Surgical Management of Juvenile Idiopathic Arthritis Related Dentofacial Deformities

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood and frequently affects the temporomandibular joints (TMJ). JIA TMJ management requires a team approach. Initially, TMJ JIA involvement is managed with systemic therapy or intra-articular medications to treat symptoms and limit the growth deformity, however may later require surgical intervention. This case series describes 4 patients with different presentations and treatments of juvenile idiopathic arthritis affecting the temporomandibular joints to illustrate a proposed treatment algorithm. This algorithm is not designed to be an absolute treatment regimen but a framework to help clarify the presenting problems and interventions that may be considered to treat JIA associated temporomandibular dysfunction and dentofacial deformity. This case series represents the variety of JIA temporomandibular joint disease and offers a graduated appropriate treatment algorithm.

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Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia

The Journal of Laryngology & Otology ◽

10.1017/s0022215115000365 ◽

2015 ◽

Vol 129 (5) ◽

pp. 410-415 ◽

Cited By ~ 8

Author(s):

I Syed ◽

V S Sunkaraneni

Keyword(s):

Assessment Tool ◽

Case Reports ◽

Treatment Algorithm ◽

Case Series ◽

Team Approach ◽

Hereditary Haemorrhagic Telangiectasia ◽

Questionnaire Studies ◽

Randomised Controlled ◽

Specific Management

AbstractBackground:There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis.Method:The Medline and Embase databases were interrogated on 15 November 2013 using the search items ‘hereditary haemorrhagic telangiectasia’ (title), ‘epistaxis’ (title) and ‘treatment’ (title and abstract), and limiting the search to articles published in English.Results:A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3in vitrostudies.Conclusion:There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

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Initial radiological signs of dentofacial deformity in juvenile idiopathic arthritis

Scientific Reports ◽

10.1038/s41598-021-92575-4 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Peter Stoustrup ◽

Michelle Sys Traberg ◽

Louise Hauge Matzen ◽

Mia Glerup ◽

Annelise Küseler ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Treatment Outcome ◽

Early Diagnosis ◽

Occlusal Plane ◽

Surface Erosion ◽

Facial Skeleton ◽

Timely Initiation ◽

Orthopaedic Treatment ◽

Dentofacial Deformities ◽

Dentofacial Deformity

AbstractJuvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood and the temporomandibular joint (TMJ) is often involved. TMJ arthritis in growing individuals can cause deformation of facial skeleton (dentofacial deformity) and TMJ components (TMJ deformity). Treatment outcome hinges on early initiation of anti-inflammatory treatment and orthopaedic treatment with dental splints. The aim of the present study was to characterize the radiological signs of dentofacial deformity in patients with a JIA-induced need for orthopaedic treatment. We retrospectively studied 96 patients with JIA and 20 non-JIA controls to identify the initial radiological signs of JIA-induced dentofacial deformity leading to initiation of orthopaedic treatment. We found that initial radiological signs of dentofacial deformities were subtle and characterized by minor mandibular asymmetry and occlusal plane steepening. Radiological findings of TMJ deformity associated with initial dentofacial deformity were frequent and characterized by condylar articular surface flattening (OR 8.42), condylar subcortical cyst (OR 5.94), condylar surface erosion (OR 5.38) and condylar deviation in form (OR 25.39). Radiological signs of TMJ deformity were also documented in TMJs considered “healthy” during initial clinical and radiological examination. This study presents new knowledge of importance for early diagnosis of dentofacial deformity in JIA. Early diagnosis of dentofacial deformity is important as treatment outcome is greatly influenced by timely initiation.

