1/15. Ligneous conjunctivitis in a girl with severe type I plasminogen deficiency.BACKGROUND: Ligneous conjunctivitis is a rare form of chronic recurrent pseudomembranous disease and may be associated with systemic membranous pathological changes. Recently ligneous conjunctivitis has been linked to severe type I plasminogen deficiency. We report on a patient with plasminogen deficiency and severe bilateral ligneous conjunctivitis. A new treatment approach and its outcome in this patient are described. CASE REPORT: We present the case of a 9-month-old Turkish girl with massive swelling of the eyelids and hard white pseudomembranes on both lids. The conjunctival smear was positive for streptococcus pneumoniae. The clinical diagnosis was: ligneous conjunctivitis with superinfection. Histological investigation showed fibrin as major component of the pseudomembranes. The coagulation analyses revealed decreased plasminogen activity (<5%; normal 80-120%) and decreased plasminogen antigen (<0.4 mg/dl; normal 6-25 mg/dl). The failure of surgical therapy led to the attempt at treatment with intravenous lys-plasminogen. A significant improvement of the ocular symptoms occurred; stabilization with no recurrent pseudomembranes could be achieved for 6 months after treatment. DISCUSSION: The initial amelioration of symptoms in our patient after systemic replacement therapy confirms the etiological importance of plasminogen deficiency in the development of ligneous conjunctivitis. Curative treatment of ligneous conjunctivitis is still not available. However, intravenous application of plasminogen offers new possibilities in therapy, although long-term treatment seems necessary.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/15. Pneumococcal arthritis affects performance status in patients with chronic GVHD of the skin following allogeneic bone marrow transplantation.We encountered 2 patients with pneumococcal arthritis following bone marrow transplantation (BMT). Both patients received grafts from unrelated human lymphocyte antigen (HLA)-matched donors and had suffered from chronic graft-versus-host disease (GVHD). One, a 10-year-old boy, suffered from Epstein-Barr virus-related lymphoproliferative disease (EB-LPD) and received oral 6-mercaptopurine and methotrexate to manage lymphadenopathy. Twenty-four months after BMT and 7 months after the onset of EB-LPD, pneumococcal arthritis occurred in both knee joints. The other patient, a 10-year-old girl, received multiagent immunosuppressive therapy for her chronic GVHD. At 51 months following BMT, pneumococcal arthritis occurred in her left knee joint. Chronic GVHD of the skin delayed the recovery from the arthritis in both patients. This complication is quite rare but can be very serious, in regard to the patient's performance status following BMT. Although vaccination against pneumococcus or preventive antibiotics should be administered to high-risk patients, early diagnosis and treatment may be the best strategy for pneumococcal arthritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/15. Coexistent yellow nail syndrome and selective antibody deficiency.BACKGROUND: yellow nail syndrome (YNS) is a rare, often underdiagnosed condition of unknown origin. The clinical features of the syndrome include yellow nails, chronic sinusitis, bronchiectasis, pleural effusion, and lymphoedema. Despite the frequent occurrence of upper and lower respiratory tract infections in patients with YNS, comprehensive analysis of their humoral immunity has not been previously reported. OBJECTIVE: To present the case of a patient with YNS whose recurrent upper and lower respiratory tract infections may have been caused by an underlying selective antibody deficiency that manifests as impaired IgG antibody response to polysaccharide antigens. methods: The patient underwent cultures of purulent sputum for streptococcus pneumoniae and Haemophilus influenzae, bronchial washings for H. influenzae, and nail scrapings for fungi. Her serum levels of IgG, IgA, IgM, IgG subclasses, and serum titers of IgG antitetanus toxoid, anti-H. influenzae, and anti-S. pneumoniae antibodies were measured. RESULTS: Cultures of purulent sputum were positive on multiple occasions for S. pneumoniae and H. influenzae and bronchial washings were positive for H. influenzae. Nail scrapings were consistently negative for fungi. She had no reductions in serum levels of IgG, IgA, IgM, or IgG subclasses and had normal serum titers of IgG antitetanus toxoid antibodies. However, she demonstrated impaired IgG antibody responses following immunization with Pneumovax and an H. influenza B vaccine. CONCLUSIONS: This case report describes the first comprehensive analysis of humoral immune function in a patient with YNS. The finding of a selective antibody deficiency in our patient provides a potential explanation for the occurrence of respiratory infections in YNS. Accordingly, we recommend that functional antibody determinations and quantitative serum immunoglobulins be evaluated in patients diagnosed as having this unusual, enigmatic syndrome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/15. Humoral immune response to pneumococcal antigen 23-F in an asplenic patient with recurrent fulminant pneumococcaemia.Host defence mechanisms were analysed in a patient with three episodes of fulminant pneumococcaemia and one episode of bacteraemic epiglottitis with haemophilus influenzae type b. The first episode took place 11 years after splenectomy for blunt abdominal trauma. Investigations revealed several host defence mechanisms to be impaired. In addition to the patient's asplenia, an inherited C2-deficiency was noted. Assessment of IgG subclasses repeatedly revealed markedly low IgG4 concentrations. These were not due to an increased turnover of IgG4, as could be shown following infusion of intravenous IgG. In addition, IgG2 concentrations were low in the patient who lacked G2M(23). Opsonic mediating antibodies against type 23-F pneumococci were in the range of those of non-immune volunteers 6 months after vaccination with a 23-valent pneumococcal vaccine. These antibodies did not increase after a septic episode with 23-F capsular-type pneumococci. Neutrophil function was apparently normal.- - - - - - - - - - ranking = 4keywords = antigen (Clic here for more details about this article) |
