1/90. The periodontal management of a patient with a profound immunodeficiency disorder.A case of severe, uncontrolled periodontal disease in a patient with a profound immunodeficiency disorder is reported. The periodontal disease was noted before the age of 10 years and is generalized, with marked associated alveolar bone loss, even threatening a mandibular fracture. Severely involved teeth were allowed to exfoliate, and extractions were avoided. At the age of 17 years, the patient now has only nine remaining teeth. The management adopted and alternative approaches are critically reviewed. The implications of the dental findings for current concepts of the pathogenesis of periodontal disease and dental caries are discussed.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
2/90. Human neutrophil immunodeficiency syndrome is associated with an inhibitory Rac2 mutation.A 5-week-old male infant presented with severe bacterial infections and poor wound healing, suggesting a neutrophil defect. neutrophils from this patient exhibited decreased chemotaxis, polarization, azurophilic granule secretion, and superoxide anion (O(2)(-)) production but had normal expression and up-regulation of CD11b. Rac2, which constitutes >96% of the Rac in neutrophils, is a member of the Rho family of GTPases that regulates the actin cytoskeleton and O(2)(-) production. Western blot analysis of lysates from patient neutrophils demonstrated decreased levels of Rac2 protein. Addition of recombinant Rac to extracts of the patient neutrophils reconstituted O(2)(-) production in an in vitro assay system. Molecular analysis identified a point mutation in one allele of the Rac2 gene resulting in the substitution of Asp57 by an Asn (Rac2(D57N)). Asp57 is invariant in all defined gtp-binding proteins. Rac2(D57N) binds GDP but not GTP and inhibits oxidase activation and O(2)(-) production in vitro. These data represent the description of an inhibitory mutation in a member of the Rho family of GTPases associated with a human immunodeficiency syndrome.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
3/90. Immunodeficiency diseases. I. T-lymphocyte precursors and T-lymphocyte differentiation in partial Di George syndrome.Immunological and pathological studies in a case of partial Di George syndrome revealed an absence of parathyroids, a major hypoplasia of thymus but a relatively moderate decrease in peripheral T-lymphocyte numbers and functions. After in vitro incubation with normal thymus extracts, a normal proportion of bone marrow cells was induced to differentiate into cells with characteristics of T lymphocytes, thus establishing the presence of T-cell precursors in the patient's bone marrow.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
4/90. cryptosporidiosis in an immunosuppressed renal-transplant recipient with iga deficiency.Cryptosporidia are sporozoan parasites that infect epithelial cells of the gastrointestinal tract. Infection with cryptosporidia has been found most commonly in a variety of animal species and only rarely in man. The authors report a case of an immunosuppressed renal-transplant recipient with iga deficiency who experienced diarrhea and fever and was found to have cryptosporidia in a jejunal biopsy specimen and in air-dried smears of the specimen. By electron microscopy, trophozoite, schizont, and macrogamete forms were identified, and these forms ahd morphologic features similar to those of cryptosporidia previously found in guinea pigs. Treatment of the cryptosporidial infection in this case was with trisulfapyrimidines. The efficacy of this treatment could not be evaluated because of complications.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
5/90. Analysis of Ig subclass deficiency: First reported case of IgG2, IgG4, and iga deficiency caused by deletion of C alpha 1, psi C gamma, C gamma 2, C gamma 4, and C epsilon in a Mongoloid patient.BACKGROUND: The cause of Ig class or subclass deficiencies has been unclear except for IgH gene deletions and a homozygous 1-base insertion (1793insG) in C gamma 2 exon 4 of IgG2 deficiency. In addition, there are no reports that IgH gene deletions are found in patients who are Mongoloid (a category that includes most of the people of asia, such as the Japanese, Chinese, Indonesians and Malaysians). OBJECTIVE: To investigate the cause of Ig subclass deficiency, we extensively investigated 4 Japanese patients with low serum IgG2 levels. Patient 1 was a 23-month-old girl whose serum IgG2, IgG4, and IgA levels were under the detection limits; she also had idiopathic thrombocytopenic purpura. Patient 2 was a 5-year-old boy whose serum IgG2 level was under the detection limit. Patient 3 was a 14-month-old boy whose serum IgG2 and IgG4 levels were under the detection limits. Patient 4 was 3-year-old girl whose IgG2 level was low and whose IgA level was under the detection limit. methods: dna was extracted from neutrophils, and Southern blot analysis was performed. RESULTS: Southern blot analysis revealed that patient 1 had lost the C alpha 1, psi C gamma, C gamma 2, C gamma 4, and C epsilon genes and was a homozygous carrier of the deletion described above. Her mother was of the heterozygous genotype. dna from patients 2, 3, and 4 revealed no deletions in comparison with control dna. CONCLUSION: This is a report of the first case of a Mongoloid in whom IgG2, IgG4, and iga deficiency was caused by deletion of C alpha 1, psi C gamma, C gamma 2, C gamma 4, and C epsilon. Our finding contributes to investigations of Ig subclass deficiency.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
