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五例 17α-羟化酶缺陷症患者临床特征及类固醇激素的质谱测定

Translated title of the contribution: Clinical characteristics of 5 patients with 17α-hydroxylase deficiency and LC-MS / MS method for themeasurement of steroid hormones
  • Gao Beibei
  • , Zhao Lin*
  • , Jiang Jingjing
  • , Guo Wei
  • , Li Xiaomu
  • , Lu Zhiqiang
  • , Li Xiaoying
  • *Corresponding author for this work
  • Fudan University

Research output: Contribution to journalArticlepeer-review

Abstract

Objective To analyze clinical characteristics of 17α-hydroxylase deficiency, and to facilitate the understanding and management of the disease. Methods A retrospective analysis of the clinical characteristics and biochemical results of 5 cases with 17α-hydroxylase deficiency diagnosed and treated from 2018 to 2020. Results All5 patients were female as social gender, and reached adulthood upon first clinic visit to our department and got diagnosed. All 5 cases had hypertension, hypokalemia, bilateral adrenal hyperplasia or adenoma, osteoporosis, and typical hormone changes related to steroid synthesis. Conclusion Steroid hormone tests with liquid chromatographytandem mass spectrometry(LC-MS / MS) enable early diagnosis of 17α-hydroxylase deficiency, assessment of the type and degree of enzyme deficiency, and choice of treatment. For such patients, it is necessary to give appropriate anti-osteoporosis therapy.

Translated title of the contributionClinical characteristics of 5 patients with 17α-hydroxylase deficiency and LC-MS / MS method for themeasurement of steroid hormones
Original languageChinese (Traditional)
Pages (from-to)132-138
Number of pages7
JournalChinese Journal of Endocrinology and Metabolism
Volume38
Issue number2
DOIs
StatePublished - 25 Feb 2022
Externally publishedYes

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

  1. SDG 3 - Good Health and Well-being
    SDG 3 Good Health and Well-being

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