Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149520 (acute cholecystitis)
 2,784 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of paroxysmal nocturnal hemoglobinuria (PNH) and review the literature regarding transfusion of red blood cells in PNH patients. A 42-year-old woman with myelodysplastic syndrome (MDS) complaining of right hypochondralgia and high fever was admitted to the hospital for acute cholecystitis with gall stone. Blood examination revealed bicytopenia (leukocytes, 1,700/microliters and hemoglobin, 8.5 g/dl) and bone marrow examination showed normocellular but hypererythroid bone marrow and dyshematopoiesis, which suggested MDS. Laboratory data revealed obstructive jaundice and hemolytic anemia. Positive sucrose and Ham tests, which were compatible with the diagnosis of PNH. Cholecystectomy was successful, and the patient showed no postoperative complications of increased hemolysis or thrombosis. As hemoglobin level gradually decreased for the first two postoperative weeks, filtrated white cell-depleted red blood cells (total, 1,000 ml) were transfused instead of washed red blood cells. No side effects of the transfusion were noted. On the basis of findings in this case and those reported in the literature, it is concluded that in some case of PNH, the use of washed red blood cells is unnecessary, and that the use of white cell-depleted red blood cells is indicated.
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PMID:[Successful cholecystectomy in a patient with paroxysmal nocturnal hemoglobinuria]. 175 63

Paroxysmal nocturnal hemoglobinuria (PNH) makes patients susceptible to intravascular hemolysis and thrombosis, and it can be life-threatening in stressful situations. Eculizumab, a humanized monoclonal antibody that inhibits the complement protein C5, has been evaluated as a novel therapy for PNH. We herein describe the case of a 59-year-old Japanese woman with classic PNH, who had been successfully treated with eculizumab, but who later developed acute cholecystitis/cholangitis from gallstones. Although the severe obstructive jaundice requiring endoscopic therapy following cholecystectomy was complicated, critical intravascular hemolysis and thrombosis were not observed. Therefore, utilizing eculizumab during the peri-operative management of PNH patients should be carefully taken into consideration.
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PMID:Successful management of obstructive jaundice due to gallstones with eculizumab in a patient with paroxysmal nocturnal hemoglobinuria. 2298 36

Invasive candidiasis remains an important cause of mortality and morbidity in patients with underlying diseases. Here, we report a case of gastric perforation due to Candia glabrata infection in a 74-year-old-male with Paroxysmal nocturnal hemoglobinuria (PNH) who received long-term corticosteroid treatment of hemophagocytic syndrome associated with acute cholecystitis. Total gastrectomy was performed, and he was treated liposomal amphotericin B. The patient was extubated successfully on the 2nd postoperative day, but the patient died of Pneumocystis jirovecii pneumonia (PJP). An autopsy revealed that there was a small amount of the cystic form of Pneumocystic jirovecii, but there was not the presence of Candida spp. Concerning the prophylaxis of invasive candidiasis, there is no strong evidence-based data in clinical practice in immunocompromised patients, such as those receiving long-term immunomodulatory therapy or corticosteroids. Our present case suggests the importance of fungal management and may indicate the need for a new approach to the fungal prophylaxis in such patients.
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PMID:Invasive candidiasis leading to gastric perforation in an immunocompromised patient. 3151 28