Citations

SummaryCoronary endothelial‐dependent microvascular dysfunction, an early reversible stage of coronary artery disease (CAD), is associated with poor clinical outcome. The current study investigated whether coronary artery distensibility index (CDI) is associated with: (i) coronary endothelial‐dependent microvascular dysfunction and (ii) vulnerable plaque composition among subjects with non‐obstructive CAD. Seventy‐four subjects with non‐obstructive CAD (luminal stenosis <30%) were studied. In 20 subjects with and without coronary endothelial‐dependent microvascular dysfunction, coronary flow reserve (CFR) of target segment during intracoronary (IC) infusion of acetylcholine (Ach) and bolus injection of adenosine as well as CDI at rest of corresponding target segment were measured. In 54 subjects, plaque compositions and CDI at rest of 154 non‐obstructive coronary segments as well as proximal segment without disease were measured by intravascular ultrasound (IVUS). CDI was defined as: [(Early‐diastolic cross‐sectional‐area (CSA) – End‐diastolic CSA of target segment)/(end‐diastolic CSA of target segment × coronary‐pulse‐pressure) × 103]. There is a direct association between endothelial dysfunction and impaired CDI of a coronary segment both in the given coronary segment and corresponding microvessels in which a strong agreement between CDI and CFR Ach (r2 = 0·85, P = 0·0001) was observed. Multivariable regression‐analysis showed that CDI was an independent predictor of the vulnerable plaque characteristics. The risk of impaired CDI was 125% higher in segments with necrotic core and 60% higher in segments with fibrofatty components as compared to normal segments (P = 0·001). In conclusions, the current study reveals that impaired CDI is an endothelial‐dependent process of both given coronary segment and corresponding microvessels and is associated with vulnerable plaque composition.

Summary Objective To describe the etiologies, clinical presentations, outcomes, and predictors of postencephalitic epilepsy ( PE ) in a large series of adult patients with acute encephalitis. Methods We conducted a retrospective review of patients diagnosed with acute encephalitis at Mayo Clinic, Rochester, between January 2000 and December 2012. The patients were categorized into two groups based on the presence or absence of PE at last follow‐up. Multivariate logistic regression analysis was used to analyze factors associated with PE . Results We identified 198 consecutive patients (100 [50.5%] male and 98 [49.5%] female) with a median age of 58 years (range 41.8–69). Etiologies included viral infection (n = 95, 48%), autoimmune (n = 44, 22%), and unknown/others (n = 59, 30%). During hospitalization, seizures were seen in patients with autoimmune encephalitis (n = 24, 54.5%), unknown/others (n = 20, 33.9%), and viral encephalitis (n = 23, 24.2%). Interictal epileptiform discharges on electroencephalography (EEG) were present in 34 (54%), whereas periodic lateralized epileptiform discharges (PLEDs) and generalized periodic discharges (GPDs) were seen in 14 (41.2%) and 2 (5.9%) patients. Forty‐six patients with seizures (70.8%) had fluid‐attenuated inversion recovery ( FLAIR )/T 2 abnormalities, 20 (31.3%) diffusion abnormalities, and 43 (66.2%) cortical involvement. Good outcome at discharge among patients with seizures was seen in 8/23 patients with viral etiology (34.8%), 10/24 patients with autoimmune encephalitis (45.5%), and 12/20 patients with unknown cause (60%). PE was present in 43 patients (29.9%). On multivariate regression analysis, the factors associated with PE were generalized seizures during hospitalization (p = 0.03), focal seizures (p ≤ 0.001), and the presence of FLAIR /T 2 abnormalities on brain magnetic resonance imaging ( MRI ) (p = 0.003). Significance The presence of seizures during hospitalization and an abnormal brain MRI are the strongest predictors of the development of PE . The etiology of encephalitis, presence of focal neurologic deficits, and interictal EEG abnormalities did not influence the development of PE .

