1/14. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/14. Allergic bronchopulmonary aspergillosis in two patients with cystic fibrosis.Allergic bronchopulmonary aspergillosis (ABPA) is hypersensitivity to aspergillus fumigatus which manifests as episodic wheezing, usually refractory to bronchodilator therapy, with fixed and transient pulmonary infiltrates, central bronchiectasis, blood eosinophilia, elevated serum IgE level, immediate skin reactivity to an A. fumigatus antigen and precipitating antibodies to A. fumigatus. It is an unusual complication of asthma and cystic fibrosis (CF). We present two cystic fibrosis patients with ABPA treated successfully with prednisone and, in Case 1 also with itraconazole. The physician should be alert to the possibility of ABPA whenever CF patients present with the new infiltrates, high serum total IgE and other positive parameters of A. fumigatus sensitization. Treatment with systemic steroids should be started in order to prevent irreversible lung damage.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/14. Efficacy of lamivudine for the treatment of hepatitis b virus infection after liver transplantation in children.BACKGROUND: There is at present very little information about hepatitis b virus (HBV) infection in children after liver transplantation. This is the first study to assess the safety and efficacy of lamivudine in this patient population. methods: We describe three children aged 5-14 years who underwent liver transplantation for fulminant hepatitis a, hyperoxaluria, and cystic fibrosis. Despite adequate immunoprophylaxis, two of the children who were serum hepatitis B surface antigen-positive before transplantation (HBV dna-negative by hybridization) had a reactivation of the disease, and one had a de novo HBV infection, at 12-18 months after transplantation. lamivudine 3 mg/kg was administered on a compassionate-use basis for 14-36 months. RESULTS: After 1 month of therapy, HBV dna disappeared from the serum in all patients by hybridization and in two patients by polymerase chain reaction. In all three children, alanine transaminase levels normalized. One child developed lamivudine resistance after 22 months with no evidence of hepatic decompensation. Repeated liver histological studies revealed progression of hepatic fibrosis in one child. All children remained serum hepatitis B surface antigen- and hepatitis B e antigen-positive. No adverse effects of the drug were noted. CONCLUSION: lamivudine is beneficial and well tolerated in children with HBV infection after liver transplantation.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
4/14. exophiala dermatitidis pneumonia in cystic fibrosis.The chest x-ray film of a girl with cystic fibrosis (CF) showed slowly increasing mottled densities during the 6th and 7th year of her life. Pulmonary symptoms and distress proceeded fast in spite of intensive treatment with antibiotics, corticosteroids, and physiotherapy. Three different fungal organisms were repeatedly cultured from the sputum: candida albicans, aspergillus fumigatus, and exophiala dermatitidis. antibodies against C. albicans were in the normal range. Candida antigen in blood and antibodies against A. fumigatus were absent. antibodies against E. dermatitidis were detected by a recently developed indirect immunofluorescence assay. It seems most probable that E. dermatitidis was the causal agent for fungal pneumonia in this case. Under therapy with amphotericin b and flucytosine the clinical course and radiological appearance improved but definitive eradication of E. dermatitidis was only achieved after treatment with itraconazole. The isolation of this fungus from the sputum of a CF patient is reported for the first time. The significance of fungal infections in CF is discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/14. Allergic bronchopulmonary aspergillosis in a patient with cystic fibrosis: diagnostic criteria when the IgE level is less than 500 IU/mL.BACKGROUND: Recently, the cystic fibrosis Foundation developed a consensus report recommending diagnostic criteria for allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis that includes a serum IgE level greater than 500 IU/mL as the "minimal diagnostic criterion." OBJECTIVE: To describe a 7-year-old girl with ABPA whose serum IgE level increased to only 398 IU/mL. methods: Total IgE and anti-Aspergillus serologic measurements were performed using enzyme-linked immunosorbent assay and standard laboratory techniques; HLA analysis was performed; interleukin 4 receptor alpha single nucleotide polymorphisms were performed using polymerase chain reaction and dna sequencing; CD23 B cells were measured using flow cytometry; and cytokine synthesis to Aspergillus purified antigens was assessed using flow cytometry. RESULTS: A 7-year-old girl with cystic fibrosis who had mild pulmonary disease and well-controlled asthma developed pulmonary infiltrates, increased wheezing, and decreased pulmonary function. Additional studies demonstrated peripheral blood eosinophilia (eosinophil count, 1807 cells/mm3 [19%]) and an increase in IgE and IgG anti-Aspergillus serology; bronchoalveolar lavage revealed septate hyphae with 45 degrees branching subsequently identified as A fumigatus and pulmonary eosinophilia. Previous HLA typing revealed that the patient was HLA-DR2 , DRB*1501, HLA-DQ2-, a pattern associated with increased risk of ABPA. In addition, there was increased up-regulation of CD23 molecules by interleukin 4 stimulation on the patient's B cells, as observed in ABPA. The patient was treated with corticosteroids and itraconazole with resolution of symptoms and pulmonary infiltrates. CONCLUSIONS: Examination of the pulmonary inflammatory response using bronchoalveolar lavage, genetic risk with HLA-DR2 DQ2- typing, and increased interleukin 4 sensitivity are useful adjunctive studies in the diagnosis of ABPA.