Chronic recurrent multifocal osteomyelitis in pediatrics in a tertiary center / Osteomielitis crónica multifocal recurrente en pediatría en un hospital terciario

ABSTRACT Introduction: Chronic recurrent multifocal osteomyelitis (CRMO), also called chronic non-bacterial osteomyelitis, is an autoinflammatory disease characterized by bone involvement, recurrent flare-ups, and the lack of microbiological isolation. It is a diagnosis of exclusion, and the fundamental basis of treatment is non-steroidal anti-inflammatory drugs. The objective of the study is to describe our experience as a result of three girls diagnosed with CRMO, highlighting the clinical presentation, the findings in the complementary tests, the treatment, and the evolution of the disease. Patients and methods: Retrospective chart review of children with CRMO in the last 5 years, being followed-up in a pediatric rheumatology clinic in a tertiary center. Results: The cases are presented of 3 patients diagnosed with CRMO, all of them young girls, with a mean age of 11 years, who consulted due to pain and functional impotence. It was in single location in two cases, and the other with several sources of pain, at cervical and lumbar level, associated with weakness of the upper and lower limbs. Two of the cases received antibiotic treatment. One girl responded to treatment with anti-inflammatory drugs and another required combining corticosteroids. The remaining case, in addition to anti-inflammatory drugs and corticosteroids, required intravenous pamidronate. Conclusions: With this study, and despite the small sample size, the aim was to highlight the importance of this, in many cases unknown and underdiagnosed, pathology, and to stress the importance of establishing a diagnostic and therapeutic protocol for the correct approach to this disease.

RESUMEN Introducción: La osteomielitis crónica multifocal recurrente (OCMR), también conocida como osteomielitis crónica no bacteriana, es una enfermedad autoinflamatoria caracterizada por afectación ósea, de curso en brotes y en ausencia de aislamiento microbiológico. El diagnóstico es de exclusión y el pilar fundamental del tratamiento son los antiinflamatorios no esteroideos (AINES). El objetivo del estudio es describir nuestra experiencia de tres niñas diagnosticadas de OCMR, destacando la presentación clínica, los hallazgos en las pruebas complementarias, el tratamiento y la evolución de la enfermedad. Pacientes y métodos: Revisión retrospectiva de historias clínicas de niños diagnosticados de OCMR en los últimos cinco años, en seguimiento en consulta de reumatología pediátrica de un hospital terciario. Resultados: Presentamos tres pacientes diagnosticadas de OCMR, todas ellas mujeres adolescentes, con media de edad de 11 años. Consultaron por dolor e impotencia funcional, dos en una única localización y la otra por varios focos de dolor, a nivel cervical y lumbar, asociando debilidad de miembros superiores e inferiores. Con respecto al tratamiento, dos recibieron tratamiento antibiótico. Una niña respondió a antiinflamatorios; otra precisó asociar corticoides, y la restante, además de antiinflamatorios y corticoides, necesitó pamidronato intravenoso. Conclusiones: Con este estudio y a pesar del pequeño tamaño muestral, se pretende resaltar la importancia de esta patología, en muchos casos desconocida e infradiagnosticada, e insistir en la importancia de establecer un protocolo diagnóstico y terapéutico para su correcto abordaje.

Rendimiento de las pruebas complementarias en el estudio de pacientes con dolor abdominal crónico / Usefulness of complementary test in the study of patients with chronic abdominal pain

Introducción: El dolor abdominal crónico (DAC) en la infancia es un motivo de consulta frecuente que afecta a la vida familiar, y en ocasiones precisa realización de pruebas complementarias. El objetivo fue realizar el análisis cualitativo, cuantitativo y económico de las pruebas que se solicitan.Pacientes y métodos: Estudio observacional, prospectivo y multicéntrico, incluyendo pacientes entre 4-15 años con DAC. Se diferenciaron 2 grupos: orgánico y funcional. Se recogieron las siguientes variables: clínicas, pruebas complementarias y su coste.Resultados: Se incluyeron 235 niños con DAC (edad media 9,7±2,7 años). Un 79% resultaron trastornos funcionales y un 21% orgánicos. Casi la mitad de los pacientes presentaba algún tipo de síntoma o signo de alarma, pero solo la clínica miccional se asoció con organicidad. La ecografía abdominal, estudio de parásitos en heces, test de hidrógeno espirado y gastroscopia son las que más se asociaron con enfermedad orgánica. Existía una diferencia apreciable entre el coste de las pruebas según cada centro. El gasto económico total fue de 52.490,8euros, siendo 195euros por paciente para los funcionales y 306euros para los orgánicos.Conclusiones: Los síntomas y signos de alarma en el DAC son frecuentes, pero poco específicos. La ecografía abdominal y el estudio de parásitos podrían ser pruebas útiles de primer nivel por su inocuidad para diferenciar TO de TDAF. La gastroscopia y el test de hidrógeno espirado fueron las pruebas más discriminativas de organicidad. El coste económico invertido en pruebas para la orientación diagnóstica del DAC de origen funcional es elevado. (AU)

