Boris Hügle

ORCID: 0000-0003-1145-6719
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About
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Research Areas
  • Autoimmune and Inflammatory Disorders Research
  • Adolescent and Pediatric Healthcare
  • Inflammasome and immune disorders
  • Immunodeficiency and Autoimmune Disorders
  • Acute Lymphoblastic Leukemia research
  • Rheumatoid Arthritis Research and Therapies
  • Ocular Diseases and Behçet’s Syndrome
  • Immune Cell Function and Interaction
  • RNA and protein synthesis mechanisms
  • melanin and skin pigmentation
  • Systemic Lupus Erythematosus Research
  • Protease and Inhibitor Mechanisms
  • Pediatric Pain Management Techniques
  • Blood Coagulation and Thrombosis Mechanisms
  • Renal Diseases and Glomerulopathies
  • Lysosomal Storage Disorders Research
  • Kawasaki Disease and Coronary Complications
  • Fibromyalgia and Chronic Fatigue Syndrome Research
  • Spondyloarthritis Studies and Treatments
  • Pharmaceutical studies and practices
  • Viral-associated cancers and disorders
  • Inflammatory Myopathies and Dermatomyositis
  • Parvovirus B19 Infection Studies
  • RNA modifications and cancer
  • Multiple Sclerosis Research Studies

German Center for Pediatric and Adolescent Rheumatology
2014-2024

Universitäts-Kinderklinik Würzburg
2018

Universitätsklinikum Würzburg
2018

Aarhus University Hospital
2017

Children's of Alabama
2017

Alberta Children's Hospital
2017

University of Calgary
2017

Klinik und Poliklinik für Mund-, Kiefer- und Plastische Gesichtschirurgie
2017

Aarhus University
2017

Hospital for Sick Children
2009-2017

Objective Aims of the study were to investigate health-related quality life (HRQOL) in adult patients with former diagnosis Juvenile Idiopathic Arthritis (JIA), compare their HRQOL general population and identify factors related a poor outcome. Methods In 2012, cross-sectional survey was performed by mailing questionnaire large cohort current German Centre for Rheumatology Children Adolescents. Only (≥18 years) compatible JIA included (n = 2592; response 66%). The information about (EQ5D),...

10.1371/journal.pone.0153267 article EN cc-by PLoS ONE 2016-04-26

<b><i>Background:</i></b> Rapid-onset dystonia-parkinsonism (RDP) is an autosomal dominant disorder linked to chromosome 19q13 that characterized by sudden onset of primarily bulbar and upper limb dystonia with parkinsonism. <b><i>Methods:</i></b> The authors evaluated 12 individuals from three generations Irish family obtained detailed medical records on a deceased member. describe the clinical, psychiatric, genetic features affected individuals. <b><i>Results:</i></b> Five eight members...

10.1212/wnl.55.7.991 article EN Neurology 2000-10-10

Upon incubation of cultured rat cells with the adenosine analogue 5,6-dichloro-1-beta-D-ribofuranosylbenzimidazole (DRB), nucleoli reversibly dissociate into their substructures, disperse throughout nuclear interior, and form nucleolar "necklaces". We have used this experimental system, which does not inhibit transcription rRNA genes, to study by immunocytochemistry distribution active genes transcriptional products during dispersal recovery normal morphology. Antibodies RNA polymerase I...

10.1083/jcb.99.2.672 article EN The Journal of Cell Biology 1984-08-01

B cell development is a highly regulated process involving multiple differentiation steps, yet many details regarding this pathway remain unknown. Sequencing of patients with cell-restricted immunodeficiency reveals autosomal dominant mutations in TOP2B. TOP2B encodes type II topoisomerase, an essential gene required to alleviate topological stress during DNA replication and transcription, no previously known role development. We use Saccharomyces cerevisiae, knockin knockout murine models,...

