RESUMO
Pneumothorax and transient pseudohypoaldosteronism have been noted as complications of obstructive uropathy in infancy. However, they are rare complications, and there have been no reports of a patient with both pneumothorax and pseudohypoaldosteronism in obstructive uropathy. Here we describe a case of pneumothorax and transient pseudohypoaldosteronism in an infant with bilateral hydronephrosis due to ureteropelvic junction obstruction. Nephrostomy improved the patient's condition and laboratory findings rapidly. While the exact pathogenic mechanism of pneumothorax in patients with obstructive uropathy is unclear, some biochemical defects due to obstructive uropathy might impair fetal lung maturation, which results in pneumothorax.
Assuntos
Hidronefrose/congênito , Hidronefrose/complicações , Pneumotórax/complicações , Pseudo-Hipoaldosteronismo/complicações , Obstrução Ureteral/fisiopatologia , Feminino , Humanos , Hidronefrose/diagnóstico por imagem , Hidronefrose/fisiopatologia , Recém-Nascido , Pneumotórax/diagnóstico por imagem , Pneumotórax/fisiopatologia , Pseudo-Hipoaldosteronismo/diagnóstico por imagem , Pseudo-Hipoaldosteronismo/fisiopatologia , Tomografia Computadorizada por Raios X , Obstrução Ureteral/complicaçõesRESUMO
In transient pseudohypoaldosteronism (TPHA), renal tubular resistance to aldosterone is thought to be secondary to renal disease. We report a case of TPHA caused by posterior urethral valves associated with urinary tract infection and review 62 cases previously reported. The infant presented with unspecific signs of vomiting and dehydration, so that pyloric stenosis was first suspected. Laboratory data and retroperitoneal sonography led to the diagnosis TPHA. This case illustrates that urine culture and renal ultrasonography should be performed in any infant with electrolyte disturbances to exclude infection or obstructive uropathy.
Assuntos
Pseudo-Hipoaldosteronismo/etiologia , Doenças Uretrais/complicações , Aldosterona/sangue , Diagnóstico Diferencial , Humanos , Hiponatremia/sangue , Hiponatremia/etiologia , Recém-Nascido , Masculino , Pseudo-Hipoaldosteronismo/sangue , Pseudo-Hipoaldosteronismo/diagnóstico por imagem , Estenose Pilórica/sangue , Estenose Pilórica/diagnóstico por imagem , Ultrassonografia , Doenças Uretrais/sangue , Doenças Uretrais/diagnóstico por imagemRESUMO
Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.