1/33. Successful treatment with methylprednisolone pulse therapy for a life-threatening pulmonary insufficiency in a patient with chronic granulomatous disease following pulmonary invasive aspergillosis and burkholderia cepacia infection.A 14-year-old boy with X-linked chronic granulomatous disease developed severe invasive pulmonary aspergillosis. He was treated with itraconazole and amphotericin b. However, he deteriorated with progressive pulmonary lesions. burkholderia cepacia was isolated from his bronchoalveolar lavage. Finally, he was given granulocyte transfusions. Following this procedure, his condition rapidly worsened leading to respiratory failure. His lung biopsy demonstrated organizing pneumonia at his right middle lobe. Then, a methylprednisolone pulse therapy was initiated together with the administration of appropriate antibiotics and adequate amounts of amphotericin b. Dramatically, his condition improved. Therefore, a methylprednisolone pulse therapy with appropriate antimicrobial drugs seems to be beneficial for severe pulmonary insufficiency in this type of patients. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/33. Failure to detect circulating Aspergillus markers in a patient with chronic granulomatous disease and invasive aspergillosis.We report a patient with chronic granulomatous disease who developed invasive pulmonary aspergillosis and a subphrenic abscess. During treatment, high levels of Aspergillus antigen were detected in the abscess, but circulating antigen and Aspergillus dna were undetectable in the serum.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
3/33. Successful treatment of invasive aspergillosis in chronic granulomatous disease by granulocyte transfusions followed by peripheral blood stem cell transplantation.Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder characterized by impaired microbial killing and susceptibility to bacterial and fungal infections. Cure of the disease can be achieved by stem cell transplantation when performed early in its course, and before severe infections have developed. Invasive aspergillosis constitutes a very high risk for transplantation. We report a 4-year-old boy with X-linked CGD who underwent successful HLA-identical peripheral blood stem cell (PBSC) transplantation during invasive pulmonary aspergillosis and osteomyelitis of the left fourth rib, which was unresponsive to antifungal treatment. During the 2 months prior to the transplant he received G-CSF-mobilized granulocyte transfusions (GTX) from unrelated donors three times a week in addition to the antifungal treatment. This resulted in clinical improvement in his respiratory status. He also received GTX during the aplastic period after the conditioning regimen, until he had engrafted. Post-transplant superoxide generation test revealed that neutrophil function was within normal range. One year post transplant the CT scan showed almost complete clearance of the pulmonary infiltrates and a marked improvement in the osteomyelitic process. Based on other reports and our own experience, GTX can serve as important treatment in patients with CGD who have failed conventional anti-fungal treatment and for whom stem cell transplantation is the only chance for cure.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
4/33. Capofungin therapy for Aspergillus lung infection in a boy with chronic granulomatous disease.neutrophils of patients with chronic granulomatous disease (CGD) are profoundly defective in killing microorganisms. As in patients with chemotherapy-induced neutropenia, CGD patients are highly susceptible to life-threatening invasive Aspergillus infections, and conventional antimycotic treatment is not always successful. The new drug, caspofungin, was used to treat Aspergillus lung infection in one child with CGD.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
5/33. Therapeutic effect of interferon-gamma for prevention of severe infection in X-linked chronic granulomatous disease.Chronic granulomatous disease (CGD) is an inherited phagocytic disorder caused by defective oxidative burst activity of neutrophils and monocytes. patients with CGD may present with recurrent, life-threatening bacterial or fungal infections and often need repeated hospitalization as early as infancy. We report a case of a boy aged 3 years and 1 month with a history of oral thrush, chronic diarrhea, skin abscesses, multiple small joints osteomyelitis, and multiple liver abscesses since 2 months of age. X-linked chronic granulomatous disease was diagnosed by nitroblue tetrazolium test and further confirmed by genetic study using single strand conformation polymorphism (SSCP) analysis and genomic dna sequencing. Two episodes of aspergillosis with severe aspergillus lung empyema and life-threatening klebsiella pneumoniae infections were noted in the first 2 years of life despite long-term prophylaxis with trimethoprim-sulfamethoxazole and itraconazole. Recombinant human interferon-gamma (rINF-gamma) 50 microg/m(2) subcutaneous injection 3 times per week was added after his last episode of severe infection. Thereafter, the boy had normal growth and development with no evidence of severe infection during 18 months of follow-up at our outpatient clinic.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
