Abstract
Cardinal features of pseudoHA include growth failure,hyperkalemia,metabolic acidosis,salt wasting and elevated peripheral renin activity(PRA) and plasma aldosterone concentrations.
Three patients were studied.Two were sibs:R.G.,7 mo girl,PRA 242.58 ng angiotensin I/ml/hr(normal 0.96-1.67),aldosterone secretion rate 10680 ng/24 hr(normal <300)and plasma aldosterone >80 ng/d1(normal 5.16-10.2);the brother's cord blood PRA 18.7 and plasma aldosterone 96.Patient J.H.,4½ mo,PRA 28,plasma aldosterone 360.Treatment with mineralocorticoids failed to normalize serum electrolytes in R.G. and J.H. but this was achieved with large quantities of oral NaCl.Features of pseudoHA were prevented in the sib with NaCl supplement.
Light microscopy of renal biopsy(R.G.):many immature glomeruli,hyperplasia and hypertrophy of the JGA,arteriolar wall thickening,and,on electronmicroscopy,fusion of podocyte foot processes, hypergranularity of JGA cells.
Similarities between pseudoHA and Bartter's syndrome include growth failure,hyponatremia,salt wasting,elevated PRA and aldosterone,JGA hyperplasia and response to indomethacin.Major differences are hyperkalemia,acidosis in pseudoHA in contrast to hypokalemia,alkalosis in Bartter's syndrome.
This appears to be the first description of changes in the kidney in pseudoHA.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Blachar, Y., Griffel, B., Levin, S. et al. 1053 PSEUDOHYPOALDOSTERONISM(pseudoHA): SIMILAR TO BARTTER'S SYNDROME?. Pediatr Res 12 (Suppl 4), 539 (1978). https://doi.org/10.1203/00006450-197804001-01059
Issue Date:
DOI: https://doi.org/10.1203/00006450-197804001-01059