Osteomielitis multifocal recurrente en niños: experiencia de un centro de tercer nivel / Recurrent multifocal osteomyelitis in children: Experience in a tertiary care center

Introducción: La osteomielitis multifocal crónica recurrente es una inflamación ósea aséptica poco frecuente en pediatría cuyo abordaje y tratamiento no está estandarizado. Métodos: Estudio descriptivo retrospectivo de menores de 14 años a quienes se diagnosticó osteomielitis crónica no bacteriana (OCNB) en un hospital de tercer nivel. Se incluyeron los pacientes diagnosticados en los últimos 6 años (2010-2015), y que cumplían los criterios de Jansson. Se analizaron las características clínicas y radiológicas, y su evolución tras las diferentes opciones terapéuticas. Resultados: Se analizaron 12 casos, con 11 años de media (±1,6desviaciones estándar [DE]), y predominio femenino (10:2). La media de focos fue de 3,5 (±2,2DE). Las localizaciones más frecuentes fueron: tobillo (58%), clavícula (50%), esternón (33%) y cadera (25%). La media de tiempo de evolución fue de 10,5 meses (±10,3DE) y la mediana hasta el diagnóstico de 2,38 meses (0,17-16). En el 33% se detectaron focos asintomáticos con gammagrafía ósea y en el 50% lesiones líticas con resonancia. Se realizó biopsia en el 60%; 2/12 (16%) asociaron patología inflamatoria y 1/12 (8,3%) desarrolló linfoma posteriormente. El 58% recibieron tratamiento antibiótico con escasa respuesta, el 100% antiinflamatorios, y el 50% corticoides sistémicos. El 41,6% requirieron metotrexato o pamidronato, y el 16% anti-TNFα. La media de tiempo de tratamiento fue de 14,8 meses (±12,4DE), presentando recurrencias el 66%. Actualmente el 83% se encuentran en remisión clínica sin tratamiento. Conclusiones: En OCNB refractarias a antiinflamatorios, el pamidronato intravenoso podría constituir una alternativa terapéutica en niños. Los fármacos anti-TNFα podrían considerarse en pacientes con fracaso a pamidronato, o aquellos que asocien entidades autoinmunes

Introduction: Chronic recurrent multifocal osteomyelitis is a rare aseptic bone inflammation that affects pediatric patients. Its management and treatment have not yet been standardized. Methods: Retrospective, descriptive study of patients under 14 years of age diagnosed with chronic nonbacterial osteomyelitis (CNBO) in a tertiary hospital. We included patients diagnosed over the last 6 years (2010-2015) who met the Jansson criteria. The clinical and radiological characteristics of CNBO were analyzed, as was the outcome after different therapeutic options. Results: We report 12 patients, with a mean age of 11 years (±1.6 standard deviation [SD]) and female predominance (10:2). The mean number of foci was 3.5 (±2.2 SD). The most common locations were ankle (58%), clavicle (50%), sternum (33%) and hip (25%). The mean disease duration was 10.5 months (±10.3 SD), and the median time to diagnosis was 2.38 months (range 0.17-16). Bone scintigraphy detected asymptomatic foci in 33% and we detected lytic lesions in 50% through magnetic resonance imaging. Biopsy was performed in 60%; 2/12 (16%) were associated with inflammatory disease and 1/12 (8.3%) later developed lymphoma. In all, 58% received antibiotic therapy with little response, 100% anti-inflammatory agents, 50% systemic corticosteroids, 41.6% methotrexate/pamidronate and 16% anti-tumor necrosis factor (TNF) α. The mean duration of treatment was 14.8 months (±12.4 SD) and 66% had recurrences. Currently, 83% are in clinical remission without treatment. Conclusions: When CNBO is refractory to treatment with anti-inflammatory drugs, intravenous pamidronate can be an alternative. Anti-TNF drugs can be considered in patients who fail with pamidronate, as can agents associated with other autoimmune conditions

Recurrent multifocal osteomyelitis in children: Experience in a tertiary care center. / Osteomielitis multifocal recurrente en niños: experiencia de un centro de tercer nivel.

INTRODUCTION: Chronic recurrent multifocal osteomyelitis is a rare aseptic bone inflammation that affects pediatric patients. Its management and treatment have not yet been standardized. METHODS: Retrospective, descriptive study of patients under 14 years of age diagnosed with chronic nonbacterial osteomyelitis (CNBO) in a tertiary hospital. We included patients diagnosed over the last 6 years (2010-2015) who met the Jansson criteria. The clinical and radiological characteristics of CNBO were analyzed, as was the outcome after different therapeutic options. RESULTS: We report 12 patients, with a mean age of 11 years (±1.6 standard deviation [SD]) and female predominance (10:2). The mean number of foci was 3.5 (±2.2 SD). The most common locations were ankle (58%), clavicle (50%), sternum (33%) and hip (25%). The mean disease duration was 10.5 months (±10.3 SD), and the median time to diagnosis was 2.38 months (range 0.17-16). Bone scintigraphy detected asymptomatic foci in 33% and we detected lytic lesions in 50% through magnetic resonance imaging. Biopsy was performed in 60%; 2/12 (16%) were associated with inflammatory disease and 1/12 (8.3%) later developed lymphoma. In all, 58% received antibiotic therapy with little response, 100% anti-inflammatory agents, 50% systemic corticosteroids, 41.6% methotrexate/pamidronate and 16% anti-tumor necrosis factor (TNF) α. The mean duration of treatment was 14.8 months (±12.4 SD) and 66% had recurrences. Currently, 83% are in clinical remission without treatment. CONCLUSIONS: When CNBO is refractory to treatment with anti-inflammatory drugs, intravenous pamidronate can be an alternative. Anti-TNF drugs can be considered in patients who fail with pamidronate, as can agents associated with other autoimmune conditions.

Successful Mercaptopurine Usage despite Azathioprine-Induced Pancreatitis in Paediatric Crohn's Disease.

BACKGROUND: Azathioprine [AZA] and mercaptopurine [MP] are recommended for maintenance of steroid-free remission in children with Crohn`s disease [CD]. Azathioprine-induced pancreatitis, an idiosyncratic and major side effect, has been considered as an absolute contraindication for the use of a second thiopurine in IBD patients. MATERIALS AND METHODS: We describe two children with CD in whom MP were successfully trialled after a confirmed azathioprine-induced pancreatitis, being well tolerated in both cases. RESULTS: Two boys [13 and 10 years old] started exclusive enteral nutrition after diagnosis of moderate (Pediatric Crohn's Disease Activity Index [wPCDAI] = 45) and mild [wPCDAI = 35] CD. Both developed an acute mild to moderate pancreatitis after 2 and 3 weeks, respectively, of AZA treatment but recovered fully in hospital after AZA withdrawal. They started on MP treatment without any adverse effect. They were tested for the presence of polymorphisms 238G>C, 460G>A, and 719A>G in the TPMT gene and 94C>A and 21>C in the ITPase. Both patients were wild-type for all tested polymorphisms. CONCLUSIONS: Azathioprine-induced acute pancreatitis should not be considered as an absolute contraindication for the use of MP. Further investigation is required to create a better understanding of the mechanism underlying the adverse events and to allow more possibilities for personalised therapy.