6/33. invasive pulmonary aspergillosis soon after therapy with infliximab, a tumor necrosis factor-alpha-neutralizing antibody: a possible healthcare-associated case?BACKGROUND: Infliximab is a chimeric monoclonal antibody against tumor necrosis factor (TNF)-alpha, used for the treatment of Crohn's disease and rheumatoid arthritis. Recently, an increased risk of infection due to mycobacterium tuberculosis and rare cases of invasive fungal disease have been reported following infliximab therapy. CASE REPORT: A 73-year-old woman with chronic rheumatoid arthritis who had been treated with methotrexate, leflunomide, and prednisone was given the first of three doses of infliximab in June 2001. In July 2001, she presented with cough, and in August, she had a right upper lobe infiltrate that was treated with levofloxacin without improvement. In October, the patient had right upper and middle lobe infiltrates on a chest X-ray and computed tomography scan. At bronchoscopy, an endobronchial mass was biopsied, which demonstrated Aspergills fumigatus. Our patient had frequently accompanied her daughter on visits to another medical center following a stem cell transplant, where her daughter was instructed to wear a mask during all visits because of extensive building construction. We postulate that our patient may have acquired pulmonary aspergillosis during this period. literature reviews on granulomatous diseases following infliximab therapy and hospital-acquired aspergillosis are presented. CONCLUSION: The temporal relationship between the administration of infliximab and A. fumigatus infection in this patient suggests a causal relationship and possible healthcare-associated acquisition. These data underscore the importance of both patient and family education on prevention strategies when potent immune-modulating medications such as infliximab have been prescribed.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
7/33. Pulmonary aspergillosis in a patient with chronic granulomatous disease: confirmation by polymerase chain reaction and serological tests, and successful treatment with voriconazole.A 25-year-old male with chronic granulomatous disease developed severe bilateral pulmonary aspergillosis confirmed by a positive result of polymerase chain reaction for Aspergillus species from sputum and positive serological tests for Aspergillus. He was successfully treated with voriconazole. The diagnostic difficulties and the new therapeutic options in the era of new antifungal agents are discussed.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
8/33. Successful elimination of an invasive aspergillus nidulans lung infection by voriconazole after failure of a combination of caspofungin and liposomal amphotericin b in a boy with chronic granulomatous disease.Chronic granulomatous disease is an inherited defect in host defense mechanisms mainly affecting neutrophil function. Pulmonary infection with aspergillus nidulans in a child with chronic granulomatous disease could not be eliminated by a combination of caspofungin and liposomal amphotericin b. Voriconazole was successful in clearing the infection.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
9/33. Microgranulomatous aspergillosis after shoveling wood chips: report of a fatal outcome in a patient with chronic granulomatous disease.Chronic granulomatous disease is characterized by recurrent infections that result from an inability of phagocytes to kill organisms effectively. We describe a patient with this disease who developed aspergillus pneumonia after shoveling moldy cedar wood chips. Despite aggressive therapy, the patient's condition deteriorated and he died. At autopsy, the lungs revealed diffuse granulomas, all of the same age, with aspergillus organisms confined to the granulomas. We propose the term "microgranulomatous aspergillosis" for this response, which does not conform to the commonly described aspergillus syndromes. We conclude that susceptible immunosuppressed patients should be advised to avoid occupational situations where high spore concentrations are generated.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
10/33. Successful treatment of systemic exophiala dermatitidis infection in a patient with chronic granulomatous disease.exophiala dermatitidis, one of the saprophytic dematiaceous fungi, is a rare cause of human infection that, when invasive, is nearly always fatal. Besides the more common subcutaneous infection usually caused by traumatic inoculation, infection can also spread hematogenously, in which case the organism has a distinct neurotropism. A patient with autosomal recessive chronic granulomatous disease of childhood who was found to have a progressive pulmonary and central nervous system infection with E. dermatitidis responded to an aggressive, multifaceted therapeutic approach. Scanning electron microscopy of the cultured conidiogenous cells confirmed that the manner of conidiogenesis is typical of the genus exophiala. We report the first successful treatment of an infection involving the lungs and central nervous system by a combination of surgical resection of the pulmonary source and medical therapy with amphotericin b, flucytosine or ketoconazole, and transfused white cells, followed by a prolonged course of fluconazole.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
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