone level. Our case makes us wonder if the elevated aldosterone was compensatory vs hypersecretory. An adrenal adenoma in this case would present with suppressed renin, elevated aldosterone with hypertension. Primary hyperaldosteronism would have suppressed renin levels in addition to elevated aldosterone levels. A salt suppression test could be used to differentiate between the etiology of primary vs secondary hyperaldosteronism if renin levels are suppressed. In conclusion, Bartter syndrome causes compensatory hyperaldosteronism with high renin levels.