Myasthenia gravis with thymoma is not associated with an increased incidence of non-muscle autoimmune disorders.

Three groups of thymectomized patients with myasthenia gravis (MG) were selected for study, 16 with thymoma, 16 with thymic atrophy and 32 with follicular hyperplasia of the thymus. All 16 patients with thymoma, 15/16 with thymus atrophy and 30/32 with follicular hyperplasia had AChR antibodies. Non-receptor muscle (CA) antibodies were found in sera of 15/16 patients with thymoma, 3/16 with thymus atrophy and in none of the sera from patients with follicular hyperplasia. There were 2 patients with thymoma and polymyositis, but none of the thymoma patients had rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or other autoimmune disorders. Among the 32 patients with follicular hyperplasia of the thymus were 2 with SLE, 2 with RA and 1 with juvenile diabetes mellitus. In this study, there was an increased incidence of non-muscle autoimmune disorders among MG patients with follicular hyperplasia of the thymus but not among MG patients with thymoma.     

精神分裂症和神经症患者心理防御机制的对照研究

目的：探讨精神分裂症患者和神经症患者在心理防御机制的使用上与正常人的差别。为临床提供辅助诊断。方法：采用心理防御方式问卷对48例精神分裂症、64例神经症患者进行测试，并与129例健康人对照。结果：精神分裂症患者和神经症患者与正常对照组相比较，多使用不成熟的防御机制，较少使用成熟防御机制；精神分裂症组与神经症组相比，更多使用分裂，而在其它防御机制的使用上无明显差异；神经症患者较少掩饰性。结论：精神分裂症病人和神经症病人在心理防御机制的使用上与正常人不同，神经症病人与精神分裂症病人使用大致相同的心理防御机制。     

Activity in vitro of twelve antibiotics against clinical isolates of extended-spectrum beta-lactamase producing Escherichia coli

Twelve beta-lactam and non-beta-lactam antibiotics were evaluated against 115 clinical isolates of extended-spectrum beta-lactamase-producing (ESBLs) Escherichia coli using a broth microdilution test in accordance with the CLSI guidelines. Susceptibility was 100% with imipenem, ertapenem and amikacin, 95.7% with piperacillin-tazobactam, 91.3% with cefoxitin, 87% with tobramycin, 81.7% with amoxicillin-clavulanate, 80% with cefepime, 67.8% with ceftazidime, 27.8% with ciprofloxacin, 27% with levofloxacin and 13% with ceftriaxone. Ertapenem was the antibiotic with the lowest minimum inhibitory concentrations (MICs) for all isolates. There were no clinically relevant differences in the activity of the antibiotics in the presence of CTX-M-9 or SHV enzymes.     

Elevated BAFF levels in the cerebrospinal fluid of patients with neuro-Behçet's disease: BAFF is correlated with progressive dementia and psychosis

Neuro‐Behçet’s disease (NBD) is a serious complication of Behçet’s disease. Generally, NBD patients with a chronic course are refractory to immunosuppressive treatment, resulting in the deterioration of personality. In this study, levels of B cell‐activating factor belonging to the TNF family (BAFF) were measured in the cerebrospinal fluid (CSF) from 18 patients with NBD, 27 patients with epidemic aseptic meningitis (AM), 24 patients with multiple sclerosis (MS) and 34 healthy controls. BAFF levels in patients with NBD were significantly elevated compared with healthy controls, but showed no statistically significant elevation compared with either of the disease controls. In contrast, CSF IL‐6 levels were slightly elevated in patients with NBD and significantly elevated in patients with AM and MS compared with healthy controls. Patients with NBD were subdivided into two groups according to their clinical course (eight patients with a slowly progressive course presenting with psychosis and dementia and 10 patients with an acute course including aseptic meningitis, brainstem involvement and myelopathy). BAFF levels were significantly increased in those with a slowly progressive course compared with those with an acute course. CSF BAFF levels did not correlate with serum BAFF levels, CSF cell counts or CSF IL‐6 levels in patients with NBD. These data suggested that BAFF was produced within the central nervous system and may be associated with the development of NBD, particularly with a progressive course.     

