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101.
Nihal Fathi Samar H. Goma Nadia M. Ismail Abeer M. Ghandour Sally S. Youssef Nisreen A. Mohammed Eman Mosad 《The Egyptian Rheumatologist》2018,40(1):45-49
Background
Systemic sclerosis (SSc) is an autoimmune connective tissue disease with vascular, fibrotic and immune changes of skin and some internal organs. Anti-heterogeneous nuclear ribonucleoproteins (anti-hnRNP) were found in SSc patients.Aim of the work
To assess anti-hnRNP A1 and A2 autoantibodies in limited SSc patients and to find their relation to clinical and hand radiographic characteristics.Patients and methods
26 limited SSc patients and 16 matched control were studied. Skin thickness was scored according to the modified Rodnan skin score method (mRss) and radiologic examination by plain X-ray of the hand and wrist was performed anti-hnRNP A1 and A2 were measured in patients and control.Results
All patients were females with a mean age of 37.5 ± 11.24 years and mean disease duration of 7.84 ± 1.19 years. 96.2% of cases showed juxta-articular osteoporosis, 38.5% with marginal erosions, 73.1% with surface erosions, 42.3% with subchondral cyst, 42.3% with metacarpophalangeal subluxation, 11.5% with marginal sclerosis, 80.8% with resorption of distal phalanges, 38.5% with resorption of distal ulna and 34.6% with calcinosis. Anti-hnRNPA1 was positive in all the patients but the anti-hnRNPA2 was positive in 21 (80.8%). Anti-hnRNP A1 and A2 showed significant difference between patients and control (5.66 ± 4.18 ng/ml vs 2.88 ± 0.82; p < 0.01 and 1.82 ± 0.36 vs 0.73 ± 0.08; p < 0.02, respectively). There was no significant correlation between the markers with the mRss or radiographic changes.Conclusion
Joint affection in SSc is more frequent than expected. Anti-hnRNP A1 and anti hnRNP A2 antigens may be useful markers for SSc patient although no significant relation was found with radiologic findings. 相似文献102.
Objective Familial Mediterranean fever (FMF) is an autosomal recessive recurrent polyserositis with a higher prevalence in some ethnic groups, including Turks. Mutations in the FMF gene (MEFV) were found associated with FMF. The aim of this study was to analyze MEFV gene mutations in FMF patients to gain insight into the mutation phenotype correlation.Objectives We analyzed the most frequent mutations (M680I, M694V, V726A, and E148Q) in a group of young male Turkish FMF patients using an amplification refractory mutation system and a commercial kit.Results M694V mutation was detected in 80% of the patients. After making a strict diagnostic discrimination between arthralgia and arthritis, arthritis was present in 71% of homozygous and 29.4% of heterozygous patients for M694V mutation. Other mutations were not found to correlate with specific symptoms or findings.Conclusion The homozygosity of M694V mutation in the MEFV gene is associated with arthritis in FMF patients. 相似文献
103.
Sousa Rd França A Dória Nòbrega S Belo A Amaro M Abreu T Poças J Proença P Vaz J Torgal J Bacellar F Ismail N Walker DH 《The Journal of infectious diseases》2008,198(4):576-585
BACKGROUND: The pathophysiologic mechanisms that determine the severity of Mediterranean spotted fever (MSF) and the host-related and microbe-related risk factors for a fatal outcome are incompletely understood. METHODS: This prospective study used univariate and multivariate analyses to determine the risk factors for a fatal outcome for 140 patients with Rickettsia conorii infection admitted to 13 Portuguese hospitals during 1994-2006 with documented identification of the rickettsial strain causing their infection. RESULTS: A total of 71 patients (51%) were infected with the Malish strain of Rickettsia conorii, and 69 (49%) were infected with the Israeli spotted fever (ISF) strain. Patients were admitted to the intensive care unit (40 [29%]), hospitalized as routine inpatients (95[67%]), or managed as outpatients (5[4%]). Death occurred in 29 adults (21%). A fatal outcome was significantly more likely for patients infected with the ISF strain, and alcoholism was a risk factor. The pathophysiology of a fatal outcome involved significantly greater incidence of petechial rash, gastrointestinal symptoms, obtundation and/or confusion, dehydration, tachypnea, hepatomegaly, leukocytosis, coagulopathy, azotemia, hyperbilirubinemia, and elevated levels of hepatic enzymes and creatine kinase. Some, but not all, of these findings were observed more often in ISF strain-infected patients. CONCLUSIONS: Although fatalities and similar clinical manifestations occurred among both groups of patients, the ISF strain was more virulent than the Malish strain. Multivariate analysis revealed that acute renal failure and hyperbilirubinemia were most strongly associated with a fatal outcome. 相似文献
104.