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POS0068 HIGH LEVELS OF PORPHYROMONAS GINGIVALIS AND PREVOTELLA INTERMEDIA ANTIBODIES IN CHILDREN WITH JUVENILE IDIOPATHIC ARTHRITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2222 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 240.2-241

Author(s):

F. Zekre ◽

R. Cimaz ◽

M. Paul ◽

J. L. Stephan ◽

S. Paul ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Juvenile Idiopathic Arthritis ◽

Periodontal Disease ◽

Porphyromonas Gingivalis ◽

Joint Disease ◽

Control Group ◽

Prevotella Intermedia ◽

Igg Antibodies ◽

Idiopathic Juvenile Arthritis ◽

High Level

Background:Idiopathic juvenile arthritis (JIA) is a heterogeneous group of pathologies whose origin remains unknown at present (1). They are characterised by a systemic inflammatory and joint disease affecting children under 16 years of age. The current classification groups the different forms of JIA into 7 distinct entities (systemic forms, polyarticular forms with or without rheumatoid factors, oligoarticular forms, inflammatory arthritis associated with enthesopathies (ERA), arthritis associated with psoriasis and unclassifiable arthritis). Exact etiology of JIA is still unknown. To date, the various hypotheses put forward on the occurrence of JIAs integrate the genetic and environmental framework.The link between periodontal disease and rheumatoid arthritis (RA) is largely reported. Recently, Porphyromonas gingivalis (P. gingivalis) infection explained the occurrence of arthritis in rodent and in RA (2). Several studies mention the beneficial effect of P. gingivalis treatment on disease activity.Currently, there are very few studies on the prevalence of P. gingivalis in patients with JIA and the possible involvement of the germ in the development of inflammatory joint diseases in the pediatric population(3)(4).Objectives:The objective of our study is to determine presence of high IgG antibodies against P. gingivalis and Prevotella Intermedia in a cohort of patients with JIA compared to a control population and to determine variation of level according to sub-classes of JIA.Methods:Sera were obtained from 101 patients satisfying the ILAR classification criteria for JIA and in 25 patients with two other dysimmune disorders (type 1 diabetes and juvenile inflammatory bowel disease). Level of IgG antibodies against P. gingivalis and Prevotella Intermedia were obtained by homemade ELISA already used previously (5).Results:In the JIA group, major children were oligarthritis (47.5%), polyarthritis represents 31.7% of JIAs, ERA and systemic forms of JIA are respectively 9 and 11%. For the control group, 10 (40%) children had diabetes and 15 (60%) had IBD.Levels of anti-P. gingivalis anti-Prevotella Intermedia antibodies were higher in AJI group compared at control groups (P<0.01, P<0.05). Theses difference are mainly related to oligoarthritis and ERA subsets for both P. gingivalis and Prevotella Intermedia.Figure 1.Relative titer of antibodies to P. gingivalis and anti Prevotella intermedia. *: P<0.05; **: P<0.01; ***: P<0.001. P. gingivalis (control vs oligoarthritis p= 0.0032. control vs ERA p= 0.0092). Prevotella intermedia (control vs oligoarthritis p= 0.0194. control vs ERA p= 0.0039).Conclusion:We confirmed high level of anti-P. gingivalis and anti-Prevotella intermedia antibodies in JIA compared to other inflammatory disorders. For the first time, we observed that this high level was mainly in oligoarthritis and ERA. Further investigations are required to investigate involvement of oral dysbiosis in AJI pathogenesis. As observed in RA, it could be a new way to integrate in JIA therapy management.References:[1]Thatayatikom A, De Leucio A. Juvenile Idiopathic Arthritis (JIA). StatPearls Publishing; 2020[2]Cheng Z, Meade J, Mankia K, Emery P, Devine DA. Periodontal disease and periodontal bacteria as triggers for rheumatoid arthritis. Best Pract Res Clin Rheumatol. 2017;31(1):19–30.[3]Romero-Sánchez C, Malagón C, Vargas C, Fernanda Torres M, Moreno LC, Rodríguez C, et al. Porphyromonas Gingivalis and IgG1 and IgG2 Subclass Antibodies in Patients with Juvenile Idiopathic Arthritis. J Dent Child Chic Ill. 2017 May 15;84(2):72–9.[4]Lange L, Thiele GM, McCracken C, Wang G, Ponder LA, Angeles-Han ST, et al. Symptoms of periodontitis and antibody responses to Porphyromonas gingivalis in juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2016 Feb 9[5]Rinaudo-Gaujous M, Blasco-Baque V, Miossec P, Gaudin P, Farge P, Roblin X, et al. Infliximab Induced a Dissociated Response of Severe Periodontal Biomarkers in Rheumatoid Arthritis Patients. J Clin Med. 2019 May 26;8(5).Disclosure of Interests:None declared.

