RESUMEN
A rare congenital malformation of the respiratory tract, bronchopulmonary sequestration generally presents in childhood and adolescence with recurrent pneumonia or in adulthood as an incidental finding on thoracic imaging. Manifesting as intrapulmonary or extrapulmonary types, bronchopulmonary sequestration characteristically receives blood supply from the systemic rather than pulmonary circulation. We present a 45-year-old male patient who received a provisional diagnosis of bronchopulmonary sequestration following an incidental finding on routine imaging. This case describes the way in which a provisional diagnosis may be made based upon imaging as well as underscoring the importance of alleviating the burden of additional imaging studies.
RESUMEN
Sickle cell disease (SCD) increases the incidence of childhood stroke eighty-fold. Stroke risk can be estimated by measurement of the blood velocity through the middle cerebral artery (MCA) using transcranial doppler ultrasound (TCD). A high MCA blood velocity indicates increased stroke risk due to cerebral vasculopathy, and first-line treatment to prevent primary or recurrent strokes in high-risk children with SCD has classically been chronic blood transfusions. Research has more recently shown that many of these patients may safely transition from transfusions to oral hydroxyurea (HU) treatment while maintaining a decreased risk of stroke. However, the effect on stroke risk of truly prophylactic HU treatment beginning in infancy, prior to the onset of cerebral vasculopathy, is less well understood. Our retrospective study aimed to document the long-term effects of HU treatment compared with no HU treatment in children with SCD, using TCD measurements as our primary outcome and a surrogate marker of stroke risk. Our results showed that when accounting for age-related variability and duration of treatment, prophylactic HU treatment was independently associated with lower TCD MCA velocities compared with no HU treatment, providing further evidence supporting its early initiation for patients with SCD.
RESUMEN
Behcet's disease (BD) is a multisystem, autoimmune vasculitis disorder affecting small, medium, and large blood vessels, with poorly understood pathogenesis. It commonly presents with recurrent aphthous ulcers, genital ulcers, skin lesions, and bilateral uveitis. Neurological symptoms are present in less than 10% of cases and develop, on average, 5-6 years after the first non-neurological symptoms. This presentation, known as Neuro-Behcet's disease (NBD), is associated with a worse prognosis of BD. Treatment for NBD is dependent on the severity of symptoms and the presence of other systemic manifestations but often initially involves glucocorticoids and a disease-modifying agent. This case report presents a 44-year-old female patient, previously diagnosed with BD, who presented with neurological symptoms and MRI findings consistent with NBD.