10.1038/s41467-019-11570-6 article EN cc-by Nature Communications 2019-08-13

Wegener's granulomatosis (WG) is a devastating small-vessel vasculitis in children. Standard treatment consists of immunosuppressive medications with cyclophosphamide potentially associated significant infectious side effects, including Pneumocystis jiroveci pneumonia (PCP). Recently, rituximab, monoclonal antibody against B cells, has successfully been used refractory disease.We describe the first pediatric patient WG sinus and lung disease who developed PCP 6 months after while being...

10.1002/acr.20279 article EN Arthritis Care & Research 2010-06-25

To describe characteristics of patients with juvenile idiopathic arthritis (JIA) presenting isolated the temporomandibular joints (TMJ).Patients JIA TMJ from 4 large tertiary pediatric rheumatology centers were included. Demographic and clinical data analyzed using descriptive statistics.Fifty-five identified (65% bilateral presentation). Six developed in other (median time 6 mos); uveitis, all prior to arthritis. At last followup, 9% still taking antirheumatic medications.JIA can occur...

10.3899/jrheum.170263 article EN The Journal of Rheumatology 2017-09-01

Systemic juvenile idiopathic arthritis (sJIA) is commonly considered an autoinflammatory disease. However, sJIA patients may develop aggressive without systemic inflammation later in the disease, resembling autoimmune phenotype similar to other subtypes of JIA. The objective this study was determine whether antinuclear antibodies (ANA) and rheumatoid factor (RF) will with over course A single center sample follow-up more than one year obtained. retrospective chart survey used extract...

10.1186/1546-0096-12-28 article EN cc-by Pediatric Rheumatology 2014-07-16

Inflammatory bowel disease can develop in the context of some rheumatic diseases childhood, including juvenile idiopathic arthritis (JIA). (IBD) is frequently associated with other immune-mediated diseases; however, systemic onset JIA (sJIA) has not previously been connected to IBD. Treatment sJIA significantly changed recent years, possibly causing changes inflammatory patterns. Therefore, data from German Center for Pediatric and Adolescent Rheumtology 2010 until 2015 were analyzed by...

10.1186/s12969-017-0147-3 article EN cc-by Pediatric Rheumatology 2017-03-14

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in pathogenesis has been ascribed proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, course adverse effects real-life setting. In 2009 research...

10.1186/s12969-018-0236-y article EN cc-by Pediatric Rheumatology 2018-04-05

Using data from the German Biologics JIA Registry (BIKER), long-term safety of biologics for systemic-onset with regard to adverse events special interest was assessed.Safety assessments were based on event reports after first dose through 90 days last dose. Rates event, serious and 25 predefined analysed. Incidence rates compared each biologic against all other combined applying a mixed-effect Poisson model.Of 260 patients in this analysis, 151 received etanercept, 109 tocilizumab, 71...

10.1093/rheumatology/kez577 article EN Lara D. Veeken 2019-11-12

Flares of juvenile idiopathic arthritis (JIA) have been described in the context various infections. rheumatic diseases adults following infection with SARS-CoV-2 several cohorts. So far, effect on course JIA is unknown.

10.1186/s12969-021-00653-8 article EN cc-by Pediatric Rheumatology 2021-11-29

Although several studies show that JIA-patients have significantly lower employment rates than the general population, research on educational and occupational attainments in patients with juvenile idiopathic arthritis (JIA) remain conflicting most likely due to small sample sizes. Therefore, aim of this study is compare achievements status 3698 German population (GGP). "SEPIA" was a large cross-sectional current historic cohort treated single center between 1952 2010. For analyses education...

10.1186/s12969-017-0172-2 article EN cc-by Pediatric Rheumatology 2017-05-22

Objectives: Chronic anterior uveitis is a serious complication of juvenile idiopathic arthritis (JIA); disease flares are highly associated with loss vision. Leflunomide (LEF) used successfully for JIA joint but its effectiveness in has not been determined. The aim this study was to determine whether LEF improves flare rates patients compared preceding methotrexate (MTX) therapy.Method: A single-centre retrospective consecutive children and chronic performed. All initially received MTX were...

10.3109/03009742.2015.1013983 article EN Scandinavian Journal of Rheumatology 2015-05-20
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