Long-term effects of surgically corrected menstrual outflow obstruction: A case-control study.

Objective: To enhance evidence-based knowledge on long-term sequalae in patients with surgically corrected obstructing Müllerian anomalies.

Methods: This long-term case-control study included patients with menstrual outflow obstruction due to congenital anomalies of the uterus or vagina, who were at least 18 years old, and for whom 2 years had elapsed since the first surgery at the start of this study. The control group consisted of women without current gynecological problems.

TurnerFertility trial: fertility preservation in young girls with Turner syndrome by freezing ovarian cortex tissue-a prospective intervention study.

Objective: To evaluate which girls with Turner syndrome (TS) could benefit from fertility preservation by ovarian tissue cryopreservation on the basis of karyotype, puberty status, and hormonal data.

Design: Prospective intervention study; participants were included between 2018 and 2020.

Setting: Tertiary hospital in the Netherlands.

[Children and adolescents with gender questions: dilemmas in the hormonal treatment by multidisciplinary teams].

In the Netherlands but also in many other countries, there is an increasing social discussion about gender identity and gender diversity, and an increasing number of children and adolescents are seeking medical help because of questions about their gender identity. The cause of this increase is still unknown. Gender questions are diverse and require an individual approach by a multidisciplinary team.

Primary Amenorrhea with Apparently Absent Uterus: A Report of Three Cases.

Background: The apparent absence of a uterus upon imaging women with primary amenorrhea appears to lead to a high risk of misdiagnosis, which will lead to significant mental distress in patients.

Case: Three young females with primary amenorrhea were referred with a diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome based on radiological findings of an apparently absent uterus. In two patients, the absence of the uterus could be confirmed, but with various diagnoses.

External quality control and training of semen analysis in the Netherlands: starting point for further reduction of outcome variability.

Semen analysis is characterized by high levels of intra- and inter-laboratory variability, due to a low level of standardization, high subjectivity of the assessments, and problems with automated procedures. To improve consistency of laboratory results, quality control and training of technicians are important requisites. The goals of this study are to evaluate the results of an external quality control (EQC) program and standardized training by ESHRE Basic Semen Analysis Courses (BSAC) on the variability in manual assessments of semen parameters.

Iatrogenic and non-iatrogenic causes of female fertility loss that may indicate fertility preservation.

Due to advances in fertility preservation options and improvements in life expectancy, there is growing need for fertility preservation counseling for women at risk for premature ovarian insufficiency. The aim of this review is to provide an overview of various causes of female fertility loss and to raise awareness for counseling women about their options for fertility preservation. Furthermore, for counseling of women not only at the time of gonadotoxic treatment but also post-treatment and also of women with other benign causes of increased risk for impaired fertility or premature ovarian insufficiency.

Pelvic organ prolapse and collagen-associated disorders.

Introduction And Hypothesis: Pelvic organ prolapse (POP) and other disorders, such as varicose veins and joint hypermobility, have been associated with changes in collagen strength and metabolism. We hypothesized that these various disorders were more prevalent in both POP patients and their family members.

Methods: In this study, the prevalence of various collagen-associated disorders, including POP, was compared between POP patients (n = 110) and control patients (n = 100) and their first and second degree family members.

Widespread non-central nervous system organ pathology in fragile X premutation carriers with fragile X-associated tremor/ataxia syndrome and CGG knock-in mice.

Fragile X-associated tremor/ataxia syndrome (FXTAS) is an adult-onset neurodegenerative disorder generally presenting with intention tremor and gait ataxia, but with a growing list of co-morbid medical conditions including hypothyroidism, hypertension, peripheral neuropathy, and cognitive decline. The pathological hallmark of FXTAS is the presence of intranuclear inclusions in both neurons and astroglia. However, it is unknown to what extent such inclusions are present outside the central nervous system (CNS).

Predictors and risk model development for menopausal age in fragile X premutation carriers.

Purpose: Women who carry a fragile X mental retardation 1 premutation are at risk for fragile X-associated primary ovarian insufficiency and should be counseled for a potentially reduced fertility. Multiple factors can affect the age of onset and severity of fragile X-associated primary ovarian insufficiency. In this study, we assessed the predictive power of several factors with menopausal age, a surrogate measure of onset of fragile X-associated primary ovarian insufficiency.

X chromosome inactivation does not define the development of premature ovarian failure in fragile X premutation carriers.

Since only 20% of female fragile X premutation carriers develop premature ovarian failure (POF, i.e., amenorrhea before age of 40 years), and since X chromosome inactivation (XCI) determines the phenotypic severity of full mutation women, we reasoned that the development of POF in fragile X premutation carriers could be due to skewed XCI (XCI ratio >80:20).

The bone-anchored hearing aid: quality-of-life assessment.

Objectives: To assess the impact of a bone-anchored hearing aid (BAHA) on the quality of life (QOL) of adults and to test the hypothesis that a BAHA improves QOL because otorrhea and/or skin irritations decrease.

Design: Prospective postal-based questionnaire study using validated health-related QOL instruments, combined with hearing-aid-related questions.

Patients And Methods: The study included 56 consecutive adult patients with acquired conductive or mixed hearing loss who were scheduled for BAHA implantation at the University Medical Centre Nijmegen, Nijmegen, the Netherlands.

A view from the bridge: agreement between the SF-6D utility algorithm and the Health Utilities Index.

Background: The SF-6D is a new health state classification and utility scoring system based on 6 dimensions ('6D') of the Short Form 36, and permits a "bridging" transformation between SF-36 responses and utilities. The Health Utilities Index, mark 3 (HUI3) is a valid and reliable multi-attribute health utility scale that is widely used. We assessed within-subject agreement between SF-6D utilities and those from HUI3.

Cost effectiveness of implantable cardioverter defibrillator therapy versus drug therapy for patients at high risk of sudden cardiac death.

The implantable cardioverter defibrillator (ICD) is a therapy for patients at risk of sudden cardiac death due to ventricular tachycardia (VT) or ventricular fibrillation (VF). But the apparent high cost of ICD therapy relative to antiarrhythmic drugs such as amiodarone has raised questions about the cost effectiveness of ICD therapy versus drug therapy. To inform this debate we reviewed the literature on ICD cost effectiveness.