Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 17-year-old man was admitted to our hospital because of exertional dyspnea, fever, and hemoptysis. Chest X-ray films disclosed diffuse alveolar infiltrates and patchy shadows in both lungs. Laboratory data included a hemoglobin level of 6.7 g/dl and white blood cell count of 8,100/microliter. Urinalysis revealed microscopic hematuria with RBC cast. Bronchoalveolar lavage fluid from the right middle lobe bronchus was bloody. Anti-GBM antibodies were detected at low levels in serum (10 EU/ml) by ELISA procedures. Renal and lung biopsies were performed. Immunofluorescent studies revealed linear deposits of IgG along glomerular basement membrane of the kidney, but not in alveolar walls of the lung. This case fulfilled the criteria for Goodpasture's syndrome. The patient was treated with methylprednisolone (1,000 mg/day, for 3 days) and plasma exchange (for 2 days), and demonstrated a dramatic improvement in his clinical condition and chest X-ray findings. We were unable to identify autoantibodies to the NC domain of the alpha 3 chain of type IV collagen. Another conformational epitope may play a role in the disease.
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PMID:[Goodpasture's syndrome initially presenting with alveolar hemorrhage]. 1049 7

Goodpasture's disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems. We present a case of particular interest in regards to atypical presentation, and the uncommon treatment that the patient underwent. A 65-year-old Afghani female arrived with complaints of nausea, vomiting, loss of appetite, malaise, decreased urine output, exertional dyspnea, and cough. The patient presented initially with renal failure and unexpectedly developed respiratory failure after hemodialysis. Initial CT of thorax revealed diffuse bilateral pulmonary edema. Subsequently, the patient received a bronchoscopy demonstrating alveolar hemorrhage, which highlights a clinician's need to maintain a differential and reassess patients. Anti-GBM antibody in the serum was detected and the renal biopsy revealed evidence of the antibody on immunofluorescence. In regards to management, the patient could only be treated with plasmapheresis as she had contraindication to initiation of immunosuppression, after which she showed significant clinical improvement. We would like to highlight the benefit of plasmapheresis without concomitant immunosuppression and recommend such an approach to be considered in similar clinical scenarios, where contraindication for immunosuppressant therapy exists.
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PMID:Goodpasture's Disease: An Uncommon Disease With an Atypical Clinical Course. 2666 84