Does Outcome/Survival of Patients With Myelodysplastic Syndromes Should Be Predicted by Reduced Levels of ADAMTS-13? Results From a Pilot Study

IntroductionVon Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy. Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study, we have evaluated whether or not deficiencies of ADAMTS-13 were present in myelodysplastic syndromes (MDS). Moreover, we assessed if a reduction in basal levels of ADAMTS-13 may play a role in the prognosis of MDS.Patients and MethodsWe measured and compared the levels of vWF cleaving protease ADAMTS-13 in 100 patients with MDS and 35 healthy controls. Patients were divided into 2 groups according to the International Prognostic Scoring System: group I consisting of 44 patients with low-risk MDS and group II of 56 patients with high-risk MDS. Patients with high-risk and low-risk MDS presented significantly lower levels of ADAMTS-13 than controls (P < .001 and P = .0177, respectively). High-risk patients had significantly lower levels of ADAMTS-13 when compared with the low-risk group (P < .001).ResultsWe found that reduced levels of ADAMTS-13 have a relationship with overall survival (P < .001). Statistical analysis showed that ADAMTS-13 correlates with cytogenetics (P < .001) and a tendency of slight correlation with platelet count and basal levels of ADAMTS-13 (R, 0.35; P value, 0.001). Moreover, we found that levels of ADAMTS-13 have correlation with response to treatment (P < .001).ConclusionsADAMTS-13 in MDS might represent a surrogate marker of prognosis, response to therapy, or disease progression. Further studies are needed.     

温病学理论指导下的新型冠状病毒肺炎诊治刍议

基于温病学理论,探讨新型冠状病毒肺炎的中医病因病机与诊治思路。温病学说源于明清之前的历代中医理论积累,经过长期的温热病防治实践而形成,其对于外感热病(包括烈性传染病)的病因病机、证治方药有着较为系统的论述,近年来在我国的新发传染病防治中发挥出积极的作用。温瘟相通,瘟疫病因多为热毒疫邪,表现为热证阳证,可按温病学中的卫气营血或三焦辨证分期论治,或治以吴又可、庞安时等的经方验方,这些因、机、证、治理论可试用于新型冠状病毒肺炎的中医诊疗。且肺与大肠相表里,胃肠道也是新型冠状病毒侵袭或繁殖的部位,采用中医清热通下之法,使邪从肠道而出,可能是减轻体内病毒感染与肺脏炎症的一种途径。     

Subacute sclerosing panencephalitis resembling Rasmussen’s encephalitis on magnetic resonance imaging

Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen’s encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.     

Fathers of children with autism spectrum disorder: Their perceptions of paternal role a predictor of caregiving satisfaction,self-efficacy and burden

BackgroundThe positive effect of a father’s involvement in children’s upbringing is now recognised. However, research on fathers raising children with autism spectrum disorder (ASD) are still few. This study examines the relationship between the perception, fathers of children with ASD have of the importance of their role in the development of their children and the feelings (self-efficacy, caregiving burden, satisfaction) they express about their parenting experience.MethodSixty-three Swiss Italian fathers of children with ASD completed The Role of the Father Questionnaire (ROFQ), three sub-scales of the Caregiver Survey, a subtest of the Child Adjustment and Parent Efficacy Scale and a home-made questionnaire measuring Perceived Social Support.ResultsThe results from hierarchical multiple regression analyses show that the importance that fathers attach to the paternal role predicts positively their caregiving satisfaction and their feeling of self-efficacy. The children’s challenging behaviours predict positively the caregiving burden whereas the assessment of social support predicts it negatively.ConclusionsThe perception of the importance of the paternal role needs to be considered in the support offered to families with a child with ASD. A better understanding of the fathers’ feelings could be of value for the programmes.     

Preparation and identification of multifunctional mesoporous silica nanoparticles for in vitro and in vivo dual-mode imaging,theranostics, and targeted tracking

Mesoporous silica nanoparticles (MSNs) can provide a structural foundation for a new generation of nanocarriers with a broad range of functionalities. Multifunctional MSNs can serve as all-in-one diagnostic and therapeutic tools that can be used to simultaneously visualize and treat various diseases, such as cancer. This research study is the first time that two lanthanide-based imaging systems have been combined to incorporate controlled drug release and targeted tracing into a single MSN-based nano-platform for a novel theranostic drug delivery system. Doping lanthanide ions, i.e., europium (Eu) and gadolinium (Gd) ions, into an MSN structure (EuGd-MSNs) imparts fluorescence and magnetism to the nanostructure that can be used to develop magnetic resonance imaging (MRI) and biological fluorescence tools. Current cancer research has revealed that most human cancer cells express a large number of folate receptors on their surface. Grafting folic acid (FA) onto the EuGd-MSN surface (EuGd-FA-MSNs) imparts a targeting function to the MSN because of the specificity of the binding of FA to cell surface receptors. Furthermore, grafting anticancer drugs, such as camptothecin (CPT), onto the surface of these MSNs by forming disulfide bonds (EuGd-SS-CPT-FA-MSNs) enables intracellular controlled drug release. A high concentration of intracellular glutathione cleaves the disulfide bond to release the drug and treat the disease. The results of in vitro and in vivo studies show that the functionalized MSNs can be successfully used as a platform to integrate dual-imaging, targeting, and therapeutic treatment in multifunctional diagnosis drug delivery systems.