97 results on '"Behniafard N"'
Search Results
2. Non-Infectious Complications in B-Lymphopenic Common Variable Immunodeficiency
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Pashangzadeh, S, primary, Delavari, S, additional, Shad, TM, additional, Salami, F, additional, Rasouli, SE, additional, Yazdani, R, additional, Mahdaviani, SA, additional, Nabavi, M, additional, Aleyasin, S, additional, Ahanchian, H, additional, Azad, FJ, additional, Chavoshzadeh, Z, additional, Nazari, F, additional, Momen, T, additional, Sherkat, R, additional, Abolnezhadian, F, additional, Esmaeilzadeh, H, additional, Fallahpour, M, additional, Arshi, S, additional, Bemanian, MH, additional, Shokri, S, additional, Ebrahimi, SS, additional, Abolmolouki, M, additional, Farid, AS, additional, Rezaei, A, additional, Esmaeili, M, additional, Kalantari, A, additional, Sadeghi-Shabestari, M, additional, Shirkani, A, additional, Behniafard, N, additional, Khalili, A, additional, Eslamian, MH, additional, Cheraghi, T, additional, Shafie, A, additional, Tavakol, M, additional, Khoshkhui, M, additional, Iranparast, S, additional, Shamshiri, M, additional, Shahri, MA, additional, Khazaei, R, additional, Asadi, M, additional, Babaha, F, additional, Aghamohammadi, A, additional, Rezaei, N, additional, and Abolhassani, H, additional
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- 2023
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3. Interleukin-6 and tumor necrosis factor-alpha gene polymorphisms in chronic idiopathic urticaria
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Tavakol, M., Amirzargar, A.A., Movahedi, M., Aryan, Z., Bidoki, A.Z., Gharagozlou, M., Aghamohammadi, A., Nabavi, M., Ahmadvand, A., Behniafard, N., Heidari, K., Soltani, S., and Rezaei, N.
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- 2014
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4. Association of single nucleotide polymorphisms of interleukin-1 family with atopic dermatitis
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Behniafard, N., Gharagozlou, M., Sotoudeh, S., Farhadi, E., Khaledi, M., Moghaddam, Z.G., Mahmoudi, M., Fathi, S.M., Darabi, B., Aghamohammadi, A., Amirzargar, A.A., and Rezaei, N.
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- 2014
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5. Correlation Between Peripheral and Central Venous Pressures in Children with Congenital Heart Disease
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Amoozgar, H., Behniafard, N., Borzoee, M., and Ajami, G. H.
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- 2008
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6. Investigating the Prevalence of Human Papilloma Virus in Squamous Cell Carcinoma of the Larynx and Its Correlation with Disease Prognosis
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saeid atighechi, Meybodian, M., Dadgarnia, M. H., Baradaranfar, M. H., and Behniafard, N.
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Risk ,Survival ,virus diseases ,Original Article ,Squamous Cell Carcinoma ,Larynx ,lcsh:Otorhinolaryngology ,Human Papilloma Virus (HPV) ,lcsh:RF1-547 - Abstract
Introduction: The human papilloma virus (HPV) can play a role in the development of head and neck squamous cell carcinoma (SCC). Our aim was to assess the prevalence of HPV DNA in SCC of the larynx. The impact of HPV infection on patient survival was also evaluated. Materials and Methods: This case-control study was performed in 44 patients with SCC of the larynx (case group), while the control group comprised samples obtained from cadavers with no previous history of malignancy. A preliminary pathologic evaluation was performed on all samples in the control group (36 samples) to ensure the absence of dysplasia or malignancy. Polymerase chain reaction (PCR) was used to detect HPV DNA. After completing the treatment protocol, patients were followed to assess the impact of HPV infection on overall survival (OS). Results: PCR evaluation in the case group showed that HPV DNA was successfully isolated from 11 (25%) samples, while only two (5.6%) HPV DNA-positive were obtained from cadavers. According to these results, a significant difference was obtained in the prevalence of HPV DNA and laryngeal SCC between cases and controls (P=0.031). No statistically significant difference was observed in the OS of patients with or without HPV infection in the case group (P=0.235). Conclusion: Based on these results, we suggest that the prevalence of HPV infection is higher in laryngeal SCC subjects compared with healthy individuals. Although a longer OS was seen in HPV-positive patients, survival analysis did not show a significant difference in the comparison of HPV-positive and negative findings in SCC patients.
