Ülger ALTUNTAŞ, İBRAHİM GÖKCE, Neslihan Çiçek DENİZ, Meryem BENZER, Halil TUĞTEPE, Serçin GÜVEN, NURDAN YILDIZ, HARİKA ALPAY

Posterior üretral valvlı iki yenidoğanda tuz kaybı: sekonder psödohipoaldosteronizm

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerininaldosterona duyarsızlığı ile karakterize, hiperkalemi,hiponatremi ve metabolik asidoz ile kendini gösteren nadirbir durumdur. Obstrüktif üropatisi olan bebeklerde vepiyelonefrit sırasında görülebilir. Gelişimi devam eden erkenbebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisive enfeksiyonu sekonder PHA hastalarında tübüluslardaaldosteron direncine neden olan üç önemli faktördür. Genelliklehiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır,tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteronseviyelerinin saptanması ile tanı konur. Süt çoçuklarındabiyokimyasal incelemede hiperpotasemi, hiponatremi vemetabolik asidoz saptandığında konjenital adrenal hiperplaziayırıcı tanısında PHA göz önünde bulundurulmalı ve buhastalarda üriner ultrasonografisi (USG) ve idrar analizi rutinolarak yapılmalıdır. Bu yazımızda sekonder PHA gelişenposterior üretral valv (PUV)‘lı iki hasta sunuldu.

Anahtar Kelimeler:

Obstrüktif üropati, Sekonder psödohipoaldosteronizm, Süt çocuğu

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

Secondary pseudohypoaldosteronism (PHA) is a rare conditionthat presents with hyperkalemia, hyponatremia, and metabolicacidosis, which develops due to renal tubular unresponsivenessto aldosterone. It may be seen in infants with obstructiveuropathy and acute pyelonephritis. Three important reasons ofthe aldosterone unresponsiveness of the tubules are early infancykidney, obstructive uropathy, and urinary tract infection. Besidesother findings, diagnosis is established with highly increasedplasma aldosterone and renin levels. In infants who present withhyperkalemia, hyponatremia, and metabolic acidosis, PHA shouldbe considered in the differential diagnosis of congenital adrenalhyperplasia. Urinary ultrasonography and urine analysis should beperformed in these patients. Herein, we present two patients withsecondary PHA caused by posterior urethral valves.

Keywords:

Obstructive uropathy, Secondary pseudohypoaldosteronism, Infant,

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