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排序方式: 共有962条查询结果,搜索用时 31 毫秒
101.
Parr DG Stoel BC Stolk J Stockley RA 《American journal of respiratory and critical care medicine》2004,170(11):1172-1178
FEV(1) is fundamental to the diagnosis and staging of chronic obstructive pulmonary disease. In emphysema, airflow obstruction usually coexists with impairment of gas exchange, but discordance is not infrequent. We hypothesized that variations in the distribution of emphysema would be associated with functional differences and therefore account for discordant physiology. We used quantitative computed tomography to assess emphysema severity and distribution in 119 subjects with alpha1-antitrypsin deficiency (PiZ phenotype) and grouped them according to distribution pattern. In the 102 subjects with emphysema, 65 had a predominantly basal pattern ("basal"), but 37 (36%) had greater involvement of the upper regions ("apical"). Subjects from each group were matched for total volume of emphysema and age, and matched pairs analysis was used to relate emphysema distribution to clinical phenotype. Basal distribution was associated with greater impairment of FEV(1) (mean difference, 9.9% predicted; 95% confidence interval, 3.8 to 16.0; p = 0.002) but less impairment of gas exchange (Pa(O(2)) mean difference, 0.5 kPa, 0.03 to 0.1; p = 0.016) and alveolar-arterial oxygen gradient (mean difference, 0.7 kPa; 0.2 to 1.2; p = 0.007) than the apical distribution. Emphysema distribution correlated with physiologic discordance (r = -0.409, p < 0.001). The use of single physiologic parameters as a surrogate measure of emphysema severity may introduce systematic bias in the staging of subjects with emphysema. 相似文献
102.
van Tuyl SA Letteboer TG Rogge-Wolf C Kuipers EJ Snijder RJ Westermann CJ Stolk MF 《European journal of gastroenterology & hepatology》2007,19(2):153-158
INTRODUCTION: Hereditary hemorrhagic teleangiectasia (HHT) is an autosomal dominant disorder with mucocutaneous teleangiectasia and visceral arteriovenous malformations. Mutations of endoglin and Activin A receptor like kinase-1 have different phenotypes, HHT1 and HHT2, respectively. The gastrointestinal tract is frequently affected, but limited information is available on the relationship with genotype. AIM: To determine whether different genotypes have different phenotypes with respect to intestinal teleangiectasia. METHODS: HHT patients, referred for anemia, underwent videocapsule endoscopy. Chart review was performed for information on genotype and HHT manifestations. RESULTS: Twenty-five patients were analyzed (men/women 13/9, mean age 49+/-15 years.), 14 HHT1, eight HHT2 and three without known mutation. Epistaxis occurred in 96% of patients. Gastroduodenoscopy revealed teleangiectasia in 7/12 (58%) HHT1 and 3/8 (38%) HHT2 patients. Videocapsule endoscopy found teleangiectasia in all HHT1 and 5/8 (63%) HHT2 patients. In 9/14 HHT1 patients, teleangiectasia were large. Teleangiectasia in the colon was restricted to 6/11 (55%) HHT1 patients. Hepatic arteriovenous malformations were present in 1/7 HHT1 and 5/6 HHT2 patients. CONCLUSION: Large teleangiectasia in small intestine and colon appear to occur predominantly in HHT1. Hepatic arteriovenous malformations are mainly found in HHT2. In HHT patients with unexplained anemia, videocapsule endoscopy should be considered to determine the size and extent of teleangiectasia and exclude other abnormalities. 相似文献
103.
Stolk P Souverein PC Leufkens HG Weil JG Egberts AC Heerdink ER 《Pharmacopsychiatry》2007,40(3):111-115
BACKGROUND: COX-2 inhibitors (COX-2i) have been reported to have beneficial effects on schizophrenia. This observational study assesses the association between exposure to COX-2i or/and NSAIDs and schizophrenia deterioration. METHODS: We conducted a case-control study within a cohort (n=3,485) of antipsychotic users with a schizophrenia diagnosis (ICD-9=295.x) in IMS-Lifelink, a US claims database. Case events indicating exacerbation of schizophrenia were: switching antipsychotic medication, starting combination therapy, using parenteral antipsychotics or an increasing dose. For each case one control was selected. Exposure to COX-2i/NSAIDs (current/recent/none) and cumulative exposure in Defined Daily Doses 90 days before the index/event date were assessed. Age, sex and co-medication were evaluated as confounders. Logistic regression analysis was used to assess the association. RESULTS: 1,443 case events occurred. For current use, no benefit on schizophrenia case events from exposure to COX-2i was found (adjusted OR 1.16; 95% CI 0.83-1.62). Instead, recent COX2i use with a duration of 0 to 93 days was associated with an increased risk for schizophrenia deterioration (adjusted OR 2.56; 95% CI 1.35-4.87). This association was strongest in rofecoxib. No relation was found for NSAIDs. CONCLUSION: The use of COX-2i was not associated with a decreased risk for schizophrenia deterioration in this population. 相似文献
104.
