A case of pregnancy and delivery complicated with salt-wasting congenital adrenal hyperplasia

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  • SATO Noriko
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • SHIOJI Mitsunori
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • KUBOTA Satoshi
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • YAMASHITA Michiko
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • TAKAHASHI Ryoko
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • FUJITANI Mayumi
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • KAMACHI Keiichi
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • TSUJIE Tomoko
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital
  • TOKUHIRA Atsushi
    Department of Obstetrics and Gynecology, Toyonaka Municipal Hospital

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Other Title
  • 先天性副腎皮質過形成（21-水酸化酵素欠損症　塩類喪失型）の妊娠分娩の1症例
  • 症例報告 先天性副腎皮質過形成(21-水酸化酵素欠損症 塩類喪失型)の妊娠分娩の1症例
  • ショウレイ ホウコク センテンセイ フクジン ヒシツ カケイセイ(21-スイサンカ コウソ ケッソンショウ エンルイ ソウシツガタ)ノ ニンシン ブンベン ノ 1 ショウレイ

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Congenital adrenal hyperplasia (CAH) is an inherited defect of cortisol biosynthesis. More than 90% of CAH cases are caused by 21-hydroxylase deficiency (21-OHD). Complete 21-OHD leads to the classical salt-wasting form, characterized by decreased cortisol and aldosterone production and androgen excess, leading to prenatal virilization of external genitalia in female fetuses and salt-wasting in both sexes. Fertility prognosis in women with classical 21-OHD is low, especially in the salt-wasting form. We report a case of pregnancy and delivery complicated with salt-wasting CAH. The patient was a 26-year-old female with CAH and salt-wasting due to 21-OHD. She had been diagnosed with salt-wasting CAH in the first month of life when she presented with dehydration, vomiting, hyperkalemia, and ambiguous external genitalia. She was treated with glucocorticoid and mineral corticoid. Surgical repair of the external genitalia was performed at 6 years, followed by vaginoplasty at 11 years. She was married at 25 years and became pregnant spontaneously at 26 years. The pregnancy was uneventful. The dose of medication remained unchanged throughout the pregnancy. Because of extensive prior vaginal reconstruction, she underwent elective caesarian section at 38 weeks gestation and delivered a healthy 2694g male infant. Before the caesarian section, 100mg hydrocortisone was administrated intravenously. She and her baby were discharged from the hospital 7 days after delivery uneventfully. [Adv Obstet Gynecol, 64 (1) : 49-53, 2012 (H24.2)]

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