Health-related quality of life of female patients with congenital adrenal hyperplasia in Malaysia.

BACKGROUND: This study investigates the health-related quality of life (HRQOL) of female patients with congenital adrenal hyperplasia (CAH) in Malaysia. The objectives were to attain socio-demographic and medical data on these Malaysian females with CAH and establish their health-related quality of life (HRQOL) in comparison to age matched diabetic controls. METHODS: A cross-sectional study was conducted over 6 months in the two main tertiary centres for CAH patients in Malaysia. Participants including 59 female-raised CAH patients (mean age ± SD = 16.3 ± 4.2 years, range 10-28 years) compared to 57 age-matched female diabetic patients (mean age ± SD = 16.5 ± 3.4 years, range 10-26 years). Socio-demographic and medical profiles was obtained through semi-structured interviews. HRQOL of participants were evaluated utilising validated, Malay translated questionnaires which were age appropriate: Pediatric Quality of Life Inventory (PedsQL v4.0) scales for Child (8-12) and Adolescent (13-18) and Medical Outcome Survey 36-item Short Form version. These were then compared to the diabetic controls. RESULTS: The CAH participants consisted of children (ages 10-12 years, n = 12), adolescents (ages 13-17 years, n = 29) and adults (≥ 18 years, n = 18). The majority were Malays (64.4%) and had salt-wasting CAH (67.8%). There were no significant differences between the total mean score of the HRQOL of the combined children and adolescents CAH group (total mean score ± SD = 81.6 ± 17.9, 95% CI = 75.6-87.6) when compared to age-matched diabetic patients (total mean score ± SD = 80.8 ± 11.0, 95% CI = 77.0-84.5, P = 0.81, effect size = 0.05); no significant difference between the adult CAH and diabetic controls in the physical [median score (IQR) CAH vs diabetics; 49.3 (11.4) vs. 50.2 (6.1), P = 0.60, effect size = 0.09] and the mental composite scores [median score (IQR) CAH vs. diabetics; 47.8 (14.1) vs. 50.0 (10.8), P = 0.93, effect size = 0.01]. CONCLUSIONS: The HRQOL of the Malaysian CAH cohort were comparable to the diabetic controls.

The Association between Childhood Adversity and Risk of Dysmenorrhea, Pelvic Pain, and Dyspareunia in Adolescents and Young Adults: A Systematic Review.

PURPOSE: Exposure to adverse childhood experiences (ACEs) has been associated with poor health outcomes, including chronic pain. However, little is known about the potential impact on the development of pelvic pain in adolescents and young adults. This systematic review was conducted to explore the association between ACEs and dysmenorrhea, pelvic pain, and dyspareunia in adolescents and young adults. METHODS: Medline, Embase, and PsycNET were searched, using keywords related to childhood adversity, dysmenorrhea, pelvic pain, and dyspareunia. RESULTS: Of the 566 articles identified, 19 studies were included. There was an association between the number and severity of ACEs and the risk of dysmenorrhea. Sexual abuse and posttraumatic stress disorder appeared to be associated with dysmenorrhea, pelvic pain, and dyspareunia, but it was unclear whether this relationship was mediated by poorer mental health. No association was found for immigration and bullying, and findings were inconsistent regarding female genital mutilation, parental separation, and parental death. CONCLUSIONS: Future research should include longitudinal follow-up and use validated tools to assess childhood adversity. A greater understanding of the risk of ACEs among adolescents and young adults with dysmenorrhea, chronic pelvic pain, and dyspareunia could provide insight into the development of these conditions.

A Challenge in Diagnosis and Management: Uterine Torsion in Childhood.

BACKGROUND: Uterine torsion is a rare event, which mostly reported in females with a gravid uterus and is exceptionally rare in children. CASE: A 9-year-old girl presented with 3 days of intermittent lower abdominal pain. Ultrasound revealed an ovarian mass, but laparotomy revealed an ischemic enlarged ovary and uterus rotated 180°. No reperfusion occurred after 60 minutes. A subtotal hysterectomy and right salpingoophorectomy were thus performed. CONCLUSION: Uterine and adnexal torsion presents with symptoms similar to those of adnexal torsion. Delays in diagnosis and referral continue to be an issue, resulting in suboptimal outcomes. Uterine torsion, although exceedingly rare in childhood, appears to occur only in the setting of ovarian masses, which provide the impetus for the rotational force to the elongated cervix of the prepubertal uterus.

A Multicenter Cross-Sectional Study of Malaysian Females With Congenital Adrenal Hyperplasia: Their Body Image and Their Perspectives on Feminizing Surgery.

