No abstract

Background and Purpose-Cerebral venous thrombosis is generally treated with anticoagulation. However, some patients do not respond to medical therapy and these might benefit from mechanical thrombectomy. The aim of this study was to gain a better understanding of the efficacy and safety of mechanical thrombectomy in patients with cerebral venous thrombosis, by performing a systematic review of the literature. Methods-We identified studies published between January 1995 and February 2014 from PubMed and Ovid. We included all cases of cerebral venous thrombosis in whom mechanical thrombectomy was performed with or without intrasinus thrombolysis. Good outcome was defined as normal or mild neurological deficits at discharge (modified Rankin Scale, 0-2). Secondary outcome variables included periprocedural complications and recanalization rates. Results-Our study included 42 studies (185 patients). Sixty percent of patient had a pretreatment intracerebral hemorrhage and 47% were stuporous or comatose. AngioJet was the most commonly used device (40%). Intrasinus thrombolysis was used in 131 patients (71%). Overall, 156 (84%) patients had a good outcome and 22 (12%) died. Nine (5%) patients had no recanalization, 38 (21%) had partial, and 137 (74%) had near to complete recanalization. The major periprocedural complication was new or increased intracerebral hemorrhage (10%). The use of AngioJet was associated with lower rate of complete recanalization (odds ratio, 0.2; 95% confidence interval, 0.09-0.4) and lower chance of good outcome (odds ratio, 0.5; 95% confidence interval, 0.2-1.0). Conclusions-Our systematic review suggests that mechanical thrombectomy is reasonably safe but controlled studies are required to provide a definitive answer on its efficacy and safety in patients with cerebral venous thrombosis.

No abstract

Premature death and cardiac abnormalities are described in individuals with sickle cell disease (SCD), but the mechanisms are not well characterized. We tested the hypothesis that cardiac abnormalities in children with SCD are related to sleep-disordered breathing. We enrolled 44 children with SCD (mean age, 10.1 years; range, 4-18 years) in an observational study. Standard and tissue Doppler echocardiography, waking oxygen saturation averaged over 5 minutes, and overnight polysomnography were obtained in participants, each within 7 days. Eccentric left ventricular (LV) hypertrophy was present in 46% of our cohort. After multivariable adjustment, LV mass index was inversely related to average asleep and waking oxygen saturation. For every 1% drop in the average asleep oxygen saturation, there was a 2.1 g/m 2.7 increase in LV mass index. LV diastolic dysfunction, as measured by the E/E ratio, was present in our subjects and was also associated with low oxygen saturation (sleep or waking). Elevated tricuspid regurgitant velocity (> 2.5 m/sec), a measure of pulmonary hypertension, was not predicted by either oxygen saturation or sleep variables with multivariable logistic regression analysis. These data provide evidence that low asleep and waking oxygen saturations are associated with LV abnormalities in children with SCD. (Blood. 2010;116(1):16-21)

SynopsisCystic Fibrosis (CF) is a multi-systemic life-limiting genetic disorder, primarily impacting respiratory functioning. Most patients with CF are diagnosed by 2 years of age, and the current median predicted survival rate is 37.4 years old, with 95% of patients dying from complications relate to pulmonary infection. Given the chronic, progressive and disabling nature of CF, multiple treatments are prescribed, most on a daily basis. Thus, this illness requires children, with the aid of their families, to adopt multiple health-related behaviors in addition to managing more typical developmental demands. The morbidity and mortality factors pose cognitive, emotional and behavioral challenges for many children with CF and their families. This article will apply a developmental perspective to describing the psychosocial factors impacting psychological adjustment and health-related behaviors relevant to infants, preschool and school age children, and adolescents with CF. Topics particularly pertinent to developmental periods and medical milestones will be noted, with clinical implications highlighted.