Urine Steroid Hormone Profile Analysis in Cytochrome P450 Oxidoreductase Deficiency: Implication for the Backdoor Pathway to Dihydrotestosterone (original) (raw)

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1Departments of Laboratory Medicine (K.H.) Tokyo 160-8582, Japan

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2Pediatrics (T.H.), Keio University School of Medicine, Tokyo 160-8582, Japan

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3Department of Pediatrics, Dokkyo University School of Medicine Koshigaya Hospital (T.N.), Koshigaya 343-8555, Japan

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4Division of Endocrinology and Metabolism, Kanagawa Children’s Medical Center (M.A.), Yokohama 232-8555, Japan

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5Division of Endocrinology and Metabolism, National Center for Child Health and Development (R.H.), Tokyo 157-8535, Japan

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6Department of Pediatrics, Tohoku University School of Medicine (I.F.), Sendai 980-0878, Japan

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7Department of Pediatrics, Hokkaido University School of Medicine (T.T.) Sapporo 060-8638, Japan

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8Department of Pediatrics, Shirakawa Kousei General Hospital (R.T.), Shirakawa 961-0907, Japan

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9Department of Endocrinology and Metabolism, National Research Institute for Child Health and Development (M.F., T.O.), Tokyo 157-8535, Japan

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9Department of Endocrinology and Metabolism, National Research Institute for Child Health and Development (M.F., T.O.), Tokyo 157-8535, Japan

*Address all correspondence and requests for reprints to: Dr. T. Ogata, Department of Endocrinology and Metabolism, National Research Institute for Child Health and Development, 2-10-1 Ohkura, Setagaya, Tokyo 157-8535, Japan.

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Received:

10 November 2005

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Keiko Homma, Tomonobu Hasegawa, Toshiro Nagai, Masanori Adachi, Reiko Horikawa, Ikuma Fujiwara, Toshihiro Tajima, Ryoujun Takeda, Maki Fukami, Tsutomu Ogata, Urine Steroid Hormone Profile Analysis in Cytochrome P450 Oxidoreductase Deficiency: Implication for the Backdoor Pathway to Dihydrotestosterone, The Journal of Clinical Endocrinology & Metabolism, Volume 91, Issue 7, 1 July 2006, Pages 2643–2649, https://doi.org/10.1210/jc.2005-2460
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Abstract

Context: Although the “backdoor” pathway to dihydrotestosterone has been postulated in the fetal-to-early-infantile period of patients with cytochrome P450 oxidoreductase deficiency (PORD), clinical data in support of this pathway remain limited.

Objective: The objective of this study was to obtain clinical evidence for the presence of the backdoor pathway in PORD.

Setting: This was a collaboration study between laboratories and hospitals.

Subjects: Twenty-two Japanese patients with molecularly confirmed PORD and 1763 control subjects participated in this study.

Intervention: Urine steroid profile analysis was performed by gas chromatography/mass spectrometry. In five patients and 776 control subjects, urine samples were obtained before 12 months of age.

Main Outcome Measure: The main outcome measure was identification of a urine steroid(s) indicating the backdoor pathway.

Results: In the PORD patients, pregnanediol, pregnanetriolone, and pregnanetriol were obviously elevated, and the urine steroid ratios reflecting CYP17A1 and CYP21A2 activities were decreased throughout the examined ages. Furthermore, etiocholanolone and 11-hydroxyandrosterone, which should originate almost exclusively from androstenedione in the conventional “frontdoor” pathway, were grossly normal or somewhat decreased since early infancy, whereas androsterone, which can be derived not only from androstenedione and dihydrotestosterone in the conventional frontdoor pathway but also from 5α-pregnane-3α,17α-diol-20-one in the backdoor pathway, was increased during early infancy and remained grossly normal thereafter. Thus, the androsterone to etiocholanolone ratio was increased during early infancy and remained grossly normal thereafter. 5α-Pregnane-3α,17α-diol-20-one was elevated throughout the examined ages.

Conclusions: The increased androsterone excretion during early infancy, as compared with the etiocholanolone and 11-hydroxyandrosterone excretions in the same period, suggests the presence of the backdoor pathway in PORD.

Copyright © 2006 by The Endocrine Society

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