Consensus document of the Spanish Society of Paediatric Infectious Diseases and the Advisory Committee on Vaccines of the Spanish Association of Pediatrics for vaccination of immunosuppressed individuals.

The number of people with immunosuppression is increasing considerably due to their greater survival and the use of new immunosuppressive treatments for various chronic diseases. This is a heterogeneous group of patients in whom vaccination as a preventive measure is one of the basic pillars of their wellbeing, given their increased risk of contracting infections. This consensus, developed jointly by the Sociedad Española de Infectología Pediátrica (Spanish Society of Pediatric Infectious Diseases) and the Advisory Committee on Vaccines of the Asociación Española de Pediatría (Spanish Association of Paediatrics), provides guidelines for the development of a personalised vaccination schedule for patients in special situations, including general recommendations and specific recommendations for vaccination of bone marrow and solid organ transplant recipients, children with inborn errors of immunity, oncologic patients, patients with chronic or systemic diseases and immunosuppressed travellers.

Documento de consenso de la Sociedad Española de Infectología Pediátrica y el Comité Asesor de Vacunas de la Asociación Española de Pediatría para la vacunación en inmunodeprimidos / Consensus document of the Spanish Society of Paediatric Infectious Diseases and the Advisory Committee on Vaccines of the Spanish Association of Pediatrics for vaccination of immunosuppressed individuals

El número de personas con inmunodepresión está aumentando considerablemente debido a su mayor supervivencia y al empleo de nuevas terapias inmunosupresoras en diversas patologías crónicas. Se trata de un grupo heterogéneo de pacientes en los que la vacunación como arma preventiva supone uno de los pilares básicos de su bienestar, por su elevado riesgo a padecer infecciones. Este consenso, elaborado conjuntamente entre la Sociedad Española de Infectología Pediátrica (SEIP) y el Comité Asesor de Vacunas de la Asociación Española de Pediatría (CAV-AEP), aporta unas directrices para programar un calendario adaptado a cada paciente en situaciones especiales que incluye recomendaciones generales, vacunación en pacientes con trasplante de médula y trasplante de órgano sólido, vacunación en niños con errores innatos de la inmunidad, vacunación en el paciente oncológico, vacunación en pacientes con enfermedades crónicas o sistémicas y vacunación en niños viajeros inmunodeprimidos.(AU)

The number of people with immunosuppression is increasing considerably due to their greater survival and the use of new immunosuppressive treatments for various chronic diseases. This is a heterogeneous group of patients in whom vaccination as a preventive measure is one of the basic pillars of their wellbeing, given their increased risk of contracting infections. This consensus, developed jointly by the Sociedad Española de Infectología Pediátrica (Spanish Society of Pediatric Infectious Diseases) and the Advisory Committee on Vaccines of the Asociación Española de Pediatría (Spanish Association of Paediatrics), provides guidelines for the development of a personalised vaccination schedule for patients in special situations, including general recommendations and specific recommendations for vaccination of bone marrow and solid organ transplant recipients, children with inborn errors of immunity, oncologic patients, patients with chronic or systemic diseases and immunosuppressed travellers.(AU)

Executive summary: Guidelines for the diagnosis and treatment of septic arthritis in adults and children, developed by the GEIO (SEIMC), SEIP and SECOT.

Infection of a native joint, commonly referred to as septic arthritis, is a medical emergency because of the risk of joint destruction and subsequent sequelae. Its diagnosis requires a high level of suspicion. These guidelines for the diagnosis and treatment of septic arthritis in children and adults are intended for use by any physician caring for patients with suspected or confirmed septic arthritis. They have been developed by a multidisciplinary panel with representatives from the Bone and Joint Infections Study Group (GEIO) belonging to the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC), the Spanish Society of Paediatric Infections (SEIP) and the Spanish Society of Orthopaedic Surgery and Traumatology (SECOT), and two rheumatologists. The recommendations are based on evidence derived from a systematic literature review and, failing that, on the opinion of the experts who prepared these guidelines. A detailed description of the background, methods, summary of evidence, the rationale supporting each recommendation, and gaps in knowledge can be found online in the complete document.

