Cerebral venous thrombosis: retrospective analysis of 49 cases

Introduction: Cerebral Venous Thrombosis (CVT) is a rare and potentially life-threatening disease, accounting for about 0.5% of stroke cases. However, it is believed to be an underdiagnosed condition. Early diagnosis requires a high degree of suspicion and appropriate use of imaging modalities. Objectives: Imagiological and clinical characterization of CVT cases diagnosed at our hospital from 2004 to 2007. Methods: This study was a retrospective, cross-sectional analysis from 2004 to 2007, using our institution database. We reviewed hospital discharge data to assess the incidence of CVT. The study population consisted of 49 patients. Retrospective review of the clinical data and imaging studies of these patients was then performed. Results: Of the 49 patients with confirmed CVT, 38 were female. Patient age varied between 16 and 75 years, with an average of 42.6 years. Thrombotic risk factors were found in 43 patients; the most frequent was dyslipidemia (n = 22) followed by oral contraceptive use (n = 18). Initial head Computerized Tomography (CT) was normal in six cases. Diagnosis was made by Magnetic Resonance (MR) in 38 cases, Cerebral CT-Venography in 10 cases and Digital Subtraction Angiography in one case. Average time from onset of symptoms to diagnosis was nine days; this was not significantly different when comparing the group diagnosed by MR with the group diagnosed by CT-Venography. Right transverse sinus was the most frequent location of thrombosis (n = 36). Only in four cases thrombosis did not involve the lateral sinuses. Conclusions: Lateral sinus thrombosis is a frequent variety of CVT, accounting for 91.8% of our cases. A negative Head CT scan does not exclude the presence of cerebral venous thrombosis; therefore appropriate imaging study should be performed whenever there's a high degree of clinical suspicion. Cerebral CT-Venography seems to be a good alternative to MR for the diagnosis of CVT

International Paediatric Mitochondrial Disease Scale

OBJECTIVE : There is an urgent need for reliable and universally applicable outcome measures for children with mitochondrial diseases. In this study, we aimed to adapt the currently available Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) to the International Paediatric Mitochondrial Disease Scale (IPMDS) during a Delphi-based process with input from international collaborators, patients and caretakers, as well as a pilot reliability study in eight patients. Subsequently, we aimed to test the feasibility, construct validity and reliability of the IPMDS in a multicentre study. METHODS : A clinically, biochemically and genetically heterogeneous group of 17 patients (age 1.6–16 years) from five different expert centres from four different continents were evaluated in this study. RESULTS : The feasibility of the IPMDS was good, as indicated by a low number of missing items (4 %) and the positive evaluation of patients, parents and users. Principal component analysis of our small sample identified three factors, which explained 57.9 % of the variance. Good construct validity was found using hypothesis testing. The overall interrater reliability was good [median intraclass correlation coefficient for agreement between raters (ICCagreement) 0.85; range 0.23–0.99). CONCLUSION : In conclusion, we suggest using the IPMDS for assessing natural history in children with mitochondrial diseases. These data should be used to further explore construct validity of the IPMDS and to set age limits. In parallel, responsiveness and the minimal clinically important difference should be studied to facilitate sample size calculations in future clinical trials.The work of SK and JS was sponsored by ZonMW (The Netherlands Organization for Health Research and Development).http://link.springer.com/journal/10545am2017Paediatrics and Child Healt

The impact of surgical delay on resectability of colorectal cancer: An international prospective cohort study

