RESUMEN
The aganglionic segment of bowel in Hirschsprung's disease (HD) varies in length. It is not clear whether total colonic aganglionosis (TCA) merely represents a long form of HD or a different phenotype of the disease. Animal model studies suggest that TCA may have a longer transition zone (TZ) than conventional colorectal HD. We compared mucosal innervation of TZ in 2 TCA cases and 10 conventional colorectal HD cases by quantifying calretinin-positive mucosal nerve fibers using image processing and analysis. One TCA was associated with esophageal atresia-tracheoesophageal fistula, the other with trisomy 21. The gradients of calretinin-stained pixel count increase per distance from the beginning of TZ (slope) for TCA were not significantly different from those for the conventional HD group. Given this observation, it is speculated that the length of TZ in TCA may fall within the range of and may not be much longer than conventional colorectal HD.
Asunto(s)
Calbindina 2/metabolismo , Neoplasias Colorrectales/patología , Enfermedad de Hirschsprung/patología , Fístula Traqueoesofágica/patología , Adolescente , Animales , Niño , Colon/inervación , Colon/metabolismo , Colon/patología , Neoplasias Colorrectales/metabolismo , Modelos Animales de Enfermedad , Femenino , Enfermedad de Hirschsprung/metabolismo , Humanos , Íleon/inervación , Íleon/metabolismo , Íleon/patología , Procesamiento de Imagen Asistido por Computador , Lactante , Estudios Longitudinales , Masculino , Fibras Nerviosas/patología , Fístula Traqueoesofágica/metabolismoRESUMEN
A 43-year-old man with history of non-Hodgkins' lymphoma presented with unilateral eye swelling, pain and vision deficits which had been progressive over 2 months. Symptoms followed a presumed bacterial pneumonia 4 months prior. Imaging demonstrated retro-orbital soft tissue swelling with bony erosion concerning for a mass; surgical decompression was performed with histology confirming disseminated Blastomyces dermatitidis Symptoms responded rapidly to antifungal therapy with amphotericin followed by itraconazole. Orbital dissemination of blastomycosis is extremely rare; accurate diagnosis requires tissue biopsy to facilitate timely targeted therapy and minimise morbidity.
Asunto(s)
Blastomicosis/diagnóstico por imagen , Enfermedades Pulmonares Fúngicas/diagnóstico por imagen , Celulitis Orbitaria/diagnóstico por imagen , Adulto , Antifúngicos/uso terapéutico , Blastomyces , Blastomicosis/terapia , Descompresión Quirúrgica , Dolor Ocular/etiología , Humanos , Masculino , Nervio Óptico , Órbita/cirugía , Celulitis Orbitaria/terapia , Muslo , Tomografía Computarizada por Rayos X , Trastornos de la Visión/etiologíaRESUMEN
Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem vasculitic disorder that predominantly affects medium- and small-sized blood vessels. EGPA belongs to a group of vasculitides known as anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV). Upper airway involvement is seen in all ANCA-associated vasculitides, but destructive upper airway disease has never been reported in patients with EGPA. We report the first case of erosive chondritis and saddle nose deformity in a 50-year-old patient suffering from EGPA.