There is no standard of care for catheter size or post-procedure supine time in cerebral angiography. Catheter sizes range from 4-Fr to 6-Fr with supine times ranging from two to over six hours. The objective of our study was to establish the efficacy, safety, and cost savings of two-hour supine time after 4-Fr elective cerebral angiography. A prospective, single arm study was performed on 107 patients undergoing elective cerebral angiography. All cerebral angiograms were performed with a 4-Fr sheath-based system without closure devices. Ten minutes of manual compression was applied to the femoral access site, with further compression held as clinically indicated. Patients were then monitored in a nursing unit for two hours supine and subsequently mobilized. Nursing discretion was allowed for earlier mobilization. Patients were called the next day to assess delayed hematoma and bleeding. Estimates of cost savings and productivity increases are provided. All patients ambulated in two hours or less. There were no strokes or vessel dissections. Five patients (4.7%) experienced a palpable hematoma, three patients (2.8%) experienced bleeding immediately following the procedure requiring further compression, and one patient (0.9%) experienced minor groin oozing at home. No patient required transfusion, thrombin injection, or endovascular/surgical management of a groin complication. A two-hour post-procedure supine time resulted in cost savings of $952 per angiogram and a total of $101,864. 4-Fr sheath based cerebral angiography with two-hour post-procedure supine time is safe and effective, and allows for a considerable increase in patient satisfaction, cost savings and productivity.

SummaryAmong 826 patients with primary myelofibrosis (PMF) and analysable metaphases on cytogenetic studies, 352 (42·6%) had abnormal karyotype, of which 240 (68·2%) were sole aberrations and 48 (13·6%) were complex; the most frequent abnormalities were 20q− (23·3%), 13q− (18·2%), +8 (11·1%), +9 (9·9%), chromosome 1q+ (9·7%) and −7/7q− (7·1%). Phenotypic correlates included: abnormal karyotype with anaemia (P = 0·02), leucopenia (P < 0·01) and thrombocytopenia (P < 0·01); complex karyotype with younger age (P = 0·04) and thrombocytopenia (P < 0·01); leucopenia with 20q−, +8 and −7/7q− and thrombocytopenia with 20q− and −7/7q−. Cytopenias were less likely to occur with 13q−. 476 patients were annotated for JAK2/CALR/MPL mutations; abnormal karyotype frequencies were 43% in JAK2, 42% CALR, 33% MPL mutated and 34% triple‐negative cases (P = 0·3). A proportion of patients were also screened for ASXL1, EZH2, IDH1, IDH2, SRSF2, U2AF1 and SF3B1 mutations; in all instances, mutational frequencies were higher in patients with normal karyotype, reaching significance for ASXL1 (P = 0·02) and U2AF1 (P = 0·01). 13q− was associated with mutant CALR (P = 0·03), +9 with mutant JAK2 (P = 0·02) and 20q− with mutant SRSF2 (P = 0·02). The current PMF study provides detailed cytogenetic information and correlations with mutations and clinical phenotype.

Background Celiac disease (CD) occurs in approximately 1% of the Western population. It is a lifelong disorder that is associated with impaired quality of life (QOL) and an excessive risk of comorbidity and death. Objectives To review the literature on screening for CD in relation to the current World Health Organization (WHO) criteria for mass screening. Methods We performed a PubMed search to identify indexed papers on CD screening with a publication date from 1900 until 1 June 2014. When we deemed an abstract relevant, we read the corresponding paper in detail. Results CD fulfills several WHO criteria for mass screening (high prevalence, available treatment and difficult clinical detection), but it has not yet been established that treatment of asymptomatic CD may reduce the excessive risk of severe complications, leading to higher QOL nor that it is cost‐effective. Conclusions Current evidence is not sufficient to support mass screening for CD, but active case‐finding may be appropriate, as we recognize that most patients with CD will still be missed by this strategy. Although proof of benefit is still lacking, screening for CD may be appropriate in high‐risk groups.