OBJECTIVE: To explore the impact of congenital adrenal hyperplasia (CAH) on body image in Malaysian females with CAH and to understand the perspectives of these young women and their parents toward feminizing genitoplasty (FG). DESIGN: Multi-center cross-sectional study. SETTING: Two tertiary medical centers in Malaysia. PARTICIPANTS: A total of 59 patients with CAH who were raised as females and more than 10 years old, and their parents. METHODS: The CAH respondents completed the validated and translated Body Image Disturbance Questionnaires (BIDQ). All CAH respondents and their parents underwent semi-structured interviews to explore their views on FG. MAIN OUTCOME MEASURES: Body image disturbance score and perspectives on FG. RESULTS: The 59 CAH respondents consisted of 12 children, 29 adolescents, and 18 adults. The majority were of Malay ethnicity (64.4%) with classical CAH (98.3%) and had undergone FG (n = 55, 93.2%). For the BIDQ scores, the median score (interquartile range) for general body image was 1.29 (0.71), range 1.00-3.29, whereas the genital appearance score was 1.07 (0.39), range 1.00-4.29, revealing a greater concern for general body parts over genitalia. With regards to FG, it was perceived as necessary. Infancy and early childhood were perceived as the best timing for first FG by both respondents and parents, most preferring single-stage over 2-stage surgery. CONCLUSIONS: General body appearance concerns were greater than for genital appearance, with more impact on the patients' lives. Contrary to much international opinion, feminizing surgery was perceived as necessary and appropriate by CAH respondents and their families, and should be offered in infancy or early childhood. Future qualitative studies are recommended.

Malaysian Females With Congenital Adrenal Hyperplasia: Surgical Outcomes and Attitudes.

Background: Girls born with congenital adrenal hyperplasia have virilized external genitalia. There is considerable debate regarding both the outcomes of feminizing genitoplasty and timing of the surgery in this population. Objective: To investigate outcomes of females 46,XX individuals with CAH in Malaysia, the surgical outcomes of feminizing genitoplasty (FG) and their attitudes toward surgery. Study Design: This is a cross-sectional study involving the two main tertiary centers in Malaysia. All 46,XX patients with CAH and raised female, who had undergone FG were identified and invited to participate. Data on socio-demographic, medical profiles, and attitudes toward surgery were collected. A standardized evaluation of the external genitalia was undertaken including the anatomic and cosmetic evaluation by independent gynecologists. Results: Of 61 individuals identified, 59 participated-consisting of children (n = 12), adolescents (n = 29) and adults (n = 18). All but one had classical CAH (98.3%) and had undergone FG (n = 55, 93.2%) with surgery mostly undertaken by pediatric surgeons trained in DSD work (n = 44, 74.6%). Complications overall were low (20.3%), with repeat surgery rate of 9.1%. External genital examination was performed in 38 participants. Overall 36.8% had absent clitoral glands and 39.5% had a persistent urogenital sinus and in 10.5%, no vaginal orifices were seen. Poor cosmetic outcomes were present in 42.1% with 55.3% recommended for further assessment under general anesthetic. Almost half participants did not venture an opinion on FG, those who did varied from having a positive attitude toward it (18 participants) to 3 opining that it should not be done, or avoided or delayed. From the participants, 35.5% preferred FG to be done early in life compared to 44.0% of the parents. Conclusions: The reoperation rates of the feminizing genitoplasty surgeries were low however due to the anatomic and cosmetic outcomes, reassessment of the external genitalia of these CAH patients may be required once they consider becoming sexually active as they may require further treatment. Many factors such as cultural sensitivities and access to medical treatment and late diagnoses have an impact on attitudes toward FG.

The Association Between Unwanted Sexual Experiences and Early-Onset Cervical Cancer and Precancer by Age 25: A Case-Control Study.

BACKGROUND: We examined the association between unwanted sexual experiences and cervical cancer, cervical intraepithelial neoplasia 3, adenocarcinoma in situ, diagnosed ≤25 years of age. METHODS: A case-control study of women ≤55 years who attended gynecological hospitals in Australia between 1983 and 2007. Cases were ≤25 years when diagnosed with disease, control group 1 were "older women" >25 years at diagnosis; control group 2 were "well women" ≤25 years attending preventive health clinics. A self-administered postal survey was utilized. The main outcome measures were prevalence of childhood sexual abuse (<16 years) and unwanted adolescent sexual experiences (between 16 and 18 years) in cases compared to controls. RESULTS: Of 400 contactable subjects, 251 participated (62.8%). Prevalence of childhood sexual abuse in cases (26.6% [25/94]) was similar to other groups. Prevalence of childhood genital contact abuse in cases with cervical cancer was 45.5% [5/11], compared to older women (20% [10/50], p = 0.08) and well women (13.8% [8/58], p = 0.01), and was marginally more common compared to well women when adjusted for other lifestyle factors (odds ratio [OR]: 4.7 [1.0-22.6], p = 0.05). Prevalence of unwanted adolescent sexual experiences in cases was 28.9% [33/114]. Prevalence of adolescent penile-genital contact experiences in cervical cancer cases was 46.7% [7/15], compared to older women (9.4%, [6/64], p < 0.001) and well women (13.7%, [10/73], p = 0.003), and was more common compared to well women when adjusted for lifestyle (OR: 5.9 [1.4-24.9], p = 0.02) and sexual health risk factors (OR: 5.6 [1.4-22.1] p = 0.01). CONCLUSIONS: Unwanted sexual experiences with genital contact were a risk factor for invasive cervical cancer ≤25 years, likely due to a complex interplay of biological and environmental factors.