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Posterior üretral valvlı iki yenidoğanda tuz kaybı: sekonder psödohipoaldosteronizm

Yıl 2017, Cilt: 30 Sayı: 2, 119 - 123, 14.05.2017
https://doi.org/10.5472/marumj.344826

Öz

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerinin

aldosterona duyarsızlığı ile karakterize, hiperkalemi,

hiponatremi ve metabolik asidoz ile kendini gösteren nadir

bir durumdur. Obstrüktif üropatisi olan bebeklerde ve

piyelonefrit sırasında görülebilir. Gelişimi devam eden erken

bebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisi

ve enfeksiyonu sekonder PHA hastalarında tübüluslarda

aldosteron direncine neden olan üç önemli faktördür. Genellikle

hiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır,

tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteron

seviyelerinin saptanması ile tanı konur. Süt çoçuklarında

biyokimyasal incelemede hiperpotasemi, hiponatremi ve

metabolik asidoz saptandığında konjenital adrenal hiperplazi

ayırıcı tanısında PHA göz önünde bulundurulmalı ve bu

hastalarda üriner ultrasonografisi (USG) ve idrar analizi rutin

olarak yapılmalıdır. Bu yazımızda sekonder PHA gelişen

posterior üretral valv (PUV)‘lı iki hasta sunuldu.

Kaynakça

  • Devuyst O, Konrad M, Jeunemaitre X, Zennaro MC. Tubular disorders of electrolyte regulation. In: Avner ED, Harmon WE, Niaudet P, Yoshikawa N, editors. Pediatric Nephrology. 6th ed. Berlin Heidelberg: Springer-Verlag, 2009; 929-77.
  • Goodyer P. Disorders of tubular transport. In: Kher KK, Schnaper HW, Makker SP, editors. Clinical Pediatric Nephrology. 2nd ed. London: Informa Healthcare, 2006; 317-36.
  • Nasir AA, Ameh EA, Abdur-Rahman LO, Adeniran JO, Abrahan MK. Posterior urethral valve. World J Pediatr 2011;7:205-16. doi: 10.1007/s12519-011-0289-1
  • Hodges SJ, Patel B, McLorie G, Atala A. Posterior urethral valves. ScientificWorldJournal 2009; 9:1119-26. doi: 10.1100/tsw.2009.127
  • Asano T, Abe M, Asai M, et al. Urinary tract malformation and infection with hyperkalemia and decreased fractional excretion of potassium in an infant. J Nippon Med Sch 2006;73:289-91. doi: http://doi.org/10.1272/jnms.73.289
  • Bogdanović R, Stajić N, Putnik J, Paripović A. Transient type 1 pseudo- hypoaldosteronism: report on an eight-patient series and literature review. Pediatr Nephrol 2009;24:2167- 75. doi: 10.1007/s00467-009-1285-8
  • Pai B, Shaw N, Högler W. Salt – losing crisis in infants – not always of adrenal origin. Eur J Pediatr 2012;171:317-21. doi: 10.1007/s00431-011-1541-3
  • Nandagopal R, Vaidyanathan P, Kaplowitz P. Transient pseudohypoaldosteronism due to urinary tract infection in infancy: A report of 4 cases. Int J Pediatr Endocrinol 2009;2009:195728. doi: 10.1155/2009/195728
  • Ağladıoğlu SY, Aycan Z, Kendirci HN, Erkek N, Baş VN. Does pseudohypoaldosteronism mask the diagnosis of congenital adrenal hyperplasia? J Clin Res Pediatr Endocrinol 2011; 3:219-21. doi:10.4274/jcrpe.369
  • Bowden SA, Cozzi C, Hickey SE, Thrush DL, Astbury C, Nuthakki S. Autosomal dominant pseudohypoaldosteronism type 1 in an infant with salt wasting crisis associated with urinary tract infection and obstructive uropathy. Case Rep Endocrinol 2013;2013:524647. doi:http://dx.doi. org/10.1155/2013/524647
  • Bayer M, Kutílek S. A hereditary form of pseudohypoaldosteronism may be manifested in the course of pyelonephritis. Acta Paediatr 1993; 82:504. doi: 10.1111/ j.1651-2227.1993.tb12736.x

Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism

Yıl 2017, Cilt: 30 Sayı: 2, 119 - 123, 14.05.2017
https://doi.org/10.5472/marumj.344826

Öz

Secondary pseudohypoaldosteronism (PHA) is a rare condition

that presents with hyperkalemia, hyponatremia, and metabolic

acidosis, which develops due to renal tubular unresponsiveness

to aldosterone. It may be seen in infants with obstructive

uropathy and acute pyelonephritis. Three important reasons of

the aldosterone unresponsiveness of the tubules are early infancy

kidney, obstructive uropathy, and urinary tract infection. Besides

other findings, diagnosis is established with highly increased

plasma aldosterone and renin levels. In infants who present with

hyperkalemia, hyponatremia, and metabolic acidosis, PHA should

be considered in the differential diagnosis of congenital adrenal

hyperplasia. Urinary ultrasonography and urine analysis should be

performed in these patients. Herein, we present two patients with

secondary PHA caused by posterior urethral valves.

