Diagnostic and therapeutic approach to spondylodiscitis in Spanish children through the RIOPed network: evaluation before and after the publication of the national consensus document.

OBJECTIVE: Pediatric spondylodiscitis (PSD) is an uncommon condition, for which there are no specific international clinical guidelines. Factors related to complications have not been stablished. Our aim was to describe clinical and epidemiological characteristics of PSD, to analyze factors associated with complications and to evaluate adherence to the recommendations of the Spanish National Consensus Document (NCD) for the diagnostic and therapeutic approach to acute osteoarticular infections. MATERIAL AND METHODS: Ambispective, multicenter, national study of two PSD cohorts: historical (2008-2012) and prospective (2015-2020, after publication of NCD). Patients with diagnosis of PSD were included. Demographic, clinical, microbiological and radiological data were recorded. Factors related to the development of complications were analized by logistic regression. Comparisons between both cohorts were performed. RESULTS: Ninety-eight PSD were included. In 84.7%, diagnosis was confirmed by magnetic resonance imaging. Microbiological isolation was obtained in 6.1%, with methicillin-sensitive S. aureus as the main etiologic agent. Complications occurred in 18.9%, the most frequent being soft tissue abscess. Of the 8.6% of patients with sequelae, persistent pain was the most common. Comparing cohorts, there was better adherence to NCD treatment recommendations in the prospective one (57.6% vs. 12.9%, p < 0.01), including a reduction in the length of intravenous antibiotic therapy (10 vs. 14 days, p < 0.01). CONCLUSIONS: The evolution of PSD in our series was favorable, with low frequency of complications and sequelae. The adherence to the recommendations of the NCD was high. Studies with larger sample size are needed to establish new recommendations to optimize the approach to these infections.

CENTRAL NERVOUS SYSTEM INFECTION BY GROUP A STREPTOCOCCUS IN CHILDREN IN SPAIN (2019-2023).

Central nervous system infections in children caused by group A Streptococcus are rare. This study, conducted across 52 hospitals in Spain from 2019 to 2023, identified 32 cases of central nervous system infections in children caused by group A Streptococcus, with a significant increase from October 2022 onward (1.1% vs. 5.9%, P = 0.002). Half required pediatric intensive care unit admission, 12.5% exhibited sequelae and the mortality rate was 6.2%. Mastoiditis was the predominant primary infection.

Consensus document of the Spanish Society of Paediatric Infectious Diseases (SEIP) and the Spanish Society of Paediatric Intensive Care (SECIP) for the diagnosis and treatment of central venous catheter-related infections in paediatric care.

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 is the infection of these devices, 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. 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.

Childhood-Onset Non-Infectious Uveitis in the "Biologic Era". Results From Spanish Multicenter Multidisciplinary Real-World Clinical Settings.

OBJECTIVE: To characterize and describe clinical experience with childhood-onset non-infectious uveitis. STUDY DESIGN: A multicenter retrospective multidisciplinary national web-based registry of 507 patients from 21 hospitals was analyzed. Cases were grouped as immune disease-associated (IMDu), idiopathic (IDIu) or ophthalmologically distinct. Characteristics of juvenile idiopathic arthritis-associated (non-HLA-B27-related) uveitis (JIAu), IDIu, and pars planitis (PP) were compared. RESULTS: IMDu (62.3%) and JIAu (51.9%) predominated in young females; and IDIu (22.7%) and PP (13.6%) in older children, without sex imbalance. Ocular complications occurred in 45.3% of cases (posterior synechiae [28%], cataracts [16%], band keratopathy [14%], ocular hypertension [11%] and cystoid macular edema [10%]) and were associated with synthetic (86%) and biologic (65%) disease-modifying antirheumatic drug (DMARD) use. Subgroups were significantly associated (p < 0.05) with different characteristics. JIAu was typically anterior (98%), insidious (75%), in ANA-positive (69%), young females (82%) with fewer complications (31%), better visual outcomes, and later use of uveitis-effective biologics. In contrast, IDIu was characteristically anterior (87%) or panuveitic (12.1%), with acute onset (60%) and more complications at onset (59%: synechiae [31%] and cataracts [9.6%]) and less DMARD use, while PP is intermediate, and was mostly bilateral (72.5%), persistent (86.5%) and chronic (86.8%), with more complications (70%; mainly posterior segment and cataracts at last visit), impaired visual acuity at onset, and greater systemic (81.2%), subtenon (29.1%) and intravitreal (10.1%) steroid use. CONCLUSION: Prognosis of childhood uveitis has improved in the "biologic era," particularly in JIAu. Early referral and DMARD therapy may reduce steroid use and improve outcomes, especially in PP and IDIu.

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.

Crohn's disease in a patient with systemic onset juvenile idiopathic arthritis. Association or associated side effect of treatment?

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.

Adiposity Is Related to Inflammatory Disease Activity in Juvenile Idiopathic Arthritis.

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 (ß [95% CI] = 0.30 [0.17-1.41]; p = 0.014), JADAS27 (ß [95% CI] = 0.45 [0.16-1.08]; p = 0.009), and physical activity (ß [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.

Skin lesions in children during the first wave of the SARS-CoV-2 pandemic.

BACKGROUND: 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-CoV2 infection, analyzing others possible infectious/autoimmune etiologies. MATERIAL AND METHODS: Observational, multicenter, cross-sectional study, about children with skin manifestations from April to May 2020. The diagnosis of SARS-CoV2 was performed by PCR in nasopharyngeal exudate and/or presence of antibodies by serology. RESULTS: 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. CONCLUSION: In our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV2 previous contact.