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Endocrine Dysfunction in Children with Zika-Related Microcephaly Who Were Born during the 2015 Epidemic in the State of Pernambuco, Brazil

Viruses ◽

10.3390/v13010001 ◽

2020 ◽

Vol 13 (1) ◽

pp. 1

Author(s):

Andréia Veras Gonçalves ◽

Demócrito de B. Miranda-Filho ◽

Líbia Cristina Rocha Vilela ◽

Regina Coeli Ferreira Ramos ◽

Thalia V. B. de Araújo ◽

...

Keyword(s):

Optic Nerve ◽

Viral Infections ◽

Case Series ◽

Optic Nerve Hypoplasia ◽

Endocrine Dysfunction ◽

Careful Monitoring ◽

Early Puberty ◽

Growth Impairment ◽

Appropriate Treatment ◽

The Brain

Congenital viral infections and the occurrence of septo-optic dysplasia, which is a combination of optic nerve hypoplasia, abnormal formation of structures along the midline of the brain, and pituitary hypofunction, support the biological plausibility of endocrine dysfunction in Zika-related microcephaly. In this case series we ascertained the presence and describe endocrine dysfunction in 30 children with severe Zika-related microcephaly from the MERG Pediatric Cohort, referred for endocrinological evaluation between February and August 2019. Of the 30 children, 97% had severe microcephaly. The average age at the endocrinological consultation was 41 months and 53% were female. The most frequently observed endocrine dysfunctions comprised short stature, hypothyroidism, obesity and variants early puberty. These dysfunctions occurred alone 57% or in combination 43%. We found optic nerve hypoplasia (6/21) and corpus callosum hypoplasia (20/21). Seizure crises were reported in 86% of the children. The most common—and clinically important—endocrine dysfunctions were pubertal dysfunctions, thyroid disease, growth impairment, and obesity. These dysfunctions require careful monitoring and signal the need for endocrinological evaluation in children with Zika-related microcephaly, in order to make early diagnoses and implement appropriate treatment when necessary.

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Repair of proximal humerus fracture nonunions using a standardized treatment algorithm: a case series

European Journal of Orthopaedic Surgery & Traumatology ◽

10.1007/s00590-020-02857-2 ◽

2021 ◽

Author(s):

Kurtis D. Carlock ◽

Sanjit R. Konda ◽

Isabella R. Bianco ◽

Joseph D. Zuckerman ◽

Kenneth A. Egol

Keyword(s):

Proximal Humerus Fracture ◽

Proximal Humerus ◽

Treatment Algorithm ◽

Case Series ◽

Humerus Fracture ◽

Standardized Treatment ◽

Fracture Nonunions

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Bouveret’s Syndrome – Case Series with Therapeutic Implications

Zeitschrift für Gastroenterologie ◽

10.1055/a-1113-7966 ◽

2020 ◽

Vol 58 (04) ◽

pp. 352-356

Author(s):

Tobias Kukiolka ◽

Jan Borovicka ◽

Stephan Baumeler ◽

Marc Schiesser ◽

Christoph Gubler

Keyword(s):

Balloon Dilatation ◽

Rare Complication ◽

Gallstone Disease ◽

Treatment Algorithm ◽

Case Series ◽

Endoscopic Technique ◽

Bouveret’S Syndrome ◽

Therapeutic Implications ◽

Electrohydraulic Lithotripsy ◽

Bouveret's Syndrome

AbstractBouveret’s syndrome is a rare complication resulting from gallstone disease. Both surgical and endoscopical procedures are performed, with the disease to be seen as strictly interdisciplinary. There are no well-established recommendations for this condition. In this paper, we want to describe our experience from 6 cases in 3 Swiss hospitals from 2015 to 2017 with emphasis on the endoscopic technique of electrohydraulic lithotripsy followed by balloon dilatation and propose a treatment algorithm.

