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