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- 2016
7. Infectious etiology of chronic diarrhea in patients with primary immunodeficiency diseases
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Parvaneh, L., primary, Sharifi, N., additional, Azizi, G., additional, Abolhassani, H., additional, Sharifi, L., additional, Mohebbi, A., additional, Bahraminia, E., additional, Delavari, S., additional, Alebouyeh, M., additional, Tajeddin, E., additional, Mohebbi, S.R., additional, Yazdani, R., additional, Behniafard, N., additional, and Aghamohammadi, A., additional
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- 2019
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8. Association Between Single Nucleotide Polymorphisms of the Interleukin-4 Gene and Atopic Dermatitis
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Gharagozlou, M., Behniafard, N., Amirzargar, A. A., Hosseinverdi, S., Sotoudeh, S., Farhadi, E., Khaledi, M., Zahra Aryan, Moghaddam, Z. G., Mahmoudi, M., Aghamohammadi, A., and Rezaei, N.
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Male ,Genotype ,Incidence ,Iran ,Polymorphism, Single Nucleotide ,Risk Assessment ,Dermatitis, Atopic ,Cohort Studies ,Haplotypes ,Reference Values ,Case-Control Studies ,Child, Preschool ,Confidence Intervals ,Odds Ratio ,Humans ,Female ,Genetic Predisposition to Disease ,Interleukin-4 ,Promoter Regions, Genetic ,Alleles - Abstract
Atopic dermatitis (AD) is an inflammatory skin disease in which both genetic and environmental factors seem to be involved. Several studies investigated the association of certain genetic factors with AD in different ethnic groups, but conflicting data were obtained. This study was performed to check the possible association between single nucleotide polymorphisms (SNPs) of interleukin 4 (IL-4) and the IL-4 receptor α chain (IL-4Rα) and AD in a group of Iranian patients. The allele and genotype frequencies of genes encoding for IL-4 and IL-4Rα were investigated in 89 patients with AD in comparison with 139 healthy controls, using methods based on polymerase chain reaction sequence-specific primers. The most frequent alleles of IL-4 in patients were T at -1098 (P Key words: atopic dermatitis; polymorphism, single nucleotide; interleukin-4 gene
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- 2015
9. Human papilloma virus and nasopharyngeal carcinoma: pathology, prognosis, recurrence and mortality of the disease
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Atighechi S, Ahmadpour Baghdadabad MR, Sa, Mirvakili, Mohammad Hasan Sheikhha, Mh, Baradaranfar, Mh, Dadgarnia, and Behniafard N
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Adult ,Aged, 80 and over ,Male ,Nasopharyngeal Carcinoma ,Adolescent ,Carcinoma ,Papillomavirus Infections ,Nasopharyngeal Neoplasms ,Chemoradiotherapy ,Middle Aged ,Prognosis ,Polymerase Chain Reaction ,Survival Rate ,Young Adult ,DNA, Viral ,Carcinoma, Squamous Cell ,Humans ,Female ,Neoplasm Recurrence, Local ,Papillomaviridae ,Aged ,Follow-Up Studies ,Neoplasm Staging - Abstract
One of the malignant tumors among head and neck cancers is nasopharyngeal carcinoma. Many studies consider human papilloma virus (HPV) as a cause for nasopharyngeal carcinoma.41 paraffin-wax-embedded block samples were examined to detect HPV DNA and its subtype's presence by polymerase chain reaction. The recurrence, prognosis and survival were evaluated for an average of 48 months.HPV DNA was positive in 9 patients (22%). The overall recurrence rate was 75% in HPV negative patients and 11% in HPV positive ones. The mortality rate in HPV negative and positive patients was 37.5% and 0%, respectively.HPV type 18 and 16 were the most common subtypes. Also, it can be implied that patients which are HPV positive had better prognosis and also less recurrence.