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AB0980 CASE-REVIEW ON FAMILIAL MEDITERRANEAN FEVER IN A SPECIALIZED CENTRE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1355 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1784.1-1784

Author(s):

R. Dos Santos Sobrín ◽

M. Martí Masanet ◽

B. Lopez-Montesinos ◽

L. Lacruz Pérez ◽

I. Calvo

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Familial Mediterranean Fever ◽

Periodic Fever ◽

Severe Disease ◽

Strong Association ◽

Mefv Gene ◽

Systemic Juvenile Idiopathic Arthritis ◽

Case Series ◽

Case Review ◽

Mediterranean Fever

Background:Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder caused mostly by mutations in MEFV gene. Its inheritance is autosomal recessive and is the most frequent periodic fever syndrome. First-line treatment is based in colchicine use, so biologics (anti-IL-1) are reserved for refractory cases1, 2.Objectives:To account for clinic and treatment features of patients with FMF in a specialized center as opposed to non-referent centers.Methods:This study was developed in the Pediatric Rheumatology Service in Hospital Universitario y Politécnico La Fe de Valencia. Demographic, clinic and treatment data were collected from patients diagnosed of FMF since January 2004 to September 2019.Results:106 patients met last FMF criteria3. 55% had a pathogenic mutation in genetic analysis. 52% were female. Before 10 years old, 71% of patients had the diagnosis (51% before 4 years old). Arthralgia/myalgia (73%), periodic fever (62%) and abdominal pain (54%) were the most common symptoms. Systemic Juvenile Idiopathic Arthritis (JIA, 6), other forms of JIA (9) and vasculitis (10) were the most prevalent comorbidities. When talking about treatment, 76,4% received Colchicine (60,5% with good response), 22,6% needed a classical disease modifying antirheumatic drug (mostly Methotrexate) and 22 patients got biologic treatment (73% anti-IL-1).Conclusion:When analyzing this case-review, JIA has a strong association with our patients, so it could explain severe disease activity and more articular involvement. This could be an illustration to the higher use of Methotrexate. Also, the most relevant symptom was arthralgia while fever is the most frequent in literature. Likewise, age of diagnosis has been earlier than other case-series (this would be more frequent in other autoinflammatory syndromes, as literature relates)1, 2, 4.References:[1]Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. (2019).[2]Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016;75:644-651.[3]Sag E, Demirel D, Demir S, et al. Performance of the new “Eurofever/PRINTO classification criteria” in FMF patients. Semin Arthritis Rheum. 2019;19:30369-5.[4]Rozenbaum M, Rosner I. Severe outcome of juvenile idiopathic arthritis (JIA) associated with familial Mediterranean fever (FMF). Clin Exp Rheumatol. 2004;22:S75-8.Disclosure of Interests:Raquel Dos Santos Sobrín: None declared, Miguel Martí Masanet: None declared, B Lopez-Montesinos: None declared, Lucía Lacruz Pérez: None declared, Inmaculada Calvo Grant/research support from: Bristol-Myers Squibb, Clementia, GlaxoSmithKline, Hoffman-La Roche, Merck Sharpe & Dohme, Novartis, Pfizer, Sanofi, Speakers bureau: AbbVie, GlaxoSmithKline, Hoffman-La Roche, Novartis

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Cryoprobe biopsy for the diagnosis of acute hypoxemic respiratory failure of undetermined origin

Journal of the Intensive Care Society ◽

10.1177/1751143719847323 ◽

2019 ◽

Vol 21 (2) ◽

pp. 119-123

Author(s):

Marcos J Las Heras ◽

Jose Dianti ◽

Manuel Tisminetzky ◽

Graciela Svetliza ◽

Sergio E Giannasi ◽

...