G Zaidi RP Sahu L Zhang G George N Bhavani N Shah V Bhatia A Bhansali G Jevalikar RV Jayakumar GS Eisenbarth E Bhatia 《Clinical genetics》2009,76(5):441-448
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare recessive disorder resulting from mutations in the autoimmune regulator ( AIRE ) gene. There is no information on AIRE mutations in Indians. In a cross-sectional study, nine patients (eight families), from four referral hospitals in India, were studied for AIRE mutations by direct sequencing. We screened for new mutations in 150 controls by allele-specific PCR. The patients had 1–7 known components of APECED. Three patients had unusual manifestations: presentation with type 1 diabetes; chronic sinusitis and otitis media; and facial dysmorphism. All patients carried homozygous, probably recessive, AIRE mutations. Two unrelated patients from a small in-bred community (Vanika Vaisya) in south India carried an unreported missense mutation, p.V80G, in the N-terminal caspase recruitment domain. Another unique mutation, p.C302X, resulting in a truncated protein with deletion of both zinc-finger domains, was detected in a patient from Gujarat. Neither mutation was detected in controls. Other mutations, previously described in Caucasians, were: 13 base pair deletion (p.C322fsX372) in 4 (38%), and Finn-major (p.R257X) and p.R139X (Sardinian) mutation in one subject each. In conclusion, in this first series of APECED in Indians, we detected AIRE mutations previously reported in Caucasians, as well as unique mutations. Of these, p.V80G is possibly an ancestral mutation in an in-bred community. 相似文献
105.
106.
Silvio ST Tafuri Domenico DM Martinelli Giovanni GC Caputi Annamaria AA Arbore Cinzia CG Germinario Rosa RP Prato 《BMC health services research》2009,9(1):100-5
Background
Mandatory vaccination has contributed to the success of immunisation programmes but voluntary vaccination allows people to be responsible for their own health. There are benefits from both policies and the arguments between them remain subject to debate within and without the scientific community, both nationally and internationally. The aim of this study is to assess the opinions of those who actually work in the Vaccination Service. 相似文献107.
108.
E.A.M. Zijtregtop J. Wilschut N. Koelma J.J.M. Van Delden R.P. Stolk J. Van Steenbergen J. Broer B. Wolters M.J. Postma E. Hak 《Vaccine》2009
Since 2008, (pre)pandemic vaccines against H5N1 influenza have been available and pandemic vaccines against new influenza H1N1 are currently produced. In The Netherlands, the vaccination call for seasonal influenza among the recommended groups approximates 70%. These statistics raise the question if adults in Western societies are willing to get a (pre)pandemic influenza vaccination, for example, against avian H5N1 or swine-like H1N1 virus. A questionnaire was performed to determine the predictors of a negative intention to be immunized against pandemic influenza among adults. Demographical, behavioural and organisational determinants were studied. Thirty-four and five percent of the respondents were negatively intended to get a pandemic influenza vaccination in a pre-pandemic or pandemic phase, respectively. On the basis of six behavioural determinants negative intention to get a pandemic influenza vaccination can be predicted correctly in almost 80% of the target group. These determinants should be targeted in pandemic preparedness plans. 相似文献
109.
Gosman MM Boezen HM van Diemen CC Snoeck-Stroband JB Lapperre TS Hiemstra PS Ten Hacken NH Stolk J Postma DS 《Thorax》2007,62(3):242-247
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a respiratory disorder with increasing prevalence and mortality. It is associated with airway obstruction, increased airway hyper-responsiveness (AHR), and ongoing airway and lung inflammation dominated by CD8 lymphocytes and neutrophils. Single-nucleotide polymorphisms (SNPs) in a disintegrin and metalloprotease 33 (ADAM33) gene have been associated with AHR and COPD. OBJECTIVE: To assess whether SNPs in ADAM33 are associated with the severity of AHR and airway inflammation in COPD. METHODS: Eight SNPs in ADAM33 (F+1, Q-1, S_1, S_2, ST+5, T_1, T_2, V_4) were genotyped in 111 patients with COPD (96 males, 69 current smokers, mean (standard deviation (SD)), aged 62 (8) years, median pack-years 42 (IQR 31-55), mean postbronchodilator forced expiratory volume in 1 s (FEV(1))% predicted 63 (9). Provocative concentration of methacholine causing a decrease in FEV(1) of 20% (PC(20) methacholine), sputum and bronchial biopsies were collected. RESULTS: Patients with the ST+5 AA genotype had more severe AHR, higher numbers of sputum inflammatory cells and CD8 cells in bronchial biopsies than patients with the GG genotype (p = 0.03, 0.05 and 0.01, respectively). CD8 cell numbers were lower in patients carrying the minor allele of SNP T_1 and T_2, and homozygotic minor variants of SNP S_2 compared with the wild type (p = 0.02, 0.01 and 0.02, respectively). CONCLUSIONS: This is the first study revealing that SNPs in a gene that confers susceptibility to COPD in the general population-that is, ADAM33-are associated with AHR and airway inflammation in COPD. These findings constitute an important step forward in linking gene polymorphisms with COPD pathophysiology, thereby possibly contributing to better treatments for this progressive and disabling disease in the future. 相似文献
110.
Maria CLG Santos P Suzanne Hart Mukundhan Ramaswami Cláudia M Kanno Thomas C Hart Sergio RP Line 《Head & face medicine》2007,3(1):1-7