Kaynakça

  • Devuyst O, Konrad M, Jeunemaitre X, Zennaro MC. Tubular disorders of electrolyte regulation. In: Avner ED, Harmon WE, Niaudet P, Yoshikawa N, editors. Pediatric Nephrology. 6th ed. Berlin Heidelberg: Springer-Verlag, 2009; 929-77.
  • Goodyer P. Disorders of tubular transport. In: Kher KK, Schnaper HW, Makker SP, editors. Clinical Pediatric Nephrology. 2nd ed. London: Informa Healthcare, 2006; 317-36.
  • Nasir AA, Ameh EA, Abdur-Rahman LO, Adeniran JO, Abrahan MK. Posterior urethral valve. World J Pediatr 2011;7:205-16. doi: 10.1007/s12519-011-0289-1
  • Hodges SJ, Patel B, McLorie G, Atala A. Posterior urethral valves. ScientificWorldJournal 2009; 9:1119-26. doi: 10.1100/tsw.2009.127
  • Asano T, Abe M, Asai M, et al. Urinary tract malformation and infection with hyperkalemia and decreased fractional excretion of potassium in an infant. J Nippon Med Sch 2006;73:289-91. doi: http://doi.org/10.1272/jnms.73.289
  • Bogdanović R, Stajić N, Putnik J, Paripović A. Transient type 1 pseudo- hypoaldosteronism: report on an eight-patient series and literature review. Pediatr Nephrol 2009;24:2167- 75. doi: 10.1007/s00467-009-1285-8
  • Pai B, Shaw N, Högler W. Salt – losing crisis in infants – not always of adrenal origin. Eur J Pediatr 2012;171:317-21. doi: 10.1007/s00431-011-1541-3
  • Nandagopal R, Vaidyanathan P, Kaplowitz P. Transient pseudohypoaldosteronism due to urinary tract infection in infancy: A report of 4 cases. Int J Pediatr Endocrinol 2009;2009:195728. doi: 10.1155/2009/195728
  • Ağladıoğlu SY, Aycan Z, Kendirci HN, Erkek N, Baş VN. Does pseudohypoaldosteronism mask the diagnosis of congenital adrenal hyperplasia? J Clin Res Pediatr Endocrinol 2011; 3:219-21. doi:10.4274/jcrpe.369
  • Bowden SA, Cozzi C, Hickey SE, Thrush DL, Astbury C, Nuthakki S. Autosomal dominant pseudohypoaldosteronism type 1 in an infant with salt wasting crisis associated with urinary tract infection and obstructive uropathy. Case Rep Endocrinol 2013;2013:524647. doi:http://dx.doi. org/10.1155/2013/524647
  • Bayer M, Kutílek S. A hereditary form of pseudohypoaldosteronism may be manifested in the course of pyelonephritis. Acta Paediatr 1993; 82:504. doi: 10.1111/ j.1651-2227.1993.tb12736.x
Toplam 11 adet kaynakça vardır.

Ayrıntılar

Konular Klinik Tıp Bilimleri
Bölüm Olgu Sunumu
Yazarlar

Ülger Altuntaş Bu kişi benim

İbrahim Gökce Bu kişi benim

Halil Tuğtepe Bu kişi benim

Meryem Benzer Bu kişi benim

Serçin Güven Bu kişi benim

Nurdan Yıldız Bu kişi benim

Harika Alpay Bu kişi benim

Yayımlanma Tarihi 14 Mayıs 2017
Yayımlandığı Sayı Yıl 2017 Cilt: 30 Sayı: 2

Kaynak Göster

APA Altuntaş, Ü., Gökce, İ., Tuğtepe, H., Benzer, M., vd. (2017). Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism. Marmara Medical Journal, 30(2), 119-123. https://doi.org/10.5472/marumj.344826
AMA Altuntaş Ü, Gökce İ, Tuğtepe H, Benzer M, Güven S, Yıldız N, Alpay H. Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism. Marmara Med J. Mayıs 2017;30(2):119-123. doi:10.5472/marumj.344826
Chicago Altuntaş, Ülger, İbrahim Gökce, Halil Tuğtepe, Meryem Benzer, Serçin Güven, Nurdan Yıldız, ve Harika Alpay. “Salt Wasting in Two Neonates With Posterior Urethral Valves: Secondary Pseudohypoaldosteronism”. Marmara Medical Journal 30, sy. 2 (Mayıs 2017): 119-23. https://doi.org/10.5472/marumj.344826.
EndNote Altuntaş Ü, Gökce İ, Tuğtepe H, Benzer M, Güven S, Yıldız N, Alpay H (01 Mayıs 2017) Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism. Marmara Medical Journal 30 2 119–123.
IEEE Ü. Altuntaş, “Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism”, Marmara Med J, c. 30, sy. 2, ss. 119–123, 2017, doi: 10.5472/marumj.344826.
ISNAD Altuntaş, Ülger vd. “Salt Wasting in Two Neonates With Posterior Urethral Valves: Secondary Pseudohypoaldosteronism”. Marmara Medical Journal 30/2 (Mayıs 2017), 119-123. https://doi.org/10.5472/marumj.344826.
JAMA Altuntaş Ü, Gökce İ, Tuğtepe H, Benzer M, Güven S, Yıldız N, Alpay H. Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism. Marmara Med J. 2017;30:119–123.
MLA Altuntaş, Ülger vd. “Salt Wasting in Two Neonates With Posterior Urethral Valves: Secondary Pseudohypoaldosteronism”. Marmara Medical Journal, c. 30, sy. 2, 2017, ss. 119-23, doi:10.5472/marumj.344826.
Vancouver Altuntaş Ü, Gökce İ, Tuğtepe H, Benzer M, Güven S, Yıldız N, Alpay H. Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism. Marmara Med J. 2017;30(2):119-23.