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- 2014
10. Human papilloma virus and nasopharyngeal carcinoma: pathology, prognosis, recurrence and mortality of the disease
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saeid atighechi, Baghdadabad, M. R. A., Mirvakili, S. A., Sheikhha, M. H., Baradaranfar, M. H., Dadgarnia, M. H., and Behniafard, N.
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virus diseases ,Short communications ,female genital diseases and pregnancy complications - Abstract
Background: One of the malignant tumors among head and neck cancers is nasopharyngeal carcinoma. Many studies consider human papilloma virus (HPV) as a cause for nasopharyngeal carcinoma. Methods: 41 paraffin-wax-embedded block samples were examined to detect HPV DNA and its subtype’s presence by polymerase chain reaction. The recurrence, prognosis and survival were evaluated for an average of 48 months. Results: HPV DNA was positive in 9 patients (22%). The overall recurrence rate was 75% in HPV negative patients and 11% in HPV positive ones. The mortality rate in HPV negative and positive patients was 37.5% and 0%, respectively. Conclusion: HPV type 18 and 16 were the most common subtypes. Also, it can be implied that patients which are HPV positive had better prognosis and also less recurrence. Key Words: human papilloma virus, nasopharyngeal cancer, polymerase chain reaction, prognosis, recurrence.
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- 2014
11. Pediatric patients with common variable immunodeficiency: long-term follow-up
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Mohammadinejad P, Aghamohammadi A, Hassan Abolhassani, Ms, Sadaghiani, Abdollahzade S, Sadeghi B, Soheili H, Tavassoli M, Sm, Fathi, Tavakol M, Behniafard N, Darabi B, Pourhamdi S, and Rezaei N
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Male ,Delayed Diagnosis ,Adolescent ,Immunoglobulins ,Iran ,Common Variable Immunodeficiency ,Phenotype ,Agammaglobulinemia ,Child, Preschool ,Humans ,Female ,Genetic Predisposition to Disease ,Child ,Follow-Up Studies - Abstract
Common variable immunodeficiency (CVID) is the most common form of symptomatic primary immunodeficiency disease. It is characterized by hypogammaglobulinemia, increased predisposition to infections, autoimmunity, and cancer.This study was performed to evaluate the clinical and immunological features of a group of pediatric patients with CVID.The study population comprised 69 individuals with CVID diagnosed during childhood.The patients were followed up for a mean (SD) period of 5.2 (4.3) years. The mean diagnostic delay was 4.4 (3.6) years, which was significantly lower in patients who were diagnosed recently. Children were classified according to 5 clinical phenotypes: infections only (n=39), polyclonal lymphocytic infiltration (n=17), autoimmunity (n=12), malignancy (n=7), and enteropathy (n=3). Postdiagnosis survival (10-year) was 71%.The high percentages of pediatric patients with CVID in Iran may be due to the considerable prevalence of parental consanguinity in the region and an underlying genetic background.
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- 2012
12. Correlation Between Peripheral and Central Venous Pressures in Children with Congenital Heart Disease
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Amoozgar, H., primary, Behniafard, N., additional, Borzoee, M., additional, and Ajami, G. H., additional
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- 2007
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13. Physicians awareness on primary immunodeficiency disorders in iran.
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Nourijelyani K, Aghamohammadi A, Salehi Sadaghiani M, Behniafard N, Abolhassani H, Pourjabar S, Rezvanizadeh A, Khadamy J, Imanzaeh A, Sedaghat M, and Rezaei N
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- 2012
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14. Evaluation of factors contributed in nonadherence to medication therapy in children asthma.