Plemper RK Erlandson KJ Lakdawala AS Sun A Prussia A Boonsombat J Aki-Sener E Yalcin I Yildiz I Temiz-Arpaci O Tekiner B Liotta DC Snyder JP Compans RW 《Proceedings of the National Academy of Sciences of the United States of America》2004,101(15):5628-5633
Measles virus (MV) constitutes a principal cause of worldwide mortality, accounting for almost 1 million deaths annually. Although a live-attenuated vaccine protects against MV, vaccination efficiency of young infants is low because of interference by maternal antibodies. Parental concerns about vaccination safety further contribute to waning herd immunity in developed countries, resulting in recent MV outbreaks. The development of novel antivirals that close the vaccination gap in infants and silence viral outbreaks is thus highly desirable. We previously identified a microdomain in the MV fusion protein (F protein) that is structurally conserved in the paramyxovirus family and constitutes a promising target site for rationally designed antivirals. Here we report the template-based development of a small-molecule MV inhibitor, providing proof-of-concept for our approach. This lead compound specifically inhibits fusion and spread of live MV and MV glycoprotein-induced membrane fusion. The inhibitor induces negligible cytotoxicity and does not interfere with receptor binding or F protein biosynthesis or transport but prevents F protein-induced lipid mixing. Mutations in the postulated target site alter viral sensitivity to inhibition. In silico docking of the compound in this microdomain suggests a binding model that is experimentally corroborated by a structure-activity analysis of the compound and the inhibition profile of mutated F proteins. A second-generation compound designed on the basis of the interaction model shows a 200-fold increase in antiviral activity, creating the basis for novel MV therapeutics. This template-based design approach for MV may be applicable to other clinically relevant members of the paramyxovirus family. 相似文献
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108.
Muammer Ozcimen Yasar Sakarya Sertan Goktas Rabia Sakarya Ismail Alpfidan Halil I. Yener 《Cutaneous and ocular toxicology》2015,34(4):313-317
Objective: The purpose of this study was to examine the vasoreactivity in retina and choroid of the healthy eyes in response to experimentally altered partial arterial pressure of carbon dioxide (PaCO2) using a non-invasive technique, spectral domain optical coherence tomography (SD-OCT).Materials and methods: The study included non-smoking participants between 18 and 35 years of age, having visual acuity of 20/20 and with no systemic and ocular diseases. At baseline, the participants breathed room air (normocapnia). Hypocapnia was created with the help of hyperventilation; for this, the participants were instructed to draw deep and quick breaths, resulting one breathing cycle per 2?s. To create hypercapnia subjects rebreathed from a 5?l bag at least 3?min. Choroidal thickness and retinal artery diameter were measured at baseline, and hyperventilation and rebreathing conditions by SD-OCT.Results: Twenty eyes of 20 healthy subjects were included in this study. Their mean age was 24.90?±?5.32 years. Hyperventilation caused a significant reduction in choroidal thickness, compared with baseline, at all points; whereas rebreathing caused no significant change at all points. The mean diameters of the arteries were 151.80?±?7.88?μm, with a significant decline to 148.90?±?7.25?μm at hyperventilation condition and a significant increase to 153.50?±?7.88?μm at rebreathing condition (p?=?0.018, p?=?0.043, respectively).Conclusion: This study demonstrated that, SD-OCT was a useful tool in measuring the ocular vascular response under hypercapnia and hypocapnia conditions. These findings may be helpful for further understanding the physiological nature of ocular blood flow and this preliminary study provides a basis for future studies. 相似文献
109.
Kamal Faisal Khan Muhammad Ali Lee-Smith Wade Sharma Sachit Marella Hemnishil K. Iqbal Umair Mcdonough Stephanie Aslam Aysha Ismail Mohammad K. Tombazzi Claudio Adler Douglas G. 《Digestive diseases and sciences》2021,66(10):3242-3249
Digestive Diseases and Sciences - Esophageal diverticula can cause significant symptoms and affect the quality of life. There has been recent interest in the use of peroral endoscopic myotomy in... 相似文献
110.
Sari I Altuntas F Hacioglu S Kocyigit I Sevinc A Sacar S Deniz K Alp E Eser B Yildiz O Kaynar L Unal A Cetin M 《American journal of hematology》2008,83(4):334-339
The aim of the study is to review the clinical manifestations and the hematological findings of brucellosis and pancytopenia, with or without hematological malignancies. The records of 202 patients with brucellosis were evaluated retrospectively. Among these cases of brucellosis seen in a 6 year period between April 1999 and June 2005, 30 patients with pancytopenia were identified. The most common manifestation was fever, followed by weight loss, anorexia, malaise, arthralgia, and hepatosplenomegaly. Bone marrow biopsies revealed hypercellularity or normocellularity. The most common findings in the bone marrow evaluation were histiocytic hemophagocytosis and granulomas. Among all cases, we diagnosed 5 hematological malignancies (1 acute myelogenous leukemia, 2 acute lymphoblastic leukemia, and 2 multiple myeloma) concurrently with brucellosis. The clinical symptoms and findings were similar in patients with and without malignancies. In cases with malignancies, the bone marrow biopsy revealed predominant primary disease involvement. Significant increases in ESR and CRP, severe anemia and thrombocytopenia were observed in patients with malignancies. Peripheral blood counts in patients without malignancies returned to normal after antibiotic treatment for brucellosis. However, pancytopenia in two patients with malignancies did not recover because of primary resistant disease. We conclude that while histiocytic hemophagocytosis may be considered as a major cause of pancytopenia, leukemic infiltration can also be an extreme and unusual cause of pancytopenia in patients in whom brucellosis was concurrently diagnosed with hematological malignancies. 相似文献