Position statement on infection screening, prophylaxis, and vaccination in pediatric patients with rheumatic diseases and immunosuppressive therapies, part 2: infection prophylaxis.

This study aims to provide practical recommendations on prophylaxis for infection in pediatric patients with immune-mediated rheumatic diseases receiving/scheduled to receive immunosuppressive therapy. A qualitative approach was applied. A narrative literature review was performed via Medline. Primary searches were conducted using MeSH terms and free text to identify articles that analyzed data on infections and vaccinations in pediatric patients with immune-mediated rheumatic diseases receiving immunosuppressive therapy. The results were presented and discussed in a nominal group meeting comprising a committee of 12 pediatric rheumatologists from the Prevention and Treatment of Infections Working Group of the Spanish Society of Pediatric Rheumatology. Several recommendations were generated. A consensus procedure was implemented via a Delphi process that was extended to members of the Spanish Society of Pediatric Rheumatology and the Vaccine Advisory Committee of the Spanish Association of Pediatrics. Participants produced a score ranging from 0 (completely disagree) to 10 (completely agree). Agreement was considered to have been reached if at least 70% of participants voted ≥ 7. The literature review included more than 400 articles. Overall, 63 recommendations were generated (23 on infection prophylaxis) and voted by 59 pediatric rheumatologists and other pediatric specialists, all of whom achieved the pre-established level of agreement. The recommendations on prophylaxis of infection cover vaccination and prophylaxis against varicella zoster virus, tuberculosis, Pneumocystis jiroveccii, and invasive fungal infections in pediatric patients with immune-mediated rheumatic diseases receiving/scheduled to receive immunosuppressive therapy.  Conclusion: Based on current evidence and a Delphi process, we provided consensus and updated recommendations on prophylaxis and treatment of infections to guide those caring for pediatric rheumatology patients. What is Known: •Data largely derived from adults find that infectious diseases and related complications are a major cause of morbidity and mortality in patients with immune-mediated rheumatic diseases. •It is crucial to be aware of the preventive measures that should be implemented to prevent these infections in children, although most guidelines are often extrapolated from adult cases. What is New: •In the absence of evidence, a literature review and a Delphi survey were conducted to establish a series of expert recommendations that could prove useful in clinical practice, providing a practical and simple day-to-day approach to be used by pediatric rheumatologists. •The recommendations focus on tuberculosis, herpes zoster virus, fungal infections, and Pneumocystis jirovecii.

Bronchiolitis before and after the SARS-CoV-2 pandemic: Twelve years of experience in a Spanish paediatric hospital.

INTRODUCTION: Acute bronchiolitis is the main cause of hospitalization in children under 2 years of age, with a regular seasonality, mostly due to the respiratory syncytial virus. OBJECTIVES: To describe the epidemiology of bronchiolitis hospitalizations in our center in the last 12 years, and analyze the changes in clinical characteristics, microbiology, and adverse outcomes during the SARS-CoV-2 pandemic. METHODS: Observational study including patients admitted for bronchiolitis between April 2010 and December 2021 in a Spanish tertiary paediatric hospital. Relevant demographic, clinical, microbiological, and adverse outcome variables were collected in an anonymized database. The pandemic period (April 2020 to December 2021) was compared to 2010-2015 seasons using appropriate statistical tests. RESULTS: There were 2138 bronchiolitis admissions, with a mean of 195.6 per year between 2010 and 2019 and a 2-4-month peak between November and March. In the expected season of 2020, there was a 94.4% reduction of bronchiolitis hospitalizations, with only 11 cases admitted in the first year of the pandemic. Bronchiolitis cases increased from the summer of 2021 during a 6-month long peak, reaching a total of 171 cases. Length of stay was significantly shorter during the pandemic, but no differences were found in clinical and microbiological characteristics or other adverse outcomes. CONCLUSIONS: The SARS-CoV-2 pandemic has modified the seasonality of bronchiolitis hospitalizations, with a dramatic decrease in cases during the expected season of 2020-2021, and an extemporaneous summer-autumn peak in 2021 with longer duration but similar patient characteristics and risk factors.

Enfermedad de Crohn en paciente con artritis idiopática juvenil de inicio sistémico: ¿asociación o complicación asociada al tratamiento? / Crohn's Disease in a Patient with Systemic Onset Juvenile Idiopathic Arthritis. Association or Associated Side Effect of Treatment?

