Asunto(s)
Miositis , Centros de Atención Terciaria , Humanos , Miositis/terapia , Miositis/etiología , Masculino , Femenino , Niño , Enfermedad Aguda , Estudios Retrospectivos , Adolescente , PreescolarRESUMEN
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.
Asunto(s)
Síndrome Coronario Agudo , Lupus Eritematoso Sistémico , Niño , Femenino , Humanos , Síndrome Coronario Agudo/complicaciones , Puente de Arteria Coronaria , Electrocardiografía , Lupus Eritematoso Sistémico/complicaciones , Tomografía Computarizada por Rayos XRESUMEN
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.
Asunto(s)
Artritis Infecciosa , Osteomielitis , Antibacterianos/uso terapéutico , Artritis Infecciosa/diagnóstico , Artritis Infecciosa/tratamiento farmacológico , Artritis Infecciosa/epidemiología , Niño , Servicio de Urgencia en Hospital , Humanos , Lactante , Masculino , Osteomielitis/diagnóstico , Osteomielitis/tratamiento farmacológico , Osteomielitis/epidemiología , Estudios Retrospectivos , España/epidemiología , Centros de Atención Terciaria , Extremidad SuperiorRESUMEN
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.