Developing the Persian Version of Sensory Gating Inventory and Assessing Its Validity and Reliability.

Introduction: Sensory Gating Inventory (SGI) measures behavioral aspects of Sensory Gating (SG), which filters irrelevant sensory inputs into the higher cortex. It modifies sensitivity to sensory stimuli. Abnormal SG leads to overloading of information in the brain and its subsequent dysfunction.

Homeostatic and pathogenic roles of PI3Kδ in the human immune system.

Phosphoinositide 3-kinase delta (PI3Kδ) mediates signaling transduction downstream of diverse immune receptors, including the T cell receptor (TCR), the B cell receptor (BCR), costimulatory molecules and cytokine receptors. Our understanding of the role of PI3Kδ in the immune system comes primarily from mice, and especially from the consequences of pharmacological inhibition of PI3Kδ in mouse models of human disease as well as the consequences of genetic manipulation, resulting in hyperactivation or loss of PI3Kδ function. In case of humans, in vitro studies with PI3Kδ-specific inhibitors, the consequences of treatment of hematologic malignancies with the PI3Kδ-specific inhibitor idelalisib and primary immunodeficiency disorders due to germline variants hyper- or underactivating PI3Kδ provide most of our knowledge on the role of PI3Kδ in immunity and immune regulation.

Correction: Long-term outcome after allogeneic hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: a retrospective analysis and a review of the literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation (SAAWP-EBMT).

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Consensus on definition and severity grading of lymphatic complications after kidney transplantation.

Background: The incidence of lymphatic complications after kidney transplantation varies considerably in the literature. This is partly because a universally accepted definition has not been established. This study aimed to propose an acceptable definition and severity grading system for lymphatic complications based on their management strategy.

Intratympanic application of triamcinolone in sudden hearing loss-radiologic anatomy in cone beam CT and its' correlation to clinical outcome.

Purpose: To evaluate temporal bone cone-beam CT in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) being treated with primary and secondary intratympanic (IT) triamcinolone and to possibly correlate these results to the clinical outcome.

Methods: Retrospective analysis of patients treated with IT triamcinolone for ISSNHL at our department in 2018. Pre- and post-therapeutic audiologic examinations included four-tone average (FTA) at 0.

Long-term outcome after allogeneic hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: a retrospective analysis and a review of the literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation (SAAWP-EBMT).

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative procedure in patients with Shwachman-Diamond syndrome (SDS) with bone marrow abnormalities. The results of 74 patients with SDS (6 acute myeloid leukemia, 7 myelodysplastic syndrome, and 61 bone marrow failure) treated with HSCT between 1988 and 2016 are reported. The donor source was: 24% sibling, 8% parent, and 68% unrelated donor.

Assessment of a Mobile App by Adolescents and Young Adults With Cystic Fibrosis: Pilot Evaluation.

Background: Cystic fibrosis (CF) continues to be the most common life-limiting chronic pulmonary disease in adolescents and young adults. Treatment of CF demands a high treatment time investment to slow the progression of lung function decline, the most important contributor to morbidity and mortality. Adherence is challenging in CF due to the high treatment burden and the lack of immediate health consequences in case of nonadherence.

Cognition in older adults with severe to profound sensorineural hearing loss compared to peers with normal hearing for age.

Cognitive performance of older adults with severe to profound sensorineural hearing loss and indication for cochlear implantation was evaluated compared to peers with age appropriate hearing. Prospective matched case control study. Study group consisted of  = 30 patients aged between 60 and 80 years, with adult onset, severe to profound sensorineural hearing loss on both sides and indication for cochlear implantation.

A novel NFKBIA variant substituting serine 36 of IκBα causes immunodeficiency with warts, bronchiectasis and juvenile rheumatoid arthritis in the absence of ectodermal dysplasia.

Genetic studies have led to identification of an increasing number of monogenic primary immunodeficiency disorders. Monoallelic pathogenic gain-of-function (GOF) variants in NFKBIA, the gene encoding IκBα, result in an immunodeficiency disorder, typically accompanied by anhidrotic ectodermal dysplasia (EDA). So far, 14 patients with immunodeficiency due to NFKBIA GOF mutations have been reported.

The patient-reported disease burden in adults with atopic dermatitis: a cross-sectional study in Europe and Canada.

Background: Cross-sectional data on patient burden in adults with atopic dermatitis (AD) from real-world clinical practice are limited.

Objective: This study compared patient-reported burden associated with adult AD across severity levels from clinical practices in Canada and Europe.

Methods: This study included adults (18-65 years) diagnosed with AD by dermatologists, general practitioners or allergists.

Toward An Exploration of Habituating to Tinnitus: Perspectives on Sensory Gating.

Background: Some tinnitus participants habituate to their tinnitus, but some others do not and complain of its annoyance so much. It has been suggested that tinnitus is a habituation deficit. Habituation and the ability to ignore a sensory input depend on the normal function of filtering mechanism of sensory gating.

CD74 is a T cell antigen in spondyloarthritis.

Objectives: Spondyloarthritis (SpA) is a chronic inflammatory disease of unknown aetiology. Previously, we identified autoantibodies against CD74 in sera of SpA patients. The aim of this study was to evaluate CD74 as a T cell antigen in SpA.

