Renal Tubular Hyperkalemia

Pérez, Guido O.; Pelleya, Rene; Oster, James R.

doi:10.1159/000166595

Cited by 16 publications

(10 citation statements)

References 14 publications

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“…Dietary K + intake, which was not stated, may have been reduced and can have a marked effect on U K V in man 41 and animals. 42 As Perez et al 43 and Sanjad et al 11 point out, reduction of plasma K + with salt restriction does not rule out a defect in K + secretion.…”

Section: Hypertensionmentioning

confidence: 99%

Pseudohypoaldosteronism Type II

Healy

2014

Hypertension

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K + during a NaHCO 3 infusion. As will be discussed below, we now know that there are 5 levels of severity of PHAII so that the response to PRAN may differ between individual cases. Given the hyperkalemia of these patients, one might have expected even a supranormal response to PRAN as seen below in the mouse model of PHAII.32 PRAN were used in 11 of the studies surveyed here and although some had a reduced kaliuretic response, in none was there a response greater than the normal controls. 3,5,[7][8][9][11][12][13]17,19,20 Therefore, given the hyperkalemia, these results indicate a mild degree of depression of ENaC function. This might have been expected to lead to a compensatory increase in activity of the major K + secretory mechanism, the renal outer medullary K + (ROMK) channels. 34,35 This might imply that ROMK also was not functioning normally (Figure, Theory 1).Another factor that could have suppressed the K + secretion, particularly ROMK, 31 was the acidosis, which is known to be because of hyperkalemia in PHAII. 5,7,11,12 If PHAII is reversed by drug treatment (thiazide) and the drug is stopped, the hyperkalemia returns in 10 days before a return of the acidosis at 17 days, 8 indicating the primacy of the hyperkalemia. If the ENaC is compromised to a degree, this period without acidosis would provide ROMK with a window of opportunity to control the rising plasma K + ; this clearly does not happen. DeFronzo et al 33 studied K + secretion in sickle cell disease that damages the renal papillae and the medulla. They found a negligible response in K + secretion to PRAN, yet hyperkalemia did not occur (average plasma K + , 3.78±0.01 mmol/L). This difference from PHAII might be explained by the fact that although ROMK shares the late DT, the connecting tubule and the collecting duct sites with ENaC, functioning isoforms ROMK 2 and 3 are present as well in the whole of the distal convoluted tubule (DCT). 34 Therefore, in sickle cell disease, these isoforms may have been intact in the DCT before the late DCT but perhaps not so in PHAII. PHAII and sickle cell disease cannot be compared pathologically, but these results reflect on the way the effects of PRAN are interpreted because they show that a negligible response in K + secretion to PRAN is not obligatorily associated with hyperkalemia in humans.Finally, Golbang et al 26 (see below) have described a father and son with PHAII caused by a mutant gene shown by genetic testing to inhibit ROMK substantially.The second view of the cause of hyperkalemia in PHAII is that a Cl -shunt may play a role 3 by increasing paracellular Cl -absorption in the early DT (Figure, Theory 2). Schambelan et al 3 in 1981 showed that with mineralocorticoid pretreatment, intravenous Na 2 SO 4 and NaHCO 3 could both invoke an increase in K + excretion in PHAII, but that intravenous sodium chloride (NaCl) was much less able to do so. They postulated that a Cl -shunt proximal to the ENaC would result in less Cl -delivery and thus less negativity at the ENaC, resulting in decreased K + sec...

