Hyporeninemic Hypoaldosteronism, Sodium Wasting and Mineralocorticoid-Resistant Hyperkalemia in Two Patients with Obstructive Uropathy

Abstract

The renin-aldosterone system and tubular responsiveness to exogenous mineralocorticoid administration was evaluated in 2 hyperkalemic patients with obstructive uropathy. Both patients manifested marked renal sodium wasting and a modest inability to lower urine pH despite systemic acidosis. None of the abnormalities was corrected with supraphysiologic doses of mineralocorticoid. In addition, plasma renin and aldosterone levels were inappropriately low. Thus, both hypoaldosteronism and renal tubular resistance to mineralocorticoids coexisted in these patients. It is likely that an association between these abnormalities is more frequent than previously recognized.

severe hyperkalemia without electrocardiographic changes in a patient with addison disease

“…Therefore, in our case nephropathy can also contribute to severe hyperkalemia together with AD. Patients with chronic renal insufficiency frequently have an impaired kaliuretic response to exogenous mineralocorticoid administration, suggesting that enhanced distal chloride reabsorption may account for many of the findings of hyporeninemic hypoaldosteronism (11). Rapid increases in K + concentrations may potentiate the cardiotoxic effects of hyperkalemia, whereas if hyperkalemia develops slowly (e.g., chronic renal failure), cardiac manifestations may be attenuated.…”

Hypertension, Mineralocorticoid-Resistant Hyperkalemia, and Hyperchloremic Acidosis in an Infant with Obstructive Uropathy

“…Obstructive nephropathy has been associated with several types of dRTA and hyperkalemia [11][12][13][14][15][16], Batlle et al [3] studied 13 patients with obstructive uropathy, hyperkalemia, and dRTA. Three types of renal tubular dysfunction were identified: selective hvpoaldosteronism, a 'voltage-dependent' acidification defect, and 3 patients with a com bination of both, while Pelleya et al [15] reported 2 adults, and Rodriquez-Soriano et al [16] have described 6 infants with salt-wasting and mineralocorticoid-resistant hyperkalemia, i.e., aldosterone resistance (pseudohy poaldosteronism type I). More recently Alon et al [17] have reported 3 neonates with unilateral kidney disease who had incomplete end-organ resistance to aldosterone (dRTA type 4, subtype 5) [5].…”

Hypothesis: A simple algorithm to distinguish between hypoaldosteronism and renal aldosterone resistance in patients with persistent hyperkalemia

“… HA.14 reports, 33 patients, plus an unreported case of our own 8–21 . Twenty‐two of these patients had associated renal impairment and two also had PHA.…”

“…Renal impairment and hyperkalemia. Ten reports, 43 patients plus two unreported cases of our own 5,9,11,13,14,16–19,40 . Of the 45 patients, 22 had been included in the HA data.…”

“… Plasma aldosterone and potassium in patients obtained from patient reports, defined with either HA 8–21 or RAR 3,18,22–37 according to criteria outlined in the text. The defined lower limit was set from the data in Fig.…”