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Autores principales: Auriane Cospain, Ludivine Dion, Maud Bidet, Krystel Nyangoh Timoh, Chloé Quelin, Isis Carton, Alinoe Lavillaureix, Karine Morcel, Paul Rollier, Laurent Pasquier, Bénédicte Nouyou, Sylvie Odent, Daniel Guerrier, Erika Launay, Marc‐Antoine Belaud Rotureau, Mélanie Fradin, Sylvie Jaillard, Vincent Lavoué
Formato: Artículo Open Access
Publicado: Wiley 2024
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Acceso en línea:https://obgyn.onlinelibrary.wiley.com/doi/10.1111/aogs.14985
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author Auriane Cospain
Ludivine Dion
Maud Bidet
Krystel Nyangoh Timoh
Chloé Quelin
Isis Carton
Alinoe Lavillaureix
Karine Morcel
Paul Rollier
Laurent Pasquier
Bénédicte Nouyou
Sylvie Odent
Daniel Guerrier
Erika Launay
Marc‐Antoine Belaud Rotureau
Mélanie Fradin
Sylvie Jaillard
Vincent Lavoué
author_facet Auriane Cospain
Ludivine Dion
Maud Bidet
Krystel Nyangoh Timoh
Chloé Quelin
Isis Carton
Alinoe Lavillaureix
Karine Morcel
Paul Rollier
Laurent Pasquier
Bénédicte Nouyou
Sylvie Odent
Daniel Guerrier
Erika Launay
Marc‐Antoine Belaud Rotureau
Mélanie Fradin
Sylvie Jaillard
Vincent Lavoué
Auriane Cospain
Ludivine Dion
Maud Bidet
Krystel Nyangoh Timoh
Chloé Quelin
Isis Carton
Alinoe Lavillaureix
Karine Morcel
Paul Rollier
Laurent Pasquier
Bénédicte Nouyou
Sylvie Odent
Daniel Guerrier
Erika Launay
Marc‐Antoine Belaud Rotureau
Mélanie Fradin
Sylvie Jaillard
Vincent Lavoué
collection Wiley Open Access
contents Optimizing care for MRKH patients: From malformation screening to uterus transplantation eligibility Auriane Cospain Ludivine Dion Maud Bidet Krystel Nyangoh Timoh Chloé Quelin Isis Carton Alinoe Lavillaureix Karine Morcel Paul Rollier Laurent Pasquier Bénédicte Nouyou Sylvie Odent Daniel Guerrier Erika Launay Marc‐Antoine Belaud Rotureau Mélanie Fradin Sylvie Jaillard Vincent Lavoué Acta Obstetricia et Gynecologica Scandinavica AbstractIntroductionMayer‐Rokitansky‐Küster‐Hauser (MRKH) syndrome with utero‐vaginal aplasia is the most severe form of the Müllerian duct anomalies and can be associated with extra‐genital abnormalities such as renal or skeletal anomalies, hearing loss, or cardiac defects. The past two decades have witnessed significant advances both in understanding the etiologies of MRKH and in the development of fertility treatments such as uterine transplantation. The present work aimed to determine the rate of women with MRKH syndrome who underwent optimal initial management (after comprehensive malformation assessment) and to establish the rate of patients eligible for uterine transplantation (i.e., those with a vaginal length ≥7 cm without reconstruction using a bowel segment, and an anti‐Müllerian hormone level >1.5 ng/mL before 35 years).Material and MethodsCohort study of 85 women with MRKH syndrome consulting in our tertiary center.Results62.4% of women with MRKH syndrome had an exhaustive malformative evaluation according to the French guidelines (Protocole National de Diagnostic et de Soin [PNDS]), of which 76.5% had associated malformations (MRKH type II). Pedigree, when available, showed a family history of infertility or a urogenital tract spectrum anomaly in 60% of cases. Concerning the uterine transplantation selection criteria, when evaluated, 22.6% of women had an anti‐Müllerian hormone level <1.5 ng/mL and 36% a vaginal length <7 cm. On the 21 women with complete evaluation of both primary and secondary outcomes, 14 of them would be eligible for a uterine transplantation program at the time of consultation according to the main inclusion criteria of uterine transplantation program.ConclusionsWomen with MRKH syndrome are often inadequately explored for associated malformations. Early assessment and monitoring of the ovarian reserve is key for fertility preservation, especially in the era of uterine transplantation. 10.1111/aogs.14985 http://creativecommons.org/licenses/by-nc/4.0/
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spellingShingle Optimizing care for MRKH patients: From malformation screening to uterus transplantation eligibility
Auriane Cospain
Ludivine Dion
Maud Bidet
Krystel Nyangoh Timoh
Chloé Quelin
Isis Carton
Alinoe Lavillaureix
Karine Morcel
Paul Rollier
Laurent Pasquier
Bénédicte Nouyou
Sylvie Odent
Daniel Guerrier
Erika Launay
Marc‐Antoine Belaud Rotureau
Mélanie Fradin
Sylvie Jaillard
Vincent Lavoué
Acta Obstetricia et Gynecologica Scandinavica
Optimizing care for MRKH patients: From malformation screening to uterus transplantation eligibility Auriane Cospain Ludivine Dion Maud Bidet Krystel Nyangoh Timoh Chloé Quelin Isis Carton Alinoe Lavillaureix Karine Morcel Paul Rollier Laurent Pasquier Bénédicte Nouyou Sylvie Odent Daniel Guerrier Erika Launay Marc‐Antoine Belaud Rotureau Mélanie Fradin Sylvie Jaillard Vincent Lavoué Acta Obstetricia et Gynecologica Scandinavica AbstractIntroductionMayer‐Rokitansky‐Küster‐Hauser (MRKH) syndrome with utero‐vaginal aplasia is the most severe form of the Müllerian duct anomalies and can be associated with extra‐genital abnormalities such as renal or skeletal anomalies, hearing loss, or cardiac defects. The past two decades have witnessed significant advances both in understanding the etiologies of MRKH and in the development of fertility treatments such as uterine transplantation. The present work aimed to determine the rate of women with MRKH syndrome who underwent optimal initial management (after comprehensive malformation assessment) and to establish the rate of patients eligible for uterine transplantation (i.e., those with a vaginal length ≥7 cm without reconstruction using a bowel segment, and an anti‐Müllerian hormone level >1.5 ng/mL before 35 years).Material and MethodsCohort study of 85 women with MRKH syndrome consulting in our tertiary center.Results62.4% of women with MRKH syndrome had an exhaustive malformative evaluation according to the French guidelines (Protocole National de Diagnostic et de Soin [PNDS]), of which 76.5% had associated malformations (MRKH type II). Pedigree, when available, showed a family history of infertility or a urogenital tract spectrum anomaly in 60% of cases. Concerning the uterine transplantation selection criteria, when evaluated, 22.6% of women had an anti‐Müllerian hormone level <1.5 ng/mL and 36% a vaginal length <7 cm. On the 21 women with complete evaluation of both primary and secondary outcomes, 14 of them would be eligible for a uterine transplantation program at the time of consultation according to the main inclusion criteria of uterine transplantation program.ConclusionsWomen with MRKH syndrome are often inadequately explored for associated malformations. Early assessment and monitoring of the ovarian reserve is key for fertility preservation, especially in the era of uterine transplantation. 10.1111/aogs.14985 http://creativecommons.org/licenses/by-nc/4.0/
title Optimizing care for MRKH patients: From malformation screening to uterus transplantation eligibility
topic Acta Obstetricia et Gynecologica Scandinavica
url https://obgyn.onlinelibrary.wiley.com/doi/10.1111/aogs.14985