A 6-year-old Hispanic boy with 3 days of hemoptysis presented with hemoglobin 4.5 g/dL. He had 3 months of iron deficiency anemia unresponsive to iron supplementation, with no gastrointestinal symptoms. Chest x-ray demonstrated right lung infiltrates with small pleural effusion (Fig. 1A). Computed tomography of the chest revealed ground glass appearance of the entire right lung and left lower lobe (Fig. 1B). Bronchoalveolar lavage showed 60% hemosiderin-laden macrophages that along with the radiologic findings is diagnostic for idiopathic pulmonary hemosiderosis (1). Severe anemia was disproportionate to hemoptysis. Searching for other causes of anemia, we found elevated tissue transglutaminase (128 U/mL) and positive antiendomysial immunoglobulin A antibody. Esophagogastroduodenoscopy demonstrated edema and minimal scalloping of duodenum. Histology confirmed flattened villi with erosion and increased intraepithelial lymphocytes (Fig. 1C and D), consistent with celiac disease. After initiating a gluten-free diet, pulmonary symptoms resolved and both followup imaging and tissue transglutaminase normalized.The association of celiac disease and idiopathic pulmonary hemosiderosis has been defined as the Lane-Hamilton syndrome. Both the disorders are believed to be immunologically mediated (1,2). Because of the good prognosis with a gluten-free diet, hemoptysis associated with pulmonary hemosiderosis in the setting of disproportionately severe anemia should prompt the evaluation for celiac disease (3-5).
rare and include worsening of demyelinating diseases, and neuropathies, often commencing months following treatment.to date. METHODS: We report the case of a 7 year old boy who was diagnosed with ulcerative colitis and primary sclerosing cholangitis at 6 and half years of age, who continued to have mild disease, manifested primarily by ongoing hematochezia and anemia despite Balsalazide and 6-Mercaptopurine therapy. His course to this point was unremarkable and his disease severity was mild with a Pediatric Ulcerative Colitis Score of 30. He was admitted for blood transfusion and underwent endoscopy and colonoscopy. He then received his first infusion of Infliximab. Other than mild sinus arrhythmia, he tolerated this well. There were no other notable events surrounding the infusion, he was interacting appropriately with normal vital signs and was discharged home. RESULTS: Within 6 hours of his infusion, he awoke from sleep from a severe headache and developed acute nausea and emesis. Half an hour later, he was slumped to one side and parents brought to a local emergency room. Upon arrival, he was completely unresponsive and emergently intubated. Given slight hypotension, he was suspected to have an anaphylactic reaction and received intravenous fluids, packed red blood cells and Epinephrine. Once transferred to our pediatric center, CT image of his brain revealed multifocal bilateral hypodense lesions with surrounding edema involving multiple lobes, the pons, and brainstem. Left sided lesions were largest and consistent with parenchymal hemorrhage. He was started on hypertonic saline and mannitol and external ventricular drains were promptly inserted, but his initial intracranial pressures were low. MRI imaging confirmed bilateral multi-territory infarcts that appeared to be arterial in nature without evidence of arterial clots, or venous infarcts. A workup for infectious encephalitis was negative, as was investigation for underlying anatomic anomalies of his vasculature. His presentation was not in keeping with an underlying vasculitis. An echocardiogram showed a dilated left ventricle, but did not show evidence of an arterial-septal defect, intracardiac thrombi or other abnormality. CONCLUSIONS: Our patient met criteria for brain death within 5 days following his single dose of Infliximab. Family refused an autopsy. We report a case of acute hemorrhagic stroke and ultimately fatal outcome following an initial infusion of Infliximab. Although the etiology of this event remains unclear, the close temporal relation to the Infliximab infusion cannot be ignored. To our knowledge, such a life threatening reaction within hours of Infliximab infusion has not been reported.
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