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