The MK2 cascade regulates mGluR-dependent synaptic plasticity and reversal learning.

The ability to either erase or update the memories of a previously learned spatial task is an essential process that is required to modify behaviour in a changing environment. Current evidence suggests that the neural representation of such cognitive flexibility involves the balancing of synaptic potentiation (acquisition of memories) with synaptic depression (modulation and updating previously acquired memories). Here we demonstrate that the p38 MAPK/MAPK-activated protein kinase 2 (MK2) cascade is required to maintain the precise tuning of long-term potentiation and long-term depression at CA1 synapses of the hippocampus which is correlated with efficient reversal learning.

First Experience With a New Thin Lateral Wall Electrode in Human Temporal Bones.

Introduction: A modern cochlear implant electrode array design must combine: improved surgical ease of use, structure preservation, particularly important for pediatric application, stable position within the cochlea over time, and a meaningful balance between hearing preservation against addressing sufficient cochlear tissue to support electrical-only hearing. The aim of this study was to investigate a new lateral wall electrode array design from Advanced Bionics on human temporal bones (TBs).

Methods: Ten fresh-frozen TBs were implanted with the SlimJ electrode array via the round window.

The potential role of auditory prediction error in decompensated tinnitus: An auditory mismatch negativity study.

Introduction: Some tinnitus subjects habituate to their tinnitus but some others do not and complain of its annoyance tremendously. Normal sensory memory and change detection processes are needed for detecting the tinnitus signal as a prediction error and habituation to tinnitus. The purpose of this study was to compare auditory mismatch negativity as the index of sensory memory and change detection among the studied groups to search for the factors involving in the perception of tinnitus and preventing habituation in decompensated tinnitus subjects.

Neuronal Development of Hearing and Language: Cochlear Implants and Critical Periods.

The modern cochlear implant (CI) is the most successful neural prosthesis developed to date. CIs provide hearing to the profoundly hearing impaired and allow the acquisition of spoken language in children born deaf. Results from studies enabled by the CI have provided new insights into () minimal representations at the periphery for speech reception, () brain mechanisms for decoding speech presented in quiet and in acoustically adverse conditions, () the developmental neuroscience of language and hearing, and () the mechanisms and time courses of intramodal and cross-modal plasticity.

The roadmap for the Allergology specialty and allergy care in Europe and adjacent countries. An EAACI position paper.

The high prevalence of allergic diseases warrants for sufficient health care provisions available to patients with allergic diseases. Allergy care should be delivered by well-trained specialists. However, the current status of allergy care is not well documented.

DNA Methylation of the Leptin Gene Promoter is Altered by Chronic Alcohol Exposure in an Animal Model for Alcohol Dependence.

Appetite-regulating peptides, such as leptin, are linked to craving and have been in the focus of alcohol dependence research for years. The objective of our study was to investigate the dynamics of leptin gene promoter methylation during alcohol withdrawal and specific treatment in a rodent (rat) model for alcohol dependence. DNA methylation was measured using direct bisulfite sequencing at 0 h, 24 h, and 6 days of alcohol withdrawal as well as after treatment with alpha-melanocyte-stimulating hormone (alpha-MSH), Beta-Endorphin, or saline.

Take a second look: it's Kikuchi's disease! A case report and review of literature.

Generalized lymphadenopathy is a common cause of concern for both patients and clinicians. Possible etiologies include infections, malignancies and autoimmune diseases. Kikuchi Fujimoto disease (KFD) is a hyperergic condition that presents with fever, lymphadenopathy and can include systemic involvement, thus being easily mistaken for the above-mentioned entities.

Physical, psychological and economic burden of two-wheel users after a road traffic injury: Evidence from intensive care units of three EU countries.

Introduction: This study aimed to assess the physical, psychological, and economic burden shouldered by severely injured two-wheel users in three European countries as well as the cost resulting from their hospitalization.

Methods: A total of seven public hospitals were involved in three countries: Greece, Italy, and Germany. Participants enrolled during a 12-month period starting in April 2013.

Tuberculous coxitis with trochanteric bursitis manifesting a year after immigration to Germany: a case report.

Background: Osteoarticular tuberculosis is rare in Germany. In particular, trochanteric bursitis is an extremely rare manifestation of osteoarticular tuberculosis. We describe a case of tuberculous coxitis with trochanteric bursitis, successfully treated with a fourfold tuberculostatic therapy.

Consider Syphilis in Case of Lymphopenia in HIV-Infected Men Who Have Sex with Men (MSM): A Single-center, Retrospective Study.

Introduction: The way syphilis affects the immunologic and virologic parameters of a human immunodeficiency virus (HIV) infection remains controversial. The aim of this study was to investigate the impact of syphilis infection on lymphocyte and lymphocyte subset counts as well as viral load in HIV-infected patients.

Methods: All HIV-infected patients attending the outpatient clinic for infectious diseases of Hannover Medical University Hospital diagnosed with syphilis between 2009 and 2016 were retrospectively evaluated for changes in total lymphocyte, B cell, CD3 T cell, CD4 and CD8 T cell counts as well as in HIV viral load.