Impacto COVID-19 en la urgencia y hospitalización de un hospital terciario. Lecciones de gestión aprendidas / COVID-19 impact on the emergency and hospitalization of a tertiary hospital. Management lessons learned

Introducción: El objetivo es analizar el impacto de la pandemia COVID-19 en las urgencias e ingresos hospitalarios pediátricos. Métodos: Estudio de cohortes retrospectivo, de los pacientes atendidos en un hospital terciario, desde el 14 de marzo hasta el 26 de abril de 2020 comparándose con el mismo periodo de los 3 años anteriores. Resultados: Se observa una notable reducción global de las visitas a urgencias e ingresos en todas las áreas pediátricas, manteniéndose la asistencia en neonatología y los ingresos programados en oncología. Discusión: La reducción de la actividad global en la urgencia pediátrica no es solo explicable por la disminución de las enfermedades transmisibles. Ha podido contribuir la disminución de la demanda inadecuada y de los ingresos inapropiados. La disponibilidad de camas pediátricas haría innecesaria la reducción de la actividad quirúrgica programada y permitiría redistribuir recursos a áreas con mayor presión asistencial.(AU)

Introduction: The objective is to analyze the impact of the COVID-19 pandemic on the pediatric emergencies and hospital admissions. Methods: Retrospective cohort study of patients treated in a tertiary hospital, from March 14 to April 26, 2020, compared to the same period of the previous 3 years. Results: A notable overall reduction in emergency room visits and admissions is observed in all pediatric areas, maintaining care in neonatology and scheduled admissions in oncology. Discussion: The reduction in global activity in pediatric emergencies is not only explained by the decrease in contagious diseases. The decrease in inadequate demand and inappropriate income may have contributed. The availability of pediatric beds would make the reduction of programmed surgical activity unnecessary and would allow the redistribution of resources to areas with greater healthcare pressure.(AU)

Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort.

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.

COVID-19 impact on the emergency and hospitalization of a tertiary hospital. Management lessons learned.

INTRODUCTION: The objective is to analyze the impact of the COVID-19 pandemic on the pediatric emergencies and hospital admissions. METHODS: Retrospective cohort study of patients treated in a tertiary hospital, from March 14 to April 26, 2020, compared to the same period of the previous 3 years. RESULTS: A notable overall reduction in emergency room visits and admissions is observed in all pediatric areas, maintaining care in neonatology and scheduled admissions in oncology. DISCUSSION: The reduction in global activity in pediatric emergencies is not only explained by the decrease in contagious diseases. The decrease in inadequate demand and inappropriate income may have contributed. The availability of pediatric beds would make the reduction of programmed surgical activity unnecessary and would allow the redistribution of resources to areas with greater healthcare pressure.

SARS-CoV-2 acute bronchiolitis in hospitalized children: Neither frequent nor more severe.

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.

Alteraciones inmunológicas asociadas a parotiditis crónica recurrente juvenil / Immune disorders associated with juvenile recurrent chronic parotitis

Introducción: La parotiditis crónica recurrente juvenil 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 parotiditis crónica recurrente juvenil 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 juvenil 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. (AU)

Introduction: Juvenile recurrent chronic parotitis 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 juvenile recurrent chronic parotitis 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. (AU)

Immune disorders associated with juvenile recurrent chronic parotitis.

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.

COVID-19 impact on the emergency and hospitalization of a tertiary hospital. Management lessons learned. / Impacto COVID-19 en la urgencia y hospitalización de un hospital terciario. Lecciones de gestión aprendidas.

INTRODUCTION: The objective is to analyze the impact of the COVID-19 pandemic on the pediatric emergencies and hospital admissions. METHODS: Retrospective cohort study of patients treated in a tertiary hospital, from March 14 to April 26, 2020, compared to the same period of the previous 3 years. RESULTS: A notable overall reduction in emergency room visits and admissions is observed in all pediatric areas, maintaining care in neonatology and scheduled admissions in oncology. DISCUSSION: The reduction in global activity in pediatric emergencies is not only explained by the decrease in contagious diseases. The decrease in inadequate demand and inappropriate income may have contributed. The availability of pediatric beds would make the reduction of programmed surgical activity unnecessary and would allow the redistribution of resources to areas with greater healthcare pressure.

[Immune disorders associated with juvenile recurrent chronic parotitis]. / Alteraciones inmunológicas asociadas a parotiditis crónica recurrente juvenil.

