At the age of 3 weeks, a girl presenting with acute dehydration was admitted to our hospital. Clinical and laboratory findings revealed malformations of the genitourinary tract, an acute urinary infection and electrolyte disturbances (severe hyponatremia at 115 mmol/l and mild hyperkalemia at 5.6 mmol/l). According to anamnestic data, the child was born to healthy, nonconsanguineous parents. Vaccum extraction was done in the 38th gestational week due to pathological cardiotocography (CTG) findings. Auxological parameters were within the normal range for gestational age. Normal values for 17-OH progesterone and ACTH ruled out congenital adrenal hyperplasia (CAH). Pathologically high aldosterone and plasma renin activity (PRA) confirmed the diagnosis of pseudohypoaldosteronism with salt-wasting crisis which proved to be transient.

Tjaden BL, Rock JA: Uterovaginal anomalies; in Carpenter SE, Rock JA (eds): Pediatric and Adolescent Gynecology. New York, Raven Press, 1992, pp 313–330.
Thompson DP, Lynn HB: Genital anomalies associated with solitary kidney. Mayo Clin Proc 1966;41:538–548.
Sarto GE, Simpson JL: Abnormalities of the Müllerian and Wolffian duct systems. Birth Defects 1978;14:37–54.
Giedion A, Prader A: Hand-foot-uterus (HFU) syndrome with hypospadias: The hand-foot-genital (HFG) syndrome. Pediatr Radiol 1976;4:96–102.
Verp MS, Simpson JL, Elias S, Carson SA, Sarto GE, Feingold M: Heritable aspects of uterine anomalies. I. Three familial aggregates with Müllerian fusion anomalies. Fertil Steril 1983;40:80–85.
Cheek DB, Perry JW: A salt wasting syndrome in infancy. Arch Dis Child 1958;33:252–256.
Kuhnle U, Guariso G, Sonega M, Hinkel GK, Hubl W, Armanini D: Transient pseudohypoaldosteronism in obstructive renal disease with transient reduction of lymphocytic aldosterone receptors: Results in two affected infants. Horm Res 1993;39:152–155.
Zennaro MC, Borensztein P, Soubrier F, Armanini D, Corvol P: The enigma of pseudohypoaldosteronism. Steroids 1994;59:96–99.
Arai K, Tsigos C, Suzuki Y, Listwak S, Zachman K, Zangeneh F, Rappaport R, Chanoine JP, Chrousos GP: No apparent mineralocorticoid receptor defect in a series of sporadic cases of pseudohypoaldosteronism. JCEM 1995;80:814–817.
Rock JA, Jones HW Jr: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 1980;138:339–342.
Rosendahl W: Diagnostic procedures in endocrine disorders of sodium regulation; in Ranke MB (ed): Diagnostics of Endocrine Function in Children and Adolescents. Heidelberg, J&J Edition (Barth), 1996, pp 241–254.
Chang SS, Grunder S, Hanukoglu A, Rösler A, Mathew PM, Hanukoglu I, Schild L, Lu Y, Shimkets RA, Nelson-Williams C, Rossier BC, Lifton RP: Mutations in subunits of the epithelial sodium channels cause salt wasting with hyperkalemic acidosis, pseudohypoaldosteronism type I. Nat Genet 1996;12:248–253.
Muhammad S, Mamish ZM, Tucci JR: Type II pseudohypoaldosteronism: Report of a case and review of the literature. J Endocrinol Invest 1994;17:453–457.
Levin TL, Abramson SJ, Burbige KA, Connor JP, Ruzal-Shapiro C, Berdon WE: Salt losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux – value of ultrasonography in diagnosis. Pediatr Radiol 1991;21:413–415.
Tolaymat A, Sandoval S, Abella E, Rawlings DJ: Clinical quiz: An infant with renal unresponsiveness. Pediatr Nephrol 1997;11:121–123.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.