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Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood inflammatory disease. SJIA accounts for approximately 5-15 per cent of all cases of JIA and has a high morbidity and mortality rate. In this disease, pulmonary complications (PC) other than pleuritis are much less frequent and not easily recognised by clinicians. Pulmonary hypertension, the most severe PC, is associated with uncontrolled disease and use of biologic therapies. We present a case of a school-age female with sJIA who died of acute cardiopulmonary instability secondary to pulmonary venous-occlusive disease demonstrated by necropsy. We describe her clinical evolution. We also undertook a narrative review of the literature about PC in sJIA to discuss the current state of the art regarding this complication. High disease activity and the use of multiple therapies include disease-modifying anti-rheumatic drugs should be a red flag for clinicians when discounting PC and pulmonary hypertension. The combination of chest X-ray, electrocardiogram and echocardiogram appear to be the best tests to achieve an early diagnosis., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background: A new clinical syndrome named Paediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 (PIMS-TS) has been described. This new disease is a leading cause of hospital and paediatric intensive care unit (PICU). It has been related to immunity dysregulation., Methods: Prospective-retrospective observational study to describe the innate cell signature and immunophenotype of children admitted to PICU because of PIMS-TS (from March 2020 to September 2020). The immunophenotype was done through the expression analysis of these proteins of mononuclear cells: CD64, CD18, CD11a and CD11b. They were compared with previous healthy controls and children admitted to PICU because of bacterial infection, viral infection and Kawasaki disease (KD). Two hundred and forty-seven children were studied: 183 healthy controls, 25 viral infections, 20 bacterial infections, 6 KD and 13 PIMS-TS., Results: PIMT-TS showed the lowest percentage of lymphocytes and monocytes with higher relative numbers of CD4+ ( p  = .000). Monocytes and neutrophils in PIMS-TS showed higher levels of CD64 expression ( p  = .000). Also, CD11a and CD11b were highly expressed ( p  =,000)., Conclusion: We observed a differential cell innate signature in PIMS-TS. These findings are consistent with a proinflammatory status (CD64 elevated expression) and lymphocyte trafficking to tissues (CD11a and CD11b). More studies should be carried out to confirm our results.

Introduction: Septic thrombosis of intracranial venous sinuses (STSV) is a rare and severe complication of cranial infections., Materials and Methods: The main objective of this paper is to describe the clinical data, diagnostic procedures, treatment and evolution of a series of cases of STSV. In addition, the current literature is reviewed. Observational retrospective study by review of medical histories (January 1995-December 2016). The data collected were: clinical, analytical, epidemiological, microbiological, radiological, management and follow-up. A descriptive and statistical analysis of the data was done., Results: Twelve children were included (86,832 admissions studied). They have a median age of 4.5 years (range 1-13) with a median time of symptoms of 6 days (range 1-25). At admission, the clinical data were: fever (11/12), vomiting (9/12) and headache (8/12). They also showed bad general status 12/12, 7/12 acute otitis media and 5/12 VI cranial nerve paresis. The lumbar puncture was pathological in 4/12. The most frequently microorganism isolated was Streptococcus sp. Prothrombotic mutations were confirmed on 2/12. Cranial computed tomography allowed diagnosis in 9/12; the magnetic resonance imaging achieves that in 12/12. Previous neurological signs or time to diagnosis did not influence the appearance of other image complications. All received antibiotic treatment, heparin 10/12 and 11/12 surgery. There were no sequels., Conclusion: In our series otitis, headache, vomiting and fever were prevalent. Complementary tests allowed the suspect but the definitive diagnosis was obtained by neuroimaging. There were no sequels and the therapies were mainly wide broad-spectrum antibiotics, heparin, and surgical.

We describe 5 children with severe SARS-CoV-2 infection, hemodynamic instability and suspected acute abdomen. This form of the disease has not been previously documented. Four of the cases were confirmed SARS-CoV-2 infection and 1 probable. All of them were previously healthy and needed a pediatric critical care unit admission. The respiratory symptoms were not dominant or were absent. Also, fever was observed. Laboratory testing revealed lymphopenia and high levels of C-reactive protein and procalcitonin with D-dimer, ferritin and interleukin-6 usually elevated. Respiratory support and inotropic support were almost always necessary. In all of them, deterioration occurred on the day of admission.

Objectives: Spain has been one of the countries most severely affected by the coronavirus disease 2019. This study aims to describe a series of children admitted to a PICU due to coronavirus disease 2019 infection., Design: Prospective observational study., Setting: Tertiary hospital in Madrid, Spain., Patients: Children admitted to the PICU with severe acute respiratory syndrome coronavirus 2 (severe acute respiratory syndrome coronavirus 2) infection, from March 1, 2020, to April 15, 2020., Interventions: Observational study., Measurements and Main Results: Epidemiologic data, previous clinical characteristics, support therapy needed, imaging tests, laboratory observations on admission, and pharmacologic therapy. Eleven children were admitted to the PICU, with suspected coronavirus disease 2019; the polymerase chain reaction test was positive in seven. The median age was 100.7 months (range, 0.5-162). Five were admitted from the emergency department and two from the ward. The Pediatric Sequential Organ Failure Assessment score was 3 (range, 0-9), and Pediatric Risk of Mortality II score was 4 (range, 0-16). All children were previously healthy except one (allogeneic hematopoietic stem cell transplantation). Respiratory symptoms and fever were prevalent. A chest radiograph led to a pneumonia diagnosis. Not all patients presented with lymphopenia on admission. D-Dimer and ferritin were elevated. All patients needed oxygen therapy through a nasal cannula; five patients received high-flow nasal cannula therapy, which was later substituted with noninvasive ventilation in four. Mechanical ventilation was necessary in two patients on the first day of PICU admission. Two children required mechanical ventilation and inotropic support. Tocilizumab was applied in two intubated children. Also, four children received heparin. No patients died., Conclusions: On the whole, the children were previously healthy and are more than 1 year old. Respiratory symptoms were the leading cause of PICU admission, making respiratory support the principal therapy. Patients requiring mechanical ventilation showed deterioration on the first day of admission. These children seemed to require close monitoring, and multicenter studies are necessary.

Background: Although necrotizing pneumonia (NN) is one of the most feared complications of community-acquired pneumonia, data in pediatric patients are scarce. The objective of this article is to describe children admitted to pediatric intensive care unit (PICU) because of NN., Methods: Retrospective-prospective observational study in children admitted with NN to PICU (from January 1, 2010, to December 31, 2018). The data collected included information on disease epidemiology, PICU management, respiratory assistance and disease evolution., Results: Fifty-one children were included, 42 of 51 had received 7-valent or 13-valent pneumococcal vaccine. Median age was 3.2 years (1.9-4.2), 15 of 51 had signs of sepsis at admission. Forty-nine patients presented pleural effusion with drainage in 46. The most common respiratory support modality was high-flow oxygen nasal cannula (17/51). Computed tomography was the gold standard for diagnosis. Etiologic diagnosis was obtained in 34 of 51, and pneumococcus was isolated in 29 of 34. In all of these cases, initial detection was made by capsular antigen in pleural fluid. Children with pneumococcal NN had fewer days of evolution prior to PICU admission (P = 0.041). Cefotaxime with clindamycin was used in 49 of 51. Surgery was necessary in 3 of 51 patients. After PICU discharge, only 5 of 51 were readmitted. There were deaths., Conclusions: In our study, the NN was mainly observed in children around 3 years old. The main causal agent was pneumococcus. The evolution towards NN appeared to be faster than in case of other etiologies. Surgery management was unusual. All children required prolonged admissions but had a full clinical recovery.