INTRODUCTION: Juvenile recurrent chronic parotitis 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 juvenile recurrent chronic parotitis 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.

Marcadores predictivos de nefritis focal bacteriana aguda. Estudio multicéntrico casos-control / Predictive markers of acute focal bacterial nephritis. A multicentre case-control study

INTRODUCCIÓN: La nefritis focal bacteriana aguda es una infección intersticial bacteriana, localizada en el parénquima renal, que entraña mayor gravedad que la pielonefritis aguda. El objetivo del estudio es el análisis de factores predictivos que permitan su diagnóstico precoz, fundamental para un adecuado abordaje terapéutico. PACIENTES Y MÉTODOS: Estudio multicéntrico de casos y control retrospectivo. Centros participantes: hospitales de Castellón y Valencia. Periodo de estudio: 2010-2018. Casos: nefritis focal bacteriana. Controles: pielonefritis aguda. RESULTADOS: Se incluyó a un total de 158 pacientes (1:1). La mediana de edad de los casos fue 2 años. El 75% de sexo femenino. No existieron diferencias en la presentación clínica. En el análisis univariante la nefritis focal se relacionó con malformaciones del tracto urinario, bacteriemia, recuento de neutrófilos y la procalcitonina, así como las convulsiones febriles en el límite de la significación. Valores de procalcitonina ≥ 2 ng/ml tiene una OR de 4,9 (IC del 95: 1,77-13,85) de presentar nefritis focal. En el análisis multivariante las malformaciones urológicas mantuvieron la significación estadística y la procalcitonina en el límite de la significación. CONCLUSIONES: Las malformaciones del tracto urinario predisponen al desarrollo de nefritis focal bacteriana. Ante pacientes con infección del tracto urinario y factores predictivos de nefritis focal bacteriana aguda, sería recomendable la realización de una ecografía Doppler renal en fase aguda para un diagnóstico y un tratamiento adecuado

INTRODUCTION: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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

[Predictive markers of acute focal bacterial nephritis. A multicentre case-control study]. / Marcadores predictivos de nefritis focal bacteriana aguda. Estudio multicéntrico casos-control.

INTRODUCTION: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Selección del tratamiento antibiótico empírico en pielonefritis según el perfil del paciente / Selection of empirical antibiotic treatment in pyelonephritis according to the patient's profile

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Prevalence and Clinical Characteristics of SARS-CoV-2 Confirmed and Negative Kawasaki Disease Patients During the Pandemic in Spain.

Introduction: COVID-19 has a less severe course in children. In April 2020, some children presented with signs of multisystem inflammation with clinical signs overlapping with Kawasaki disease (KD), most of them requiring admission to the pediatric intensive care unit (PICU). This study aimed to describe the prevalence and clinical characteristics of KD SARS-CoV-2 confirmed and negative patients during the pandemic in Spain. Material and Methods: Medical data of KD patients from January 1, 2018 until May 30, 2020 was collected from the KAWA-RACE study group. We compared the KD cases diagnosed during the COVID-19 period (March 1-May 30, 2020) that were either SARS-CoV-2 confirmed (CoV+) or negative (CoV-) to those from the same period during 2018 and 2019 (PreCoV). Results: One hundred and twenty-four cases were collected. There was a significant increase in cases and PICU admissions in 2020 (P-trend = 0.001 and 0.0004, respectively). CoV+ patients were significantly older (7.5 vs. 2.5 yr) and mainly non-Caucasian (64 vs. 29%), had incomplete KD presentation (73 vs. 32%), lower leucocyte (9.5 vs. 15.5 × 109) and platelet count (174 vs. 423 × 109/L), higher inflammatory markers (C-Reactive Protein 18.5vs. 10.9 mg/dl) and terminal segment of the natriuretic atrial peptide (4,766 vs. 505 pg/ml), less aneurysm development (3.8 vs. 11.1%), and more myocardial dysfunction (30.8 vs. 1.6%) than PreCoV patients. Respiratory symptoms were not increased during the COVID-19 period. Conclusion: The KD CoV+ patients mostly meet pediatric inflammatory multisystem syndrome temporally associated with COVID-19/multisystem inflammatory syndrome in children criteria. Whether this is a novel entity or the same disease on different ends of the spectrum is yet to be clarified.

Mutations in the human UBR1 gene and the associated phenotypic spectrum.

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.