No abstract

Background Females with congenital adrenal hyperplasia (CAH) and atypical genitalia often undergo complex surgeries; however, their outcomes remain largely uncertain. Methods We searched several databases through 8 March 2016 for studies evaluating genital reconstructive surgery in females with CAH. Reviewers working independently and in duplicate selected and appraised the studies. Results We included 29 observational studies (1178 patients, mean age at surgery, 2.7 ± 4.7 years; mostly classic CAH). After an average follow-up of 10.3 years, most patients who had undergone surgery had a female gender identity (88.7%) and were heterosexual (76.2%). Females who underwent surgery reported a sexual function score of 25.13 using the Female Sexual Function Index (maximum score, 36). Many patients continued to complain of substantial impairment of sensitivity in the clitoris, vaginal penetration difficulties, and low intercourse frequency. Most patients were sexually active, although only 48% reported comfortable intercourse. Most patients (79.4%) and treating health care professionals (71.8%) were satisfied with the surgical outcomes. Vaginal stenosis was common (27%), and other surgical complications, such as fistulas, urinary incontinence, and urinary tract infections, were less common. Data on quality of life were sparse and inconclusive. Conclusion The long-term follow-up of females with CAH who had undergone urogenital reconstructive surgery shows variable sexual function. Most patients were sexually active and satisfied with the surgical outcomes; however, some patients still complained of impairment in sexual experience and satisfaction. The certainty in the available evidence is very low.

Severe obesity is associated with abnormal lipids and increased risk for cardiovascular disease. Obesity is a risk factor for vitamin D deficiency. We examined relationship between 25-hydroxy vitamin D (25(OH)D) concentrations and lipids in children with severe obesity. Medical records of 376 children were reviewed. Linear regression models and logistic regression were used to examine the relationship between 25(OH)D and lipids after adjustment for age, gender, season of blood draw, body mass index (BMI) z-score, and BMI % of 95th percentile. Two-hundred sixty-three out of 376 children (70%) had 25(OH)D concentrations < 30 ng/mL. Concentrations of 25(OH)D were positively correlated with those of high-density lipoprotein cholesterol (HDL-C) (r2 = 0.08, r = 0.22, β = 0.16, 95% CI = 0.05–0.27, p = 0.004). HDL-C was lower in children with 25(OH)D < 30 ng/mL (n = 263) compared to those with 25(OH)D ≥ 30 ng/mL (n = 113) (41.3 ± 10.2 vs. 46.4 ± 12 mg/dL, p < 0.0001). Children with 25(OH)D concentrations < 30 ng/mL had greater adjusted odds of low HDL-C (<40 mg/dL) compared with those with 25(OH)D ≥ 30 ng/mL (47.9% vs. 29.2%, OR 2.15 (1.33–3.51), p = 0.0019). Total cholesterol and non-HDL-C were not correlated with 25(OH)D concentrations. Vitamin D deficiency is highly prevalent in children with severe obesity. Prospective clinical trials are warranted to determine if vitamin D supplementation can improve HDL-C and potentially decrease risk for cardiovascular disease in children with obesity.

Mild SCH in children and adolescents was associated with higher rates of elevated total cholesterol and elevated non-HDL cholesterol. Randomized placebo controlled studies are warranted to determine if treatment of mild SCH in children leads to improvement in lipid profile.

Rationale, aims, and objectives: Waist circumference (WC) and waist-to-height ratio (WHtR) are superior surrogate markers of central obesity than body mass index.However, WC is not measured routinely in paediatric clinics. The objective of this study was to implement measurement of WC during routine assessment of children in an ambulatory outpatient clinic setting and subsequent dissemination of cardiometabolic risk counselling in children with central obesity (defined as WHtR ≥0.5). Method: Prospective cohort of patients aged 6 to 20 years. Study period was divided into three phases: baseline (3 months), process improvement (2 months), and implementation (6 months). Define-Measure-Analyse-Improve-Control (DMAIC) strategy was applied. Measurement of WC was implemented as a component of the physical examination in patients. Outcome measures were (1) improvement in frequency of WC measurement and (2) utilization of WHtR in cardiometabolic risk counselling.Results: Waist circumference was not measured in any patient during baseline phase (n = 551). During process improvement phase, of the total 347 patients, WC was measured in 35% vs target of 30%. In the implementation phase, WC was measured in 37% patients (365 out of 964). Of these 365 patients, 175 (48%) had elevated WHtR, and 73% of them (n = 128) were counselled about their increased cardiometabolic risk. Conclusions:Application of an evidence-based DMAIC protocol led to significant improvement in assessment for central obesity in an ambulatory clinic practice and appropriate counselling regarding cardiometabolic risk reduction in children and adolescents with central obesity over an 8-month period. Meticulous planning and execution, frequent reinforcement, and integrating feedback from the involved multi-disciplinary team were important factors in successful implementation of this quality improvement project.