mohammad bemanian

Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare primary immunodeficiency disorder characterized by recurrent infections and low immunoglobulin levels due to variable combined immunodeficiency, and centromeric region instability, and facial dysmorphism. We describe a 12-year-old boy with recurrent respiratory tract infections, facial anomalies, scoliosis, and psychomotor retardation. He had recurrent pneumonia with low serum IgG and IgM levels during infancy and preschool age. Later at the age of 10, he developed recurrent ear infections. An IgA and IgM deficiency was found accompanied by a normal B-cell and T-cell count as well as an impaired candida-induced T-cell proliferation. Further evaluations revealed a missense mutation in the DNMT3B gene on chromosome 20. Chromosomal analysis showed a sunburst multi-radial feature on chromosome 1, which is a hallmark of ICF syndrome. The genetic mutation and chromosomal abnormality along with clinical findings are compatible with the diagnosis of ICF syndrome. To the best of our knowledge, this is the first time that scoliosis is observed in an ICF patient. The additional variable clinical symptoms in the case were the presence of spastic gait as well as hypogammaglobulinemia with immunoglobulin isotype switch at different ages.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may be triggered by some diseases and medications. For the latter one, non-steroidal anti-inflammatory drugs (NSAIDs) have been identified as one of the potential causative agents to develop LABD. Here, a rare case of drug-induced LABD is introduced. A 13-month-old Iranian boy presented with a history of generalized blisters, displaying the classic “string of pearls” sign who was eventually diagnosed as a case of LABD. In his admission, he was diagnosed whit Mucocutaneous lymph node syndrome and treated with aspirin.  Some features like appearing the characteristic lesions one week following the administration of aspirin, rapid clearance of lesions after the withdrawal of the drug, and reappearance of new lesions after readministration of aspirin were highly suggestive of aspirin-induced LABD. To establish the diagnosis, we used the “Naranjo probability score” which determined the probable causative role of aspirin. The diagnosis was confirmed by showing the positive IgA deposition in the basement membrane zone in a direct immunofluorescence study of the skin biopsy. The child was treated with dapsone with dramatical response to the drug.

Asthma induced by ingestion of aspirin occurs when symptoms arise within 30 minutes to three hours after aspirin consumption. Previous data indicate that sensitivity to aspirin may be associated with poorly controlled asthma. This study aims to evaluate the frequency of aspirin sensitivity in patients with moderate to severe asthma receiving conventional asthma therapy. This clinical trial was conducted on 65 patients aged 18 to 65 years with moderate to severe asthma from February 2015 to February 2016 at the Allergy Department, Hazrat-e-Rasoul Hospital, Iran University of Medical Sciences, Tehran. To assess treatment responses in patients, forced expiratory volume in the first second (FEV1) and asthma control test (ACT) scores were measured at baseline and after 3 months. The results of the oral aspirin challenge revealed a prevalence of 35.38% for sensitivity to aspirin. Hypersensitivity reactions to aspirin were detected in 60.9% of the patients with moderate asthma and 39.1% of the patients with severe asthma. All patients with positive aspirin challenge tests suffered from rhinosinusitis and in 56.5% of cases, history of previous hypersensitivity reactions to non-steroidal anti-inflammatory drugs (NSAIDs) was detected. No meaningful differences were found between those patients with aspirin sensitivity and those with aspirin tolerance neither in mean pre-bronchodilator FEV1 nor in ACT scores pre- and post-treatment. To conclude, aspirin sensitivity was not found to have an association with an unfavorable response to conventional treatment in patients with uncontrolled asthma.

Sesame food allergy (SFA); especially anaphylaxis, is a life-threatening condition. The accurate diagnosis of SFA is done by skin prick test (SPT), skin prick to prick (SPP) or specific IgE (sIgE) and is confirmed by oral food challenge (OFC). Since there are few studies evaluating and comparing the utility of these methods for diagnosis of sesame anaphylaxis in adult patients, we aimed to compare OFC with diagnostic tests, including SPT, SPP, and sesames IgE; using ImmunoCAP considering the sensitivity and specificity issues in patients with sesame anaphylaxis. Twenty patients with sesame anaphylaxis were diagnosed based on OFC. Then SPT, SPP, and sIgE were evaluated. Sixteen patients had positive OFC; while 4 patients had negative results. Out of 16 OFC+ patients, 7 patients were SPT+, 15 patients were SPP+, and 2 patients had detectable sIgE. A positive SPT indicated 44% sensitivity and 50% specificity. A positive SPP showed 87.5% sensitivity and 75% specificity. A positive ImmunoCAP test demonstrated 12.5% sensitivity and 75% specificity. The AUC of SPP was significant for the diagnosis of sesame anaphylaxis (p=0.038). In conclusion, when the OFC is not possible, the SPP test with natural sesame seed may be applicable in patients with a convincing history instead of the artificial or commercial extracts of sesame used for SPT. Positive SPP is a good alternative diagnostic method for patients with sesame anaphylaxis. Also, the poor sensitivity of SPT and sIgE may indicate the poor discriminative capability of these tests.

