Resumo Objetivo: Descrever as características clínicas e os diferentes fenótipos de crianças com síndrome inflamatória multissistêmica na criança temporalmente relacionada com a COVID-19 (do inglês multisystem inflammatory syndrome in children — MIS-C) e avaliar as condições de risco que favorecem a maior gravidade da doença durante um período de 12 meses em um hospital pediátrico de referência na Colômbia. Métodos: Estudo retrospectivo de 12 meses de observação de crianças menores de 18 anos que cumprem os critérios para o MIS-C. Resultados: Vinte e oito crianças foram apresentadas com os critérios do MIS-C. A idade média era de sete anos, e 54% eram do sexo masculino. Para além da febre (100%) (com início quatro dias antes da admissão), as características clínicas mais frequentes eram gastrointestinais (86%) e mucocutâneas (61%). Quatorze crianças (50%) apresentavam sintomas semelhantes aos de Kawasaki. As anomalias ecocardiográficas mais frequentes foram derrame pericárdico (64%), envolvimento valvar (68%), disfunção ventricular (39%) e anomalias coronárias (29%). Tinham linfopenia 75% das crianças. Todas tinham algum teste de coagulação anormal. A maioria recebeu imunoglobulina intravenosa (89%), glucocorticoides (82%), vasopressores (54%) e antibióticos (64%). Tiveram envolvimento mais grave 61% dos pacientes, que precisaram ser internados em unidade de terapia intensiva (mediana de quatro dias, média de seis dias); os preditores de gravidade foram pacientes com fenótipo inflamatório/ MIS-C (odds ratio — OR 26,5; intervalo de confiança — IC95% 1,4–503,7; p=0,029) e erupção cutânea (OR 14,7; IC95% 1,2–178,7; p=0,034). Dois pacientes (7%) apresentavam síndrome de ativação macrofágica. Conclusões: Alteração da artéria coronária, disfunção ventricular e internação na unidade de terapia intensiva foram frequentes, o que nos alerta sobre a importância da suspeita clínica precoce.

Abstract Objective: This study aimed to describe the clinical characteristics and the different phenotypes of children with multisystem inflammatory syndrome in children (MIS-C) temporally related to COVID-19 and to evaluate the risk conditions that favored a greater severity of the disease during a 12-month period at a pediatric reference hospital in Colombia. Methods: A 12-month retrospective observational study of children under the age of 18 years who met criteria for MIS-C. Results: A total of 28 children presented MIS-C criteria. The median age was 7 years. Other than fever (100%) (onset 4 days prior to admission), the most frequent clinical features were gastrointestinal (86%) and mucocutaneous (61%). Notably, 14 (50%) children had Kawasaki-like symptoms. The most frequent echocardiographic abnormalities were pericardial effusion (64%), valvular involvement (68%), ventricular dysfunction (39%), and coronary artery abnormalities (29%). In addition, 75% had lymphopenia. All had at least one abnormal coagulation test. Most received intravenous immunoglobulin (89%), glucocorticoids (82%), vasopressors (54%), and antibiotics (64%). Notably, 61% had a more severe form of the disease and were admitted to an intensive care unit (median 4 days, mean 6 days); the severity predictors were patients with the inflammatory/MIS-C phenotype (OR 26.5; 95%CI 1.40–503.7; p=0.029) and rash (OR 14.7; 95%CI 1.2–178.7; p=0.034). Two patients had macrophage activation syndrome. Conclusions: Coronary artery abnormalities, ventricular dysfunction, and intensive care unit admission were frequent, which needs to highlight the importance of early clinical suspicion.

Resumen Justificación: La uveítis idiopática y la asociada a artritis idiopática juvenil son condiciones oculares que llevan a deterioro visual. En Costa Rica, la incidencia en pediatría se desconoce; aunque existe una clínica especializada en el Hospital Nacional de Niños para el manejo de estas patologías, no hay guías ni recomendaciones de manejo que hayan sido publicadas. Objetivos: Resumir las recomendaciones de manejo vigentes para uveítis no infecciosas y ofrecer una guía adaptada a la realidad costarricense, específicamente para población pediátrica. Métodos: Se efectuó una revisión no sistemática de bibliografía médica indexada en las plataformas PubMed y Scielo, sobre el diagnóstico y manejo de uveítis no infecciosa orientado a población pediátrica y en artritis idiopática juvenil. Se revisaron las guías recomendadas en Europa y se ajustaron a lo que se realiza en la Clínica de Uveítis del Hospital Nacional de Niños. Conclusiones: Se realiza una propuesta de manejo que incluye una valoración oftalmológica oportuna, diagnóstico certero, y tratamiento temprano y agresivo para prevención de complicaciones y minimizar secuelas.