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Mirsadraee R, Gharagozlou M, Movahedi M, Behniafard N, and Nasiri R
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- 2012
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15. Proliferating Myositis: An Inflammatory Lesion often Misdiagnosed as A Malignant Tumor
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Binesh Fariba, Sobhanardekani Mohammad, Zabihi Somayeh, and Behniafard Nasim
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ds proliferative myositis ,sarcoma ,diagnosis ,pathology ,Internal medicine ,RC31-1245 - Abstract
Proliferative myositis (PM) is a rare inflammatory disease. Most commonly, the lesion occurs in the extremities. Regarding its fast growth and bizarre shape of the cellular components this entity commonly misdiagnosed and the patients undergo improper therapeutic approaches. In other words, it is often misdiagnosed as sarcoma. The diagnosis can only be made by the microscopic examination, so biopsy is mandatory. Here the authors report a patient with PM who was initially misdiagnosed as pleomorphic sarcoma of the lower extremity and explain this rare entity.
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- 2016
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16. Filaggrin single nucleotide polymorphisms in atopic dermatitis
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Khaledi, M., Fotouhi, A., Farhadi, E., Mahdaviani, B., Sotoudeh, S., Tavakoli, M., Behniafard, N., Gharagozlou, M., and Nima Rezaei
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Male ,Genotype ,Intermediate Filament Proteins ,Case-Control Studies ,Humans ,Female ,Filaggrin Proteins ,Iran ,Real-Time Polymerase Chain Reaction ,Polymorphism, Single Nucleotide ,Alleles ,Dermatitis, Atopic - Abstract
Atopic Dermatitis (AD) is a relapsing chronic pruritic inflammatory disease of skin in which no monogenic cause has been identified so far. Meanwhile Filaggrin (FLG) gene is considered as the most important gene associated with predisposition to the disease.One hundred and six patients with AD and 105 healthy individuals were enrolled in this study. Real time polymerase chain reaction was performed to determine frequencies of alleles and genotype in six variants of FLG gene.Atopic dermatitis (AD) is a relapsing chronic pruritic inflammatory disease of skin for which no monogenic cause has been identified so far. Meanwhile, the filaggrin (FLG) gene is considered as the most important gene associated with predisposition to the disease.One hundred and six patients with AD and 105 healthy individuals were enrolled in this study. Real time polymerase chain reaction was performed to determine frequencies of alleles and genotype in six variants of the FLG gene. The frequencies of allele A in variants of rs3126065, rs2786680, and rs1933063 as well as allele C in variant rs3814300 were 100%. There was no significant difference between allele frequencies in variants rs2485518 and rs3814299. The only genotypes in variants of rs3814299 and rs2485518 were GG and CC, respectively, with no significant difference between the patients and controls. This study demonstrated that there was no significant association between polymorphisms of FLG gene variants and AD.
17. Does malleolus non-lifting tympanoplasty have any advantage over malleus lifting techniques?
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Vahidi, M. R., Mollasadeghi, A., honeyeh shahbazian, Behniafard, N., and Dadgarnia, M. H.
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Lifting ,Tympanoplasty ,Original Article ,Auditory Threshold ,Hearing loss ,Otitis media - Abstract
Introduction: In order to achieve a higher success rate for tympanoplasty, different techniques have been developed, and a wide variety of grafting materials have been developed. One of the techniques currently receiving considerable attention involves not lifting the remaining of eardrum from the malleus and embedding the graft underneath in order to repair the eardrum correctly in its original position, as well as minimizing graft lateralization leading to progression of hearing rehabilitation. We compared the effects of tympanoplasty with and without malleus lifting on hearing loss in patients with chronic otitis media. Materials and Methods: In this study, 30 consecutive patients diagnosed as having chronic otitis media without cholesteatoma were randomly assigned to two tympanoplasty groups; with or without malleus lifting. Air and bone conduction thresholds were recorded before and 45 days after the intervention. Results: In groups, except for 8000 Hz, the air conduction was significantly improved following surgery. According to air conduction there was no difference between the groups before surgery at different frequencies, although it was improved to a greater degree in the group without lifting at 250 Hz postoperatively. The average post-operative air-bone gap (ABG) gain was significantly higher in all study frequencies in the target group. One of the effects of this technique is inner-ear protection from physical trauma to the ossicular chain, and prevention of damage to bone conduction. Conclusion: A higher hearing threshold and also higher ABG gain can be achieved by not lifting the remaining eardrum from the malleus and embedding the graft undereath it, especially at lower frequencies.