The birth prevalence of malformation syndromes in Tokyo infants: A survey of 14,430 newborn infants

A survey of the birth prevalence of congenital anomalies among newborn infants in Japan is under way at a large maternity hospital in Tokyo. Of 14,430 consecutive newborn babies (7,455 M; 6,975 F), 33 had a multiple congenital anomalies (MCA) syndrome. These included 2 with trisomy 13 (including a mosaic), 3 with trisomy 18 (including 1 mosaic), 16 with trisomy 21 (including 1 mosaic), 1 with cri-du-chat syndrome, 1 with 5p partial trisomy, 1 with Hallermann-Streiff syndrome, 1 with Treacher-Collins syndrome, 1 with achondroplasia, 2 with arthrogryposis multiplex congenita, 1 with hemihypertrophy, 1 with Wiedemann-Beckwith syndrome, 1 with asplenia syndrome, 1 with Klippel-Trenaunay-Weber syndrome, and 1 with probable Marfan's syndrome. Except for one infant with Ullrich-Turner syndrome, cases with sex-chromosome aberrations could not be diagnosed neonatally on a clinical basis.     

Targets and dimensions of social comparison among people with spinal cord injury and other health problems

The present research examined comparison targets and comparison dimensions among two Spanish samples of individuals facing serious illnesses and diseases. In Study 1, 90 older patients (mean age 66.36) with various age‐related diseases, particularly cardiovascular diseases and diabetes, indicated that they compared themselves most often with others with the same disease, next with others with another disease and least with people without health problems. They compared themselves more often on their mental state, symptoms and physical activities than on their social activities. Social comparison orientation (SCO) as an individual difference characteristic was associated with more frequent comparisons with particularly similar targets, and with more frequent comparisons of one's symptoms and physical activities. Neuroticism was correlated only with more comparisons of one's symptoms. Study 2 was conducted in a sample of 70 relatively young patients (mean age 43.97) with spinal cord injury (SCI). Overall, they compared themselves more often with others than the participants in Study 1, and they compared themselves to a similar extent with people with SCI as with people with another disease and with people without health problems. While they felt on average better off than people with other diseases and other people with SCI, people with SCI felt on average worse off than people without health problems. They compared themselves more often on physical activities than on any other dimension. Higher levels of stress and uncertainty were associated with more frequent comparisons with people without SCI, and with more frequent comparisons of one's mental state, one's symptoms and one's future perspectives. The discussion focuses on the theoretical relevance of the results for social comparison theory, and on the practical relevance of the findings for interventions.     

Sm14 of Schistosoma mansoni in fusion with tetanus toxin fragment C induces immunoprotection against tetanus and schistosomiasis in mice

We have constructed vectors that permit the expression in Escherichia coli of Schistosoma mansoni fatty acid-binding protein 14 (Sm14) in fusion with the nontoxic, but highly immunogenic, tetanus toxin fragment C (TTFC). The recombinant six-His-tagged proteins were purified by nickel affinity chromatography and used in immunization and challenge assays. Animals inoculated with TTFC in fusion with or coadministered with Sm14 showed high levels of tetanus toxin antibodies, while animals inoculated with Sm14 in fusion with or coadministered with TTFC showed high levels of Sm14 antibodies. In both cases, there were no changes in the type of immune response (Th2) obtained with the fusion proteins compared to those obtained with the nonfused proteins. Mice immunized with the recombinant proteins (TTFC in fusion with or coadministered with Sm14) survived the challenge with tetanus toxin and did not show any symptoms of the disease. Control animals inoculated with either phosphate-buffered saline (PBS) or Sm14 died with severe symptoms of tetanus after 24 h. Mice immunized with the recombinant proteins (Sm14 in fusion with or coadministered with TTFC) showed a 50% reduction in worm burden when they were challenged with S. mansoni cercariae, while control animals inoculated with either PBS or TTFC were not protected. The results show that the expression of other antigens in fusion at the carboxy terminus of TTFC is feasible for the development of a multivalent recombinant vaccine.     

Association between IgE levels and asthma severity among African American, Mexican, and Puerto Rican patients with asthma

BACKGROUND: High levels of IgE are associated with asthma. Whether higher levels of IgE are associated with more severe asthma is still unclear. OBJECTIVE: To determine whether IgE is associated with asthma severity among Latino and African American subjects with asthma. METHODS: We assessed lung function and asthma severity among African American, Mexican, and Puerto Rican patients with asthma with high IgE levels (> or =100 IU/mL; n = 492) and compared these values to those of patients with asthma with low IgE levels (<100 IU/mL; n = 247). We also examined IgE as a continuous variable among these groups. RESULTS: Patients with asthma with high IgE had a lower mean FEV(1) (87.6 +/- 17.1, percent of predicted) than patients with asthma with low IgE (91.5 +/- 17.0; P = .031). Regardless of race and ethnicity, baseline FEV(1), forced expiratory flow, and FEV(1)/forced vital capacity were lower among subjects with high IgE than among subjects with low IgE (P = .031, P < .0001, P = .0001, respectively). In addition, 54.7% of patients with asthma with high IgE had been previously hospitalized, compared with 44.1% of patients with asthma with low IgE (odds ratio, 1.33; 95% CI, 1.04-1.71). CONCLUSION: Higher IgE is associated with lower baseline lung function and more severe asthma among these populations. CLINICAL IMPLICATIONS: Among patients with asthma from 3 ethnically distinct groups, total IgE levels are inversely correlated with baseline lung function and asthma severity.     