6/90. Conjunctival squamous cell carcinoma combined with malignant lymphoma.A 31-year-old white woman with a poorly differentiated malignant lymphoma and a compromised immune system atypically contracted squamous cell carcinoma. Data obtained after her death indicate that her immune system was severely altered by either the primary disease or the chemotherapeutic regimen, or both.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
7/90. Transient B cell immaturity with intractable diarrhoea: a possible new immunodeficiency syndrome.A male boy is described, who suffered from an intractable diarrhoea and several infections and who died in a severe marasmic state at the age of 8 months. Immunological studies revealed a block in the normal differentiation of B cells to Ig-producing plasma cells. After the age of 5 months, however, this block disappeared, leading to a dramatic increase in circulating Ig, most pronounced in the IgM class. In the intestine, plasma cells could only be detected after the age of 5 months, and then with a marked preponderance of IgM cells. Our results thus indicate a reversible block in the normal maturation of B cells in our patient. An older brother may have had a similar disease, suggesting a possible genetic basis for the disorder.- - - - - - - - - - ranking = 5keywords = tract (Clic here for more details about this article) |
8/90. Hyper-IgM syndrome: a case report.Hyperimmunoglobulin M syndrome is a rare primary immunodeficiency disorder. We report a case of a 6-month-old boy who suffered from developmental delays, frequent respiratory tract infection, and unusual fungal and bacterial infection. X-linked hyperimmunoglobulin M syndrome was ultimately diagnosed with decreasing immunoglobulin-G, A, and E (immunoglobulin g = 51.3 mg/dL, immunoglobulin a = 8.32 mg/dL, immunoglobulin e <17.5 mg/dL), elevating immunoglobulin m (immunoglobulin m = 140 mg/dL), and decreasing T-cell expression of the cd40 ligand over flow cytometry. Seizure episodes and hypotonia developed with greater signal intensity at the putamen in a brain magnetic resonance imaging, which is compatible with hypoxic ischemic encephalopathy. This is the youngest proven case of hyper-IgM syndrome in taiwan ever reported.- - - - - - - - - - ranking = 2528.6316613257keywords = respiratory tract infection, respiratory tract, tract infection, tract (Clic here for more details about this article) |
9/90. 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 = 5057.2970551526keywords = respiratory tract infection, respiratory tract, tract infection, tract, upper (Clic here for more details about this article) |
10/90. Cellular immunodeficiency and autoimmunity in long-term mineral oil administration.BACKGROUND: Subcutaneous mineral oil injection is an old-fashioned practice used mostly for cosmetic purposes. Infection, ulceration, subcutaneous nodules, and autoimmune activation are among the known adverse effects. Immunodeficiency has not been previously reported in association with mineral oil injection. OBJECTIVE: To report the case of a 43-year-old woman who performed long-term self-administration of mineral oil and was found to have both cellular immunodeficiency and autoimmunity. methods: We performed an immunological evaluation. Throat, induced sputum, urine, and blood cultures were examined for microorganisms. Pelvic computed tomography, inguinal lymph node biopsy, bone marrow biopsy, and liver biopsy were also performed. RESULTS: Laboratory results revealed peripheral lymphopenia, very low absolute numbers of lymphocyte subpopulations, and a markedly impaired lymphocyte proliferative response to mitogens (phytohemagglutinin and concanavalin a) and recall antigens (mumps, candida albicans, purified protein derivative, and tetanus toxoid). The cultures were negative for microorganisms. The pelvic computed tomogram demonstrated areas of diffuse oil-density signals throughout the subcutaneous tissue in the gluteal area and proximal lower extremities, as well as bilateral inguinal lymphadenopathy. A lymph node biopsy specimen showed lipid granulomas and necrotizing lymphadenitis. A bone marrow biopsy specimen demonstrated hypercellular marrow with normal trilineage hematopoesis. Increased serum transaminase levels, hypoalbuminemia, positive anti-extractable nuclear antigen and anti-Ro antibodies, and plasma cells in the liver suggested an autoimmune process. CONCLUSIONS: mineral oil administration may be associated with both cellular immunodeficiency and autoimmunity. patients who have received long-term administration of a foreign substance should undergo a comprehensive immunological evaluation.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
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