Purpose: This study examines quality of life and psychological distress in individuals with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) and Complete Androgen Insensitivity Syndrome (CAIS), two syndromes belon...Purpose: This study examines quality of life and psychological distress in individuals with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) and Complete Androgen Insensitivity Syndrome (CAIS), two syndromes belonging to the field of “disorders of sex development”. Methods: Fifty women with MRKHS and eleven individuals with CAIS participated. The German versions of the World Health Organization Quality of Life Questionnaire (short version, WHOQOL-Bref), the Brief Symptom Inventory (BSI), and the Patient Health Questionnaire (PHQ-D) were used as standardized instruments to measure quality of life and psychopathology. Additional questions concerning demographic variables and suicidality were included. Results: In both patient samples examined, general quality of life reported was in the average range (CAIS: mean z-score = ﹣0.43, SD = 1.05;MRKHS: mean z-score = ﹣0.11, SD = 1.06). The standardized instrument assessment revealed increased psychological distress (proportion of clinical cases according to the BSI: CAIS: 54.5%;MRKHS: 55.1%). A correlation between psychological distress and time span from first suspicion to diagnosis was found in women with MRKHS (Spearman’s rho = 0.35, p = 0.018). Conclusions: The results illustrate the importance of individualised and thorough diagnostics when dealing with patients with MRKHS or CAIS. Psychological distress might be an issue for these individuals and therefore should be considered in treatment and counselling.展开更多
Background:As a congenital malformation that results in infertility and an inability to have vaginal intercourse,Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome places a considerable psychological burden on patients...Background:As a congenital malformation that results in infertility and an inability to have vaginal intercourse,Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome places a considerable psychological burden on patients,which results in anxiety symptoms.However,only single case studies or a few small to medium-sized cross-sectional studies were identified to focus on anxiety symptoms in MRKH patients.Thus,the aim of this study was to explore the status of anxiety symptoms and the related factors in patients with MRKH syndrome.Methods:This cross-sectional study involving 141 patients with MRKH syndrome and 178 healthy women was conducted from January 2018 to December 2018.All participants were required to complete a demographic questionnaire and the Generalized Anxiety Disorder 7-item scale(GAD-7),Patient Health Questionnaire-9,Eysenck Personality Questionnaire-Revised,Short Scale for Chinese,and Chinese Version of the Female Sexual Function Index.The main outcome was the anxiety symptoms measured by the GAD-7.Main outcome was compared between the MRKH syndrome group and the healthy control group.Then,we explored the related factors by comparing patients with and without anxiety symptoms.Results:Of the respondents,24.1%experienced moderate to severe anxiety symptoms.Patients with MRKH syndrome manifested more severe anxiety symptoms than healthy women.Negative self-evaluation of femininity(odds ratio[OR]2.706,95%confidence interval[CI]1.010-7.247),neurotic personality traits(OR 1.100,95%CI 1.029-1.175),and coexisting depressive symptoms(OR 4.422,95%CI 1.498-13.049)were more prevalent in anxious patients.Conclusion:The findings stress the importance of anxiety symptom screening in MRKH patients and identify patients at risk of anxiety symptoms,providing a possible basis for future intervention.展开更多
This review presents an update of Mayer—Rokitansky—Küster—Hauser(MRKH)syndrome on its etiologic,clinical,diagnostic,psychological,therapeutic,and reproductive aspects.The etiology of MRKH syndrome remains uncl...This review presents an update of Mayer—Rokitansky—Küster—Hauser(MRKH)syndrome on its etiologic,clinical,diagnostic,psychological,therapeutic,and reproductive aspects.The etiology of MRKH syndrome remains unclear due to its intrinsic heterogeneity.Nongenetic and genetic causes that may interact during the embryonic development have been proposed with no definitive etiopathogenesis identified.The proportion of concomitant extragenital malformations varies in different studies,and the discrepancies may be explained by ethnic differences.In addition to physical examination and pelvic ultrasound,the performance of pelvic magnetic resonance imaging is crucial in detecting the presence of rudimentary uterine endometrium.MRKH syndrome has long-lasting psychological effects on patients,resulting in low esteem,poor coping strategies,depression,and anxiety symptoms.Providing psychological counseling and peer support to diagnosed patients is recommended.Proper and timely psychological intervention could significantly improve a patient’s outcome.Various nonsurgical and surgical methods have been suggested for treatment of MRKH syndrome.Due to the high success rate and minimal risk of complications,vaginal dilation has been proven to be the first-line therapy.Vaginoplasty is the second-line option for patients experiencing dilation failure.Uterine transplantation and gestational surrogacy are options for women with MRKH syndrome to achieve biological motherhood.展开更多
