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1.
Viswanath P Kurup Alan P Knutsen Richard B Moss Naveen K Bansal 《Clinical and molecular allergy : CMA》2006,4(1):1-7
Background
Aspergillus fumigatus, a widely distributed fungus, has been implicated in causing life threatening infections as well as severe asthma and allergic diseases in man. Allergic affliction like allergic bronchopulmonary aspergillosis (ABPA) is a disabling lung disease frequently seen in patients with asthma and cystic fibrosis. Immunodiagnosis of the former is comparatively easier due to the availability of purified antigens and sensitive methods. However, this is not true with cystic fibrosis patients where the prevalence of ABPA is fairly high and the morbidity and mortality are significant.Methods
In the present study, we have evaluated purified recombinant allergens from A. fumigatus, namely Asp f 1, f 2, f 3, f 4, and f 6 using ELISA and a semi-automated method (ImmunoCAP). We studied 17 patients each from cystic fibrosis with ABPA, and cystic fibrosis with asthma, 22 cystic fibrosis with no ABPA or asthma, and 11 age matched controls.Results
The results indicate that no antigen, antibody or method is capable of differentiating cystic fibrosis (CF) with ABPA from other CF patients, although some allergens showed strong reaction or showed more prevalence among the patients studied.Conclusion
When results of several allergens such as Asp f 1, f 2, f 3, f 4, and f 6 in their binding to IgA, IgG, and IgE antibodies were analyzed, a more strong discrimination of CF patients with ABPA was possible from the other groups studied. 相似文献2.
Salvador Raga M Rosa Julià Catalina Crespí Joan Figuerola Natalia Martínez Joan Milà Núria Matamoros 《Respiratory research》2003,4(1):1-14
Background
γδ T cells have an important immunoregulatory and effector function through cytokine release. They are involved in the responses to Gram-negative bacterium and in protection of lung epithelium integrity. On the other hand, they have been implicated in airway inflammation.Methods
The aim of the present work was to study intracytoplasmic IL-2, IL-4, IFN-γ and TNF-α production by γδ and αβ T lymphocytes from cystic fibrosis patients and healthy donors in response to Pseudomonas aeruginosa (PA). Flow cytometric detection was performed after peripheral blood mononuclear cells (PBMC) culture with a cytosolic extract from PA and restimulation with phorbol ester plus ionomycine. Proliferative responses, activation markers and receptor usage of γδ T cells were also evaluated.Results
The highest production of cytokine was of TNF-α and IFN-γ, γδ being better producers than αβ. No differences were found between patients and controls. The Vγ9δ2 subset of γδ T cells was preferentially expanded. CD25 and CD45RO expression by the αβ T subset and PBMC proliferative response to PA were defective in cystic fibrosis lymphocytes.Conclusion
Our results support the hypothesis that γδ T lymphocytes play an important role in the immune response to PA and in the chronic inflammatory lung reaction in cystic fibrosis patients. They do not confirm the involvement of a supressed Th1 cytokine response in the pathogenesis of this disease. 相似文献3.
Shramana Mandal Dipti Mahajan Somak Roy Meeta Singh Nita Khurana 《Diagnostic pathology》2007,2(1):1-4
Background
Glomerulocystic kidney disease is an uncommon type of cystic renal disease. It is characterized by cortical microsysts, which are represented by cystic dilatation of Bowman's spaces.Case presentation
We describe a case of glomerulocystic disease in a neonate and another in an abortus associated with tracheo-oesophageal fistula and megacystic-megaureter syndrome. The kidney on autopsy was sponge-like and revealed presence of cysts corresponding to dilatations of Bowman's space microscopically. In these two cases, the Glomerulocystic Kidney Disease in one case corresponded to a sporadic form and, in the other, to a syndromic, non-heritable form of glomerulocystic kidney disease.Conclusion
The associated anomalies in Glomerulocystic Kidney disease are well described in the literature. Two more new unrelated associations are described in this article. 相似文献4.
