Objective: To identify factors associated with the higher proportion of fatty tissue and overweight/obesity observed in patients with juvenile idiopathic arthritis (JIA). Patients and methods: We ...performed a cross-sectional study of 80 JIA patients aged 4–15 years with 80 age- and sex-matched healthy controls. Body composition was assessed using dual-energy x-ray absorptiometry. The 27-joint Juvenile Arthritis Disease Activity score (JADAS27) was calculated. Two multivariate models were constructed to identify factors associated with overweight/obesity and fat mass index (FMI). Results: No differences were found between cases and controls in body mass index (BMI) or body composition. However, compared with controls, patients with a high inflammatory activity (JADAS27 > 4.2 for oligoarticular JIA or >8.5 for polyarticular disease) had higher values for BMI (p = 0.006); total fat mass (p = 0.003); FMI (p = 0.001); and fat in the legs (p = 0.001), trunk (p = 0.001), and arms (p = 0.002). The factors associated with overweight/obesity in patients were the duration of therapy with biological drugs, measured in months (OR 95% CI = 1.12 1.02–1.04; p = 0.037), and physical activity (OR 95% CI = 0.214 0.07–0.68; p = 0.010), while the factors associated with FMI were age (β 95% CI = 0.30 0.17–1.41; p = 0.014), JADAS27 (β 95% CI = 0.45 0.16–1.08; p = 0.009), and physical activity (β 95% CI = −0.22 −5.76 to 0.29; p = 0.031). Conclusion: Our study revealed no differences between JIA patients with well-controlled disease and low disability and the healthy population in BMI or body composition. Furthermore, the association observed between inflammatory activity and adiposity could be responsible for poorer clinical course.
HIV infection was the main risk of suffering Pneumocystis jirovecii pneumonia (PJP). The clinical-epidemiological characteristics of PJP have currently changed, with there being few studies on this.
...A retrospective observational study was carried out on paediatric patients diagnosed with PJP over a 17 year period in a third level hospital in Spain.
A total of 23 patients were included, of whom 7/23 (47.8%) suffered a haematological disease, 5/23 (21.7%) a primary immunodeficiency, and 4/23 (17.4%) an HIV infection. Prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX) was received by 11/23 (47.8%) patients. All were treated with TMP-SMX and 18/23 (78.3%) with systemic glucocorticoids. There were 6(26.1%) deaths, of which one of them (16.7%) suffered an HIV infection. A higher mortality was seen in the non-HIV patients with greater leucocytosis, greater CO2 retention, and a higher heart rate at onset, differences not observed in HIV patients. No differences were found in mortality in relation to the predisposing factor, use of pTMP-SMX, or treatment with glucocorticoids.
Paediatric patients with haematological cancers are currently the main risk group of developing PJP in this age group. No differences were found in mortality between patients with or without HIV infection as predisposing factor. The mortality among non-HIV patients was higher in those that had greater leucocytosis, greater CO2 retention, and increased heart rate at onset. A better prognosis was not seen in patients that received prophylaxis with TMP-SMX prior to the development of the PJP, or in those that received glucocorticoids as part of the treatment.
Cutaneous manifestations have been included in COVID-19 patients' clinical spectrum. Our objective was to determine the association between skin lesions in children and SARS-CoV-2 infection, ...analyzing others possible infectious/autoimmune etiologies.
Observational, multicenter, cross-sectional study, about children with skin manifestations from April to May 2020. The diagnosis of SARS-CoV-2 was performed by PCR in nasopharyngeal exudate and/or presence of antibodies by serology.
Sixty-two children were included, 9 (14.5%) presented positive antibodies to SARS-CoV-2, with no positive PCR to SARS-Cov-2 in those patients in whom it was made. Patients with positive serology to SARS-CoV-2 presented chilblains and/or vesicular-bullous skin lesions more frequently (66.7% vs. 24.5%, p=0.019). Generalized, urticarial and maculopapular rash was more common in patients with negative antibodies (37.7 vs. 0%, p=0.047), others pathogens were isolated in 41.5% of these patients. There were no significant differences in the positivity for autoantibodies between both groups.
In our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV-2 previous contact.
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.
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.
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.
Los dispositivos intravasculares son esenciales para el abordaje diagnóstico y terapéutico de múltiples enfermedades en pediatría, siendo especialmente importantes los catéteres venosos centrales ...(CVC). Una de las complicaciones más frecuentes es la infección de estos dispositivos, lo cual conlleva una elevada morbimortalidad. Estas infecciones presentan una gran complejidad, precisando de un elevado consumo de recursos, tanto para su diagnóstico como para su tratamiento, afectando de manera más frecuente a pacientes pediátricos vulnerables ingresados en unidades de alta complejidad. La evidencia para su abordaje en pediatría es menor que en adultos, y no hay documentos de consenso realizados en nuestro medio. El objetivo de este documento, realizado entre la Sociedad Española de Enfermedades Infecciosas Pediátricas (SEIP) y la Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), es dar recomendaciones de consenso basadas en la mayor evidencia disponible para optimizar el diagnóstico y tratamiento de las bacteriemias y fungemias relacionadas con el catéter. Este documento se centrará en pacientes pediátricos no neonatales, sin entrar en discusión sobre la prevención de estas infecciones.
