Elimination of Pain and Improvement of Exercise Capacity in Camurati-Engelmann Disease With Losartan

Background: Camurati-Engelmann disease (CED) is a rare disorder, with approximately 250 described cases in the literature. Treatment options are limited and have been suboptimal so far

Metabolic, cardiovascular and anthropometric differences between prepubertal girls and boys

Summary Objective We aimed to assess possible differences in insulin sensitivity and other metabolic, anthropometric and cardiovascular parameters between boys and girls prior to puberty. Methods We studied 85 healthy prepubertal children (33 girls and 52 boys) aged 8Á7 AE 1Á9 years (range 4Á0-11Á9 years), born 38-40 weeks gestation, and of birth weight appropriatefor-gestational-age. Insulin sensitivity was measured using frequently sampled intravenous glucose tests and Bergman's minimal model. Other clinical assessments included anthropometric measures, fasting lipid and hormonal profiles, body composition from whole-body dual-energy X-ray absorptiometry and 24-h ambulatory blood pressure monitoring. Results Prepubertal girls and boys were of similar parentadjusted height SDS (P = 0Á26), but girls had considerably more body fat (P < 0Á0001), less fat-free mass (P = 0Á0002) and greater abdominal adiposity (P < 0Á0001). These differences in body composition were independent of adrenal androgens. Insulin sensitivity was 18% lower in girls (11Á0 vs 13Á4 9 10 À4 /min (mU/l); P = 0Á028), but this difference disappeared with adjustment for adiposity and DHEAS concentrations. There were, however, some apparent sex differences in cardiovascular parameters, with girls displaying increased heart rate and reduced blood pressure dipping. Girls also had higher triglyceride concentrations (+23%; P = 0Á036). Conclusion There are a number of anthropometric, metabolic and cardiovascular differences between sexes prior to the appearance of external signs of puberty. Although differences in insulin sensitivity were eliminated when adiposity and DHEAS concentrations were accounted for, there were independent differences in body composition and cardiovascular parameters. Thus, gender, adrenarche and adiposity should be accounted for in studies examining metabolic and cardiovascular outcomes prior to puberty. (Receive

Surgically Induced Necrotizing Scleritis Following Strabismus Surgery Treated Successfully with Topical N-acetylcysteine in a Child with Congenital Fibrosis of Extraocular Muscles and Varadi Papp Syndrome

<p><i>Introduction</i>: Surgically induced necrotizing scleritis (SINS) is a rare but serious disorder that can develop many years after strabismus surgery. It is generally treated with high-dose steroids or immunosuppression. <i>Case report</i>: We describe a patient with Varadi Papp syndrome and congenital fibrosis of the extraocular muscles, who developed surgically induced necrotizing scleritis a month after strabismus surgery and was successfully managed by oral vitamin C and topical N-acetylcysteine 10%. <i>Discussion</i>: While SINS is conventionally treated with steroids/immunosuppression, a conservative approach may be tried in milder cases. The role of topical N-acetylcysteine in managing this complication needs to be explored.</p

Pre-Pubertal Children Born Post-Term Have Reduced Insulin Sensitivity and Other Markers of the Metabolic Syndrome

<div><p>Background</p><p>There are no data on the metabolic consequences of post-term birth (≥42 weeks gestation). We hypothesized that post-term birth would adversely affect insulin sensitivity, as well as other metabolic parameters and body composition in childhood.</p> <p>Methods</p><p>77 healthy pre-pubertal children, born appropriate-for-gestational-age were studied in Auckland, New Zealand: 36 born post-term (18 boys) and 41 (27 boys) born at term (38–40 weeks gestation). Primary outcome was insulin sensitivity measured using intravenous glucose tolerance tests and Bergman’s minimal model. Other assessments included fasting hormone concentrations and lipid profiles, body composition from whole-body dual-energy X-ray absorptiometry, 24-hour ambulatory blood pressure monitoring, and inflammatory markers.</p> <p>Results</p><p>Insulin sensitivity was 34% lower in post-term than in term children (7.7 vs. 11.6 x10^-4·min^-1·(mU/l); p&lt;0.0001). There was a compensatory increase in acute insulin response among post-term children (418 vs 304 mU/l; p=0.037), who also displayed lower glucose effectiveness than those born at term (2.25 vs 3.11 x10^-2·min^-1; p=0.047). Post-term children not only had more body fat (p=0.014) and less fat-free mass (p=0.014), but also had increased central adiposity with more truncal fat (p=0.017) and greater android to gynoid fat ratio (p=0.007) compared to term controls. Further, post-term children displayed other markers of the metabolic syndrome: lower normal nocturnal systolic blood pressure dipping (p=0.027), lower adiponectin concentrations (p=0.005), as well as higher leptin (p=0.008) and uric acid (p=0.033) concentrations. Post-term boys (but not girls) also displayed a less favourable lipid profile, with higher total cholesterol (p=0.018) and LDL-C (p=0.006) concentrations, and total cholesterol to HDL-C ratio (p=0.048).</p> <p>Conclusions</p><p>Post-term children have reduced insulin sensitivity and display a number of early markers of the metabolic syndrome. These findings could have important implications for the management of prolonged pregnancies. Future studies need to examine potential impacts later in life, as well as possible underlying mechanisms.</p> </div