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.
View Article and Find Full Text PDFA 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 Symptoms responded rapidly to antifungal therapy with amphotericin followed by itraconazole.
View Article and Find Full Text PDFEosinophilic 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.
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