Introduction
Coronary stenting has previously been considered to be less feasible in children under 12 years old due to the limitation of vascular access. We report the case of a six-year-old boy who successfully underwent stent implantation for his totally occluded right coronary artery.Case presentation
A Taiwanese boy aged six years and nine months old was found to have giant aneurysms after an acute episode of Kawasaki disease. An angiography revealed that his middle right coronary artery was totally occluded. A 0.014-inch guidewire was advanced to cross the totally occluded site. After pre-dilating the middle portion of his right coronary artery with a 1.5 mm balloon, stenting of his right coronary artery was accomplished using a 2.5 × 28 mm and a 2.5 × 18 mm bare metal stent. A final angiography demonstrated no residual stenosis or dissection.Conclusion
Coronary stenting could be a therapeutic option for children as young as six years old. Close follow-up is mandatory because the long-term outcome is still unclear, especially in a small child. 相似文献5.
Background
Systemic sclerosis (SSc), a progressive disease characterized by excessive accumulation of connective tissue components. Although most patients have long survival, some of them progress rapidly to death. Pulmonary system involvement and pulmonary hypertension are the most frequent cause of death. When the patient with SSc is to be operated, the anesthetic procedure could be a serious problem. In this article, we report a combined spinal – epidural technique in a patient with progressive SSc and the anesthetic considerations that could be recommended for these patients.Case presentation
A 68-year-old woman who had a history of progressive systemic sclerosis, pulmonary fibrosis, kyphoscoliosis and decreased oral apertura underwent total hip arthroplasty. This operation was performed successfully under combined spinal epidural anesthesia.Conclusion
Systemic sclerosis is a complex disease that involves multiple organ systems. Every aspects of anesthetic care may be altered or hindered by the pathogenesis of disease. Although the choice of regional or general anesthesia is unclear, to choose combined spinal epidural anesthesia may be useful. 相似文献6.
Panagiota Latsi Panagiotis Pantelidis Dimitris Vassilakis Hiroe Sato Kenneth I Welsh Roland M du Bois 《Respiratory research》2003,4(1):1-5
Background
Genes encoding cytokine mediators are prime candidates for genetic analysis in conditions with T-helper (Th) cell disease driven imbalance. Idiopathic Pulmonary Fibrosis (IPF) is a predominantly Th2 mediated disease associated with a paucity of interferon-gamma (IFN-γ). The paucity of IFN-γ may favor the development of progressive fibrosis in IPF. Interleukin-12 (IL-12) plays a key role in inducing IFN-γ production. The aim of the current study was to assess whether the 1188 (A/C) 3'UTR single nucleotide polymorphism (SNP) in the IL-12 p40 subunit gene which was recently found to be functional and the 5644 (G/A) 3' UTR SNP of the IFN-γ gene were associated with susceptibility to IPF.Methods
We investigated the allelic distribution in these loci in UK white Caucasoid subjects comprising 73 patients with IPF and 157 healthy controls. The SNPs were determined using the polymerase chain reaction in association with sequence-specific primers incorporating mismatches at the 3'-end.Results
Our results showed that these polymorphisms were distributed similarly in the IPF and control groupsConclusion
We conclude that these two potentially important candidate gene single nucleotide polymorphisms are not associated with susceptibility to IPF. 相似文献7.
8.