Summary Importance: Idiopathic uveitis and that associated with juvenile idiopathic arthritis are ocular conditions that lead to visual impairment. In Costa Rica, the incidence in pediatric population is unknown; although there is a specialized clinic at the National Children’s Hospital for the management of these pathologies, there are no guidelines or recommendations published in our country. Aims: To summarize the current management recommendations for non-infectious uveitis and offer a guide adapted to the Costa Rican reality, specifically for the pediatric population. Methods: A non-systematic review of indexed medical literature on PubMed and Scielo platforms was carried out on the diagnosis and management of non-infectious uveitis aimed to pediatric patients and in juvenile idiopathic arthritis. The recommended guidelines in Europe were reviewed and adjusted to what is done at the Uveitis Clinic of the National Children’s Hospital. Conclusions: A management proposal is made that includes a timely ophthalmological evaluation, accurate diagnosis; early and aggressive treatment to prevent complications and minimize sequelae

Abstract Background: Hereditary angioedema is classified as a primary immunodeficiency of the complement system because it is characterized by the absence of C1 esterase inhibitor (C1-INH) and by the periodic edema of any region of the body that involves soft tissue. Objective: To characterize the adult patients with a confirmed diagnosis of hereditary angioedema who have been treated in the Clinical Allergology Department of Hospital México de la Caja Costarricense del Seguro Social. Methods: The study was retrospective and observational. The information was obtained from the clinical records of the patients with a confirmed diagnosis of hereditary angioedema that were being controlled in the Allergology Department of the “Hospital México de la Caja Costarricense del Seguro Social”. Results: A total of 14 patients; seven men and seven women, were identified. The average was of 36.6 years of age. The most frequent clinical manifestations were peripheral edema and abdominal pain. Only three patients presented laryngeal edema at some point in their evolution. 12 cases corresponded to hereditary angioedema type I. Conclusion: The clinical characteristics of all the documented cases corresponded to those described for this pathology, although only a few patients had a history of severe manifestations.

Resumen Antecedentes: El angioedema hereditario se encuentra clasificado como una inmunodeficiencia primaria del sistema de complemento, debido a que se caracteriza por la ausencia de C1 inhibidor esterasa y por edema periódico de cualquier región del cuerpo que involucre tejido blando. Objetivo: Caracterizar a los pacientes adultos con diagnóstico de angioedema hereditario atendidos en el Servicio de Alergología Clínica del Hospital México de la Caja Costarricense del Seguro Social. Métodos: Estudio observacional retrospectivo. Los datos fueron obtenidos de los expedientes clínicos de los pacientes con diagnóstico confirmado de angioedema hereditario que estaban en seguimiento en el Servicio de Alergología del Hospital México, Caja Costarricense del Seguro Social. Resultados: Se identificaron 14 pacientes, siete hombres y siete mujeres. El promedio de edad fue de 36.6 años. Las manifestaciones clínicas más frecuentes fueron edema de extremidades y dolor abdominal. Solo tres pacientes en algún momento durante su evolución presentaron edema laríngeo; 12 casos correspondieron a angioedema hereditario tipo I. Conclusión: Las características clínicas de todos los casos documentados correspondieron con las descritas para angioedema, aunque solo algunos pacientes contaban con historial de manifestaciones graves.

La tuberculosis (TB) se declaró por la OMS como una "emergencia global" La TB congénita (TBC) es muy rara, siendo rápida y progresiva. Los criterios para su diagnóstico son: (1) lesiones en la primera semana de vida; (2) complejo hepático primario o granulomas caseosos; (3) infección tuberculosa documentada en endometrio o placenta y (4) exclusión de infección tuberculosa luego del nacimiento de otras fuentes. Es difícil encontrar estos criterios por lo que la TBC debe ser considerada en todo neonato enfermo, con pobre respuesta a la terapia antibiótica convencional, especialmente si la madre tiene factores de riesgo para esta infección. Los exámenes de TB en niños son poco sensibles. El diagnóstico diferencial entre TBC y TB postnatal es puramente académico y epidemiológico, ya que el manejo es el mismo para ambas entidades. El objetivo de este reporte es presentar un caso de TBC