5/15. Combined IgG2, IgG4 and iga deficiency: low C1q concentrations and the presence of excess C1r and C1s in an adult patient with recurrent pneumococcal infections.The complement (C) profile was investigated in an adult patient with combined IgG2, IgG4 and iga deficiency and recurrent pneumococcal infections. The analysis revealed no gross impairment of the classic and alternative pathways of C activation. However, the concentrations of circulating C1q were persistently decreased, and the sera contained an excess of C1r-C1s complexes, resembling the C1 aberrations previously found in children with recurrent acute otitis media. The concentrations of C4 in the patient were persistently low. This could be ascribed to partial C4 deficiency with lack of C4A variants. The patient's IgG and IgM antibody responses to pneumococcal capsular polysaccharides and to other bacterial carbohydrate antigens were very poor. Interestingly, pneumococcal C-polysaccharide (CPS) could be detected in serum obtained during infection-free periods. Since CPS has been shown to bind C1q without causing C1 activation, the possibility was considered that the C1 aberrations in serum were due to circulating CPS. After administration of intramuscular gammaglobulin to the patient, the serum C1q levels were observed to return to normal.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/15. C2 deficiency, moderately low IgG2 concentrations and lack of the G2m(23) allotype marker in a child with repeated bacterial infections.C2 deficiency was demonstrated in an 11-year-old boy with a past history of recurrent purulent otitis media, pneumonia, H. influenzae meningitis and S. pneumoniae septicaemia. The major histocompatibility complex haplotypes present, A10, B18, DR2, BF*S, C2*QO, C4*A4, C4*B2 and A28, B18, DR2, BF*S, C2*Q0, C4*A4, C4*B2, were in accord with previous observations in C2 deficiency. The concentrations of C1q, C5, factor B and factor D were in the low normal range and the hemolytic activity of the alternative pathway was slightly decreased. In addition, the patient showed moderately low IgG2 concentrations and lacked the IgG2 subclass marker G2m(23). The findings indicate that the patient's susceptibility to bacterial infections may be due to C2 deficiency in combination with the presence of an IgG allotype associated with impaired antibody responses to carbohydrate antigens.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/15. purpura fulminans in pneumococcal sepsis.Two cases of pneumococcal sepsis in splenectomized patients were complicated by purpura fulminans. In addition, acute renal failure developed in both patients, and myolysis in one. Immunological findings in the patient with myolysis suggest a possible role of pneumococcal antigen-containing circulating immune complexes in the pathogenesis of these complications.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/15. Antigenemia in fulminant pneumococcemia.Two asplenic patients with fulminant pneumococcemia developed purpura and coagulopathy. Levels of C3 and C4 in the serum were low. Both patients had high levels of circulating capsular polysaccharide, and one patient had visible diplococci on a smear of the peripheral blood. Pneumococcal group C polysaccharide (C-substance) was detected in the serum of one of the patients. Possible pathogenetic relation of circulating pneumococcal antigens to the development of coagulopathy in pneumococcemia is discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/15. Avoidance of false-negative blood culture results by rapid detection of pneumococcal antigen.False-negative blood culture results may occur in children with pneumococcal bacteremia due to bacterial autolysis. We describe four patients with pneumococcal bacteremia whose aerobic blood cultures showed partial or complete autolysis of the pneumococci. Pneumococcal antigen, however, was rapidly detected in media from the blood culture bottles, using an agglutination assay. Processing of the media before analysis was necessary to prevent nonspecific agglutination and to allow the detection of a specific reaction. It is important that physicians and laboratory personnel be aware that pneumococci may rapidly autolyze during incubation, yielding false-negative culture results. Antigen detection methods may provide rapid and specific identification of the etiologic agent.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
10/15. Rapid diagnosis of septic arthritis by coagglutination.Pneumococcal capsular antigens were detected and serotyped by coagglutination in joint fluids and serum of a patient with septic arthritis within 1 h of obtaining the specimens. Pneumococcal antigens continued to be detected by coagglutination for 3 days, whereas cultures and Gram stains were negative after 1 day of antibiotic therapy.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
| | Next -> |