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/14. Clonal analysis of Inquilinus limosus isolates from six cystic fibrosis patients and specific serum antibody response.Inquilinus limosus is a novel Gram-negative bacterium of the subdivision alpha-proteobacteria recently found in the airways of patients with cystic fibrosis (CF). Here, the authors report on the clinical courses of six CF patients colonized with I. limosus. Five patients suffered from either an acute respiratory exacerbation or a progressive loss of pulmonary function, whereas one patient was in a stable clinical situation. This study focused on two aims: (i) the clonal analysis of I. limosus isolates by random amplified polymorphic dna (RAPD)-PCR, and (ii) the clarification of whether the presence of I. limosus in the respiratory tract is associated with a specific serum antibody response. serum IgG was detected by immunoblotting using I. limosus whole-cell-lysate proteins as antigens. Sera from healthy blood donors (n=10) and from CF patients colonized with pseudomonas aeruginosa (n=10) were found to be immunoblot negative. All six Inquilinus-positive patients raised serum IgG antibodies against various I. limosus antigens. Surprisingly, in one patient, a specific I. limosus serum antibody response was already detected 1 year prior to Inquilinus-positive sputum cultures. Two prominent antigens were characterized by MALDI-MS: a 23 kDa protein revealed homology to the outer membrane lipoprotein OmlA of actinobacillus pleuropneumoniae, and an 18 kDa protein to a protein-tyrosine phosphatase of burkholderia cepacia. In conclusion, detection of I. limosus is accompanied by a specific serum antibody response and may reflect the infectious/pathogenic potential of I. limosus. Moreover, IgG immunoblotting may be useful to detect early infection with I. limosus and may support the selective cultivation of this novel emerging pathogen.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
7/14. cystic fibrosis: production of high levels of uromodulin-like protein by HLA-DR blood monocytes differentiating towards a fibroblastic phenotype.We report here the spontaneous in vitro transformation of blood monocytes into fibroblasts, in a patient suffering from cystic fibrosis (CF). The blood monocytes with this capacity express HLA-DR specificity. monocytes were identified by non-specific esterase activity and by immunofluorescence using monoclonal antibodies against monocytes/macrophages antigens. Neo-fibroblasts were identified by electron microscopy and immunofluorescence using monoclonal antibodies against a cytoplasmic enzyme specifically involved in the synthesis of collagen. The secretion of collagen was evidenced using antibodies against type I collagen. Both monocytes/macrophages and neo-fibroblasts express the monocytic and the fibroblastic markers and synthesize type I collagen. This transformation observed in vitro might mimick the process of fibrosis development which takes place in vivo, particularly in pancreatic acini, lungs and intestine of patients with CF. Interestingly, the whole process in vitro is inhibited when T lymphocytes are properly stimulated by IL2. In addition, both monocytes and neo-fibroblasts secrete high quantities of uromodulin-like glycoprotein. The significance of this finding is discussed in relation to the thick mucus secretion which characterizes the disease. In addition, from a fundamental point of view, it confirmed in a large series of patients that this observation may have significant implications, since CF mutation impairs the gene coding for cAMP-regulated Cl- channel and that it has been proposed that uromodulin might be implicated in Cl- transport. Therefore the question of the relationships between uromodulin and the cAMP-regulated Cl- channel arises.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/14. vasculitis complicating cystic fibrosis.Twelve patients with skin vasculitis complicating cystic fibrosis are described. Seven of these were proven histologically and of these two had systemic vascultitis. Staining of vasculitic tissue by the avidin-biotin immunoperoxidase technique using both monoclonal and polyclonal antisera directed against haemophilus influenzae, staphylococcus aureus and pseudomonas aeruginosa did not consistently reveal any bacterial antigens in these tissues. In one patient the vasculitis appeared secondary to ranitidine. There was no evidence of autoimmune disease in any of the patients. Antineutrophil cytoplasmic antibodies were detected in the serum of 40 per cent of the patients with vasculitis complicating cystic fibrosis but in none of 61 controls with cystic fibrosis (but without vasculitis) matched for age and sex and with similar bacteriological flora of sputum.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/14. An immunohistological evaluation of pseudomonas aeruginosa pulmonary infection in two patients with cystic fibrosis.pseudomonas aeruginosa is the principal pulmonary pathogen in patients with cystic fibrosis. All attempts to date to prevent or eradicate P. aeruginosa infections in these patients have been unsuccessful. vaccination against P. aeruginosa has been proposed as a preventive strategy but it has not been adequately evaluated. The purpose of this study was to determine whether P. aeruginosa, present in the lungs of patients with cystic fibrosis, express surface antigens similar to those grown in vitro; this issue is of critical importance when choosing bacterial products as vaccine candidates. Lung sections from two patients who died of the pulmonary complications of cystic fibrosis were studied. Bacteria, both in lung sections and isolated from the lung sections and grown in vitro, reacted strongly with polyclonal and monoclonal antibodies against P. aeruginosa mucoid exopolysaccharide and outer membrane proteins F and H2; this suggested that these antigens are surface exposed in vivo. It was also found that bacteria in both lung sections were associated in situ with IgG, IgA, and C3 but not with IgM or C4.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/14. Recurrent cutaneous vasculitis in cystic fibrosis.We treated a patient with cystic fibrosis who experienced recurrent episodes of palpable purpura and arthralgias associated with exacerbations of her pulmonary disease. skin biopsy demonstrated the classic findings of leukocytoclastic vasculitis, and C3 deposits were detected in the dermal blood vessels. Chronic bacterial infection and antibiotics are possible sources of antigen involved in immune complex formation in patients with chronic lung diseases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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