Coronavirus Disease 2019 in Autoimmune Hepatitis: A Lesson From Immunosuppressed Patients.

Chronic immunosuppression is associated with increased and more severe viral infections. However, little is known about the association between immunosuppression and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Our aim was to describe the clinical course of patients with immunosuppressed autoimmune hepatitis (AIH) during coronavirus disease 2019 (COVID-19) infection in Italy.

Establishment of patient-derived tumor xenograft models of mucinous ovarian cancer.

Mucinous ovarian carcinoma (mEOC) represents a rare subtype of epithelial ovarian cancer, accounting for 3-4% of all ovarian carcinomas. The rarity of this tumor type renders both the preclinical and clinical research compelling. Very few preclinical and models exist.

An Imatinib-non-responsive patient with an Leu387Trp mutation achieves cytogenetic and molecular response under bosutinib: Case report and biological characterization.

Leu387Trp mutation, aroused in an imatinib-non-responsive CML patient, was selected by imatinib treatment along with other unknown factors responsible for resistance, and then it was overcome by bosutinib. These results will be useful for treating patients with this rare mutation and will advise against automatically considering a new mutation as the cause of TKI resistance.

Substantia Nigra Swelling and Dentate Nucleus T2 Hyperintensity May Be Early Magnetic Resonance Imaging Signs of β-Propeller Protein-Associated Neurodegeneration.

Background And Methods: Mutations in cause β-propeller protein-associated neurodegeneration (BPAN), a type of neurodegeneration with brain iron accumulation (NBIA). We reviewed clinical and MRI findings in 4 patients with de novo mutations.

Results: Psychomotor delay and movement disorders were present in all cases; early-onset epileptic encephalopathy was present in 3.

Efficacy and safety of perampanel oral loading in postanoxic super-refractory status epilepticus: A pilot study.

Refractory nonconvulsive status epilepticus (NCSE) occurs in 10%-30% of patients following resuscitation after cardiac arrest. Both the optimal treatment and prognosis of postanoxic status epilepticus remain uncertain. We analyzed acute electroencephalographic changes, neurological outcome at 3 months, and adverse effects in consecutive postanoxic patients with super-refractory NCSE treated with add-on oral loading of perampanel.

Systematic review and meta-analysis of frailty as a predictor of morbidity and mortality after major abdominal surgery.

Background: Frailty is associated with poor prognosis, but the multitude of definitions and scales of assessment makes the impact on outcomes difficult to assess. The aim of this study was to quantify the effect of frailty on postoperative morbidity and mortality, and long-term mortality after major abdominal surgery, and to evaluate the performance of different frailty metrics.

Methods: An extended literature search was performed to retrieve all original articles investigating whether frailty could affect outcomes after elective major abdominal surgery in adult populations.

Sevoflurane improves respiratory mechanics and gas exchange in a case series of infants with severe bronchiolitis-induced acute respiratory distress syndrome.

This report describes the successful use of a new intervention to improve respiratory mechanics and gas exchange in a relatively homogeneous group of infants with severe bronchiolitis-induced PARDS after failure of conventional treatment. These results may open a new interesting area of research and management for PARDS patients.

Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices.

We present a consensus report pertaining to the improved clarity of definitions and classification of glomerular lesions in lupus nephritis that derived from a meeting of 18 members of an international nephropathology working group in Leiden, Netherlands, in 2016. Here we report detailed recommendations on issues for which we can propose adjustments based on existing evidence and current consensus opinion (phase 1). New definitions are provided for mesangial hypercellularity and for cellular, fibrocellular, and fibrous crescents.

How to describe univariate data.

Univariate analysis has the purpose to describe a single variable distribution in one sample. It is the first important step of every clinical trial. In this short review, we focus on this analysis, the methods that authors should use to report this type of data, information that they should not miss and mistakes that they must avoid.

Photometric evaluation of cranial and facial symmetry in hemicoronal single suture synostosis treated with surgical fronto-orbital remodeling.

Objective: Evaluation of frontal vault symmetry and progressive facial symmetrization in a cohort of patients with hemicoronal single suture synostosis treated with a standardized cranioplasty and rigid fixation.

Patients And Methods: Fifty-four patients with hemicoronal synostosis operated between 1999 and 2014 were reviewed retrospectively. Pre, immediately postoperative and yearly photographs from the top of the skull and frontal views of the face were taken with the same head position and projection.