La evolución de la artritis idiopática juvenil de inicio sistémico (AIJs) hacia las diferentes formas de presentación de enfermedad inflamatoria intestinal es extremadamente infrecuente. Presentamos la que, hasta ahora, es la primera comunicación de un paciente con AIJs con evolución a enfermedad de Crohn en el que se han detectado mutaciones en genes responsables de la adecuada regulación del sistema inmune innato.(AU)

The progression of systemic-onset juvenile idiopathic arthritis (JIAs) to the different forms of presentation of inflammatory bowel disease is extremely rare. We present the first report of a patient with SJIA that progressed to Crohn's disease in which mutations have been detected in genes responsible for the adequate regulation of the innate immune system.(AU)

Escala para la identificación del paciente pediátrico crónico complejo (Escala PedCom). Estudio piloto / A scale for the identification of the complex chronic pediatric patient (PedCom Scale): A pilot study

Introducción y objetivos: La condición crónica compleja (CCC) es una realidad cada vez más prevalente en pediatría. Sin embargo, padecer una CCC no supone necesariamente ser un paciente crónico complejo (PCC). Desde esta perspectiva, nos propusimos el desarrollo de un instrumento (Escala PedCom) que facilitase la identificación del PCC. Material y métodos: Inicialmente se definieron aspectos generales para la clasificación de un paciente como PCC. Posteriormente se desarrollaron los ítems de la escala puntuándolos de 0,5 a 4 puntos. Se realizó análisis factorial confirmatorio (AFC) y se estudió la consistencia interna mediante alfa de Cronbach. La concordancia se evaluó mediante estudio intra- e interobservador. El gold standard fue la clasificación realizada por 2 evaluadores tras valoración de la historia clínica del paciente. El punto de corte para considerar al paciente como PCC se estableció mediante curva ROC. Resultados: La versión inicial incluyó 43 ítems con índice de validez de contenido global (IVC) de 0,94. Para el estudio se incluyeron 180 pacientes. Tras el AFC se eliminó un ítem, por lo que la versión final consta de 42 ítems con IVC de 0,95. El valor alfa de Cronbach fue 0,723. El índice de correlación intraclase del análisis test-retest fue de 0,998 y 0,996 para el estudio interobservador. El punto de corte para considerar a un paciente como PCC se estableció en 6,5 puntos, con el que se obtuvo una sensibilidad del 98% y especificidad del 94%. Conclusiones: La Escala PedCom es una herramienta de fácil uso enfocada a la identificación del PCC. En nuestra muestra, presentó adecuada consistencia interna y niveles adecuados de concordancia intra- e interobservador; con buenos resultados de sensibilidad y especificidad para la identificación del PCC. (AU)

Introduction and objectives: The complex chronic condition (CCC) is an increasingly prevalent reality in pediatrics. However, having a CCC does not necessarily mean being a complex chronic patient (CCP). From this perspective, we developed an instrument (PedCom Scale) that would facilitate the identification of the PCC. Material and methods: Initially, general aspects for the classification of patients as CCP were defined. Subsequently, the items of the scale were developed, scoring them from 0.5 to 4 points. We performed a confirmatory factor analysis (CFA) and the internal consistency was studied using alpha-Cronbach. Concordance was evaluated by intra- and inter-observer study. The gold standard was the classification performed by two evaluators after assessing the patient's medical history. The cut-off point for considering the patient as a CCP was established using the ROC curve. Results: The initial version included 43 items with a global content validity index (CVI) of 0.94. A total of 180 patients were included. After the CFA, one item was eliminated, so the final version consists of 42 items with an CVI of 0.95. The alpha-Cronbach value was 0.723. The intraclass correlation coefficient of the test–retest analysis was 0.998 and 0.996 for the inter-observer study. The cut-off point for considering a patient as a CCP was established at 6.5 points, with these results we obtained a sensitivity of 98% and specificity of 94%. Conclusions: The PedCom Scale is an easy-to-use tool focused on the identification of the CCP. In our sample, it presented satisfactory levels of internal consistency and adequate levels of intra- and inter-observer agreement, with good sensitivity and specificity for the identification of the PCC. (AU)

Are infections in children with juvenile idiopathic arthritis more frequent than in healthy children? A prospective multicenter observational study.