Background
To assess psychosocial functioning and distress of children and adolescents with cystic fibrosis compared to healthy controls.Methods
Thirty-six patients with cystic fibrosis aged 8–18 years (24 boys, mean age?±?SD: 11.5?±?2.6 years) and 31 sex- and age-matched healthy control subjects (18 boys, mean age?±?SD: 12?±?2.5 years) were enrolled in the study. In order to assess the self-esteem, social adjustment, and family functioning of these young people, the Culture-free Self-esteem Inventory, the Social Adjustment Scale–Self-Report, and the Family Assessment Device were administered. Emotional/ behavioral problems were assessed through the Youth Self Report and the Child Behavior Checklist given to both the subjects and their parents.Results
No significant differences were found for self-esteem between the two study groups. Regarding social adjustment, children with cystic fibrosis reported significantly worse friendship and overall adjustment (P?<?0.05). Moreover, no difference was found in the levels of family functioning between the two groups. No significant differences between the groups were found in emotional/ behavioral problems from the self-reports. On the contrary, parents of children with cystic fibrosis reported significantly higher levels of withdrawal/ depression, thought problems, and delinquent behavior (P?≤?0.01) as compared to controls.Conclusions
Children and adolescents with cystic fibrosis appear to be a psychosocially vulnerable group. A biopsychosocial approach should emphasize the assessment and treatment of the psychosocial distress of these patients alongside multiple somatic treatments.9.
Dorothy A Sparks Daniel M Chase Mark Forsyth Gregg Bogen Jon Arnott 《Journal of medical case reports》2010,4(1):1-3
Introduction
Craniosynostosis can affect the skull in various ways. The most common forms are abnormal skull shape and beaten copper pattern, while Lückenschädel (or lacunar skull) is one of the least common forms.Case presentation
We report the case of a 3-month-old Caucasian boy with multiple suture craniosynostosis and with acquired craniomeningocele presenting as a bulging mass in the lateral occipital area.Conclusion
To the best of our knowledge, this is the first report of a patient with multiple suture craniosynostosis and acquired craniomeningocele. 相似文献10.
Endoscopic application of n-butyl-2-cyanoacrylate on esophagojejunal anastomotic leak: a case report
Manousos-Georgios Pramateftakis Georgios Vrakas Ioannis Kanellos Ioannis Mantzoros Stamatis Angelopoulos Efthymios Eleftheriades Charalampos Lazarides 《Journal of medical case reports》2011,5(1):1-3
Introduction
Chryseobacterium indologenes is an uncommon human pathogen. Most infections have been detected in hospitalized patients with severe underlying diseases who had indwelling devices implanted. Infection caused by C. indologenes in a newborn has not been previously reported.Case presentation
We present a case of ventilator-associated pneumonia caused by C. indologenes in a full-term Caucasian newborn baby boy with congenital heart disease who was successfully treated with piperacillin-tazobactam.Conclusion
C. indologenes should be considered as a potential pathogen in newborns in the presence of invasive equipment or treatment with long-term broad-spectrum antibiotics. Appropriate choice of effective antimicrobial agents for treatment is difficult because of the unpredictability and breadth of antimicrobial resistance of these organisms, which often involves resistance to many of the antibiotics chosen empirically for serious Gram-negative infections. 相似文献11.
12.
David Cassiman Renate Zeevaert Elisabeth Holme Eli-Anne Kvittingen Jaak Jaeken 《Orphanet journal of rare diseases》2009,4(1):1-6
Background
A case series of the cardiac magnetic resonance imaging findings in seven adult Alström patients.Methods
Seven patients from the National Specialist Commissioning Group Centre for Alström Disease, Torbay, England, UK, completed the cardiac magnetic resonance imaging protocol to assess cardiac structure and function in Alström cardiomyopathy.Results
All patients had some degree of left and right ventricular dysfunction. Patchy mid wall gadolinium delayed enhancement was demonstrated, suggesting an underlying fibrotic process. Some degree of cardiomyopathy was universal. No evidence of myocardial infarction or fatty infiltration was demonstrated, but coronary artery disease cannot be completely excluded. Repeat scanning after 18 months in one subject showed progression of fibrosis and decreased left ventricular function.Conclusion
Adult Alström cardiomyopathy appears to be a fibrotic process causing impairment of both ventricles. Serial cardiac magnetic resonance scanning has helped clarify the underlying disease progression and responses to treatment. Confirmation of significant mutations in the ALMS1 gene should lead to advice to screen the subject for cardiomyopathy, and metabolic disorders. 相似文献13.