Antinuclear Antibodies and Anti-DNA Antibodies in Scleroderma

Antinuclear antibodies (ANA), including anti-DNA antibodies, and rheumatoid factors (RAT, Waaler-Rose) were determined prospectively during a 3-year period in 40 patients with localized scleroderma (LS) compared with 77 patients with generalized scleroderma (GS). ANA were increased in 26% of patients with LS, and in 47% with GS, anti-DNA antibodies in 23% of patients with LS, and in 34% with GS. Thus, the anti-DNA antibody level was lower compared with the known level in systemic lupus erythematosus. Rheumatoid factors were present in 6-7% of patients with LS, and in 14-15% of patients with GS. Increased antinuclear antibodies were not associated with any specific type of localized scleroderma, nor with internal disorders, and no case of clinical overlap to discoid or systemic lupus erythematosus was observed. However, six patients with localized scleroderma and complaints of arthralgia all presented increased antibodies, and one patient showed overlap to rheumatoid arthritis. It is suggested that increased ANA and anti-DNA antibodies in localized scleroderma, associated with joint manifestations, represents a systemic component in this type of scleroderma, with activation of the immune system and similarities with generalized collagen diseases.     

Hauttumoren als Markerläsionen hereditärer Tumorsyndrome

A number of hereditary tumor syndromes are associated with characteristic dermal neoplasms and knowledge and early diagnosis of these lesions may facilitate the diagnostic of the underlying syndrome. These syndromes include Muir-Torre syndrome, associated with cystic sebaceomas, Cowden syndrome, associated with multiple tricholemmomas, Carney complex associated with multiple superficial angiomyxomas, Birt-Hogg-Dubé syndrome associated with multiple fibrofolliculomas, tuberous sclerosis associated with multiple facial angiofibromas and so-called Koenen tumors, patients with renal cell cancer associated with pilar leiomyomatosis and uterine leiomyomas, Gardner syndrome associated with Gardner fibromas and nevoid basal cell carcinoma associated with multiple basal cell carcinomas in young patients.     

Specificity of immunoglobulin E in coccidioidomycosis and correlation with disease involvement.

Serum immunoglobulin E (IgE) antibodies were quantitated in 26 patients with active pulmonary coccidioidomycosis, 59 patients with active disseminated disease, 12 patients in clinical remission, and 91 healthy subjects. Significant differences were obtained in IgE serum levels of patients with active disease versus healthy subjects (P less than 0.0001). Patients with pulmonary coccidioidomycosis did not differ in their IgE levels when compared with patients with disseminated disease. However, serum IgE levels were significantly increased in patients with disease involving two or more organ systems when compared with patients with pulmonary disease or extrapulmonary disease involving a single organ system (P less than 0.02). Total serum IgE correlated with anti-Coccidioides IgE (P less than 0.001), but with only six exceptions, patients with anti-Coccidioides IgE also exhibited IgE antibodies to 1 or more of 12 common allergens. The correlation between hyperproduction of IgE and disease severity coupled with the depressed cell-mediated immune status of patients with this disease suggests a defect(s) in the T-lymphocyte population which functions to regulate IgE synthesis.     

动态脑电图对晕厥患者的诊断意义

目的:探讨动态脑电图(AEEG)在晕厥患者中的诊断价值。方法:采用十六导联动态脑电图分析仪连续监测24小时的脑电活动。结果:242例晕厥发作患者中AEEG结果示:28例为正常脑电图,95例为轻度异常脑电图,97例为中度异常脑电图,22例为重度异常脑电图,中、重度异常脑电图的异常率为49.2%。而REEG结果示:191例为正常脑电图,38例为轻度异常脑电图,13例为轻-中度异常脑电图,有异常表现者51例,异常率为21.1%。结论:AEEG较REEG在对脑源性晕厥患者的中有明显的优势,对临床诊疗有很大的价值。     

Unusual manifestations of primary cytomegalovirus infection in patients without HIV infection and without organ transplants

