Risk-prediction tool for identifying hospitalized children with a predisposition for development of venous thromboembolism: Peds-Clot clinical Decision Rule.

BACKGROUND: The prevalence of VTE is increasing in tertiary pediatric hospitals. Identification of high-risk populations using uniform criteria is required to develop evidence-based VTE prevention guidelines. OBJECTIVE: To develop a VTE risk prediction rule, the Peds-Clot clinical Decision Rule (PCDR), to identify high-risk children who were at increased risk of developing VTE. METHODS: This retrospective case-control study developed the PCDR using a derivation cohort (173 cases, 346 controls) and validated it on a separate validation cohort (100 cases, 100 controls). A uniform data collection strategy was applied to derive both the samples. Conditional logistic regression analyses were used to develop a risk-prediction model. Each significant predictor was assigned a score based on its beta coefficient and the PCDR was developed. ROC curves were derived to test the performance of the PCDR. RESULTS: Characteristics of derivation and validation cohorts were comparable. Six risk factors (positive blood stream infection, central venous catheter, direct admission to ICU/NICU, hospitalization for ≥ 7 days, immobilization for > 72 h, and use of birth control pills) formed the final risk prediction model (risk score range, 0.5-9.5). A risk score of 3 or more identified high-risk children at a sensitivity of 70% and specificity of 80% and AUC of 0.852 (95% confidence interval, 0.814-0.890). The application of a risk score to the validation sample showed sensitivity 57% and specificity 88% and an AUC of 0.875 (95% confidence interval, 0.82-0.924). CONCLUSION: Incorporation of the PCDR in routine clinical care can be an attractive strategy to identify high-risk hospitalized children with a predisposition for VTE. The clinical utility of the PCDR needs validation in prospective studies.

Prevalence and risk factors of cardiovascular disease (CVD) events among patients with haemophilia: experience of a single haemophilia treatment centre in the United States (US).

The primary objective of the study was to examine the prevalence of cardiovascular disease (CVD) events and their known risk factors among persons with haemophilia (PWH). This cross-sectional study, covering a 5-year period, included PWH aged ≥35 years who were cared for at a single haemophilia treatment centre in the United States. Medical records were extensively reviewed to collect the information about CVD events and their risk factors such as obesity, hypertension, diabetes, hypercholesterolemia and smoking. Prevalence rates were compared with national population estimates and associations between risk factors and CVD events were examined using logistic regression. The study cohort comprised 185 PWH (102 haemophilia A and 83 haemophilia B). Lifetime prevalence of a CVD event was 19.5% (36/185, 95% confidence interval [CI] 13.8-25.2%). CVD mortality was 5.4% (10/185, 95% CI 2.7-8.1). Compared with US non-Hispanic White males (NHWH), PWH had about twice the prevalence of coronary artery disease, stroke and myocardial infarction. The prevalence of CVD risk factors for PWH was similar to that for US NHWM with 39.5% of PWH exposed to two or more of these risk factors. Both hypertension and smoking were associated significantly with CVD events, with odds ratios of 4.9 and 6.3, respectively. In conclusion, this study revealed that both CVD events and its risk factors were at least equally prevalent among PWH and might have been even higher than among the US NHWM in the United States. Therefore, it is imperative to implement strategies for CVD prevention among PWH.

Hereditary haemorrhagic telangiectasia.

Hereditary haemorrhagic telangiectasia (also known as Osler-Weber-Rendu syndrome) is a relatively common, under-recognized autosomal-dominant disorder that results from multisystem vascular dysplasia. It is characterized by telangiectases and arteriovenous malformations of skin, mucosa and viscera. This article summarizes the clinical manifestations and the management of this disorder and its management. This review underscores an urgent need to conduct prospective multicentre studies to develop evidence-based management guidelines for this disease.