Liotta A, Maggio M C, Iachininoto R, Bellipanni P F, Calì G, Arena V, Arena E
Dipartimento Materno-Infantile, Università di Palermo.
Pediatr Med Chir. 2003 Sep-Oct;25(5):375-7.
PHA is a rare cause of hydramnios, characterized by increased amniotic fluid levels of aldosterone and sodium. Two distinct genetic entities (PHA type I and PHA type II) are included. Both are stemmed by a target organ defect with diminished renal tubular responsiveness to aldosterone. The AA present a case in which pregnancy resulted in a preterm infant with severe hydramnios, metabolic acidosis, hyponatriemia, hyperkaliemia. Salt and fluid replacement significantly improved clinical and metabolic condition. However a growth deficiency (-2 SDS) persists at follow-up.
原发性醛固酮增多症(PHA)是羊水过多的罕见病因,其特征是羊水内醛固酮和钠水平升高。它包括两种不同的遗传类型(I型PHA和II型PHA)。两者均由肾小管对醛固酮反应性降低的靶器官缺陷引起。AA报告了一例妊娠导致早产婴儿出现严重羊水过多、代谢性酸中毒、低钠血症、高钾血症的病例。补充盐和液体显著改善了临床和代谢状况。然而,随访时生长发育迟缓(标准差评分-2)仍然存在。