RESUMEN
BACKGROUND: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. METHODS: We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. RESULTS: One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. CONCLUSIONS: We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.
Asunto(s)
Dermatomiositis , Sistema de Registros , Humanos , Dermatomiositis/epidemiología , Dermatomiositis/diagnóstico , Femenino , Niño , Masculino , España/epidemiología , Pronóstico , Estudios Retrospectivos , Preescolar , Autoanticuerpos/sangre , Debilidad Muscular/etiología , Debilidad Muscular/epidemiología , Trastornos de Deglución/etiología , Trastornos de Deglución/epidemiologíaRESUMEN
[This corrects the article DOI: 10.3389/fped.2022.1017035.].
RESUMEN
OBJECTIVE: To analyse factors involved in the decision to optimise biologics in juvenile idiopathic arthritis. METHODS: A "discrete-choice" methodology was used. In a nominal group meeting, factors which may influence physicians' decisions to optimise biological dose were identified, together with decision nodes. 1000Minds® was used to create multiple fictitious clinical scenarios based on the factors identified, and to deploy surveys that were sent to a panel of experts. These experts decided for each item which of two clinical scenarios prompted them to optimise the dose of biologic. A conjoint analysis was carried out, and the partial-value functions and the weights of relative importance calculated. RESULTS: In the nominal group, three decision nodes were identified: (1) time to decide; (2) to maintain/reduce or prolong interval; (3) what drug to reduce. The factors elicited were different for each node and included patient and drug attributes. The presence of macrophage activation syndrome (MAS), systemic involvement, or subclinical inflammation made the decision easier (highest weights). The presence of joints of difficult control and year of debut influenced the decision in some but not all, and in different directions. Immunogenicity, adherence, and concomitant treatments were also aspects taken into account. CONCLUSIONS: The decision to optimise the dose of biological therapy in children and youngster can be divided into several nodes, and the factors, both patient and therapy-related, leading to the decision can be detailed. These decisions taken by experts may be transported to practice, study designs, and guidelines.
Asunto(s)
Artritis Juvenil , Humanos , Niño , Artritis Juvenil/tratamiento farmacológico , Artritis Juvenil/complicaciones , Factores Biológicos/uso terapéutico , Terapia Biológica/métodos , Encuestas y CuestionariosRESUMEN
Tumor-necrosis-factor-α inhibitors (anti-TNF-α) are associated with an increased risk of tuberculosis (TB) disease, primarily due to reactivation of latent TB infection (LTBI). We assessed the performance of parallel LTBI screening with tuberculin skin test (TST) and QuantiFERON-TB Gold In-Tube assays (QFT-GIT) before anti-TNF-α treatment in children with immune-mediated inflammatory disorders in a low TB-burden setting. We conducted a multicenter cohort study involving 17 pediatric tertiary centers in Spain. LTBI was defined as the presence of a positive TST and/or QFT-GIT result without clinical or radiological signs of TB disease. A total of 270 patients (median age:11.0 years) were included, mainly with rheumatological (55.9%) or inflammatory bowel disease (34.8%). Twelve patients (4.4%) were diagnosed with TB infection at screening (LTBI, n = 11; TB disease, n = 1). Concordance between TST and QFT-GIT results was moderate (TST+/QFT-GIT+, n = 4; TST-/QFT-GIT+, n = 3; TST+/QFT-GIT-, n = 5; kappa coefficient: 0.48, 95% CI: 0.36-0.60). Indeterminate QFT-GIT results occurred in 10 patients (3.7%) and were associated with young age and elevated C-reactive protein concentrations. Eleven of 12 patients with TB infection uneventfully completed standard LTBI or TB treatment. During a median follow-up period of 6.4 years, only 2 patients developed TB disease (incidence density: 130 (95% CI: 20-440) per 100,000 person-years), both probable de novo infections. CONCLUSION: A substantial number of patients were diagnosed with LTBI during screening. The dual strategy identified more cases than either of the tests alone, and test agreement was only moderate. Our data show that in children in a low TB prevalence setting, a dual screening strategy with TST and IGRA before anti-TNF-α treatment is effective. WHAT IS KNOWN: ⢠The optimal screening strategy for latent tuberculosis in children with immune-mediated inflammatory disorders remains uncertain. ⢠Children receiving anti-TNF-α drugs are at increased risk of developing severe tuberculosis disease. WHAT IS NEW: ⢠A dual screening strategy, using TST and an IGRA assay, identified more children with latent tuberculosis than either of the tests alone. ⢠Identification and treatment of latent tuberculosis before initiation of anti-TNF-α therapy averted incident tuberculosis cases.
