Application of the new PRINTO classification criteria for juvenile idiopathic arthritis in a sample of Portuguese patients.

BACKGROUND: The International League of Associations for Rheumatology (ILAR) classification system for juvenile idiopathic arthritis (JIA) does not depict homogenous subgroups of disease. As to unify our language with the adult rheumatic diseases, the Pediatric Rheumatology International Trials Organization (PRINTO) is attempting to revise these criteria. OBJECTIVE: To reclassify a JIA sample according to the new provisional PRINTO subsets: systemic JIA (sJIA), RF-positive JIA (RF-JIA), early-onset ANA-positive JIA (eoANA-JIA), enthesitis/spondylitis-related JIA (ESR-JIA), "other JIA" and "unclassified JIA". METHODS: Retrospective study including JIA patients followed in a Pediatric Rheumatology Unit at a university hospital. Medical records were reviewed, and patients were reclassified as per the provisional PRINTO criteria. RESULTS: Of a total of 104 patients, 41 (39.4%) were reclassified as "other JIA", 36 (34.6%) as eoANA-JIA, 15 (14.4%) as ESR-JIA, 8 (7.7%) as sJIA and 4 (3.8%) as RF-JIA. More than 90% of the oligoarticular JIA were reclassified into either eoANA-JIA or "other JIA". Only one negative RF polyarticular JIA converted to RF-JIA due to the presence of a positive anti-citrulinated peptide antibody (ACPA). The psoriatic arthritis (PsA) subgroup disappeared into eoANA-JIA (25%), ESR-JIA (25%) or "other JIA" (50%). There were significant differences in age of onset, but not on the gender ratio or uveitis presence. Antinuclear antibody was more frequent in females (p=0.035) and younger patients (p<0.001). CONCLUSION: The number of affected joints and PsA features elapsed in favour of laboratory RF, ACPA and ANA traits. PsA and oligoarticular JIA were abolished. The "other JIA" entity is heterogenous and prevalent, claiming reformulation.

Impacto de la COVID-19 en niños con trastorno del espectro autista / The impact of COVID-19 on children with autism spectrum disorder

INTRODUCCIÓN: Los niños con trastorno del espectro autista (TEA) a menudo experimentan el cambio de rutinas como un desafío importante. La necesidad de adaptación durante la pandemia por la COVID-19 puede haber causado problemas a estos niños. OBJETIVO: Conocer cómo vivieron los niños con TEA y sus familias el aislamiento social durante la cuarentena. Sujetos y métodos. Se realizó un estudio observacional, transversal y analítico. Se aplicó un cuestionario anónimo que incluía las características demográficas y clínicas de los niños, junto con el impacto que tuvo la pandemia en diferentes aspectos de la vida diaria de las familias. RESULTADOS: De los 99 cuestionarios obtenidos, 43 eran niños con TEA y 56 niños del grupo control. Los niños con TEA tuvieron predominantemente cambios en el comportamiento, a diferencia de los del grupo control. La mayoría de los niños con TEA tuvo un impacto negativo en el manejo de las emociones frente a los del grupo control, que expresaron un impacto mayoritariamente positivo/nulo. Los cuidadores puntuaron niveles de ansiedad más altos en ellos mismos que en sus hijos. Los niños con TEA y sus padres tenían niveles más altos de ansiedad que los controles. En el grupo con TEA, los niños que no mantuvieron las rutinas tuvieron niveles de ansiedad más altos. CONCLUSIÓN: Los resultados muestran un potencial impacto psicológico de la pandemia de la COVID-19 no sólo en los niños con trastornos del neurodesarrollo, sino también en sus cuidadores. Debe estarse preparado para la vigilancia de los trastornos mentales en las familias tras la pandemia de la COVID-19

INTRODUCTION. Children with autism spectrum disorder (ASD) often experience changing routines as a major challenge. For that reason, the need for adaptation during COVID-19 pandemic may have brought major problems to families with children with this pathology. AIM. To explore how children with ASD and their parents experienced the social isolation during COVID-19 outbreak period. SUBJECTS AND METHODS. We conducted an observational, cross-sectional and analytical study. We applied an anonymous questionnaire that included children’s demographic and clinical characteristics, along with the impact of the COVID-19 outbreak in different aspects of family’s daily life. RESULTS. Out of 99 questionnaires obtained, 43 were related to children with ASD and 56 to control group. Children with ASD predominantly had changes in behavior, while children from control group mostly found no changes. The majority of parents of ASD children reported a negative impact in emotion management against those in control group reporting mostly positive or no impact. Caregivers reported higher mean scores of anxiety levels in themselves than in their children. ASD children and their parents had higher levels of anxiety than healthy ones. In the group with ASD, children that did not maintain routines had higher mean levels of anxiety than children that maintained routines. CONCLUSION. Our results show a potential important psychological impact of the COVID-19 pandemic not only in children with neurodevelopmental disorders but in their caregivers as well. Physicians must be prepared for the post-pandemic surveillance of mental disorders among families

¿Distrés respiratorio persistente o algo más? / Persistent respiratory distress or something else?

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Sclerosing angiomatoid nodular transformation of the spleen: case report of a metastatic carcinoma-simulating disorder.

Sclerosing angiomatoid nodular transformation (SANT) is a rare nonneoplastic splenic disorder of unknown etiopathogenesis. This condition is usually found incidentally on imaging studies. Because of its similar features, SANT can wrongly be described as metastatic carcinoma. A 61-year-old Caucasian male was referred to our general surgery outpatient clinic regarding unusual splenic nodular formations in a routine abdominal ultrasound. All diagnostic exams performed confirmed metastatic splenic lesions, but no primary tumor was found. A laparoscopic splenectomy was performed for diagnostic purposes. Histopathology revealed SANT. Benign tumors of the spleen are uncommon entities and can easily be mistaken by malignant secondary lesions. The differential diagnosis of SANT should include other vascular lesions as well as metastatic carcinoma and inflammatory pseudotumor. It is widely recommended that a splenectomy should be performed because only by histopathology and immunohistochemistry staining, the definitive diagnosis of SANT can be made.