Introduction: Chronic abdominal pain (CAP) in children is a symptom that frequently leads to a visit to the paediatrician, which affects family life and occasionally requires the need to perform diagnostic studies (DS). The objective was to carry out a qualitative, quantitative, and economic analysis on the tests requested.Patients and methods: An observational, prospective and multicentre study was conducted that included children between 4-15 years old affected by CAP. The difference between organic and functional disorders was taken into account. The following variables were collected: history, warning signs and symptoms, DS, and the cost of these.Results: The study included 235 children with CAP (Age; mean 9.7±2.7 SD). The large majority (79%) were functional disorders and 21% organic disorders. Almost half of the patients had some warning sign or symptom, but urinary symptoms were only associated with organic disorders. The abdominal ultrasound, faecal parasites, breath test, and endoscopy were the most associated with organic disorders. There was a difference between the costs of the DS according to each centre. The total economic cost was 52,490.80 euros, with 195 euros per patient for functional disorders and 306 euros for organic disorders.Conclusion: Signs and symptoms of alarm in CAP were very frequent, but had low discriminative capacity. The abdominal ultrasound and faecal parasites are innocuous DS, and could be useful as a first level study. The endoscopy and the breath test were the most discriminative of organic disease. The economic cost of DS arising from the diagnosis of exclusion in CAP was high. (AU)

Usefulness of complementary test in the study of patients with chronic abdominal pain.

INTRODUCTION: Chronic abdominal pain (CAP) in children is a symptom that frequently leads to a visit to the paediatrician, which affects family life and occasionally requires the need to perform diagnostic studies (DS). The objective was to carry out a qualitative, quantitative, and economic analysis on the tests requested. MATERIAL AND METHODS: An observational, prospective and multicentre study was conducted that included children between 4-15 years old affected by CAP. The difference between organic and functional disorders was taken into account. The following variables were collected: history, warning signs and symptoms, DS, and the cost of these. RESULTS: The study included 235 children with CAP (Age; mean 9.7 ± 2.7 SD). The large majority (79%) were functional disorders and 21% organic disorders. Almost half of the patients had some warning sign or symptom, but urinary symptoms were only associated with organic disorders. The abdominal ultrasound, faecal parasites, breath test, and endoscopy were the most associated with organic disorders. There was a difference between the costs of the DS according to each centre. The total economic cost was 52,490.80 euros, with 195 euros per patient for functional disorders and 306 euros for organic disorders. CONCLUSION: Signs and symptoms of alarm in CAP were very frequent, but had low discriminative capacity. The abdominal ultrasound and faecal parasites are innocuous DS, and could be useful as a first level study. The endoscopy and the breath test were the most discriminative of organic disease. The economic cost of DS arising from the diagnosis of exclusion in CAP was high.

Edema palpebral bilateral: un primer signo infrecuente de la leucemia mieloide aguda / Bilateral eyelid edema: an infrequent first sign of acute myeloid leukemia

El sarcoma granulocítico es una manifestación extramedular de la leucemia mieloide aguda (LMA) poco frecuente. Es más común en la población pediátrica y puede presentarse en cualquier momento en el curso de la enfermedad. En ocasiones, puede preceder a la aparición clínica de leucemia mieloide aguda. Se presenta el caso de un niño de tres años con infiltración orbitaria bilateral que precipitó el diagnóstico de LMA

Granulocytic sarcoma is an unusual extramedullary manifestation of acute myeloid leukemia (AML). It is more common in the pediatric population and it may present at any time in the course of the disease. Occasionally, it may precede the clinical onset of acute myeloid leukemia. The case of a 3-year-old male child with a bilateral orbital infiltration that precipitated the diagnosis of AML is presented