Intravascular devices are essential for the diagnostic and therapeutic approach to multiple diseases in paediatrics, and central venous catheters (CVCs) are especially important. One of the most frequent complications of these devices is the infection, which is associated with a high morbidity and mortality. These infections are highly complex, requiring the use of substantial resources, both for their diagnosis and treatment, and affect vulnerable paediatric patients admitted to high-complexity units more frequently vulnerable. There is less evidence on their management in paediatric patients compared to adults, and no consensus documents on the subject have been published in Spain. The objective of this document, developed jointly by the Spanish Society of Paediatric Infectious Diseases (SEIP) and the Spanish Society of Paediatric Intensive Care (SECIP), is to provide consensus recommendations based on the greatest degree of evidence available to optimize the diagnosis and treatment of catheter-related bloodstream infections (CRBSIs). This document focuses on non-neonatal paediatric patients with CRBSIs and does not address the prevention of these infections.
La infección por VIH era el principal factor de riesgo para presentar neumonía por Pneumocystis jirovecii (NPJ). En la actualidad, las características clínico-epidemiológicas de la NPJ en niños han ...cambiado, existiendo pocos estudios en este sentido.
Realizamos un estudio observacional retrospectivo en pacientes pediátricos diagnosticados de NPJ durante 17 años en un hospital de tercer nivel en España.
Se recogió a 23 pacientes, de los que 11/23 (47,8%) presentaban enfermedad hematológica, 5/23 (21,7%) inmunodeficiencia primaria y 4/23 (17,4%) infección por VIH. Recibían profilaxis con trimetoprima-sulfametoxazol (TMP-SMX) 11/23 pacientes (47,8%). Todos recibieron tratamiento con TMP-SMX y 18/23 (78,3%), glucocorticoides sistémicos. Fallecieron 6/23 pacientes (26,1%), de los que 1/6 (16,7%) presentaba infección por VIH. En los pacientes no VIH con mayor leucocitosis, mayor retención de CO2 y mayor frecuencia cardíaca al inicio, se evidenció mayor mortalidad, diferencias no objetivadas en pacientes con VIH. No se encontraron diferencias en mortalidad en relación con el factor predisponente, empleo de TMP-SMX ni tratamiento con glucocorticoides.
En la actualidad, los pacientes pediátricos con neoplasias hematológicas constituyen el principal grupo de riesgo de desarrollar NPJ en este grupo etario. No hemos encontrado diferencias de mortalidad entre pacientes con o sin infección por VIH como factor predisponente. Entre los pacientes no VIH la mortalidad fue mayor en aquellos que presentaron mayor leucocitosis, mayor retención de CO2 y mayor frecuencia cardíaca al inicio. No se objetivó mejor pronóstico en pacientes que recibían profilaxis con TMP-SMX previamente al desarrollo de la NPJ ni en los que recibieron glucocorticoides sistémicos como parte del tratamiento.
HIV infection was the main risk of suffering Pneumocystis jirovecii pneumonia (PJP). The clinical-epidemiological characteristics of PJP have currently changed, with there being few studies on this.
A retrospective observational study was carried out on paediatric patients diagnosed with PJP over a 17 year period in a third level hospital in Spain.
A total of 23 patients were included, of whom 7/23 (47.8%) suffered a haematological disease, 5/23 (21.7%) a primary immunodeficiency, and 4/23 (17.4%) an HIV infection. Prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX) was received by 11/23 (47.8%) patients. All were treated with TMP-SMX and 18/23 (78.3%) with systemic glucocorticoids. There were 6(26.1%) deaths, of which one of them (16.7%) suffered an HIV infection. A higher mortality was seen in the non-HIV patients with greater leucocytosis, greater CO2 retention, and a higher heart rate at onset, differences not observed in HIV patients. No differences were found in mortality in relation to the predisposing factor, use of pTMP-SMX, or treatment with glucocorticoids.
Paediatric patients with haematological cancers are currently the main risk group of developing PJP in this age group. No differences were found in mortality between patients with or without HIV infection as predisposing factor. The mortality among non-HIV patients was higher in those that had greater leucocytosis, greater CO2 retention, and increased heart rate at onset. A better prognosis was not seen in patients that received prophylaxis with TMP-SMX prior to the development of the PJP, or in those that received glucocorticoids as part of the treatment.
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