Posterior üretral valvlı iki yenidoğanda tuz kaybı: sekonder psödohipoaldosteronizm

Abstract

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.

Keywords

• Obstrüktif üropati,Sekonder psödohipoaldosteronizm,Süt çocuğu

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

Abstract

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.

Keywords

• Obstructive uropathy,Secondary pseudohypoaldosteronism,Infant

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