Pseudohypo-Adrenalocorticism

Donnell, George N.

doi:10.1001/archpedi.1959.02070010815008

Cited by 47 publications

(15 citation statements)

References 68 publications

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“…Tu bular unresponsiveness to mineralocorticoid is rare in adults but more than 20 cases have been described in children. The term pseudohypoaldosteronism has been used to characterize this syndrome which usually occurs in infancy, responds well to salt supplementation but not to mineralocorticoid, and which usually clears during the first several years of life [5][6][7].…”

Section: Discussionmentioning

confidence: 99%

Pseudohypoaldosteronism following Kidney Transplantation

Uribarri¹,

Oh²,

Butt³

et al. 1982

Nephron

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A 56-year-old woman received a kidney transplant and presented subsequently with evidence of volume contraction, hyponatremia and hyperkalemia. Urinary sodium excretion was inappropriately high for the degree of volume contraction and urinary potassium excretion inappropriately low for the degree of hyperkalemia. Marked elevation of plasma renin activity and plasma aldosterone suggested that the renal tubules were unresponsive to mineralocorticoids. The defect was shown to be transient. The mechanisms leading to the defect are discussed.

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Section: Discussionmentioning

confidence: 99%

Pseudohypoaldosteronism following Kidney Transplantation

Uribarri¹,

Oh²,

Butt³

et al. 1982

Nephron

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“…In other cases no renal abnormalities are present. In general, patients fall into two categories: (a) those with hyper kalemia, renal saIt wasting and hypotension, normal GFR, elevated plasma renin and plasma aldosterone levels, and failure of mineralocorticoids to correct the hyperkalemia and salt wasting (165)(166)(167)(168)(169)(170)(171). High plasma aldoster one levels distinguish this group of patients from those with a defect in aldosterone biosynthesis; (b) those with hyperkalemia, normal renal Na conservation and hypertension, normal GFR, low plasma renin activity, low to normal plasma aldosterone levels, and a normal antinatriuretic response to mineralocorticoids without any kaliuretic effect (172)(173)(174)(175)(176)(177)(178)(179)(180)(181).…”

Section: Impaired Renin-aldosterone Axismentioning

confidence: 99%

Clinical Disorders of Hyperkalemia

DeFronzo¹,

BIa²,

Smith³

1982

Annu. Rev. Med.

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Potassium DistributionTotal body potassium in a healthy adult is approximately 50 meqlkg body weight or about 3500 meq for a 70-kg man (1, 2). Most of this is located within cells (Figure 1), primarily muscle, at a concentration of about 150 meq/L. Only 2% of total body potassium is located in the extracellular fl uid, normally at concentrations of 3.5 -5.0 meq/L. The maintenance of this high intracellular to extracellular K concentration depends upon the Na-K ATPase pump (3, 4) as well as other fac tors, including H+ balance, plasma tonicity, and plasma insulin, epinephrine, and aldosterone concentrations (5). The large difference in potassium concentration across the cell mem brane is critical for normal cell fu nction since it is the primary determinant of the resting membrane potential. Small changes in the intracellular/ex tracellular K ratio can severely disturb neuromuscular fu nction, particu larly of the heart. A gain (or loss) fr om the extracellular space of an amount of potassium equal to only I % of the total body K content (35 meq) can cause a 50% increase (or decrease) in plasma K concentration. Such a change would have pronounced effects on neuromuscular func ti on. Like wise, redistribution of a similar amount of potassium fr om the intracellular to extracellular (or vice versa) fluid compartmen t without net gain or loss of K fr om the body, would similarly affect neuromuscular excitability. Thus, it is important that both the total amount of potassium within the body and the distribution of potassium between intracellular and extracellu lar fl uid compartments be closely regulated.In healthy man, potassium enters only via the gastrointestinal tract. Approximately 100 meq of K is ingested per day and essentially all of this is absorbed fr om the stomach and upper gastrointestinal tract. Approxi-521 0066-4219/8 2/040 1-0521 $02.00 Annu. Rev. Med. 1982.33:521-554. Downloaded from www.annualreviews.org Access provided by Haifa University on 01/05/15. For personal use only.Quick links to online content Further ANNUAL REVIEWS

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“…The clinical features of pseudohypoal dosteronism include failure to thrive, anorexia, vomiting, irritability, hyponatremia from renal salt wasting in the presence of normal glomerular filtration rate and adrenal functions [16,20]. The laboratory features are character ized by the presence of hyponatremia, hyperkalemic met abolic acidosis and marked elevation of plasma renin activity and serum aldosterone concentrations [22][23][24][25][26][27][28][29], Pseudohypoaldosteronism is further characterized by lack of response to mineralocorticoids, good response to sodium supplementation and spontaneous improvement with age [23,24,26], We. therefore, have several reasons to conclude that the hyponatremia, renal sodium wasting, hyperkalemic metabolic acidosis as seen in our patient are compatible with the diagnosis of type 4 renal tubular acidosis [30,31].…”

Section: Dr James CM Chanmentioning

confidence: 99%