Analysis on Meaning Quality of Urban Public Places, with Special Focus on Yazd, Iran

The discussion below can be read as a call for more rigorous and transparent explication of philosophical commitments and implications of one’s chosen methodological standpoint.Physical structure, function and perception is led to place. Place by imagination and memory is defied. Nowadays, urban public spaces are in the lack of meaning they cannot perfectly be percepted by citizens. The purpose of this research is proposing, effective indicators in perception of citizens in urban public places. Meaning quality of urban public places is a big concern. Various indices shape meaning quality of urban public places. Some indices have more roles in perception quality and some of them less. Finding these indices could help planners and designers to improve meaning quality from the perspective of citizens.

Studied area The studied area is Yazd city in Iran country. So, Yazd province is located in the center of the country, and its center is city of Yazd. This city have about 582682 population. The city of Yazd is the economic and administrative capital of the province and therefore the most heavily populated. Yazd is one of the best Iranian Architecture samples. It is one of the major and historical cities in the world and this city is preparing a historic texture portfolio to register in UNESCO. This research done on Yazd Materials and methods The aim of research is, introducing indices in perception the quality of urban public places in the view of Yazd (Iran) citizens. So, the research method is "descriptive- analytical". Also, survey analysis method was used. At first, the literature was reviewed due to proposed indicators. In order to analyzing indicators the sample size is 384. So, the questionnaire was used. On the other hand, T-test and Friedman test (Non Parametric Test, K Related Samples), in SPSS, used to analysis data.

a. Proposed indicators Based on research method, at first scientific studies has been reviewed. So, indicators have been concluded from some ideas such as: Lynch (1981), Violich (1983), Bentli (2005), Coleman (1987), Alen Jacobs & Appelyard (1998), South worth, (1989), Greene(1992), Haughton & Hunter (1994), Punter & Carmona (1997), Carmona (2003), Also, National and international institutions such as PPS, theoretical issues about the quality and the quality of the survey have developed during the past few decades. Table 1 shows, the components of urban space quality in scientific ideas. According to this table twenty two indexes select for analysis in the semi Delfi process. These twenty tow indexes are: Eco-friendly form, Visual compatibility, Visual character, Accessibility, Walk- ability, Environment for all, Social reaction, Variety land use, Customizable, Efficiency, Happiness, Richness, Friendly, According to past values or historical, Eligibility, Learning, Personalization, Security, Sense of time, Culturally, Meanings, manifestations and spiritual themes, Permeability.Table 1: components of urban space quality in scientific ideas

b. Descriptive findingsIn terms of gender structure about half of the respondents were male and half of them were female. Also, more than half of them were single and others were married. The average age of the subjects was 34 years. In terms of education, about 40 percent of respondents had a master's degree and 30 percent of participation had bachelor degree. The birthplace of half respondents in this study is Yazd city and others were born in other cities. The participants were residing in Yazd city.

c. Analytical findingsMeasuring the perception of residents (In terms of divided squares)The participants were asked to express their judgments about the quality of urban public places in order to evaluate the effect of the 22 indicators on the perception of respondents and their judgment about the meaning quality. Answers were analyzed using one-sample t-test and the results are presented in

Table2

Table 2: analyzed indicators The values more than 3 for each indicator represent significant effect on the quality of judgment in the perception of citizens. According to this analysis, these indicators i.e., variety of land use, Social interactions, Sense of time, The environment for all, Welcoming and friendly, Culturally, efficiency have average above 3 and have been effective to understand the meaning of place quality in Yazd city. The results showed that other indicators are not effective on respondents' perception of the quality squares.Respondent’s prioritization of the meaning quality indicesAchieving to the importance of each indicator in shaping the perceptual quality of the squares was important result of this study that will be used in the future by others. To prioritize the respondents' perception of meaning quality indicators, Friedman test was used. Table 3 shows the results of this analysis.Table 3.The results of Friedman testThe results of Friedman test confirms that there are significant difference between twenty tow indicator’s impacts on meaning quality of spaces perception. It should be noted that the most important indicator in the formation of meaning quality are Environment for all, accessibility and efficiency.Conclusion Analyzing the meaning quality of urban public spaces by means of quantitative methods and experimental models is an approach that is used to identify the overall level of quality. Meanwhile, access to reliable results, close to reality, can be used to planning and decision-making around proceeding strategy and physical, non-physical intervention on public places. This study was done based on using these approaches of measuring environmental quality. This paper indicate that among the indices for all 22 indicators, three indicators (Environment for all, accessibility and efficiency) have the most effect on percept meaning quality. Amir Chaqmaq square has the best quality between Yazd squares. According to past values or historical was the main index in meaning quality of Amir Chaqmaq square.