Asunto(s)
Tuberculosis Latente , Tuberculosis , Humanos , Niño , Prueba de Tuberculina/métodos , Tuberculosis Latente/diagnóstico , Tuberculosis Latente/tratamiento farmacológico , Tuberculosis Latente/epidemiología , Tuberculina/uso terapéutico , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico , Factor de Necrosis Tumoral alfa/uso terapéutico , España/epidemiología , Estudios de Cohortes , Ensayos de Liberación de Interferón gamma/métodosRESUMEN
INTRODUCTION/OBJECTIVES: Asian scores developed to predict unresponsiveness to intravenous immunoglobulin (IVIG) or development of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) are not appropriate in Western populations. The purpose of this study is to develop 2 scores, to predict unresponsiveness to IVIG and development of CAA, appropriate for Spanish population. METHOD: Data of 625 Spanish children with KD collected retrospectively (2011-2016) were used to identify variables to develop the 2 scores of interest: unresponsiveness to IVIG and development of CAA. A statistical model selected best variables to create the scores, and scores were validated with data from 98 patients collected prospectively. RESULTS: From 625 patients of the retrospective cohort, final analysis was performed in 439 subjects: 37 developed CAA, and 212 were unresponsive to IVIG. For the score to predict CAA, a cutoff ≥ 8 was considered for high risk, considering a score system with a different weight for each of the eight variables. External validation showed a sensitivity of 22% and a specificity of 75%. The score to predict unresponsiveness to IVIG established a cutoff ≥ 8 for high risk, considering a score system with a different weight for each of the nine variables. External validation showed a sensitivity of 78% and a specificity of 50%. CONCLUSIONS: Two risk scores for KD were developed from Spanish population, to predict development of CAA and unresponsiveness to IVIG; validation in other cohorts could help to implement these tools in the management of KD in other Western populations.
Asunto(s)
Aneurisma Coronario , Kava , Síndrome Mucocutáneo Linfonodular , Niño , Humanos , Lactante , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Estudios Retrospectivos , Aneurisma Coronario/etiología , Aneurisma Coronario/epidemiología , Factores de RiesgoRESUMEN
BACKGROUND: Osteoarticular infections (OAIs) are typically treated initially with intravenous antibiotics. The objective of this study was to evaluate whether an exclusive oral treatment in selected children may be appropriate. METHODS: The Spanish Network of Osteoarticular Infections is a nationwide multicenter registry comprising 37 hospitals in Spain. The registry prospectively includes clinical characteristics and outcome of children with OAI. One of the hospitals from RioPed offers oral treatment to children meeting certain criteria. Patients were classified into 2 groups. Group 1: management with initial intravenous antibiotic therapy. Group 2: patients exclusively treated with oral antibiotics. A comparison between the 2 groups was performed. RESULTS: We compared 893 children who initially received intravenous antibiotics (group 1) with 64 children who received exclusively oral therapy (group 2). Patients from group 2 were younger (33.9 vs. 20.3 months; P = 0.001), had a lower percentage of Staphylococcus aureus (23.3% vs. 3.1%; P < 0.001), a higher proportion of Kingella kingae (12.1% vs. 28.1%; P = 0.001), higher erythrocyte sedimentation rate/C-reactive protein (CRP) ratio (1.4 interquartile range 0.6-3.6 vs. 3.3 interquartile range 1.7-5.7; P < 0.001) and showed lower rate of fever (63% vs. 48.8%; P = 0.024) than in group 1. Complications were not found in group 2. CONCLUSIONS: An exclusively oral administration could be a safe option in selected patients with OAI. Low-risk criteria are proposed: good general condition, no underlying disease, 6 months to 3 years old, appropriate oral tolerance, C-reactive protein <80 mg/L, erythrocyte sedimentation rate/C-reactive protein ratio ≥0.67, no skin injury, no recent surgery, no cervical spondylodiscitis and no local complications at onset.
