Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 74-year-old man presented sudden onset hoarseness and dysphagia. Two months before this event, he had developed arthralgia of the shoulders, elbows, hands and foot and pleuritis which had been alleviated by a treatment with prednisolone. On admission, the patient could not phonate nor swallow at all. His soft palate was elevated at the right side. The uvula moved left when the patient tried to speak. Laryngoscopic examination revealed the paralysis of right vocal cord. The erythrocyte sedimentation rate (79mm/1h), C-reactive protein (5.3mg/dl), rheumatoid factor (310 IU/ml) and Clq-binding immune complex (4.5 micrograms/ml) were elevated. Hepatitis C virus antibody titer was more than 10.8 IU/l. Anti-nuclear antibody was 1:20 (normal < 1:20) and anti-neutrophil cytoplasmic antibody (p-ANCA) was positive. Blood study also revealed the evidences of hemolytic anemia and hypoproteinemia. Hepatitis B virus markers, cryoglobulin, anti-ds DNA, anti-Sm, anti-RNP, anti-SS-A, anti-SS-B antibodies were negative. Magnetic resonance imaging of the brainstem was normal. A sural nerve biopsy revealed patchy demyelination of the fascicles. The teasing of nerve fibers showed segmental demyelination. Chest X-ray showed the interstitial pneumonia and pleuritis in the right lower lung. Otological examination revealed the bilateral secretory otitis media. A treatment with high dose prednisolone, ciclosporin and cyclophosphamide was partially effective. However we could not continue these medication because of the induction of liver damage. The patient died of multi-organ failure around a year after the emergence of aphonia and dysphagia. The autopsy specimen of the right vagus nerve showed the similar patchy damage of nerve fibers as was observed in the biopsied sural nerve. The present case was diagnosed as systemic rheumatoid vasculitis. The syndrome of aphonia and dysphagia due to paralysis of the unilateral soft palate and vocal cord is called Avellis syndrome. This syndrome has been reported mainly in relation with the infarction of lateral medulla. The present case shows that Avellis syndrome can be produced by mononeuritis of the vagus nerve.
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PMID:[Avellis syndrome in systemic rheumatoid vasculitis]. 882 3

Here we report a case of microscopic polyangitis (MPA) with anti-neutrophil cytoplasmic antibodies against myeloperoxidase (MPO-ANCA) accompanied by perceptive dominant hearing loss as the initial sign of the disease. A 52-year-old woman was admitted to Tokyo Metropolitan Ohtsuka Hospital in April 1996 because of bilateral progressive hearing loss. On admission, she had presented bilateral perceptive hearing loss, fever, myalgia, and weight loss. Laboratory data showed accelerated erythrocyte sedimentation rate, proteinuria, microscopic hematuria, leukocytosis, hypoproteinemia, and elevated creatinine level. Serum level of MPO-ANCA was elevated. Chest X-ray and computed tomography showed mild pleural effusion and interstitial shadow in bilateral lung fields. The biopsy findings detected that focal glomerulonephritis, and necrotizing angitis of small artery in kidney. The diagnosis of MPA was made with clinical course, laboratory findings, radiographic findings, and biopsy finding. Treatment was initiated with 45 mg of prednisolone, followed by marked improvement of hearing loss and decreased titer of serum MPO-ANCA. There are only seven reports showing hearing loss as the initial sign of the disease occurred in polyarteritis nodosa and MPA. The present case suggests the possibility that hearing loss may be the symptom of MPO-ANCA associated vasculitis.
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PMID:[A case of microscopic polyangitis accompanied by hearing loss as the initial sign of the disease]. 949 68

De novo systemic vasculitis after renal transplant is a rare complication. We report a patient who developed rapid, catastrophic necrotizing vasculitis of the gastrointestinal tract 11 months after renal transplant. A 60-year-old man was admitted for persistent pain in the right abdomen and mild intestinal hemorrhage. After some days the patient presented partial intestinal occlusion, severe hypoproteinemia and acute renal insufficiency. The patient was urgently operated with resection of a tract of the jejunum where there was a venous infarct. Laboratory tests were not significant and the search for hepatitis B, C viruses and ANCA was negative. After some days of irrepressible intestinal hemorrhage, total gastrectomy, splenectomy and resection of the duodenum and pancreas were performed. Histological pictures showed vascular lesions pathognomonic of systemic polyarteritis nodosa (PAN). After thirty days the patient died. The autopsy confirmed atypical systemic PAN with involvement of the pulmonary arteries besides the gastrointestinal tract and pancreas. Gastrointestinal (GI) complications are not frequent in renal transplant recipients, but 30% of patients with such lesions die as a direct consequence of visceral vascular damage. To the best of our knowledge, de novo PAN has been reported as the cause of catastrophic gastrointestinal involvement in only one renal transplant recipient. This case therefore raises the number of reports of PAN in kidney transplantation recipients who had no history of underlying connective tissue disease.
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PMID:Catastrophic visceral involvement secondary to de novo systemic vasculitis in a renal transplant recipient. 1201 38