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Summary Previous studies in acquired haemophilia A have reported on cohorts of patients referred to specialist centres or were retrospective surveys of specialist centre experience. This may have resulted in the literature representing a more severe group of patients than seen in routine haematological practice. We report on a consecutive, unselected cohort of all patients in south and west Wales who presented with acquired haemophilia A between 1996 and 2002. There were 18 patients, an incidence of 1·34/million/year. Compared with previously reported cohorts our patients were older, with a median age of 70 years, and less likely to have an underlying diagnosis (27%). The bleeding phenotype was less severe, with only 27% having life or limb threatening bleeds and 41% required no haemostatic treatment. One patient died of bleeding, but three died of complications related to immunosuppression. Response to immunosuppression was high compared with other series, with 88% of treated patients attaining an undetectable inhibitor and normal factor VIII level. These data suggest that previously reported cohorts may represent more severely affected patients and, whilst guidelines for treatment based on these assumptions may be valid for severely affected patients, they may not be universally applicable.

PurposeTo determine the incidence and presenting features of congenital dacryocystocele in the United Kingdom. To report on those cases complicated by dacryocystitis, respiratory compromise, and the treatment undertaken.MethodsA prospective observational study of cases of congenital dacryocystocele presenting in the United Kingdom between September 2014 and October 2015. Infants <3 months of age presenting with a cystic swelling in the medial canthal area were included. Cases were identified via the British Ophthalmology Surveillance Unit (BOSU) reporting system.ResultsA total of 49 cases were reported during the study period. This gives an incidence of 1 in 18 597 live births. There was a 71% response rate to the questionnaire. The average age at presentation was 16.94 days. Dacryocystoceles were unilateral in 91% of cases. Dacryocystitis was a complicating factor in 49% of patients and 17% had respiratory distress. Uncomplicated dacryocystocele responded well to conservative measures in 86%. Surgical intervention was required in 23% of patients. Those cases complicated by dacryocystitis (29%) and nasal obstruction (17%) were more likely to require surgical intervention compared to those with dacryocystocele alone (14%). Digital massage appears to reduce the likelihood of requiring surgical intervention. The mean time to resolution was 19 days.ConclusionsCongenital dacryocystocele is a rare presentation in the United Kingdom. Dacryocystitis and respiratory compromise commonly complicate a dacryocystocele. The use of digital massage as an early intervention is advocated and conservative measures may be sufficient in cases of uncomplicated dacryocystocele.

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The current work develops forming-limit diagrams (FLDs) for weld materials in aluminum tailorwelded blanks (TWBs) under biaxial stretching conditions. Aluminum TWBs consist of multiplethickness and alloy sheet materials welded together into a single, variable-thickness blank. The manufacture of TWBs and their application in automotive body panels requires their constituent weld material to deform under biaxial loading during sheet-metal stamping. The weld geometry is typically nonuniform and relatively small, causing difficulty if one attempts to determine the weld metal FLDs via traditional experimental methods. The subject work primarily relies on theoretical FLD calculation techniques using the Marciniak and Kuczynski (M-K) method. This numerical technique requires the use of material constants and levels of initial material imperfection that have been experimentally determined using unique miniature tensile specimens to isolate and characterize the weld metal. The experimental and numerical work, together with statistical analysis of the level of initial imperfection, allows generation of both an average and safe FLD. The weld metals studied in this work were produced via autogeneous gas tungsten arc welding of a 1-to 2-mm-thick 5000 series aluminum alloy sheet.