Asunto(s)
Artritis Infecciosa , Kingella kingae , Osteomielitis , Administración Oral , Antibacterianos/uso terapéutico , Artritis Infecciosa/tratamiento farmacológico , Proteína C-Reactiva , Niño , Humanos , Osteomielitis/tratamiento farmacológicoRESUMEN
OBJECTIVES: Negative health care encounters have psychological and behavioral consequences for patients, particularly for minority and low socioeconomic populations. Guided by an integrative model of culture, psychological processes, and health behavior, this study examined whether provider cultural competence reduces the emotional and behavioral consequences of negative health care encounters among Latina and non-Latino White American women in the United States. METHOD: A total of 335 women participated in the study, of which 236 (Latina = 112; non-Latino White = 124) reported at least one negative health care encounter during a preventive medical screening exam. Structural equation causal modeling was used to examine whether provider cultural competence, as perceived by the patient, influenced emotions associated with negative health care encounters and subsequent medical avoidance. RESULTS: When both Latina and non-Latino White American patients perceived their provider to be higher in cultural competence, they experienced less shame and embarrassment related to the negative encounter. Lower levels of shame and embarrassment in turn, predicted less medical avoidance for Latina, but not non-Latino White American women. CONCLUSIONS: Findings revealed that provider cultural competence reduces some of the consequences of negative health care encounters that are relevant to health behavior. These findings shed light on the complexity of how providers' cultural competence impacts patient behavior, highlighting the importance of including psychological variables when investigating the role of cultural competence in health behavior and outcome. (PsycInfo Database Record (c) 2020 APA, all rights reserved).
Asunto(s)
Competencia Cultural/psicología , Conductas Relacionadas con la Salud , Grupos Minoritarios/psicología , Relaciones Profesional-Paciente , Adulto , Actitud del Personal de Salud , Femenino , Hispánicos o Latinos/psicología , Humanos , Persona de Mediana Edad , Estados Unidos , Población Blanca/psicologíaRESUMEN
AIM: The traditional approach for acute paediatric osteoarticular infections (OAI) has comprised initial intravenous antibiotics followed by prompt oral antibiotics. We assessed how providing just oral antibiotics compared to the traditional two-step approach. METHODS: This prospective study was performed at the Hospital La Paz, Madrid, Spain, from September 2015 to September 2016. We compared 25 outpatients, with good general health and a mean age of 25 months who received just oral antibiotics, with 228 hospitalised children of a similar age who received intravenous and oral antibiotics from other hospitals in the Spanish Network of Osteoarticular Infections. RESULTS: The groups were comparable in terms of age, sex, fever, erythrocyte sedimentation rate value, C-reactive protein and diagnosis. The oral group comprised 15 with osteomyelitis, seven with septic arthritis, two with osteoarthritis and one with spondylodiscitis. This group had a lower percentage of Staphylococcus aureus (8% vs 26%, p = 0.06) and higher proportion of Kingella kingae (24% vs 9%, p = 0.017) than the intravenous group. There were complications (24%) and follow-up sequelae (6%) in the intravenous group, but none in the oral group. CONCLUSION: Outpatients with OAI who were in good general health had favourable outcomes when they received oral antibiotics without intravenous antibiotics.