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Section: Hypertensionmentioning

confidence: 99%

Pseudohypoaldosteronism Type II

Healy

2014

Hypertension

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“…For this condition, the term 'renal tubular hyperkalemia' has recently been in troduced by Perez et al [9], They attributed this syn drome (which was also found in subjects with a normal glomerular filtration rate) to a tubular defect to excrete potassium in the presence of a high PAC. This descrip tion fits for 1 patient in the present study (No.…”

Section: Discussionmentioning

confidence: 99%

“…If reactivity to aldosterone were also increased in renal insufficiency, this might explain the poor relationship between the degree of renal failure and PAC [4], In some patients with renal insufficiency, renal potassium excretion can be increased by mineralocorticoid administration [6,7], but in others, tubular resistance has been reported [8,9]. No systematic com parative studies on the renal response to an excess of aldosterone in patients with renal insufficiency as com pared to normal subjects are available.…”

Section: Introductionmentioning

confidence: 99%

Effect of High-Dose Aldosterone Infusions on Renal Electrolyte Excretion in Patients with Renal Insufficiency

Hené¹,

Koomans²,

Boer³

et al. 1987

Am J Nephrol

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We investigated the effect of aldosterone infusion (0.5 mg/h for 6 h) on electrolyte excretion in 11 patients with severe renal insufficiency (creatinine clearance 6–20 ml/min), with normal or elevated serum potassium levels and a wide range of plasma aldosterone levels, and compared the data with those obtained in 7 healthy subjects. The studies were done under conditions of fixed sodium and potassium intake. In the normal subjects, aldosterone infusion caused a significant rise in potassium excretion and a significant fall in sodium and chloride excretion (p < 0.01). In 1 patient with a high plasma aldosterone, virtually no response occurred to the aldosterone infusion. In the others, the increase in potassium excretion and reduction in chloride excretion were not different from the changes observed in the normals, but the fall in sodium excretion was less due to a higher urinary sodium before infusion in the normals (p < 0.05). Fractional electrolyte excretions as well as the changes in fractional excretion by aldosterone were larger in the patients (p < 0.05). Apparently, the renal tubules of patients with chronic renal failure are still responsive to maximal stimulation with aldosterone, in spite of their basically elevated fractional electrolyte output. These findings suggest that, with some exceptions, the hyperkalemia in patients with chronic renal failure is in part due to relative hypoaldosteronism.

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“…In addition, there were hyperchloremic metabolic acidosis with an apparently normal anion gap, even in the presence of monoclonal IgM, and hyporeninemic hypoaldosteronism. Perez et al [16] have recently proposed the term renal tubular hyperkalemia for the various conditions associated with hyperkalemia and impaired tubular ex cretion of potassium. According to their classification of renal tubular hyperkalemia, the present case is likely to belong to the group 111(b), i.e., a syndrome associated with mild to moderate renal insufficiency, decreased po tassium excretion, and low plasma aldosterone.…”

Section: Discussionmentioning

confidence: 99%

IgM Monoclonal Gammopathy Accompanied by Nodular Glomerulosclerosis, Urine-Concentrating Defect, and Hyporeninemic Hypoaldosteronism

et al. 1985

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A 54-year-old male had monoclonal IgM-kappa light chains in the serum and free monoclonal kappa light chains in the urine. Renal biopsy revealed nodular glomerulosclerosis associated with the accumulation of kappa light chains. Isolated microscopic hematuria was present for over 1 year. He also showed a defect in urine concentration for which the light chains deposited along the basement membrane in the inner medullary tubules were judged to be responsible. When the glomerular filtration rate fell to 30 ml/min, the syndrome of mild renal failure, decreased potassium excretion, and hyporeninemic hypoaldosteronism developed, suggesting that kappa light chain nephropathy is a cause of this syndrome. Chemotherapy appeared to have some beneficial effects in maintaining renal function.

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Renal Tubular Hyperkalemia

Cited by 16 publications

References 14 publications

Pseudohypoaldosteronism Type II

Pseudohypoaldosteronism Type II

Effect of High-Dose Aldosterone Infusions on Renal Electrolyte Excretion in Patients with Renal Insufficiency

IgM Monoclonal Gammopathy Accompanied by Nodular Glomerulosclerosis, Urine-Concentrating Defect, and Hyporeninemic Hypoaldosteronism

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