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Introduction: Endemic coronaviruses have been found in acute bronchiolitis, mainly as a coinfecting virus. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been responsible for respiratory illness in hospitalized children. The characteristics of patients with bronchiolitis have not been extensively described. Methods: Cross-sectional study of patients with bronchiolitis and SARS-CoV-2 infection enrolled in a prospective multicenter cohort of children hospitalized with COVID-19 in Spain from March 1, 2020 to February 28, 2021. Results: Twelve of 666 children infected with SARS-CoV-2 who required hospital admission met the diagnostic criteria for bronchiolitis (1.8%). Median age was 1.9 months (range: 0.4-10.1). Six cases had household contact with a confirmed or probable COVID-19 case. Main complaints were cough (11 patients), rhinorrhea (10), difficulty breathing (8), and fever (8). Eleven cases were classified as mild or moderate and one as severe. Laboratory tests performed in seven patients did not evidence anemia, lymphopenia, or high C-reactive protein levels. Chest X-rays were performed in six children, and one case showed remarkable findings. Coinfection with metapneumovirus was detected in the patient with the most severe course; Bordetella pertussis was detected in another patient. Seven patients required oxygen therapy. Albuterol was administered in four patients. One patient was admitted to the pediatric intensive care unit. Median length of admission was 4 days (range: 3-14). No patient died or showed any sequelae at discharge. Two patients developed recurrent bronchospasms. Conclusion: SARS-CoV-2 infection does not seem to be a main trigger of severe bronchiolitis, and children with this condition should be managed according to clinical practice guidelines. Instituto de Salud Carlos III (Ministry of Economy, Industry and Competitiveness) 4.090 JCR (2021) Q1, 21/130 Pediatrics 0.927 SJR (2021) Q1, 49/320 Pediatrics, Perinatology and Child Health No data IDR 2021 UEM

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.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.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%.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.

Objectives: To evaluate electrocardiogram markers to predict coronary involvement in patients with Kawasaki disease by assessing measures of ventricular repolarization parameters on the 12-lead electrocardiogram. Study design: This cross-sectional study included 180 Spanish and Japanese patients ≤14 years of age with Kawasaki disease, with or without coronary involvement, from 2011 to 2016. We manually measured the Tp-Te/QT ratio and QTc interval (with Bazett's formula) in 12-lead electrocardiogram in the acute and recovery period and explored their potential association with coronary involvement. Results: No association was found between Tp-Te/QT ratio obtained manually in V5 and V6 leads and coronary involvement in the acute (V5:0.25 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .80; V6:0.24 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .86) or the recovery (V5: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.19-0.25], P = .68; V6: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.17-0.25], P = .50) period. By contrast, QTc in V5 and V6 was significantly lower in patients with Kawasaki disease and coronary involvement in the acute period (V5: 378 ms [IQR, 364-395 ms] vs 390 ms [IQR, 371-411 ms], P = .04; V6: 377 ms [IQR, 364-392 ms] vs 390 ms [IQR, 371-410 ms], P = .01). A QTc interval of

Resumen Introduccion El objetivo es analizar el impacto de la pandemia COVID-19 en las urgencias e ingresos hospitalarios pediatricos. Metodos Estudio de cohortes retrospectivo, de los pacientes atendidos en un hospital terciario, desde el 14 de marzo hasta el 26 de abril de 2020 comparandose con el mismo periodo de los 3 anos anteriores. Resultados Se observa una notable reduccion global de las visitas a urgencias e ingresos en todas las areas pediatricas, manteniendose la asistencia en neonatologia y los ingresos programados en oncologia. Discusion La reduccion de la actividad global en la urgencia pediatrica no es solo explicable por la disminucion de las enfermedades transmisibles. Ha podido contribuir la disminucion de la demanda inadecuada y de los ingresos inapropiados. La disponibilidad de camas pediatricas haria innecesaria la reduccion de la actividad quirurgica programada y permitiria redistribuir recursos a areas con mayor presion asistencial.

We conducted a multicenter clinical validity study of the Panbio coronavirus disease 2019 Antigen Rapid Test of nasopharyngeal samples in pediatric patients with coronavirus disease 2019-compatible symptoms of ≤5 days of evolution. Our study showed limited accuracy in nasopharyngeal antigen testing: overall sensitivity was 45.4%, and 99.8% of specificity, positive-predictive value was 92.5%.

