Ovarian hyperstimulation syndrome with low oestradiol in non-classical 17 alpha-hydroxylase, 17,20-lyase deficiency: what is the role of oestrogens?

Human Reproduction
D MeirowA Rosler

Abstract

Ovarian stimulation in a patient who suffered from partial (non-classical) 17 alpha-hydroxylase 17,20 lyase deficiency of the adrenal cortex and gonads is described. Diagnosis was based on measurements of high concentrations of steroid metabolites proximal to the enzymatic block (progesterone, 17-hydropregnenolone and 17-hydroprogesterone); with further rise following adrenocorticotrophic hormone (A-CTH) stimulation, and low steroid concentrations distal to the block. Her basal plasma oestradiol values were low and did not rise even during repeated treatment cycles with maximal ovarian stimulation. However, clinical presentation of ovarian hyperstimulation syndrome (OHSS) developed despite very low oestradiol concentrations, thus seriously questioning the role of oestradiol in the pathogenesis of this condition. The poor correlation between clinical presentation of OHSS and plasma oestradiol values, as presented in this case, supports other data which conclude that oestradiol measurements alone are not sufficient to alert the physician to the possible development of OHSS.

Citations

Feb 18, 2004·Fertility and Sterility·Tommaso FalconeJeffery M Goldberg
Jul 31, 1999·European Journal of Obstetrics, Gynecology, and Reproductive Biology·J G Schenker
Jan 29, 1999·Endocrinology and Metabolism Clinics of North America·S N KalantaridouL M Nelson
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May 1, 2006·Women's Health·Mohamed A Bedaiwy, Tommaso Falcone
Feb 15, 2002·Human Fertility : Journal of the British Fertility Society·Rina Agrawal
Apr 30, 2005·Journal of Medical Primatology·Nobuhiko YoshimotoIkuo Hayasaka
Dec 28, 1999·Human Reproduction·P E Egbase
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Nov 10, 2004·Cancer·Carina G HildersJ Baptist Trimbos
Aug 26, 2014·American Journal of Reproductive Immunology : AJRI·Yechiel S Mor, Joseph G Schenker

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