INTRODUCCIÓN: Las manifestaciones cutáneas se han incluido en el espectro clínico de los pacientes con COVID-19. Nuestro objetivo fue determinar la asociación entre las lesiones cutáneas observadas en niños durante la primera ola de la pandemia y la infección por SARS-CoV-2, analizando otras posibles etiologías infecciosas o autoinmunes. MATERIAL Y MÉTODOS: Estudio observacional, multicéntrico, de corte transversal, desarrollado en niños con manifestaciones cutáneas desde abril hasta mayo de 2020. La determinación de SARS-CoV-2 se realizó mediante PCR en exudado nasofaríngeo y/o serología. RESULTADOS: Se seleccionó a 62 niños; 9 (14,5%) presentaron serología positiva para SARS-CoV-2, siendo la PCR negativa en todos los casos en los que se realizó. Los pacientes con serología positiva para SARS-CoV-2 presentaron con más frecuencia lesiones pernióticas y/o vesiculosas (66,7 vs. 24,5%; p = 0,019). El exantema generalizado, urticarial y maculopapuloso fue más habitual en el grupo de pacientes con serología negativa (37,7 vs. 0%; p = 0,047); se aislaron otros patógenos en el 41,5%. No hubo diferencias significativas en cuanto a la positividad de autoanticuerpos entre ambos grupos. CONCLUSIÓN: En nuestro estudio, las lesiones de tipo perniosis o vesiculosas se relacionaron significativamente con el contacto previo con SARS-CoV-2.

Clinical-epidemiological characteristics of Pneumocystis jirovecii pneumonia in a tertiary hospital in Spain.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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 six (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. CONCLUSIONS: 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.

[Skin lesions in children during the first wave of the SARS-CoV-2 pandemic]. / Lesiones cutáneas en niños durante la primera ola de la pandemia por SARS-CoV-2.

BACKGROUND: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: In our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV-2 previous contact.

Skin manifestations during the COVID-19 pandemic in the pediatric emergency department.

BACKGROUND: The role of SARS-CoV-2 as the cause of chilblains in children remains a matter of debate but it is important to elucidate it for patient isolation and contact tracing. We sought to define the etiology, clinical presentation, time course, and outcomes of children presenting to the emergency department (ED) with cutaneous manifestations shortly after the first pandemic peak of COVID-19 in Spain. METHODS: A prospective, observational study in children <15 years of age evaluated for skin lesions in the EDs of three pediatric hospitals. Children underwent a comprehensive work-up including tests for SARS-CoV-2 antibodies and polymerase chain reaction (PCR), and serology and PCR tests for other viruses and bacteria. A 1 month follow-up visit was conducted. RESULTS: From April 14 through May 8, 2020, we enrolled 62 children. Of those, 34 had acro-ischemic skin lesions and 28 had a variety of skin rashes. Overall, 40% of children had mild systemic symptoms. Children with chilblains were older, had pain more frequently and a more prolonged duration of skin lesions, while those with non-specific rashes had fever more frequently. Lesions were resolved in 75% of children at follow up. Five patients demonstrated SARS-CoV-2 antibodies, and none tested positive with PCR. Three additional patients tested positive with PCR for rhinovirus, Mycoplasma pneumoniae and Chlamydia pneumoniae. CONCLUSIONS: The number of ED visits for chilblains, which are rare in pediatrics, was high soon after the first peak of COVID-19 in Spain. The disease course was self-limited, outcomes were favorable, and the possibility of viral transmission was negligible as all patients tested negative for SARS-CoV-2 by PCR.

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

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.

[Low bone mineral density in juvenile idiopathic arthritis: Prevalence and related factors]. / Baja densidad mineral ósea en artritis idiopática juvenil: prevalencia y factores relacionados.

INTRODUCTION: Height adjustment is currently recommended for Z-score bone mineral density (BMD) assessed by dual energy X-ray absorptiometry. At present there are no studies that evaluate the prevalence of low BMD in paediatric patients with Juvenile Idiopathic Arthritis (JIA) in Spain following current recommendations. OBJECTIVES: To evaluate low BMD in JIA in paediatric patients with JIA in Spain following the latest recommendations, as well as to assess associated factors. METHODS: Observational cross-sectional study of Spanish JIA patients from 5 to 16 years-old, followed-up in a Paediatric Rheumatology Unit between July 2014 and July 2015. Anthropometric, clinical and treatment data were recorded. Dual energy X-ray absorptiometry, and bone metabolism parameters were collected, and a completed diet and exercise questionnaire was obtained. RESULTS: A total of 92 children participated. The population prevalence estimation of low BMD was less than 5% (95% CI). A significant positive correlation was found in the multiple linear regression analysis between the body mass index percentile (B: 0.021; P<.001) and lean mass index (B: 0.0002; P=.012), and BMD Z-score adjusted for height (Z-SAH). A significant negative correlation was found between fat mass index (B: -0.0001; P=.018) and serum type I collagen N-propeptide (B: -0,0006; P=.036) and Z-SAH. CONCLUSIONS: Low BMD prevalence in JIA patients in our population is low. An adequate nutritional status and the prevalence of lean over fat mass seem to promote the acquisition of bone mass. Those JIA patients with lower BMD could be subjected to an increase of bone turnover.