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A human skull, buried about 2500 years ago in a Bronze Age cemetery at Jinggouzi, a site of an important ethnic group in ancient China, appeared to have characteristics of fibrous dysplasia. The CT images indicated a reduction in bone density and relatively homogeneous lesions. More features were revealed using CT reconstruction techniques. Lesions seen in low‐magnification images using a 3D deep‐field microscope had an irregular honeycomb‐like structure. At higher magnification, the trabeculae morphology and the gaps between the trabeculae were irregular and varied in size and shape. Paraffin‐embedded specimens stained with HE showed trabeculae with tortuous irregular arrangements varying in shape and width. The irregular trabeculae of woven bone has been described as having fibrous dysplasia. Molecular analysis of the GNAS gene indicated no mutation. This provides a non‐invasive approach for us to make more comprehensive diagnoses and to assist research into ancient human diseases. [ABSTRACT FROM AUTHOR]

Introduction: Due to lack of patient/health care provider awareness causing delayed diagnosis, the bleeding phenotype and provider interventions in adolescents with heavy menstrual bleeding (HMB) and bleeding disorders (BD) may be different when compared to adults. Aim: The aim of this study was to compare/characterize bleeding phenotype and provider interventions in postmenarchal adolescents < 18 years and premenopausal adults  ≥ 18 years with HMB and BD. Methods: Patient demographics, BD, and provider interventions/therapy details for HMB were compared between both age groups enrolled in the Centers for Disease Control and Prevention (CDC) Female Universal Data Collection (UDC) surveillance project in United States hemophilia treatment centres. Cross‐sectional descriptive analyses including frequency distributions, summary statistics, bivariate and logistic regression analyses were performed. Results: Of 269 females (79 adolescents; median age 16 years, interquartile range (IQR) = 2; 190 adults; median age 27 years, IQR = 13) evaluated, BD distribution was similar in both groups. Compared to adolescents, adults more often had family history of bleeding (Adjusted odds ratios [AOR] = 2.6, 1.3‐5.6), delay in diagnosis (AOR = 2.5, 1.2‐4.9), bleeding with dental procedures (AOR = 2.0, 1.0‐4.0), gastrointestinal bleeding (AOR = 4.6, 1.0‐21.9), anaemia (AOR = 2.7, 1.4‐5.2), utilized desmopressin less often (AOR = 0.4, 0.2‐0.8) and underwent gynaecologic procedure/surgery more frequently (AOR = 5.9, 1.3‐27.3). Conclusion: Bleeding phenotypes of adolescents and adults with HMB and BD were different with more frequent bleeding complications, anaemia, gynaecologic procedures/surgeries, less desmopressin use and more delay in diagnosing BD in adults. Longitudinal studies are needed to determine whether improved patient/provider awareness and education will translate to early diagnosis and timely management of BD/HMB in adolescents that may prevent/reduce future haematologic/gynaecologic complications. [ABSTRACT FROM AUTHOR]

Introduction Prophylaxis is considered the optimal treatment for persons with moderate to severe haemophilia (factor activity between 1-5% of normal and <1% of normal respectively) in countries where safe factor concentrates are available and economically feasible. Historically, prophylactic treatment has not been well studied in the haemophilia B ( HB) population due to difficulties in obtaining a sufficiently large sample. Aim This study examines the prevalence of prophylaxis use among a robust sample of persons with HB in the United States and its association with specific demographic and clinical characteristics. Methods Using data collected between 1998 and 2011 for the Centers for Disease Control and Prevention's Universal Data Collection project, we analysed data on 2428 males with moderate to severe HB aged 2-79 years who were seen at 135 federally funded haemophilia treatment centres. Results Prevalence of prophylactic treatment in our sample was 35% among children and youth (ages 2-19) and 14% among adults (age 20 and older). Increased HB prophylaxis use was significantly associated with younger age (<40 years), Hispanic ethnicity, severe disease and self-infusion, while decreased use was associated with above-normal body mass index ( BMI) in adults. Health care coverage was vital, although type of coverage did not appear to influence access. Conclusions Our analysis confirms previous reports of lower prevalence of prophylaxis use among individuals with HB compared to those with haemophilia A and adds to the body of knowledge regarding treatment patterns among a historically understudied population. [ABSTRACT FROM AUTHOR]

An elevated body mass index ( BMI) may make venipuncture more difficult, potentially impacting the use of home infusion ( HI) and self-infusion ( SI). We sought to determine whether above-normal BMI is associated with decreased use of HI treatment and SI of clotting factor concentrate among haemophilic persons. We analysed data from 10 814 male patients with haemophilia A and B (45% with severe disease) aged 6-79 years enrolled in the Centers for Disease Control and Prevention Universal Data Collection surveillance project between 1998 and 2008. Associations between the use of HI and SI and BMI were evaluated using logistic regression. Fifty per cent of haemophilic men were overweight or obese, similar to rates reported among the general US population by the 2007-2008 National Health and Nutrition Examination Survey [Flegal, KM et al., JAMA 2010; 303:235-241;]. Twenty per cent of children and 22% of teens were obese, as were 28% of adults [Ogden, CL et al., JAMA 2010;303:235, 242]. Overall, 70% of the study sample used HI; 44% of those who used HI also used SI. Overweight and obese men were each less likely to use HI than those of normal weight [odds ratio ( OR) 0.8; 95% confidence interval ( CI) 0.7-1.0 and OR 0.7; 95% CI 0.6-0.8 respectively]. Obese teens and adult men were also less likely to practice SI than teens and adults of normal weight ( OR 0.8; 95% CI 0.7-0.9 for each). We conclude that overweight and obese haemophilic men are less likely to use HI and obese men are less likely to use SI than their normal-weight counterparts. [ABSTRACT FROM AUTHOR]