1/55. sarcoidosis with selective involvement of a second liver allograft: report of a case and review of the literature.A case of sarcoidosis recurrent in a patient's second liver allograft is described. There was no granulomatous disease seen in the patient's first liver allograft. After the second orthotopic liver transplantation (OLT), the patient was successfully treated for acute rejection, aspergillus infection, and cytomegalovirus viremia. Approximately 2 months after the second OLT, the patient was treated with long-term interferon-alpha for recurrent hepatitis c. Five years after the operation, he experienced liver failure secondary to recurrent hepatitis and underwent a third OLT. This is only the second reported case of sarcoidosis recurrent in the liver parenchyma of a transplanted organ and the first in which interferon-alpha might have played a role.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/55. 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 = 5keywords = granulomatous disease (Clic here for more details about this article) |
3/55. 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 = 5keywords = granulomatous disease (Clic here for more details about this article) |
4/55. Sensitization to Aspergillus species in the congenital neutrophil disorders chronic granulomatous disease and hyper-IgE syndrome.BACKGROUND: Hyper-IgE syndrome (HIE) and chronic granulomatous disease (CGD) are congenital immunodeficiency diseases with increased susceptibility to bacterial and fungal infections. Both carry significant morbidity and mortality rates because of invasive infections by Aspergillus species. We encountered 2 patients, one with HIE and one with CGD, in whom detection of sensitization to Aspergillus species preceded the diagnosis of immunodeficiency. With high-dose systemic corticosteroids for allergic bronchopulmonary aspergillosis (ABPA), an inflammatory disorder caused by sensitization to Aspergillus species, pulmonary abscesses developed in the patient with HIE, and the patient with CGD succumbed to an overwhelming Aspergillus species-induced pneumonia. OBJECTIVE: We sought to assess the prevalence of sensitization to aspergillus fumigatus and the presence of diagnostic criteria for ABPA in patients with CGD and HIE. methods: We measured A fumigatus-specific serum IgE, IgG, and precipitating antibodies as indicators for A fumigatus sensitization in the sera of 18 patients with neutrophil disorders (7 with HIE and 11 with CGD). hospital records were reviewed for the presence of other diagnostic criteria for ABPA (asthma, elevated total serum IgE concentration, and radiographic abnormalities). RESULTS: Twelve (67%) of 18 patients were sensitized to A fumigatus, as evidenced by precipitating A fumigatus-specific antibodies. Six (33%) of 18 patients had serologic evidence of ABPA. Five of those 6 patients had radiologic abnormalities consistent with a diagnosis of ABPA. One patient with HIE also had asthma, thus fulfilling minimal essential criteria for concurrent ABPA. CONCLUSIONS: patients with HIE syndrome and CGD have a high incidence of sensitization to Aspergillus species. A clinical picture indistinguishable from ABPA may coexist or emerge in patients with CGD or HIE and create a major management dilemma because systemic corticosteroids may accelerate tissue damage and invasive fungal infections. It is important to distinguish individuals with congenital neutrophil disorders from uncomplicated classic ABPA.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
5/55. 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 = 5keywords = granulomatous disease (Clic here for more details about this article) |
6/55. 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 = 5keywords = granulomatous disease (Clic here for more details about this article) |
7/55. Increased susceptibility of a carrier of X-linked chronic granulomatous disease (CGD) to aspergillus fumigatus infection associated with age-related skewing of lyonization.Chronic granulomatous disease (CGD) is an inherited disorder characterized by the inability of phagocytes to generate normal amounts of superoxide (O2-), leaving patients susceptible to life-threatening infections. It was previously assumed that once carriers of the X-linked form of CGD were found to have 30% or more of functionally normal neutrophils, they would be free of risk for infection because the lyonization ratio was believed to be constant. Our report strongly contradicts this assumption. A 45-year-old X-CGD carrier had approximately 40% of normal neutrophils in her peripheral blood at age 21 years. Recently, she contracted a life-threatening pulmonary infection with aspergillus fumigatus. After recovery, the ratio of normal-to-nonfunctional neutrophils was re-evaluated. She was found to have only 6-8% of normal neutrophils, suggesting that a striking decrease in the number of normal cells over the past 25 years was the reason for an increased susceptibility to Aspergillus infection. We conclude that age-related acquired skewing of the lyonization ratio can result in an increased susceptibility to life-threatening infections in X-CGD carriers.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
8/55. Successful treatment of aspergillus brain abscess with itraconazole and interferon-gamma in a patient with chronic granulomatous disease.This report describes the successful treatment of Aspergillus brain abscess in a boy with chronic granulomatous disease.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
9/55. Aspergillus spinal epidural abscess.Spinal abscess due to Aspergillus is rare. A young boy with chronic granulomatous disease and aspergillosis of the rib had been treated with antifungal treatment 3 months earlier. The patient presented with a brief history of progressive paraparesis. Imaging showed D9--11 vertebral involvement and destruction of the D10 vertebral body with angulation and a large dorsally placed, multiloculated epidural abscess extending from D6 to L2. There was also extensive granulation anterior to and on either side of the vertebrae. The patient underwent extensive laminectomy and decompression of all the loculi and partial removal of the granulation tissue. Aggressive medical treatment was started. The authors recommend an aggressive surgical and medical approach in such cases of disseminated invasive aspergillosis, even though the result may not be very satisfactory. This report discusses the full clinical profile and management of Aspergillus spinal epidural abscess and emphasizes the need to follow up these cases to detect recurrence and new lesions, even if the patients are on adequate medical treatment. In spite of all efforts, high morbidity and mortality is common in such patients.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
10/55. 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 = 5keywords = granulomatous disease (Clic here for more details about this article) |
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