Crons disease

Primary motor cortex (M1) consists of a stack of interconnected but distinct layers (L1-L6) which affect motor control through large-scale networks. However, the brain-wide functional influence of each layer is poorly understood. We sought to expand our knowledge of these layers' circuitry by combining Cre-driver mouse lines, optogenetics, fMRI, and electrophysiology. Neuronal activities initiated in Drd3 neurons (within L2/3) were mainly confined within M1, while stimulation of Scnn1a, Rbp4, and Ntsr1 neurons (within L4, L5, and L6, respectively) evoked distinct responses in M1 and motor-related subcortical regions, including striatum and motor thalamus...

OBJECTIVES: Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, dysfunction and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. OBJECTIVES: 1) To develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA...

Objective: Macrophage activation syndrome (MAS) is a life-threatening, potentially fatal condition associated with systemic juvenile idiopathic arthritis (sJIA). Interleukin-1 (IL-1) is a key cytokine in the pathogenesis of sJIA MAS. Many cases of MAS are medically refractory to traditional doses of biologic cytokine inhibitors and may require increased dosing. When MAS occurs in the setting of sJIA treated with the IL-1 receptor antagonist (IL-1Ra), anakinra, increased anakinra dosing may be beneficial...

Deceased donor kidney procurement biopsies findings are the most common reason for kidney discard. Retrospective studies have found inconsistent associations with post-transplant outcomes but may have been limited by selection bias because kidneys with advanced nephrosclerosis from high-risk donors are typically discarded. We conducted a retrospective cohort study of kidneys transplanted in the United States from 2015 to 2019 with complete biopsy data available, defining "suboptimal histology" as glomerulosclerosis ≥11%, IFTA ≥mild, and/or vascular disease ≥mild...

PURPOSE OF REVIEW: Multisystem inflammatory syndrome in children (MIS-C) is a postinfectious complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection primarily affecting children. MIS-C shares features with Kawasaki disease (KD) and cytokine storm syndrome (CSS) frequently requiring intensive care support. Although intravenous immunoglobulin (IVIg) and glucocorticoids (GCs) are effective therapeutics for most, refractory MIS-C is treated with various biologic disease-modifying antirheumatic drugs (bDMARDs)...

OBJECTIVE: To evaluate the relationship between maternal COVID-19 infection and the odds of in-hospital exclusive breastfeeding for term newborns. DESIGN: Retrospective descriptive quantitative. SETTING: A large, urban hospital with more than 6,000 births annually. SAMPLE: Term newborns born between March 1, 2020, and March 31, 2021 (N = 6,151). METHODS: We retrospectively extracted data from electronic health records to evaluate the relationship of maternal COVID-19 infection with the odds of in-hospital exclusive breastfeeding using univariate analysis and logistic regression models...

Recent development of new medications has changed the juvenile idiopathic arthritis (JIA) treatment goal to inactive disease. With numerous options, how does a clinician choose which medication to use? Treatment options may depend on the clinical classification and a new paradigm considers the JIA subtypes in reference to categories of adult inflammatory arthritis; poligo JIA, spondyloarthritis JIA, and systemic JIA that can help guide a clinician in determining treatment options. Treatment strategies such as consensus treatment plans can provide guidance on treatment escalation...

Background: The objective of this study was to evaluate the relia-bility and validity of the lifestyle (Miller-Smith) and life expectancy (Schneider) questionnaires in the Italian setting in order to make this instrument available for the determination of lifestyle and hope level in the different domains of everyday life. Method: Before testing their psychometric properties, the original versions of the two questionnaires, lifestyle (Miller-Smith) and life expectancy (Schneider), were translated into the Italian language...

STUDY OBJECTIVE: We evaluated whether and to what extent a novel medical student rotation in pediatric and adolescent gynecology (PAG) increases clinical knowledge and skills and meets student needs and expectations. DESIGN: Constructivist prospective pre-post study and post-rotation student survey SETTING: Academic medical center PARTICIPANTS: Pilot study of 9 medical students, which represents the entire population of those who completed the rotation. INTERVENTIONS: Four-week clinical rotation in PAG MAIN OUTCOME MEASURES: Changes in clinical knowledge were measured by a pre- and post-intervention multiple-choice assessment, and clinical skills were assessed before and after the intervention using entrustable professional activities (EPAs); these data were analyzed with paired Student's t tests...