Introduction: Juvenile recurrent chronic parotitis (JRCP) is a rare disease of unknown cause. There is a growing interest in its autoimmune aetiology and its relationship with dysfunctions of cellular and humoral immunity, although there is no agreed protocol for complementary investigations for its study. A consecutive series of cases is presented where the immune alterations and associated autoimmune disorders are investigated, proposing a study algorithm. Patients and methods: A retrospective study was carried out on patients who had JRCP during the period from 2013 to 2016 and a follow-up of at least 2 years. After its clinical and ultrasound diagnosis, complementary examinations were systematically carried out to investigate infectious, immune, and autoimmune diseases. Results: Of a total of 36 patients with inclusion criteria, 16 (44%) were found with some analytical alteration of a non-specific immunological nature (positive ANA, high IgG, low complement factor 4), or associated with a specific diagnosis, as occurred in 11 patients: Selective IgA deficiency (2), Sjögren’s syndrome associated or not with systemic lupus erythematosus (3), coeliac disease associated or not with diabetes mellitus (4), Hashimoto’s thyroiditis (1), and acquired immunodeficiency syndrome (1). Conclusion: Juvenile recurrent chronic parotitis can be considered a sentinel sign of other diseases of immunological/autoimmune aetiology for which the diagnosis, monitoring and early treatment can improve its prognosis. Viral infectious aetiology, with the exception of HIV, is not a priority in the study of recurrences. Resumen: Introducción: La parotiditis crónica recurrente juvenil (PCRJ) es una enfermedad infrecuente de causa desconocida. Existe un creciente interés por su etiología autoinmune y su relación con disfunciones de la inmunidad celular y humoral aunque no existe un protocolo consensuado de investigaciones complementarias para su estudio. Se presenta una serie consecutiva de casos donde se investigan las alteraciones inmunes y trastornos autoinmunes asociados, proponiendo un algoritmo de estudio. Pacientes y métodos: Se realizó un estudio retrospectivo de pacientes que presentaron PCRJ durante el periodo de 2013 a 2016 y seguimiento de al menos 2 años. Tras su diagnóstico clínico y ecográfico se realizaron de forma sistemática exámenes complementarios para investigación de patologías infecciosas, inmunes y autoinmunes asociadas. Resultados: De un total de 36 pacientes con criterios de inclusión, se encontraron 16 (44%) con alguna alteración analítica de carácter inmunológico inespecífico (ANA positivo, IgG elevada, factor 4 del complemento bajo) o asociada a un diagnóstico específico como ocurrió en 11 pacientes: déficit selectivo de IgA (2), síndrome de Sjögren asociado o no a lupus eritematoso sistémico (3), celiaquía asociada o no a Diabetes mellitus (4), tiroiditis de Hashimoto (1) y síndrome de inmunodeficiencia adquirida (1). Conclusión: La parotiditis crónica recurrente juvenile puede considerarse un signo centinela de otras enfermedades de etiología inmunológica/autoinmune cuyo diagnóstico, seguimiento y tratamiento precoz puede mejorar su pronóstico. La etiología infecciosa vírica, exceptuando el VIH, no es prioritaria en el estudio de recurrencias.

Acute focal bacterial nephritis is an interstitial bacterial infection, localised in the renal parenchyma, which can be more serious than acute pyelonephritis. The aim of this study is the analysis of predictive factors that may lead to its early diagnosis, which is essential for an adequate therapeutic approach.A retrospective, multicentre case and control study. The participant centres were hospitals in Castellon and Valencia. The study period was 2010-2018, with the cases being patients with focal bacterial nephritis and the patients with pyelonephritis as controls.A total of 158 (1:1) patients were included. The median age of the cases was 2 years and there were 75% females. There were no differences in the clinical presentation. In the univariate analysis, focal nephritis was associated with malformations of the urinary tract, bacteraemia, the neutrophil count, and procalcitonin, as well as febrile convulsions of borderline significance. Procalcitonin values ≥2 ng/ml had an OR of 4.9 (95%CI; 1.77-13.85) of presenting with focal nephritis. In the multivariate analysis, the urological malformations still maintained statistical significance and borderline significance for procalcitonin.The urinary tract malformations predispose the development of focal bacterial nephritis. In patients with a urinary tract infection and predictive factors of acute focal bacterial nephritis it would be worthwhile performing a renal Doppler ultrasound in the acute phase for its appropriate diagnosis and treatment.

Bertola, Debora/0000-0002-4701-6777; Rezaei, Nima/0000-0002-3836-1827; Lerch, Markus M./0000-0002-9643-8263; Richmond, Erick/0000-0001-9946-3686; Mayerle, Julia/0000-0002-3666-6459 WOS: 000334658800003 PubMed: 24599544 Johanson-Blizzard syndrome (JBS) is a rare, autosomal recessive disorder characterized by exocrine pancreatic insufficiency, typical facial features, dental anomalies, hypothyroidism, sensorineural hearing loss, scalp defects, urogenital and anorectal anomalies, short stature, and cognitive impairment of variable degree. This syndrome is caused by a defect of the E3 ubiquitin ligase UBR1, which is part of the proteolytic N-end rule pathway. Herein, we review previously reported (n=29) and a total of 31 novel UBR1 mutations in relation to the associated phenotype in patients from 50 unrelated families. Mutation types include nonsense, frameshift, splice site, missense, and small in-frame deletions consistent with the hypothesis that loss of UBR1 protein function is the molecular basis of JBS. There is an association of missense mutations and small in-frame deletions with milder physical abnormalities and a normal intellectual capacity, thus suggesting that at least some of these may represent hypomorphic UBR1 alleles. The review of clinical data of a large number of molecularly confirmed JBS cases allows us to define minimal clinical criteria for the diagnosis of JBS. For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. German Research FoundationGerman Research Foundation (DFG) [DFG ZE 524/2-3] Contract grant sponsor: German Research Foundation (DFG ZE 524/2-3).