title: [Pseudohypoaldosteronism type 1 in an infant with bilateral primary obstructive megaureter : a case report].
abstract: A 35-day-old male infant was referred to our hospital for bilateral hydroureteronephrosis on postnatal ultrasonography. He was born at 37 weeks and 2 days of gestation, weighing 2,765 g. He was diagnosed with bilateral primary obstructive megaureter. During observation, at 3 months and 2 weeks of age, he presented with dehydration and showed hyponatremia and hyperkalemia. An endocrinological examination led to a diagnosis of pseudohypoaldosteronism type 1 (PHA1). After bilateral percutaneous nephrostomy, PHA1 resolved. He underwent bilateral ureteral tapering and ureteroneocystostomy at 5 months and 3 weeks of age. Since PHA1 recurred after removal of urethral catheter, urethral catheter and bilateral double-J-stent was kept until 8 months old. He was followed-up until 3 years old with no signs of PHA1 recurrence. For an infant with obstructive uropathy or vesicoureteral reflux showing hyponatremia and hyperkalemia, PHA1 should be considered.
authors.affiliation: The Department of Urology, Kyoto University, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Makino
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kanematsu
authors.forename: Masaaki
authors.initials: M
authors.lastname: Imamura
authors.forename: Koji
authors.initials: K
authors.lastname: Yoshimura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nishiyama
authors.forename: Tohru
authors.initials: T
authors.lastname: Yorifuji
authors.forename: Osamu
authors.initials: O
authors.lastname: Ogawa
mesh_terms: D006801:Humans; D007223:Infant; D008297:Male; D009403:Nephrostomy, Percutaneous; D011546:Pseudohypoaldosteronism / Q000209:etiology*; D015607:Stents; D014513:Ureter / Q000002:abnormalities* / Q000601:surgery; D014517:Ureteral Obstruction / Q000209:etiology