Background: Children with juvenile idiopathic arthritis (JIA) might be at a higher risk of infection. Our objectives are to describe and compare infection rates in patients with JIA vs. healthy patients. Methods: A prospective, multicenter observational study was performed in Spain from January 2017 to June 2019. Patients with JIA from 7 participating hospitals and children without JIA (siblings of patients with JIA, and non-JIA children from primary health centers) were followed up with quarterly questionnaires to record infection episodes. Tuberculosis, herpes zoster, and infections requiring hospital admission were considered severe infections. Rates of infection (episodes/patient/year) were compared using a generalized estimating equations model. Results: A total of 371 children (181 with and 190 without JIA) were included. The median age was 8.8 years (IQR 5.5-11.3); 75% of the patients with JIA received immunosuppressive treatment (24% methotrexate, 22% biologic, 26% both). A total of 667 infections were recorded; 15 (2.2%) were considered severe. The infection rate was 1.31 (95%CI 1.1-1.5) in JIA and 1.12 (95%CI 0.9-1.3) in non-JIA participants (p = 0.19). Age <4 years increased the infection rate by 2.5 times (2.72 vs. 1.12, p < 0.001) in both groups. The most frequent infection sites were upper respiratory (62.6% vs. 74.5%) and gastrointestinal (18.8% vs. 11.4%). There were no differences in severe infections (2.5% vs. 2%, p = 0.65) between the groups. In children with JIA, younger age and higher disease activity (JADAS71) were associated with a higher infection rate. Conclusion: We found no differences in the infection rate or infection severity between patients with and without JIA. Most infections were mild. An age younger than 4 years increased the infection risk in both groups. Higher disease activity was associated with a higher infection rate.

A scale for the identification of the complex chronic pediatric patient (PedCom Scale): A pilot study.

INTRODUCTION AND OBJECTIVES: The complex chronic condition (CCC) is an increasingly prevalent reality in pediatrics. However, having a CCC does not necessarily mean being a complex chronic patient (CCP). From this perspective, we developed an instrument (PedCom Scale) that would facilitate the identification of the CCP. MATERIAL AND METHODS: Initially, general aspects for the classification of patients as CCP were defined. Subsequently, the items of the scale were developed, scoring them from 0.5 to 4 points. We performed a confirmatory factor analysis (CFA) and the internal consistency was studied using alpha-Cronbach. Concordance was evaluated by intra- and inter-observer study. The gold standard was the classification performed by two evaluators after assessing the patient's medical history. The cut-off point for considering the patient as a CCP was established using the ROC curve. RESULTS: The initial version included 43 items with a global content validity index (CVI) of 0.94. A total of 180 patients were included. After the CFA, one item was eliminated, so the final version consists of 42 items with an CVI of 0.95. The alpha-Cronbach value was 0.723. The intraclass correlation coefficient of the test-retest analysis was 0.998 and 0.996 for the inter-observer study. The cut-off point for considering a patient as a CCP was established at 6.5 points, with this results we obtained a sensitivity of 98% and specificity of 94%. CONCLUSIONS: The PedCom Scale is an easy-to-use tool focused on the identification of the CCP. In our sample, it presented satisfactory levels of internal consistency and adequate levels of intra- and inter-observer agreement, with good sensitivity and specificity for the identification of the CCP.

Longitudinal Study of Cognitive Functioning in Adults with Juvenile Idiopathic Arthritis.

