1/13. Aspergillus osteomyelitis in a child who has p67-phox-deficient chronic granulomatous disease.Here we describe Aspergillus osteomyelitis of the tibia in a 9-year-old boy who has an autosomal recessive form of chronic granulomatous disease (CGD). The patient showed a p67-phagocyte oxidase (phox) deficiency, which is rare type of CGD in japan. The initial treatment which consisted of surgical debridement and antibiotic therapy with amphotericin b (AMPH), did not control the infection. aspergillus fumigatus (A. fumigatus) pure isolated from drainage fluid and necrotic bone tissue demonstrated less susceptible to antifungal agents, including AMPH, fluconazole and flucytosine. Recombinant interferon gamma was then administrated, and it was effective in controlling the course of severe invasive aspergillosis. This report indicates the use of interferon gamma might be helpful in control for Aspergillus osteomyelitis of the tibia in a child with CGD demonstrated p67-phox deficiency refractory to conventional therapy with AMPH.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/13. 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) |
3/13. Successful unrelated bone marrow transplantation for a patient with chronic granulomatous disease and associated resistant pneumonitis and Aspergillus osteomyelitis.We describe the successful treatment of a 20-year-old patient with chronic granulomatous disease (CGD), by unrelated bone marrow transplantation (UBMT). The patient is relatively old compared to other CGD patients treated with BMT. He had had repeated serious infections from early childhood and was diagnosed as CGD, gp91-phox deficiency. Prolonged antibiotic-resistant pneumonitis worsened when the patient was 18 years old. In addition, he suffered Aspergillus osteomyelitis and acute renal failure due to amphotericin b. He received 94 granulocyte transfusions from 94 adult donors and the infections gradually improved. In September 1998, at 20 years of age, he underwent UBMT from an HLA 6 antigen-matched male donor, with CY and TBI conditioning. He received MTX and CsA as prophylaxis against GVHD. No serious complications occurred and rapid engraftment was achieved. Acute GVHD (grade 2, at day 19) and chronic GVHD (limited, at day 192) occurred. However, both were easily controlled. The patient is alive and well with no late rejection 26 months after UBMT.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
4/13. Femoral osteomyelitis due to aspergillus nidulans in a patient with chronic granulomatous disease.13 cases of osteomyelitis caused by aspergillus nidulans have been previously reported in patients with chronic granulomatous disease (CGD). All of them have been associated with simultaneous pulmonary infection and have had an extremely poor outcome. We report an unusual case of femoral osteomyelitis due to A. nidulans in a 16-year-old male with CGD, without pulmonary involvement. Treatment with liposomal amphotericin b and granulocyte colony-stimulating factor as well as extensive surgical debridement followed by prolonged treatment with itraconazole resulted in an excellent clinical response.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
5/13. Blastomycotic cranial osteomyelitis.This is the second case report of a temporal bone osteomyelitis caused by blastomyces dermatitidis, which presented as a chronic serous otitis media. The presenting serous otitis media was refractory to conventional medical and surgical management and progressed to a temporal bone osteomyelitis prior to diagnosis. B. dermatitidis is a rare fungal pathogen that causes a systemic pyogranulomatous disease that primarily manifests itself in the skin, bones, pulmonary, and genitourinary systems. If left untreated it is associated with a high rate of mortality. The otologic presentation of this rare disease is emphasized, while the clinical and therapeutic features are reviewed.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
6/13. Chronic granulomatous disease presenting with osteomyelitis: favorable response to treatment with interferon-gamma.A 3-year-old boy with chronic granulomatous disease presented with abscesses related to underlying osteomyelitis. Treatment with appropriate antibiotics resulted in resistance of the organisms. Treatment with interferon-gamma was encouraging in that it caused the osteomyelitis to heal and prevented its relapse when used as long-term prophylaxis.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
7/13. burkholderia gladioli osteomyelitis in association with chronic granulomatous disease: case report and review.We describe a case of insidious small bone osteomyelitis and soft tissue abscess with burkholderia gladioli in a 6-year-old Caucasian boy with chronic granulomatous disease. dna sequencing of the 16S ribosomal rna gene confirmed the bacterial identification. Clinical cure was achieved with a combination of antimicrobial therapy and surgical debridement. A review of infections caused by Burkholderia spp., other than burkholderia cepacia complex, in pediatric patients with chronic granulomatous disease is provided.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
8/13. serratia marcescens osteomyelitis in an infant.Neutrophil dysfunction can result from oxidative burst defect or from glucose-6-phosphate dehydrogenase (G6PD) deficiency; we noted both in the same patient. A 4-month-old male infant with G6PD deficiency presented with swelling of the left middle finger, left leg, and right big toe. At 5 weeks of age he was hospitalized for fever for 2 days. A maternal uncle died at 5 years of age and a male maternal cousin died at the age of 21 months, both reportedly diagnosed with chronic granulomatous disease (CGD). On physical examination, he had a swollen erythematous left third finger, left distal leg swelling, and right big toe abscess. None of these areas was significantly tender. WBC was 18.7 x 10(3)/mm(3) with 37% PMN and 5% bands. The x-ray films showed osteomyelitis in the left third proximal phalanx and the distal right first metatarsal. culture from the toe abscess grew serratia marcescens. His neutrophil oxidative burst was tested by the dihydrorhodamine-123 assay and was markedly suppressed, typical of CGD. The mother and maternal grandmother were found to be CGD carriers. He was treated with i.v. antibiotics for 4 weeks and was discharged on prophylactic trimethoprim, itraconazole and interferon gamma, with substantial reduction in infections. infection in this infant was unusual in its nature, in affecting multiple sites, and in its causative organism. Immune deficiency was suspected, particularly of the phagocytic component, but could not be attributed to his moderate degree of primary G6PD deficiency. Additional immunologic evaluation and the family history led to the diagnosis of X-linked CGD.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
9/13. Skeletal involvement in children who have chronic granulomatous disease.Chronic granulomatous disease is a rare disorder of the oxidative metabolism of the white blood cells that results in immunodeficiency. In a retrospective review of the records of forty-two patients who had chronic granulomatous disease, we identified thirteen patients who had a total of twenty skeletal infections. Two types of infection were noted: Type 1, which resulted from a direct spread of the infection from an adjacent focus, usually of fungus or mycobacteria, and Type 2, which resulted from hematogenous spread, usually of nocardia or more common bacteria. The thoracic vertebrae, ribs, and metatarsals were the most commonly involved bones. All four patients in whom the vertebrae were involved had a Type-1 fungal infection. paresis developed in three of these patients, and two of them died. Antibiotics alone effectively eradicated the infection in only one patient. Wide operative debridement combined with administration of antibiotics was the most successful treatment. Comprehensive preoperative imaging and several cultures are necessary to locate the infection in the bone and to identify the organism.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
10/13. Treatment of invasive aspergillosis with itraconazole in a patient with chronic granulomatous disease.An 18-year-old boy with X-linked chronic granulomatous disease (CGD) developed aspergillus fumigatus pneumonia and multifocal osteomyelitis. Treatment with amphotericin b resulted in only moderate improvement of the lesions and was accompanied by considerable toxicity. In contrast, administration of the new triazole drug itraconazole led to complete disappearance of all signs of infection. We conclude that itraconazole may be a valuable new drug for treating invasive aspergillosis in patients with CGD, although the duration of treatment remains to be established.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
| | Next -> |