Keyword(s):

Respiratory Failure ◽

Diagnostic Yield ◽

Specific Treatment ◽

Case Series ◽

Safe Procedure ◽

Hypoxemic Respiratory Failure ◽

Acute Hypoxemic Respiratory Failure ◽

Select Group ◽

Lung Sample ◽

Appropriate Treatment

Rationale Acute hypoxemic respiratory failure is a condition that comprises a wide array of entities. Obtaining a histological lung sample might help reach a diagnosis and direct an appropriate treatment in a select group of patients. Objective To describe our experience in the use of cryobiopsy for the diagnosis of acute hypoxemic respiratory failure of undetermined origin. Methods Retrospective analysis of case series of patients with acute hypoxemic respiratory failure who underwent lung cryobiopsy at the Intensive Care Unit of the Hospital Italiano de Buenos Aires, Argentina. Results Cryobiopsy yielded a histological diagnosis in all patients ( n = 10, 100%). This led to either a change in therapy or continuation of a specific treatment in eight of these patients. Cryobiopsy was found to be contributive in all the patients who did not meet Berlin criteria for acute respiratory distress syndrome. No major complications were associated with the procedure. Conclusions Cryobiopsy is a safe procedure with a high diagnostic yield in a selected group of patients.

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Clinical Presentation, Management, and Prognosis of Pseudogout in Joint Arthroplasty: A Retrospective Cohort Study

Journal of Bone and Joint Infection ◽

10.7150/jbji.29983 ◽

2019 ◽

Vol 4 (1) ◽

pp. 20-26 ◽

Cited By ~ 6

Author(s):

Merit P. George ◽

Floranne C. Ernste ◽

Aaron Tande ◽

Douglas Osmon ◽

Tad Mabry ◽

...

Keyword(s):

Clinical Presentation ◽

Medical Center ◽

Case Reports ◽

Joint Infection ◽

Case Series ◽

Calcium Pyrophosphate ◽

Degenerative Joint Disease ◽

Joint Disease ◽

Antibiotic Overuse ◽

Concomitant Infection

Abstract. Introduction: Calcium pyrophosphate deposition disease (CPPD), or pseudogout, is rare in prosthetic joints, but can mimic prosthetic joint infection (PJI) according to case reports. The purpose of this case series is to describe the demographics, presentation, management, and outcomes of a cohort of these patients seen at our academic medical center.Methods: Patients with post-implant pseudogout, who were evaluated at our medical center between January 1, 2000 and June 30, 2016, were identified from our EHR. Data pertaining to demographics, presentation, management, and outcomes were abstracted, and patients were categorized into two groups based on presence of concomitant infection along with positive CPDD findings in synovial fluid.Results: 22 patients were included. 90.9% of cases involved a TKA. The most common indication for arthroplasty was degenerative joint disease. Only four patients had a history of previous gout or pseudogout, three of which belonged to the group with no evidence of concomitant joint infection. Clinical features for patients without concomitant infection included pain (100%), swelling at the joint (88.9%), redness (33.3%), fever (22.2%), and decreased range of motion (100%). 45.5% of patients received antibiotics prior to joint aspiration (44.4% of patients with negative synovial fluid cultures, 46.2% of patients with concomitant infection).Conclusion: Our study suggests similar clinical presentation between post-implant pseudogout and PJI. Among patients with pseudogout as well as in those with PJI, the first dose of antibiotics should not be given before sampling for synovial culture. Unfortunately, many patients receive antibiotics prior to culture ascertainment, which raises concern for antibiotic overuse.

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