OBJECTIVE: To prospectively evaluate possible decline of cognitive functions in adult patients with juvenile idiopathic arthritis (JIA) and identify associated factors. PATIENTS AND METHODS: We performed a 24-month prospective observational study of adults (≥16 years) with JIA. The primary outcome measure was decline in cognitive function defined as a worsening of ≥2 points on the scales of the subsets administered to evaluate the different cognitive areas using the Wechsler Adult Intelligence Scale (WAIS) after 24 months: attention/concentration (digit span); verbal function (vocabulary); visual-spatial organization (block design); working memory (letter-number sequencing); and problem solving (similarities). Other variables included average inflammatory activity using C-reactive protein and composite activity indexes, comorbidity, and treatment. Logistic regression was performed to identify factors associated with cognitive decline. RESULTS: The study population comprised 52 patients with JIA. Of these, 15 (28.8%) had cognitive decline at V24. The most affected functions were working memory (17.3%), attention/concentration (9.6%), verbal function (7.7%), visual-spatial organization (7.7%), and problem solving (3.8%). There were no significant differences in the median direct or scale scores for the cognitive functions evaluated between V0 and V24 for the whole sample. The factors associated with cognitive decline in patients with JIA were average C-reactive protein (OR [95% CI], 1.377 [1.060-1.921]; p = 0.039), depression (OR [95% CI], 3.691 [1.294-10.534]; p = 0.015), and treatment with biologics (OR [95% CI], 0.188 [0.039-0.998]; p = 0.046). CONCLUSION: Cognitive decline was detected in almost one third of adults with JIA after 24 months of follow-up. Systemic inflammatory activity in JIA patients was related to cognitive decline. Patients treated with biologics had a lower risk of decline in cognitive functions.

Afectación pulmonar rápidamente progresiva secundaria a dermatomiositis juvenil anti-MDA5(+) con evolución devastadora / Interstitial lung disease in anti-MDA5 positive dermatomyositis with devastating evolution

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Digital ischemia as the initial presentation of catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.

Resultados de una encuesta nacional sobre conocimiento y uso de pseudociencias por parte de los pediatras / Results of a national survey on knowledge and use of complementary and alternative medicine by paediatricians

Introducción: El uso de determinadas pseudociencias en niños está documentado en España. El objetivo principal del estudio es estimar el grado de conocimiento, la recomendación y el uso de algunas pseudoterapias por parte de los pediatras españoles. Material y métodos: Estudio transversal, descriptivo y de ámbito nacional, mediante encuesta en línea, enviada por correo electrónico a pediatras socios de la Asociación Española de Pediatría (AEP), entre junio y julio de 2020. (AU)

Introduction: The use of certain Complementary and Alternative Medicines (CAM) in children has been documented in Spain. The main aim of this study is to estimate the knowledge, recommendations, and use of CAM by Spanish paediatricians. Material and methods: A national study was conducted from June to July 2020 using an online questionnaire. Two e-mails were sent to paediatricians who were members of the Spanish Association of Paediatrics (AEP). (AU)

Isquemia digital como forma de presentación de síndrome antifosfolípido catastrófico pediátrico / Digital Ischemia as the Initial Presentation of Catastrophic Antiphospholipid Syndrome

El síndrome antifosfolípido catastrófico es una entidad infrecuente en Pediatría, pero con importante relevancia dada la elevada morbimortalidad. Se expone el caso de un niño con isquemia digital en el que, tras realizar despistaje etiológico de diferentes entidades infecciosas e inflamatorias, se llegó al diagnóstico de síndrome antifosfolípido catastrófico primario.(AU)

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.(AU)

Results of a national survey on knowledge and use of complementary and alternative medicine by paediatricians.

INTRODUCTION: The use of certain Complementary and Alternative Medicines (CAM) in children has been documented in Spain. The main aim of this study is to estimate the knowledge, recommendations, and use of CAM by Spanish paediatricians. MATERIAL AND METHODS: A national study was conducted from June to July 2020 using an online questionnaire. Two e-mails were sent to paediatricians who were members of the Spanish Association of Paediatrics (AEP). RESULTS: Out of 1414 responses received, acupuncture was considered as a science by 31.8%. Homeopathy was recommended to parents by 28.1%. CAM was used by 21.3% of physicians, at least once, to improve their own health. Only 3.8% had ever replaced a conventional treatment with CAM. The following variables were associated with a greater disposition to prescribe homeopathy: female, age over 45 years old, paediatricians working in Primary Care, and paediatricians working in private healthcare. CONCLUSIONS: This AEP Committee on Medicines questionnaire provides new data that should be considered alarming and should ask for a serious thinking on the use of CAM in Spain. Some paediatricians are recommending parents to give treatments not supported by scientific evidence to their children. This practice could be potentially harmful, especially when conventional treatment is being replaced.