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Background <p>Pseudohypoaldosteronism (PHA) is a rare disorder characterized by aldosterone resistance, leading to hyperkalemia, hyponatremia, and metabolic acidosis. It typically presents in infancy and often resolves spontaneously in childhood. Reactivation or recurrence during pregnancy is extremely rare and poses significant challenges for maternal and fetal management.</p> Case <p>A 34-year-old woman pregnant G5P4 presented with threatened preterm labor and pruritus at 32 weeks of gestation. Laboratory findings revealed severe hyperkalemia (K + 6.2 mmol/L), hyponatremia (Na + 124 mmol/L). Her medical history was notable for childhood-onset pseudohypoaldosteronism, which resolved by the age of 5. Growth scan revealed fetal growth restriction, electrolyte disturbances were treated with insulin/dextrose infusion, sodium bicarbonate, and calcium gluconate, resulting in normalization of potassium levels. A 2190-gram (7%) female infant was delivered at 36 weeks of gestation for non-reassuring fetal CTG via uncomplicated cesarean section; the newborn had normal Apgar scores and umbilical cord pH.</p> Conclusions <p>To the best of our knowledge, this is the first documented case of reactivation of PHA 1 during pregnancy. The possibility that pregnancy can induce the recurrence of PHA underscores the importance of close monitoring and multidisciplinary management approach to improve maternal electrolyte complications and adverse fetal outcomes, such as growth restriction. Further studies are needed to better understand this rare phenomenon and improve care.</p>

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Repeated pseudohypoaldosteronism (PHA1) in pregnancy associated with fetal growth restriction: case report and review of the literature

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月24日

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  • Hudi Youshaa,
  • Yehia El Khawly,
  • Saheed Shittu,
  • Stephen Beer,
  • Mohamed Abohafs,
  • Sanaa Al Hassan,
  • Kholode Al-Maslamani,
  • Lolwa Alansari,
  • Abdul Hadi Salem,
  • Hakem Nazdaf

摘要

Background

Pseudohypoaldosteronism (PHA) is a rare disorder characterized by aldosterone resistance, leading to hyperkalemia, hyponatremia, and metabolic acidosis. It typically presents in infancy and often resolves spontaneously in childhood. Reactivation or recurrence during pregnancy is extremely rare and poses significant challenges for maternal and fetal management.

Case

A 34-year-old woman pregnant G5P4 presented with threatened preterm labor and pruritus at 32 weeks of gestation. Laboratory findings revealed severe hyperkalemia (K + 6.2 mmol/L), hyponatremia (Na + 124 mmol/L). Her medical history was notable for childhood-onset pseudohypoaldosteronism, which resolved by the age of 5. Growth scan revealed fetal growth restriction, electrolyte disturbances were treated with insulin/dextrose infusion, sodium bicarbonate, and calcium gluconate, resulting in normalization of potassium levels. A 2190-gram (7%) female infant was delivered at 36 weeks of gestation for non-reassuring fetal CTG via uncomplicated cesarean section; the newborn had normal Apgar scores and umbilical cord pH.

Conclusions

To the best of our knowledge, this is the first documented case of reactivation of PHA 1 during pregnancy. The possibility that pregnancy can induce the recurrence of PHA underscores the importance of close monitoring and multidisciplinary management approach to improve maternal electrolyte complications and adverse fetal outcomes, such as growth restriction. Further studies are needed to better understand this rare phenomenon and improve care.