18. Evaluation of factors contributed in nonadherence to medication therapy in children asthma
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Mirsadraee, R., Mohammad Gharagozlou, Movahedi, M., Behniafard, N., and Nasiri, R.
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Male ,Parents ,Health Knowledge, Attitudes, Practice ,Adolescent ,Substance-Related Disorders ,Child Behavior ,lcsh:Medicine ,Iran ,Risk Assessment ,Medication Adherence ,Risk Factors ,Humans ,Anti-Asthmatic Agents ,Child ,Chi-Square Distribution ,lcsh:R ,Age Factors ,Infant, Newborn ,Infant ,Fear ,Treatment Adherence ,Asthma ,Adolescent Behavior ,Child, Preschool ,Drug Therapy, Combination ,Female ,Self Report ,Children Asthma ,Compliance ,Nonadherence - Abstract
Asthma is one of the most common chronic inflammatory disorders in children. Nonadherence to medical therapy is a major cause of poor clinical outcome the objective of this study was evaluating factors, which are resulted in nonadherence to medical therapy in children with asthma. In this descriptive study, 150 children with asthma and nonadherent to medication therapy were enrolled. General information and probable causes of nonadherence were recorded in self-report questionnaire and data were analyzed. In our study, 57.3% of children were male. Approximately 43%of children belonged to age group 6-9 years old. Prevalence of probable causes of nonadherence to treatment were concern about treatment expenses(34.7%) ,fear of cardiac complications(34.7%), concern about drug dependency(38.7%), belief to growth inhibition(30.7%) and fear of osteopenia (32%). There was statistically significant reverse association between treatment with multi- drug regimens and concern about bone mineral abnormalities, cardiac complications and drug dependency (p=0.0001, 0.014 and 0.012 respectively). In addition, there was a significant association between mild asthma and fear about drug dependency (p=0.001). According to our results, factors such as prolonged duration of treatment, various therapeutic regimens, and receiving multiple drugs before diagnosis of asthma pose the highest frequencies for nonadherence.
19. Physicians awareness on primary immunodeficiency disorders in Iran
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Nourijelyani, K., Aghamohammadi, A., Sadaghiani, M. S., Behniafard, N., Abolhassani, H., Pourjabar, S., Rezvanizadeh, A., Joobin Khadamy, Imanzaeh, A., Sedaghat, M., and Rezaei, N.
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Adult ,Aged, 80 and over ,Male ,Health Knowledge, Attitudes, Practice ,Delayed Diagnosis ,Attitude of Health Personnel ,lcsh:R ,General Practice ,Immunologic Deficiency Syndromes ,lcsh:Medicine ,Awareness ,Iran ,Middle Aged ,Pediatrics ,Primary immunodeficiency diseases ,General Practitioners ,Predictive Value of Tests ,Physicians ,Surveys and Questionnaires ,Humans ,Female ,Pediatricians ,Clinical Competence ,Aged - Abstract
Primary immunodeficiency diseases (PIDs) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. The aim of this study was to assess the knowledge of Iranian general practitioners and pediatricians about PIDs. A questionnaire consisting 52 closed questions on clinical symptoms, laboratory data,associated syndromes and management of PIDs patients was made valid and reliable by a pair pilot study. Then the questionnaire was filled by pediatricians, general practitioners and pediatric residents from different regions of Iran. Totally, 333 physicians (50 general practitioners, 52 pediatric residents, 182 pediatric specialists, and 49 pediatric sub specialists) participated in this study. The mean total score was 55.9±14.3 (i.e. about 29 correct answers out of 52 questions). One hundred and five participants (31.9%) answered correctly more than two third of all questions. In order to qualitatively compare the groups a ranking system was used. Total scores was significantly different between physicians groups (p
20. Severe primary antibody deficiency due to a novel mutation of μ heavy chain
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Mohammadzadeh, I., Yeganeh, M., Aghamohammadi, A., Parvaneh, N., Behniafard, N., Abolhassani, H., Tabassomi, F., Hemmat, M., Kanegane, H., Miyawaki, T., Ohara, O., and Nima Rezaei
21. The effect of gelfoam impregnated with botulinum toxin on allergic rhinitis
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Zand, V., Mohammad Hossein Baradaranfar, Dadgarnia, M., Meybodian, M., Vaziribozorg, S., Mandegari, M., Behniafard, N., and Dehghani, A.