Xander W Huijsdens Beatrix J van Dijke Emile Spalburg Marga G van Santen-Verheuvel Max EOC Heck Gerlinde N Pluister Andreas Voss Wim JB Wannet Albert J de Neeling 《Annals of clinical microbiology and antimicrobials》2006,5(1):1-4
Background
Female genital tuberculosis is an uncommon disease that is rarely diagnosed in developed countries.Case presentation
A 61-year-old postmenopausal woman who had undergone surgery and treated with adjuvant chemotherapy for infiltrating ductal carcinoma of the breast five years ago, presented with bloody vaginal discharge, fatigue, weight loss, and low grade fevers at night for two months. Histological examination of the endometrium, done based on the suspicion of a second primary cancer due to the tamoxifen therapy, revealed a granulomatous reaction. Liquid and solid mycobacterial cultures of the tissues were performed. Although the acid fast staining was negative, the liquid culture was positive for Mycobacterium tuberculosis. Involvement of other systems was not detected. The patient was treated with a three-drug antituberculosis regimen for 9 months and recovered fully.Conclusion
Female genital tuberculosis is a rare but curable disease that should be included in the differential diagnosis of women with menstrual problems. Early diagnosis is important and may prevent unnecessary invasive procedures for the patient. 相似文献14.
Harriet Corvol Nadia Nathan Celine Charlier Katarina Chadelat Philippe Le Rouzic Olivier Tabary Brigitte Fauroux Alexandra Henrion-Caude Josue Feingold Pierre-Yves Boelle Annick Clement 《Respiratory research》2007,8(1):88-9
Background
The variability in the inflammatory burden of the lung in cystic fibrosis (CF) patients together with the variable effect of glucocorticoid treatment led us to hypothesize that glucocorticoid receptor (GR) gene polymorphisms may affect glucocorticoid sensitivity in CF and, consequently, may contribute to variations in the inflammatory response.Methods
We evaluated the association between four GR gene polymorphisms, TthIII, ER22/23EK, N363S and BclI, and disease progression in a cohort of 255 young patients with CF. Genotypes were tested for association with changes in lung function tests, infection with Pseudomonas aeruginosa and nutritional status by multivariable analysis.Results
A significant non-corrected for multiple tests association was found between BclI genotypes and decline in lung function measured as the forced expiratory volume in one second (FEV1) and the forced vital capacity (FVC). Deterioration in FEV1 and FVC was more pronounced in patients with the BclI GG genotype compared to the group of patients with BclI CG and CC genotypes (p = 0.02 and p = 0.04 respectively for the entire cohort and p = 0.01 and p = 0.02 respectively for F508del homozygous patients).Conclusion
The BclI polymorphism may modulate the inflammatory burden in the CF lung and in this way influence progression of lung function. 相似文献15.
Katja Lakota Jun Wei Mary Carns Monique Hinchcliff Jungwha Lee Michael L Whitfield Snezna Sodin-Semrl John Varga 《Arthritis research & therapy》2012,14(3):R102-6
Introduction
Progressive fibrosis in systemic sclerosis (SSc) is linked to aberrant transforming growth factor beta (TGF-beta) signaling. Peroxisome proliferator-activated receptor gamma (PPAR-gamma) blocks fibrogenic TGF-beta responses in vitro and in vivo. Reduced expression and function of PPAR-gamma in patients with SSc may contribute to progression of fibrosis. Here we evaluated the levels of adiponectin, a sensitive and specific index of PPAR-gamma activity, as a potential fibrogenic biomarker in SSc.Methods
Adiponectin levels were determined in the sera of 129 patients with SSc and 86 healthy controls, and serial determinations were performed in 27 patients. Levels of adiponectin mRNA in skin biopsies from SSc patients were assessed in an expression profiling microarray dataset. Regulation of adiponectin gene expression in explanted human subcutaneous preadipocytes and fibroblasts was examined by real-time quantitative PCR.Results
Patients with diffuse cutaneous SSc had reduced serum adiponectin levels. A significant inverse correlation between adiponectin levels and the modified Rodnan skin score was observed. In longitudinal studies changes in serum adiponectin levels were inversely correlated with changes in skin fibrosis. Skin biopsies from a subset of SSc patients showed reduced adiponectin mRNA expression which was inversely correlated with the skin score. An agonist ligand of PPAR-gamma potently induced adiponectin expression in explanted mesenchymal cells in vitro.Conclusions
Levels of adiponectin, reflecting PPAR-gamma activity, are correlated with skin fibrosis and might have potential utility as a biomarker in SSc. 相似文献16.