Asunto(s)
Antibacterianos/administración & dosificación , Artritis Infecciosa/tratamiento farmacológico , Osteomielitis/tratamiento farmacológico , Administración Intravenosa , Administración Oral , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Acute osteoarticular infection (OAI) is a potentially severe disease. The aim of this study was to evaluate the etiology, clinical characteristics and therapeutic approach of OAI in children in Spain. METHODS: Medical records from children <14 years with OAI from 25 hospitals between 2008 and 2012 were reviewed. Confirmed osteomyelitis (OM) and septic arthritis (SA) required a positive bacterial isolate; otherwise, they were considered probable. Probable SA with <40,000 cells/mm in joint fluid was not included. RESULTS: A total of 641 children were evaluated. Two hundred and ninety-nine cases (46%) were OM, 232 (36%) SA, 77 (12%) osteoarthritis and 33 (5%) spondylodiscitis. Children with OM were older (63 vs. 43 months for SA; P < 0.001). Magnetic resonance imaging and bone scintigraphy had the highest yield for OM diagnosis (94%). Arthrocentesis was performed in 96% of SA. A microorganism was isolated in 246 patients (38%: 33% OM vs. 55% SA; P < 0.001): Staphylococcus aureus was the most common (63%), followed by Kingella kingae (15%) and Streptococcus pyogenes (9%). Ninety-five percent of children initially received IV antibiotics, mostly cefotaxime + cloxacillin (60%) or cloxacillin (40%). Total treatment duration was 38 (±31) days for OM and 28 (±16) days for SA (P < 0.0001). Twenty percent of children with OM (46% because of complications) and 53% with SA (95% initial arthrotomy) underwent surgery. Patients with SA were compared according to initial arthrotomy (n = 123) versus arthrocentesis (n = 109), and no clinical differences were observed, except for higher rate of hip SA in the former (50% vs. 9%; P < 0.001). Children with arthrocentesis had less sequelae [6.6% vs. 1%; P = 0.03, odds ratio = 0.58 (95% confidence interval: 0.45-0.76)], but not in the multivariate analysis. CONCLUSIONS: This is the largest pediatric cohort of OAI in Spain. S. aureus was the most common isolate, although K. kingae was recovered in a high proportion of cases. Conservative management was applied in half of the patients. There was a low rate of sequelae, even with nonsurgical approaches.
Asunto(s)
Artritis Infecciosa/epidemiología , Artritis Infecciosa/terapia , Osteomielitis/epidemiología , Osteomielitis/terapia , Antibacterianos/uso terapéutico , Artritis Infecciosa/complicaciones , Artritis Infecciosa/microbiología , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Procedimientos Ortopédicos , Osteomielitis/complicaciones , Osteomielitis/microbiología , Estudios Retrospectivos , España/epidemiologíaRESUMEN
To develop recommendations on the transition from pediatric care to adult care in patients with chronic inflammatory rheumatic diseases with childhood onset based. Recommendations were generated following nominal group methodology and Delphi technique. A panel of 16 experts was established. A systematic literature review (on transitional care) and a narrative review were performed and presented to the panel in the first panel meeting to be discussed. A first draft of recommendations was generated and circulated. Focal groups with adolescents, young adults and parents were organized. In a second meeting, the focus group results along with the input from invited psychologist were used to establish definitive recommendations. Then, a Delphi process (two rounds) was carried out. A group of 72 pediatric and adult rheumatologists took part. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70 % voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford center for evidence-based medicine levels of evidence. Transition care was defined as a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic inflammatory rheumatic diseases with childhood onset as they move from child-centered to adult-oriented healthcare systems. The consensus covers: transition needs, barriers and facilitators, transitional issues (objectives, participants, content, phases, timing, plans, documentation and responsibilities), physicians' and other health professionals' knowledge and skill requirements, models/programs, and strategies and guideline for implementation. Preliminary recommendations and agreement grade are shown in the Table (first Delphi round). These recommendations are intended to provide health professionals, patients, families and other stakeholders with a consensus on the transition process from pediatric to adult care.
Asunto(s)
Pediatría , Enfermedades Reumáticas/terapia , Reumatología , Transición a la Atención de Adultos , Adolescente , Adulto , Consenso , Humanos , España , Adulto JovenRESUMEN
Regular screening methods may miss the diagnosis of occult hepatitis B infection and seronegative hepatitis C virus infection in immunocompromised patients. A cross-sectional study within a Spanish cohort of HIV-infected children yielded 6 of 254 (2.4%) possible occult hepatitis B infection cases and 2 of 254 (0.8%) seronegative hepatitis C virus-infected patients. Implementation of occult hepatitis screening in the routine care of these children may be warranted.