Biological therapy in refractory chronic nonbacterial osteomyelitis: A case series of 19 patients.

INTRODUCTION: Chronic non-bacterial osteomyelitis (CNO) represents an autoinflammatory bone disorder. Currently there are no standardized diagnostic or treatment guidelines. The objective of the study is to describe our experience with biological therapy in children with the disease. METHODS: Retrospective chart review of patients with CNO treated with biological therapy followed at two tertiary hospitals from January 2007 to April 2020. Biologicals were started in most patients due to persistent disease activity after receiving standard therapy with at least 2 drugs (NSAIDs and corticosteroids and/or pamidronate). RESULTS: Twenty-five patients were diagnosed with CNO. Out of those, 19 patients (15 females) failed conventional therapy. The mean age at diagnosis was 8.8±2.9 years and the mean diagnostic delay was 6.9±8.3 months. All patients presented with bone pain and 6/19 also had fever. The most frequently affected bones were femur (9 patients), followed by clavicle, tibia and vertebrae (6, 6 and 5 patients respectively). Nine children had skin lesions. C-reactive protein was elevated in 13/19 patients (mean 20.2mg/L±11.7) and ESR in 16/19 (mean 48mm/h±29). All patients received nonsteroidal anti-inflammatory drugs, 15/19 pamidronate, 10/19 corticosteroids and 19 anti-TNF-therapy. At the last follow-up visit, 10/19 patients were still on biological therapy (8 adalimumab, 2 infliximab) and 18 out of 19 remained asymptomatic. In regards to adverse effects, one patient receiving infliximab developed S. aureus osteomyelitis and another cutaneous leishmaniosis. CONCLUSIONS: This research emphasizes that anti-TNF-therapy represents an effective and safe alternative for patients with CNO refractory to conventional treatments.

Adalimumab for the treatment of refractory noninfectious paediatric uveitis.

To report the experience of our center with the use of adalimumab (ADA) for the treatment of severe refractory noninfectious paediatric uveitis. The study is a retrospective case series of all paediatric patients with refractory uveitis who were treated with ADA at the Paediatric Uveitis Unit of our center from 2008 to 2015. We present 12 patients (6 Juvenile idiopathic arthritis-associated uveitis, 4 idiopathic panuveitis, 1 early-onset sarcoidosis-associated panuveitis, and 1 intermediate uveitis), with uveitis in 19/24 eyes. Once ADA therapy was started, all the patients presented improved activity according to Standardization of Uveitis Nomenclature (SUN) criteria. Nine out of the 12 patients had structural damage before ADA could be started: cataract (n = 4), glaucoma (n = 2), cystic macular edema (n = 1), exudative retinal detachment (n = 1), and optic disk edema (n = 5). Visual acuity improved or maintained stable in 17/19 affected eyes, and only 2 eyes decreased its visual acuity because of structural damage, which was already present before ADA therapy. In our experience, ADA presents a good safety profile and is efficacious in the treatment of paediatric patients with different forms of refractory noninfectious uveitis.

Intravenous immunoglobulin therapy for refractory recurrent pericarditis.

Recurrent pericarditis is a troublesome complication of idiopathic acute pericarditis and occurs more frequently in pediatric patients after cardiac surgery (postpericardiotomy syndrome). Conventional treatment with nonsteroidal antiinflammatory drugs, corticosteroids, and colchicine is not always effective or may cause serious adverse effects. There is no consensus, however, on how to proceed in those patients whose disease is refractory to conventional therapy. In such cases, human intravenous immunoglobulin, immunosuppressive drugs, and biological agents have been used. In this report we describe 2 patients with refractory recurrent pericarditis after cardiac surgery who were successfully treated with 3 and 5 monthly high-dose (2 g/kg) intravenous immunoglobulin until resolution of the effusion. Our experience supports the effectiveness and safety of this therapy.

Incidencia de traumatismo craneoencefálico asociada con la edad, el sexo, y la condición socio económica de los niños ingresados a la Unidad de Cuidados Intensivos del Hospital de Niños “Baca Ortiz”: enero-agosto del 2005

Esta investigación pretende evidenciar que siendo el traumatismo craneoencefálico una de las principales causas de morbi-mortalidad general que afecta a la población ecuatoriana en general y progresivamente presente en la edad pediátrica, es un fenómeno que debe ser enfrentado desde diferentes áreas de la salud, es decir se constituye en un desafío para la salud pública y ella ligada profundamente a los requerimientos, características de los servicios hospitalarios de especialidad quienes se encargan de los aspectos clínico quirúrgicos para el diagnóstico, tratamiento y recuperación del trauma cráneoencefálico. Para el desarrollo de este estudio se optó por la metodología no experimental, transversal y descriptiva, fue realizado en el servicio de terapia intensiva del Hospital de Niños "Baca Ortiz", durante el periodo de Enero- Agosto de 2005, se consideró como casos a los niños con diagnóstico de Traumatismo Craneoencefálico, mediante observación y manejo de registros que se utilizan en la unidad (37 pacientes e historias clínicas respectivas), la entrevista a padres o familiares, apoyada en las encuestas que maneja servicio social. Fue digitada en Excel para el análisis estadístico. Los resultados obtenidos en este estudio evidencian que de 179 pacientes que ingresaron a la DCI, entre los meses de enero a agosto del 2005, los mismos que presentan diversas patologías, el 21 % que corresponde a 37 pacientes presentan Traumatismo Craneoencefálico. En este tiempo y espacio establecido la patología investigada es la primera causa de ingreso hospitalario. El 83 % de las familias estudiadas, se ubican en el nivel socio económico medio y bajo, ello se ve reflejado en su condición de vida, su nivel de escolaridad, vivienda, la estructura familiar, en un salario pauperizado que alcanza un máximo de $100,00 dólares para el 62% de las familias estudiadas. Las condiciones de extrema pobreza impiden a la...