Primary cytomegalovirus (CMV) infection with marked constitutional symptoms is rare in immunocompetent individuals and in those with iatrogenic immunosuppression, except transplant recipients. Four patients admitted to hospital with clinical illnesses associated with primary CMV infection were identified over a 12-month period. Their medical records were reviewed with regard to clinical and laboratory data, and outcome. Primary CMV infection was defined by the concomitant presence of CMV IgM and low avidity CMV IgG antibody. Of two patients with no known underlying illness, one presented with thrombocytopenic purpura and the other with vasculitis. Two patients receiving immunosuppressants for underlying ulcerative colitis presented with CMV-induced pancytopenia and CMV colitis. Atypical lymphocytosis was a feature on blood film examination in three of the four cases. One patient with disseminated CMV infection died of progressive multiorgan failure despite antiviral treatment. CMV disease following primary CMV infection should be considered in otherwise immunocompetent individuals with atypical lymphocytosis on blood film analysis, and particularly in patients on immunosuppressants such as those with ulcerative colitis, since early diagnosis and treatment with antiviral drugs may improve outcome.     

Identification of Mycobacterium tuberculosis antigens in Seibert fractions by immunoblotting.

Seibert fractions prepared from Mycobacterium tuberculosis culture filtrates were evaluated by immunoblotting with a serum pool from patients with active pulmonary tuberculosis. Antibody activity was observed primarily with antigens in the polysaccharide II and A protein fractions; these fractions were further evaluated by immunoblotting with sera from individual patients with tuberculosis, from individuals without tuberculosis and positive for the purified protein derivative antigen skin test, and from individuals negative for the purified protein derivative antigen skin test. The antigens identified in the protein A fraction, a 32,000-molecular-weight antigen and a heterogeneous high-molecular-weight antigen, reacted with antibody found in sera from all patients with tuberculosis and with antibody from over 25% of the control individuals. A 10,000-molecular-weight antigen, a 30,000- to 44,000-molecular-weight antigen, and a heterogeneous high-molecular-weight antigen were observed in the polysaccharide II fraction; these antigens reacted with serum antibody from 70% or more of the patients with tuberculosis and with antibody from 20 to 70% of the control individuals. One of the antigens, with a molecular weight ranging from 17,000 to 28,000 in the polysaccharide II fraction, reacted with antibody in 64% of the sera from patients with tuberculosis but with only 1 of 15 control normal sera. This antigen may elicit an antibody response specifically associated with tuberculosis.     

Energostim in Therapy of Chronic Ischemia of the Lower Limbs

Positive changes were more pronounced in patients with chronic ischemia of the lower limbs treated with energostim alone and in combination with trental in comparison with patients receiving trental monotherapy. The best effect was attained in patients treated with energostim in combination with trental.     

Effects of immunization with Cryptococcus neoformans cells or cryptococcal culture filtrate antigen on direct anticryptococcal activities of murine T lymphocytes.

Immunizing CBA/J mice with intact Cryptococcus neoformans cells or with a cryptococcal culture filtrate antigen (CneF) induces an anticryptococcal delayed-type hypersensitivity response. Recently, it has been shown that two phenotypically different T-cell populations are responsible for delayed-type hypersensitivity reactivity in mice immunized with intact cryptococcal cells, whereas only one of those populations is present in mice immunized with soluble cryptococcal antigens in complete Freund's adjuvant (CFA). The purpose of this study was to determine if differences occur with regard to direct anticryptococcal activity between T-lymphocyte-enriched populations from mice immunized with intact viable or dead cryptococcal cells and similar cell populations from mice immunized with the soluble cryptococcal culture filtrate antigen, CneF, emulsified in CFA. The percentage of lymphocytes which form conjugates with C. neoformans and the percentage of cryptococcal growth inhibition in vitro are greater with T-lymphocyte-enriched populations from mice sublethally infected with C. neoformans or from mice immunized with intact heat-killed cryptococcal cells in the presence or absence of CFA than with lymphocyte populations from mice immunized with CneF-CFA. Enhanced anticryptococcal activity of T lymphocytes could be induced by immunizing mice with heat-killed C. neoformans cells of serotype A, B, C, or D as well as by immunizing with a similar preparation of an acapsular C. neoformans mutant but not by immunizing with CFA emulsified with CneF prepared from any one of the C. neoformans isolates. These data indicate that the soluble cryptococcal culture filtrate antigens do not induce the same array of functional T lymphocytes as whole cryptococcal cells.     