Identification of point mutations and large intragenic deletions in Fanconi anemia using next-generation sequencing technology.

Fanconi anemia (FA) is a rare bone marrow failure disorder characterized by clinical and genetic heterogeneity with at least 17 genes involved, which make molecular diagnosis complex and time-consuming. Since next-generation sequencing technologies could greatly improve the genetic testing in FA, we sequenced DNA samples with known and unknown mutant alleles using the Ion PGM (™) system (IPGM). The molecular target of 74.

Disentangling cardiovascular control mechanisms during head-down tilt via joint transfer entropy and self-entropy decompositions.

A full decomposition of the predictive entropy (PE) of the spontaneous variations of the heart period (HP) given systolic arterial pressure (SAP) and respiration (R) is proposed. The PE of HP is decomposed into the joint transfer entropy (JTE) from SAP and R to HP and self-entropy (SE) of HP. The SE is the sum of three terms quantifying the synergistic/redundant contributions of HP and SAP, when taken individually and jointly, to SE and one term conditioned on HP and SAP denoted as the conditional SE (CSE) of HP given SAP and R.

COPD: maximization of bronchodilation.

The most recent guidelines define COPD in a multidimensional way, nevertheless the diagnosis is still linked to the limitation of airflow, usually measured by the reduction in the FEV1/FVC ratio below 70%. However, the severity of obstruction is not directly correlated to symptoms or to invalidity determined by COPD. Thus, besides respiratory function, COPD should be evaluated based on symptoms, frequency and severity of exacerbations, patient's functional status and health related quality of life (HRQoL).

Muscle ultrasonography for detecting fasciculations in frontotemporal dementia.

Ultrasound detection of muscle fasciculations was recently proposed for assessing lower motor neuron (LMN) dysfunction in ALS patients. Given the continuum between ALS and frontotemporal degeneration (FTD), the aim of the present study was to evaluate muscle ultrasound (MUS) in FTD both for feasibility and prevalence of fasciculations. Twenty-two FTD patients were examined (five muscles bilaterally: biceps brachii, first dorsalis interosseous, T10 paraspinalis, vastus lateralis, tibialis anterior) with a 7-MHz linear array transducer and a fasciculation score (FS) computed.

Vacuum assisted closure therapy in the management of cervico-facial necrotizing fasciitis: a case report and review of the literature.

Necrotizing fasciitis is a rare polymicrobial, infection, characterized by a rapid and destructive spread within the subcutaneous tissue and along the superficial fascial planes, initially not affecting underlying muscles but with a common systemic involvement. Necrotizing fasciitis confined to the cervico-facial region is extremely rare (10% of cases) and is characterized by a high mortality rate due to airway compromise and to involvement of the supra-aortic vessels. The prognosis for survival is based on the severity of the necrotizing fasciitis, on the patient's systemic condition and on the prompt diagnosis and adequate surgical and medical management.

Identification of novel point mutations in splicing sites integrating whole-exome and RNA-seq data in myeloproliferative diseases.

Point mutations in intronic regions near mRNA splice junctions can affect the splicing process. To identify novel splicing variants from exome sequencing data, we developed a bioinformatics splice-site prediction procedure to analyze next-generation sequencing (NGS) data (SpliceFinder). SpliceFinder integrates two functional annotation tools for NGS, ANNOVAR and MutationTaster and two canonical splice site prediction programs for single mutation analysis, SSPNN and NetGene2.

Early alterations in left ventricular diastolic function in normotensive diabetic patients.

In diabetes mellitus, structural and functional alterations of the heart can be already present at the time of first diagnosis. However, how early these alterations may occur has never been fully clarified. The present study aimed at investigating cardiac functional abnormalities in uncomplicated hypertensive or normotensive patients with a recent diagnosis of diabetes mellitus.

Flip-flop kinetics of ropivacaine during continuous epidural infusion influences its accumulation rate.

Background: Ropivacaine has an optimal toxicity profile for epidural anesthesia in adults, but there are currently no studies concerning its pharmacokinetics during continuous infusion. The primary objective of this study was to evaluate the pharmacokinetics and safety of ropivacaine in adults during a 48-h continuous epidural infusion.

Materials And Methods: We enrolled 43 adults (ASA I-II) scheduled for major abdominal or urologic surgery with postoperative continuous epidural analgesia with ropivacaine 0.