The mechanisms explaining progression to severe COVID-19 remain poorly understood. It has been proposed that immune system dysregulation/over-stimulation may be implicated, but it is not clear how such processes would lead to respiratory failure. We performed comprehensive multiparameter immune monitoring in a tightly controlled cohort of 128 COVID-19 patients, and used the ratio of oxygen saturation to fraction of inspired oxygen (SpO2 / FiO2) as a physiologic measure of disease severity. Machine learning algorithms integrating 139 parameters identified IL-6 and CCL2 as two factors predictive of severe disease, consistent with the therapeutic benefit observed with anti-IL6-R antibody treatment...

PURPOSE: The purpose of this study was 1) to compare condyle - fossa relationships in the temporomandibular joint (TMJ), and 2) to score condylar resorption by using a TMJ indexing system in patients with JIA and without JIA. METHODS: The present retrospective cross-sectional study included cone-beam computed tomography (CBCT) images obtained from the sagittal, coronal, and axial slices. In the multidisciplinary Pediatric Rheumatology Outpatient Clinic at The University of Alabama at Birmingham (UAB) children with JIA are also examined by a group of orthodontists working in the same institute from October 2018 to July 2019...

OBJECTIVE: The objective of this study is to evaluate pediatric patients using existing macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) scoring systems to determine how these systems identify patients with cytokine storm syndrome (CSS) in the setting of a multisystem inflammatory syndrome in children (MIS-C) and active coronavirus disease 2019 (COVID-19) infection. METHODS: Hospitalized pediatric patients with MIS-C and active COVID-19 infection at a single institution were identified...

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Various professional organizations have weighed in on the practice utility of speculum examinations. The US Preventive Services Task Force (USPSTF), American Academy of Pediatrics (AAP), and American College of Physicians (ACP) have recommended against internal pelvic examinations in asymptomatic patients, whereas the American College of Obstetricians and Gynecologists (ACOG) has encouraged shared decision making. Although a pelvic examination can be a stressful event for adolescents, studies have shown that early discussions and performing the examination in a trusting environment can mitigate the worries and improve the patient-provider relationship...

OBJECTIVE: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA). STUDY DESIGN: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS...

PURPOSE: To determine if pharmacokinetic modeling of DCE-MRI can diagnose CS-PCa in PI-RADS category 3 PZ lesions with subjective negative DCE-MRI. MATERIALS AND METHODS: In the present IRB approved, bi-institutional, retrospective, case-control study, we identified 73 men with 73 PZ PI-RADS version 2.1 category 3 lesions with MRI-directed-TRUS-guided targeted biopsy yielding: 12 PZ CS-PCa (ISUP Grade Group 2; N = 9, ISUP 3; N = 3), 27 ISUP 1 PCa and 34 benign lesions...

The COVID-19 pandemic has resulted in more than 2 million deaths globally. Two interconnected stages of disease are generally recognised; an initial viral stage and a subsequent immune response phase with the clinical characteristics of hyperinflammation associated with acute respiratory distress syndrome. Therefore, many immune modulators and immunosuppressive drugs, which are widely used in rheumatological practice, have been proposed as treatments for patients with moderate or severe COVID-19. In this Review, we provide an overview of what is currently known about the efficacy and safety of antirheumatic therapies for the treatment of patients with COVID-19...

There is a critical shortage of deceased human donor organs for transplantation. The need is perhaps most acute in neonates and infants with life-threatening congenital heart disease, in whom mechanical support devices are largely unsuccessful. If orthotopic (life-supporting) heart transplantation (OHTx) were consistently successful in the genetically engineered pig-to-nonhuman primate (NHP) model, a clinical trial of bridging with a pig heart in such patients might be justified. However, the results of pig OHTx in NHPs have been mixed and largely poor...

Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease affecting the joints and other organs that occurs in 1 in 1,000 children in the United States. Given the various categories of JIA, interpretation of the literature can be difficult. In this review, new developments in understanding non-systemic JIA and its treatment will be covered. Recent advances in the journey toward personalized treatment in JIA will be highlighted, including a review of currently available biologic modifiers. Uveitis and the temporomandibular joint will be discussed as particularly challenging treatment issues...

IMPORTANCE: Active pediatric COVID-19 pneumonia and MIS-C are two disease processes requiring rapid diagnosis and different treatment protocols. OBJECTIVE: To distinguish active pediatric COVID-19 pneumonia and MIS-C using presenting signs and symptoms, patient characteristics, and laboratory values. DESIGN: Patients diagnosed and hospitalized with active COVID-19 pneumonia or MIS-C at Children's of Alabama Hospital in Birmingham, AL from April 1 through September 1, 2020 were identified retrospectively...