Before the initial visit, caregivers consented to answer the questionnaire via telephone. Patients’ medical records were reviewed after haematological evaluation. VWF:Ag or VWF:RCo<30 IU dL−1 were labelled ‘definite type 1 click here VWD’ while 30–50 IU dL−1 were labelled ‘Low VWF’. PFA-100 screening followed by abnormal electron microscopy and/or platelet aggregation studies diagnosed a PFD. At least one haemorrhagic

symptom was present in 99 of the 104 children who completed the study (mean number of symptoms 2.87, mean Vicenza score 3.24). Eight met criteria for ‘definite type 1 VWD’, 23 for ‘low VWF’ and 13 for ‘PFD’. The sensitivity, specificity, and positive and negative predictive value (NPV) of the Vicenza score demonstrated poor diagnostic utility with the exception of high specificity in ruling out ‘definite type

1 VWD’. The NPV was comparably high with qualitative (>2 bleeding symptoms) and quantitative (Vicenza score ≥2) criteria. The Vicenza score has limited predictive value in paediatric tertiary care settings. While the NPV of excluding ‘definite type 1 VWD’ is high, simpler qualitative criteria is similarly predictive. “
“Most countries still do not achieve 1 IU of factor VIII/capita sufficient for survival. Selleckchem Venetoclax Although primary prophylaxis prevents synovitis, is not universally used. Chronic synovitis is treated with arthroscopy at expense of considerable amount of coagulation factors, and specialized surgeons. Radioactive synovectomy (RS) is a minimally invasive and cost effective alternative to arthroscopy, often considered first the option for persistent synovitis. Even without established causation with cancer, RS is avoided

by some, due to this concern. We aim contributing to the understanding of RS safety regarding malignancy, presenting a large number of treated patients, and a single case of cancer. Three centres in Brazil applied RS with 90Yttrium Citrate, 90Yttrium hydroxyapatite or 153Samarium hydroxyapatite selleck screening library in haemophilic joints and performed a survey addressing cancer in these patients. Four hundred and eighty eight patients (ages 3–51) received 1–3 RS (total 842) and follow-up was 6 months to 9 years. One patient aged 14 years presented Ewing sarcoma, 11 months after RS. The tumour was treated successfully with surgery and chemotherapy. Causality of cancer by RS is improbable in this case. Accordingly, latency here is far below minimum 5–10 years for radio-induction of solid tumours. Moreover, ES is not a typically radio-induced tumour, even at high doses. In agreement with others, though recognizing limitations, this study suggests RS is safe regarding cancer induction. Synovitis is a known burden for patients. The decision of making reasonable usage of RS should be outweighed with the risks of leaving synovitis untreated. “
“Summary.