Mania, glutamate/glutamine and risperidone in pediatric bipolar disorder: a proton magnetic resonance spectroscopy study of the anterior cingulate cortex

BACKGROUND: The purpose of this study was to investigate the anterior cingulate cortex (ACC) glutamate/glutamine (Glx) to creatine ratio (Glx/Cr) in two groups of children with Bipolar Disorder (BPD): those exhibiting manic symptoms requiring treatment and those being stably treated with the atypical antipsychotic risperidone. Atypical antipsychotics have been shown to increase serum glutamate levels and ACC Glx/Cr in subjects with schizophrenia. In this study, we hypothesized that the children with BPD in need of treatment would have lower Glx/Cr compared with the children with BPD being stably treated with risperidone. METHODS: Proton MR spectra were acquired, at 1.5 T, from the ACC of eighteen subjects with a DSM-IV diagnosis of BPD: ten (11.10+/-3.48 years; five female) were manic and not medicated with any antipsychotic and eight (10.88+/-2.99 years; one female) were medicated with the atypical antipsychotic risperidone. RESULTS: Children with BPD exhibiting manic symptoms requiring treatment had lower Glx/Cr than children with BPD being stably treated with the atypical antipsychotic risperidone. The children treated with risperidone also had significantly lower YMRS and CGI-Mania scores than the children not treated with risperidone. Both YMRS and CGI-Mania scores correlated negatively with ACC Glx/Cr levels. LIMITATIONS: The cross-sectional design, small sample size, the use of Glx rather than glutamate or glutamine and the use of Cr ratios rather than absolute concentrations are limitations of this study. CONCLUSIONS: Children with mania have lower Glx/Cr levels than children with BPD being stably treated with the atypical antipsychotic risperidone. Mania may be associated with reduced glutamate/glutamine levels in the ACC: other imaging studies have shown mania associated with hypometabolism in the ACC. These reductions in glutamate/glutamine may be increased following successful treatment with glutamatergic agents.     

DNA polymerase activity in proliferating and antigen-stimulated tissues

The activity of DNA polymerase was measured with activated DNA or with polydT-rA as templates in the particulate and in the supernatant fraction of proliferating tissues. The activity with polydT-rA was not higher in proliferating tissues (liver and brain of newborn rats, regenerating rat liver) as compared with resting tissues, with the exception of the brain supernatant, in which the activity was higher at birth and decreased with age. In the spleen of mice immunized with sheep erythrocytes there was a substantial increase of the DNA-polymerase activity with DNA as template, but not with polydT-rA as template.     

3067例遗传咨询患者的遗传代谢病筛查

本文应用尿筛查试验、尿氨基酸双相纸层析、尿粘多糖双向电泳和糖醛酸测定，对3067例遗传咨询患者进行遗传代谢病筛查。诊断苯丙酮尿症199例，粘多糖沉积症2l例，同型胱氨酸尿症3例，巨幼细胞贫血伴同型胱氨酸尿症、甲基丙二酸尿症3例，胱氨酸尿症1例，亚氨基甘氨酸尿症1例，Hartnup病1例，一过性酪氨酸血症1例。     

Enhanced histamine metabolism: a comparative analysis of collagenous colitis and food allergy with respect to the role of diet and NSAID use

OBJECTIVES AND DESIGN: To compare clinical data and histamine metabolism of patients with collagenous colitis with those of food allergy. METHODS: In 17 patients with collagenous colitis, clinical findings (diarrhoea, abdominal pain) were recorded. Plasma (for histamine) and 12-h-urine (for histamine and n-methylhistamine, all measured by RIA) were collected during two days with an unrestricted diet followed by two days with an hypoallergenic. The clinical data and measured values were compared with those from patients with confirmed food allergy (n = 21) and controls (n = 41). RESULTS: Patients with collagenous colitis were found to present with significantly more liquid stools than patients with food allergy (p < 0.001) during both types of diet, but they did not experience more abdominal pain. N-methylhistamine in 12-h-urine was significantly increased during both types of diet in patients with collagenous colitis and food allergy when compared with controls (p < 0.001 for all). Patients with food allergy--but not those with collagenous colitis--showed a significant decrease of severity of pain (p < 0.05) when the diet was changed to the elimination protocol. CONCLUSION: Histamine is extensively produced and metabolised in patients with collagenous colitis. In contrast to food allergy, the allergenic potency of the administered food seems not to influence histamine production in collagenous colitis. However, histamine metabolism corresponds with the clinical activity in both patients with food allergy and collagenous colitis.