文摘Purpose: This study examines quality of life and psychological distress in individuals with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) and Complete Androgen Insensitivity Syndrome (CAIS), two syndromes belonging to the field of “disorders of sex development”. Methods: Fifty women with MRKHS and eleven individuals with CAIS participated. The German versions of the World Health Organization Quality of Life Questionnaire (short version, WHOQOL-Bref), the Brief Symptom Inventory (BSI), and the Patient Health Questionnaire (PHQ-D) were used as standardized instruments to measure quality of life and psychopathology. Additional questions concerning demographic variables and suicidality were included. Results: In both patient samples examined, general quality of life reported was in the average range (CAIS: mean z-score = ﹣0.43, SD = 1.05;MRKHS: mean z-score = ﹣0.11, SD = 1.06). The standardized instrument assessment revealed increased psychological distress (proportion of clinical cases according to the BSI: CAIS: 54.5%;MRKHS: 55.1%). A correlation between psychological distress and time span from first suspicion to diagnosis was found in women with MRKHS (Spearman’s rho = 0.35, p = 0.018). Conclusions: The results illustrate the importance of individualised and thorough diagnostics when dealing with patients with MRKHS or CAIS. Psychological distress might be an issue for these individuals and therefore should be considered in treatment and counselling.
文摘Background:As a congenital malformation that results in infertility and an inability to have vaginal intercourse,Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome places a considerable psychological burden on patients,which results in anxiety symptoms.However,only single case studies or a few small to medium-sized cross-sectional studies were identified to focus on anxiety symptoms in MRKH patients.Thus,the aim of this study was to explore the status of anxiety symptoms and the related factors in patients with MRKH syndrome.Methods:This cross-sectional study involving 141 patients with MRKH syndrome and 178 healthy women was conducted from January 2018 to December 2018.All participants were required to complete a demographic questionnaire and the Generalized Anxiety Disorder 7-item scale(GAD-7),Patient Health Questionnaire-9,Eysenck Personality Questionnaire-Revised,Short Scale for Chinese,and Chinese Version of the Female Sexual Function Index.The main outcome was the anxiety symptoms measured by the GAD-7.Main outcome was compared between the MRKH syndrome group and the healthy control group.Then,we explored the related factors by comparing patients with and without anxiety symptoms.Results:Of the respondents,24.1%experienced moderate to severe anxiety symptoms.Patients with MRKH syndrome manifested more severe anxiety symptoms than healthy women.Negative self-evaluation of femininity(odds ratio[OR]2.706,95%confidence interval[CI]1.010-7.247),neurotic personality traits(OR 1.100,95%CI 1.029-1.175),and coexisting depressive symptoms(OR 4.422,95%CI 1.498-13.049)were more prevalent in anxious patients.Conclusion:The findings stress the importance of anxiety symptom screening in MRKH patients and identify patients at risk of anxiety symptoms,providing a possible basis for future intervention.
基金the National Key Research and Development Program of China(Nos.2021YFC2701401 and 2021YFC2701405)the Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences(Nos.2020-PT320-003 and 2021-PT320-001)the National Natural Science Foundation of China(Nos.82171614 and 81830043).
文摘This review presents an update of Mayer—Rokitansky—Küster—Hauser(MRKH)syndrome on its etiologic,clinical,diagnostic,psychological,therapeutic,and reproductive aspects.The etiology of MRKH syndrome remains unclear due to its intrinsic heterogeneity.Nongenetic and genetic causes that may interact during the embryonic development have been proposed with no definitive etiopathogenesis identified.The proportion of concomitant extragenital malformations varies in different studies,and the discrepancies may be explained by ethnic differences.In addition to physical examination and pelvic ultrasound,the performance of pelvic magnetic resonance imaging is crucial in detecting the presence of rudimentary uterine endometrium.MRKH syndrome has long-lasting psychological effects on patients,resulting in low esteem,poor coping strategies,depression,and anxiety symptoms.Providing psychological counseling and peer support to diagnosed patients is recommended.Proper and timely psychological intervention could significantly improve a patient’s outcome.Various nonsurgical and surgical methods have been suggested for treatment of MRKH syndrome.Due to the high success rate and minimal risk of complications,vaginal dilation has been proven to be the first-line therapy.Vaginoplasty is the second-line option for patients experiencing dilation failure.Uterine transplantation and gestational surrogacy are options for women with MRKH syndrome to achieve biological motherhood.
