bechet's disease

Krabbe's disease (KD) is caused by mutations in the lysosomal enzyme galactocerebrosidase and is associated with psychosine toxicity. The sphingosine 1-phosphate receptor (S1PR) agonist fingolimod (FTY720) attenuates psychosine-induced cell death of human astrocytes, demyelination in cerebellar slices, as well as demyelination in the central nervous system of twitcher mice. Psychosine also accumulates in the peripheral nervous system in twitcher mice; however, effects of fingolimod on this peripheral myelin have not been examined...

Acute lung injury (ALI) represents an aetiologically diverse form of pulmonary damage. Part of the assessment and diagnosis of ALI depends on skilled observer-based scoring of brightfield microscopy tissue sections. Although this readout is sufficient to determine gross alterations in tissue structure, its categorical scores lack the sensitivity to describe more subtle changes in lung morphology. To generate a more sensitive readout of alveolar perturbation we carried out high resolution immunofluorescence imaging on 200 μm lung vibratome sections from baseline and acutely injured porcine lung tissue, stained with a tomato lectin, Lycopersicon Esculentum Dylight-488...

Ketamine is a common anesthetic used in human and veterinary medicine. This drug has recently received increased medical and scientific attention due to its indications for neurological diseases. Despite being applied for decades, ketamine's entire metabolism and pharmacological profile have not been elucidated yet. Therefore, insights into the metabolism and brain distribution are important toward identification of neurological effects. Herein, we have investigated ketamine and its metabolites in the pig brain, cerebrospinal fluid, and plasma using mass spectrometric and metabolomics analysis...

AIM: The aim of this in vitro study was to investigate the influence of scan paths on the accuracy (trueness and precision) of intra-oral scanning of an implant impression on an edentulous patient. MATERIAL AND METHODS: An epoxy resin maxillary model was made with 6 bone level implants (NobelParallel Conical Connection RP, NobelBiocare®). The implants were placed at the spot of the first incisor, the canine and the first molar. The trans gingival component (Multi-unit, NobelBiocare®) was screwed onto the implants...

Aortic pseudoaneurysms (PA) vary in size and may remain asymptomatic. PAs may be caused by vascular injury, such as trauma or surgery, or other non-traumatic causes, such as Bechet disease, infection, or penetrating atherosclerotic ulcers. The diagnosis of PAs may have been delayed for decades. We present a case of a PA detected incidentally in a male patient who experienced traumatic bowel perforation due to blunt abdominal trauma 30 years before presentation. Computed tomography (CT) displayed a 9.2 cm mass in the pelvis, initially considered a neoplasm of small bowel origin...

BACKGROUND: In autumn 2022, the epidemics due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), respiratory syncytial virus (RSV), and influenza overlapped, and these diseases can present with the same symptomatology. The use of a triple antigen test (SARS-CoV-2 + influenza A/B + RSV) seems crucial for accurate viral diagnosis in the context of implementing long-acting monoclonal antibody vaccination against RSV in the upcoming RSV season. METHODS: We assessed the usefulness of the triple test in real life in this prospective study performed from October 2022 to May 2023 and involving 116 pediatricians (2 emergency department pediatricians and 114 ambulatory pediatricians)...

AIM: A20 haploinsufficiency (HA20) is a recently described autoinflammatory disease that manifests symptoms similar to those of Bechet's disease. However, little is known about the involvement of the liver in HA20. Herein, we report a case of HA20 complicated by autoimmune hepatitis (AIH). CASE PRESENTATION: A 33-year-old woman was previously diagnosed with HA20 and chronic thyroiditis and was treated with prednisolone (PSL) (7.5 mg/day) and levothyroxine sodium hydrate (125 µg/day)...

BACKGROUND: In France, vaccination has been implemented against Hi serotype b (Hib), pneumococcus with pneumococcal conjugate vaccines (PCV), and Neisseria meningitidis serogroup C (MenC). These interventions with different coverage and uptake have disrupted the epidemiology of vaccine-preventable acute bacterial meningitis (ABM). METHODS: We analyzed data from a French prospective surveillance network of ABM in children ≤15 years old enrolled by 259 pediatric wards (estimated national coverage: 61%)...

BACKGROUND: Behçet disease (BD) is a type of vasculitis with relapsing episodes and multisystemic clinical features, associated with significant morbidity and impact on patients' lives. People affected by BD often participate in discussions of their illness experiences. In-person support groups have limited physical accessibility and a relative lack of anonymity; however, online communities have become increasingly popular. OBJECTIVE: This study investigates the perspectives and experiences of people affected by BD by examining the content shared and discussed on a subforum of the website Reddit-a popular online space for anonymous discussions...