AIM: The SARS-CoV-2 pandemic has provided a unique opportunity to explore the impact of surgical delays on cancer resectability. This study aimed to compare resectability for colorectal cancer patients undergoing delayed versus non-delayed surgery. METHODS: This was an international prospective cohort study of consecutive colorectal cancer patients with a decision for curative surgery (January-April 2020). Surgical delay was defined as an operation taking place more than 4 weeks after treatment decision, in a patient who did not receive neoadjuvant therapy. A subgroup analysis explored the effects of delay in elective patients only. The impact of longer delays was explored in a sensitivity analysis. The primary outcome was complete resection, defined as curative resection with an R0 margin. RESULTS: Overall, 5453 patients from 304 hospitals in 47 countries were included, of whom 6.6% (358/5453) did not receive their planned operation. Of the 4304 operated patients without neoadjuvant therapy, 40.5% (1744/4304) were delayed beyond 4 weeks. Delayed patients were more likely to be older, men, more comorbid, have higher body mass index and have rectal cancer and early stage disease. Delayed patients had higher unadjusted rates of complete resection (93.7% vs. 91.9%, P = 0.032) and lower rates of emergency surgery (4.5% vs. 22.5%, P < 0.001). After adjustment, delay was not associated with a lower rate of complete resection (OR 1.18, 95% CI 0.90-1.55, P = 0.224), which was consistent in elective patients only (OR 0.94, 95% CI 0.69-1.27, P = 0.672). Longer delays were not associated with poorer outcomes. CONCLUSION: One in 15 colorectal cancer patients did not receive their planned operation during the first wave of COVID-19. Surgical delay did not appear to compromise resectability, raising the hypothesis that any reduction in long-term survival attributable to delays is likely to be due to micro-metastatic disease

Cryptococcosis in liver and kidney transplant recipients receiving anti-thymocyte globulin or alemtuzumab

Rabbit anti-thymocyte globulin (ATG) and alemtuzumab have been used for induction or preconditioning and for the treatment of acute rejection in organ transplant recipients in many centers. Such regimens may lead to a substantial decline in the CD4 lymphocyte count to levels seen in other population groups at high risk of cryptococcosis. In view of this, we examined the impact of such therapy on the cumulative incidence of cryptococcosis among liver and kidney recipients. A total of 834 liver and 727 kidney transplants were performed during the study period. Seven hundred and eighty-one patients did not receive ATG or alemtuzumab; 646 received 1 dose of either drug, and 134 patients received 2 doses of either drug. The cumulative incidence of cryptococcosis was 0.26% (2/781) among those who did not receive ATG or alemtuzumab; 0.3% (2/646) among those who received only 1 dose, and 2.24% (3/134) among those who received 2 doses (P = 0.03). There were 5 cases of cryptococcosis in liver recipients and 2 in kidney recipients. There were 3 cases of cryptococcal meningitis, 3 of pneumonia, and 1 of disseminated disease. The 2 kidney recipients had meningitis. Diagnosis occurred at a median of 255 days (range 7-517) after transplantation. The mortality rate was 14.2%. We conclude that the use of 1 dose of ATG or alemtuzumab is not associated with an increased cumulative incidence of cryptococcosis, but that those patients receiving 2 doses are at increased risk

Correlation between motor performance scales, body composition, and anthropometry in patients with duchenne muscular dystrophy

The aim of this study is to investigate the relationship between body composition, anthropometry, and motor scales in patients with Duchenne muscular dystrophy (DMD). Twenty six patients with DMD were evaluated by Expanded Hammersmith Functional Motor Scale (HFMSE), gross motor function classification system (GMFCS), multifrequency bioelectrical impedance analysis, and anthropometric measurements. Seventeen healthy children served as control group. There were 26 patients with a mean age of 9.5 +/- A 4.8 years. Ages and anthropometric measurements did not differ between groups. Of the 26 patients, nine were level I, seven were level II, two were level III, seven were level IV, and one was level V, according to the GMFCS. Despite the similar percentage of total body water, extracellular water/intracellular water ratio was significantly elevated in DMD patients (p = 0.001). Increased values of fat percentage and body fat mass index (BFMI) correlated positively with elevated GMFCS levels (r = 0.785 and 0.719 respectively). Increased fat-free mass index (FFMI) correlated negatively with elevated GMFCS levels (r = -0.401). Increased fat percentage and BFMI correlated negatively with HFMSE scores (r = -0.779 and -0.698, respectively). Increased values of FFMI correlated positively with HFMSE scores. There was also a negative correlation between increased skin fold measurements from triceps and scapula and HFMSE scores (r = -0.618 and -0.683, respectively). Increased skin fold values from the same regions correlated positively with elevated GMFCS levels (r = 0.643 and 0.712, respectively). Significant body composition changes occur in patients with DMD. Anthropometric and multifrequency bioelectrical impedance analyses measurements show good correlation between motor function scales. These results may also be helpful to evaluate the effects of new treatment strategies