Abstract
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.
Highlights
Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) and Complete Androgen Insensitivity Syndrome (CAIS) are classified as “disorders of sex development” (DSD), and are further defined as “congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical” [1]
Given the current paucity in data reporting quality of life and psychological distress in CAIS and MRKHS, the purpose of the present study was to examine the impact of these diagnoses later in life and compare the two diagnostic groups with each other, and to non-clinical samples previously reported in the literature
Results for the Brief Symptom Inventory (BSI) and World Health Organization Quality of Life (WHOQOL)-Bref are reported as standardized values to allow comparison to the nonclinical population
Summary
Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) and Complete Androgen Insensitivity Syndrome (CAIS) are classified as “disorders of sex development” (DSD), and are further defined as “congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical” [1]. Due to this atypical development, at birth individuals with MRKHS and CAIS have an unremarkable female appearance and are raised as girls, but are unable to carry a pregnancy. Two predominating issues are described in the literature and are often the central focus of medical treatment: First, the inability to engage in vaginal intercourse without some form of treatment becomes problematic. The majority of the medical literature has focused on treatments to create a so-called neovagina in order to allow sexual intercourse; various methods have been described, ranging from non-surgical approaches to different surgical procedures [4] [5]
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