Yenidoğan Döneminde Bir Psödohipoaldosteronizm Olgusu

Abstract

Psödohipoaldosteronizm, aldosterona hedef organlarda direnç sonucu aşırı tuz kaybı, hiponatremi, hiperkalemi, metabolik asidoz ve hiperreninemik hiperaldosteronizm olarak tanımlanan nadir görülen bir hastalık grubudur. Olgu sunumunda postnatal 8. gününde dehidratasyon, hiperkalemi ve hiponatremi kliniği ile başvuran bir psödohipoaldosteronizm olgusu sunulması ve tedavisinin tartışılması amaçlanmıştır.

Keywords

• Hiperkalemi, Hiponatremi, Psödohipoaldosteronizm, Yenidoğan

A Rare Case: Pseudohypoaldosteronism in the Neonatal Period

Abstract

Pseudohypoaldosteronism is a rare disease and develops as a result of resistance to aldosterone in target organs that causes excessive salt lost, hyponatremia, hyperkalemia, metabolic acidosis and hyperreninemia. An 8-day-old boy was transferred to our hospital because of dehydration, hyperkalemia and hyponatremia and was diagnosed pseudohypoaldosteronism. We aimed to discuss our case and the treatment of pseudohypoaldosteronism

Keywords

• Hyperkalemia, Hyponatremia, Pseudohypoaldosteronism, Newborn

References

1. Cheek DB, Perry JW. A salt wasting syndrome in infancy. Arch Dis Child 1958;33:252–6.
2. Arai K, Tsigos C, Suzuki Y, Irony I, Karl M, Listwak S et al. Physiological and molecular aspects of mineralocoticoid receptor action in pseudohypoaldosteronism: A responsiveness test and therapy. J Clin Endocrinol Metab 1994;79:1019-23.
3. Akçay A, Yavuz T, Semiz S, Bundak R, Demirdöven M. Pseu- dohypoaldosteronism type 1 and respiratory distress syndrome. J Pediatr Endocrinol Metab 2002;15:1557-61.
4. Yılmaz S, Aycan Z, Peltek K, Erkek N, Bas V. Does Pseudohypoaldosteronism mask the diagnosis of congenital adrenal hyperplasia? J Clin Res Pediatr Endocrinol 2011;3:219-21.
5. Melzi ML, Guez S, Sersale G, Terzi F, Secco E, Marra G, et al. Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations. Pediatr Infect Dis J 1995;14:56-9.
6. Kurtoglu S, Hatipoglu N, Büyükkayhan D. Neonatal tuz kaybı tablosu. Turk Klin J Pediatr Sci (Özel Sayı) 2008;4:90-105.
7. Schaedel C, Marthinsen L, Kristoffersen A, Kornfait R. Lung symptoms in pseudohypoaldosteronism type 1 are associated with deficiency of the α-subunit of the epithelial sodium channel. J Pediatr 1999;739-45.