Coronary Bypass in an Adolescent With Systemic Lupus Erythematosus and Acute Coronary Syndrome.

A 12-year-old female with active pediatric juvenile systemic lupus erythematosus presented to the emergency department because of episodes of oppressive central thoracic pain associated with pallor, sweating, and muscle weakness that persisted for >30 minutes. During the last episode, the electrocardiogram revealed alterations in cardiac repolarization coincident with progressive troponin T elevation. An angio computed tomography revealed a 20 mm long complete segmental obstruction of the proximal anterior descending artery that was confirmed by angiography. Because this extensive occlusion did not permit a noninvasive procedure, an off-pump coronary bypass of the internal mammary artery to the anterior descending artery was performed without complication. Six months after the procedure, myocardial function was good. To our knowledge, this is the first case report of an adolescent girl with acute coronary syndrome complicating juvenile systemic lupus erythematosus that was treated with a surgical procedure.

Characteristics of Upper Limb Osteoarticular Infections at the Emergency Department of a Tertiary University Hospital in Spain.

OBJECTIVES: Osteoarticular infections are infrequent in pediatric patients, although their incidence seems to be increasing. They usually affect children younger than 5 years and tend to localize in the lower limbs. Because of their nonspecific symptoms, especially at onset, a timely diagnosis is difficult to achieve, with the subsequent risk of a delay in treatment. We hereby report the management of osteoarticular infections in our pediatric emergency department. METHODS: This is a retrospective descriptive study of patients diagnosed with osteoarticular upper limb infection in the pediatric emergency department of a tertiary hospital from January 2011 to December 2016. RESULTS: From an initial global sample of 170 patients diagnosed with osteomyelitis or septic arthritis at any location at the pediatric emergency department, 32 children (18.82%) with upper limb involvement were included in the study. Of them, 22 were male and the mean age at diagnosis was 14.5 months (interquartile range, 2-106). Eighteen patients (56%) were diagnosed with septic arthritis, and 14 (44%) had a diagnosis of osteomyelitis.The most frequent symptom was pain (50%). More than one third of patients (11) had received a different diagnosis in a previous hospital visit. A traumatic etiology was suspected in 7 cases (21%).Regarding acute phase reactants, the mean value for C-reactive protein was 21.3 mg/L, and erythrocyte sedimentation rate was elevated in 27 cases (84%). In 28 patients, blood cultures were obtained, 24 of which came back negative. All children received antibiotic treatment and achieved a full recovery. CONCLUSIONS: One third of patients were misdiagnosed at the first consultation, which stresses the importance of a high clinical suspicion to avoid delays in diagnosis and treatment of osteoarticular infections. This study also shows a lower mean age of children with upper limb infection as compared with those with lower limb infection. All patients recovered fully with oral antibiotics.

Systemic Type I IFN Inflammation in Human ISG15 Deficiency Leads to Necrotizing Skin Lesions.

Most monogenic disorders have a primary clinical presentation. Inherited ISG15 deficiency, however, has manifested with two distinct presentations to date: susceptibility to mycobacterial disease and intracranial calcifications from hypomorphic interferon-II (IFN-II) production and excessive IFN-I response, respectively. Accordingly, these patients were managed for their infectious and neurologic complications. Herein, we describe five new patients with six novel ISG15 mutations presenting with skin lesions who were managed for dermatologic disease. Cellularly, we denote striking specificity to the IFN-I response, which was previously assumed to be universal. In peripheral blood, myeloid cells display the most robust IFN-I signatures. In the affected skin, IFN-I signaling is observed in the keratinocytes of the epidermis, endothelia, and the monocytes and macrophages of the dermis. These findings define the specific cells causing circulating and dermatologic inflammation and expand the clinical spectrum of ISG15 deficiency to dermatologic presentations as a third phenotype co-dominant to the infectious and neurologic manifestations.

¿Debe mejorar la formación en reumatología pediátrica? Resultados de una encuesta nacional / Should training in paediatric rheumatology be improved? Results of a national survey

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Artritis de cadera en probable relación con la administración de la vacuna antimeningocócica 4CMenB / Hip arthritis probably related to antimeningococcal vaccine 4CMenB

La vacuna meningocócica del grupo B 4CMenB está indicada para la inmunización activa de individuos a partir de los dos meses de edad frente a la enfermedad meningocócica invasora por Neisseria meningitidis del serogrupo B. Aunque está recomendada, actualmente no se encuentra incluida dentro del calendario de vacunación infantil. En menores de dos años las reacciones locales más frecuentes son dolor y eritema en el sitio de inyección y las sistémicas la aparición de irritabilidad y fiebre, en general de corta duración. En la ficha técnica de la vacuna no se indica como efecto secundario específico la artritis. No obstante, hasta marzo de 2017 se han documentado en la base de datos europea de informes de presuntas reacciones adversas 29 casos de artritis u otra patología similar. A continuación, presentamos las características clínicas y analíticas de dos nuevos casos de artritis en probable relación con la vacuna

Meningococcal group B vaccine is indicated for active immunization of individuals from two months of age against invasive meningococcal disease caused by Neisseria meningitidis serogroup B. Although it is recommended, currently it is not included in the childhood immunization schedule. In children under two years the most common reactions are pain and erythema at the injection site and fever and irritability, generally of short duration. In the technical specifications of the vaccine it is not indicated the arthritis. However, until March 2017 it has been documented 29 cases of arthritis in the European database of suspected adverse reactions reports. Here we report clinical and laboratory characteristics of two new cases of arthritis probably related to the administration of the vaccine

Eficacia de anakinra como tratamiento de la pericarditis recurrente / Efficacy of anakinra as a treatment for recurrent pericarditis

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En respuesta a: Deformidad de Madelung / Response to: Madelung deformity

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