Jai Prakash Muyal Vandana Muyal Brajesh Pratap Kaistha Carola Seifart Heinz Fehrenbach 《Diagnostic pathology》2009,4(1):1-8
Background
Adenoid cystic carcinomas are rare tumors with an indolent clinical course, but frequent local relapses. The identification of tumors with a higher relapse risk seems to be interesting. Hence we investigated parameters of glucose metabolism, which were found associated with poor prognosis in other malignancies.Methods
Specimen of 29 patients were investigated immunohistochemically with antibodies against p-AKT, TKTL-1 (transketolase-like 1), M2PK (M2 pyruvate kinase), and GLUT-1. Proliferation was investigated by staining with Ki67. The tumors were located at the major or minor salivary glands. Only the typical cribriform subtype was investigated. The initial tumor stage was pT1 or pT2.Results
Expression of p-AKT was significantly (P = 0.036) associated with a higher relapse risk in multivariate analysis. Low expression of M2PK was non-significantly (P = 0.065) predictive for a higher risk. TKTL-1 and GLUT-1 were expressed in the majority of cases, albeit not associated with relapse risk.Conclusion
Adenoid cystic carcinomas positive for p-AKT show a higher relapse risk. However, other parameters of glucose metabolism investigated here or proliferation (Ki67) were not predictive in this entity. Our findings demonstrate a possible background for therapeutic approaches targeting the inhibition of PI3K/AKT pathway. 相似文献17.
Introduction
Secondary infertility is defined as the inability to achieve a new pregnancy for a couple that has been able to procreate in the past.Material and methods
Over a 16-year period, 49 patients consulted for male secondary infertility. Clinical, laboratory or ultrasound assessment demonstrated the cause of infertility.Results
These patients had a mean age of 33.6 years (range: 26 to 44 years). These couples had an average of 1 living child per family, corresponding to a girl in two-thirds of cases and a boy in one-third. In these patients, infertility was due to the presence of varicocele in 15 patients (31% of cases), infection in 14 patients (29%), and varicocele associated with infection in 11 cases (22%).Conclusion
This assessment revealed two main aetiologies, but some causes remain obvious, such as surgery with resection of the prostate or testicles, or certain cytotoxic therapies. 相似文献18.
19.
Yamamoto Daigo Yamada Masanori Okugawa Homa Tanaka Kanji 《World journal of surgical oncology》2004,2(1):1-4
Background
Malignant pleural mesothelioma is a rare malignancy. The outcome remains poor despite complete surgical resection.Patients and methods
Eleven patients with histologicaly proven epithelial type malignant pleural mesothelioma undergoing extrapleural pneumonectomy with systemic chemotherapy and/or radiotherapy before and after surgical resection were retrospectively reviewed.Results
Ten out of 11 patients underwent complete surgical resection, of these 7 patients had stage I disease. Of these 7 patients, 5 are alive without any recurrence, a 2-year survival rate of 80% was observed in this group. There was no operative mortality or morbidity.Conclusion
Extrapleural pneumonectomy with perioperative adjuvant treatment is safe and effective procedure for epithelial type malignant pleural mesothelioma. 相似文献20.
Halil Ibrahim Secer Ilker Solmaz Ihsan Anik Yusuf Izci Bulent Duz Mehmet Kadri Daneyemez Engin Gonul 《Journal of brachial plexus and peripheral nerve injury》2009,4(1):1-10