Using multiple national surveillance systems, we found an increase in invasive pneumococcal disease (IPD) incidence in the early post-COVID-19 pandemic period that strongly varied by age. Age-groups with higher incidence of respiratory syncytial virus and influenza also experienced higher increase in IPD incidence, with no change in pneumococcal carriage.

We aimed to estimate the respiratory syncytial virus positivity rate among ambulatory children with bronchiolitis according to the bronchiolitis epidemic period as defined by the French Public Health Institute. The positivity rate was 28.9% during the nonepidemic period and 50.6% during the epidemic period, which suggests continuous virus circulation between bronchiolitis annual peaks.

BACKGROUND: Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema. CASE PRESENTATION: An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months...

BACKGROUND: Economic prosperity has fostered numerous changes that may translate into better or worse outcomes across all domains of health. This study aims to explore the associations of economic development with uveitis onset in mainland China. METHODS: We used Poisson regression with generalized estimated equations to quantify the associations of per capita gross domestic product (GDP) with uveitis onset in 31 provinces of mainland China from 2006 to 2017. We further estimated the effects mediated by economic growth on the temperature-uveitis and PM2...

BACKGROUND: In children, respiratory infections such as SARS-CoV-2, respiratory syncytial virus (RSV), and influenza share similar clinical signs and symptoms. Here we compared the performance of a rapid antigen diagnostic test using a self-collected anterior nasal swab (COVID-VIRO ALL IN TRIPLEX) and multiplex RT-PCR. METHODS: From October to December 2022, in the emergency pediatrics unit of Orleans Hospital, France, we evaluated the diagnostic accuracy of the triplex test...

BACKGROUND: Behcet's disease (BD) is a systemic disease characterized by recurrent oral and genital ulcers. The underlying disease pathway likely involves interleukin (IL)-17 A, a proinflammatory cytokine that is implicated in Behcet's uveitis. Secukinumab is an anti-IL-17 A drug that may have an emerging role in the treatment of refractory BD. This is the first known case report of gastrointestinal BD flare up after anti-IL-17 A therapy. CASE PRESENTATION: We presented a case of BD with cutaneous and articular features being treated with secukinumab...

Group A Streptococcus is one of the leading causes of otorrhea. The performance of rapid antigen tests in 256 children with otorrhea showed excellent sensitivity, 97.3% (95% confidence interval: 90.7%-99.7%), and specificity, 100% (95% confidence interval: 98.0%-100%). In a period of increasing invasive and noninvasive group A Streptococcus infections, an early diagnosis could be useful.

OBJECTIVES: The aim of this study was to describe the bacterial profile of middle ear fluid from spontaneous perforation of the tympanic membrane (SPTM) prior to widespread utilization of third- generation pneumococcal conjugate vaccines (PCVs). PATIENTS AND METHODS: From October 2015 to January 2023, children with SPTM were prospectively enrolled by pediatricians. RESULTS: Among the 852 children with SPTM, 73.2% were less than 3 years old; more frequently than older children, they were and suffering from complex acute otitis media (AOM) (27...

OBJECTIVES: We evaluated the burden of non-invasive group A Streptococcus (GAS) infections in ambulatory pediatrics before and during the COVID-19 pandemic in France. METHODS: We analyzed data from a national network of ambulatory pediatricians between 2018 and 2022. Clinicians evaluating children ≤15 years old for tonsillopharyngitis, perianal infections, paronychia/blistering dactylitis and scarlet fever were invited to perform a rapid antigen detection test (RADT) for GAS...

OBJECTIVES: Following various changes in the vaccine strategy in 2013 and the mandatory vaccination in 2018, we aimed to analyze the vaccination status, age, and source of contamination of pertussis and parapertussis cases in outpatient surveillance. PATIENTS AND METHODS: Confirmed pertussis and parapertussis cases were enrolled by 35 pediatricians. RESULTS: From 2014 to 2022, 73 confirmed cases of pertussis (n=65) and parapertussis (n=8) were reported...

UNLABELLED: Although central nervous system demyelinating lesions as a side effect of tumor necrosis factor (TNF)-alpha inhibitors have been reported, this treatment is still used in some autoimmune diseases. CASE PRESENTATION: A 34-year-old Syrian male presented with difficulty walking and tingling, and numbness on the left side of his body over the next 4 days, during golimumab treatment. Over the past 2 months, fatigue, recurrent calf spasms, and extremity numbness were found...