22. Severe Primary Antibody Deficiency Due to a Novel Mutation of mu Heavy Chain
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Mohammadzadeh, I., Yeganeh, M., Aghamohammadi, A., Parvaneh, N., Behniafard, N., Hassan Abolhassani, Tabassomi, F., Hemmat, M., Kanegane, H., Miyawaki, T., Ohara, O., and Rezaei, N.
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Male ,Young Adult ,Agammaglobulinemia ,Immunoglobulin mu-Chains ,Mutation ,Humans
23. Single-nucleotide polymorphisms of TNFA and IL1 in allergic rhinitis
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Nasiri, R., Akbar Amirzargar, A., Movahedi, M., Hirbod-Mobarakeh, A., Farhadi, E., Behniafard, N., Tavakkol, M., Ansaripour, B., Moradi, B., Ahad Zare, and Rezaei, N.
24. Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.
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Pashangzadeh S, Delavari S, Moeini Shad T, Salami F, Rasouli SE, Yazdani R, Mahdaviani SA, Nabavi M, Aleyasin S, Ahanchian H, Jabbari-Azad F, Chavoshzadeh Z, Nazari F, Momen T, Sherkat R, Abolnezhadian F, Esmaeilzadeh H, Fallahpour M, Arshi S, Bemanian MH, Shokri S, Ebrahimi SS, Abolmolouki M, Farid AS, Rezaei A, Esmaeili M, Kalantari A, Sadeghi-Shabestari M, Shirkani A, Behniafard N, Khalili A, Eslamian MH, Cheraghi T, Shafie A, Tavakol M, Khoshkhui M, Iranparast S, Shamshiri M, Shahri MA, Khazaei R, Asadi M, Babaha F, Aghamohammadi A, Rezaei N, and Abolhassani H
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- Humans, Female, Male, Adult, Retrospective Studies, Middle Aged, Young Adult, Autoimmunity, Adolescent, Aged, Child, Common Variable Immunodeficiency complications, Common Variable Immunodeficiency immunology, B-Lymphocytes immunology, Lymphopenia immunology
- Abstract
Background: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients., Methods: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated., Results: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups., Conclusions: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.
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- 2024
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25. Association between DASH diet and asthma symptoms among a large sample of adolescents: a cross-sectional study.
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Arabi V, Sasanfar B, Toorang F, Nafei Z, Behniafard N, and Salehi-Abargouei A
- Abstract
Background: The Dietary Approaches to Stop Hypertension (DASH) diet, which has a lot of emphasis on the consumption of fruits, vegetables, and whole grains, and on the other hand, the consumption of red meat and sodium is limited, due to its anti-inflammatory properties, which can be related to reducing the risk of asthma., Objectives: The aim of this study was to determine the relationship between the DASH diet and asthma symptoms among children and adolescents., Methods: This cross-sectional study was conducted among7667 children (3414 boys and 4253 girls) aged 6-7 and 13-14 years living in central Iran. Dietary food consumption was assessed using a multiple-choice questionnaire. Logistic regression was used to obtain odds ratios for the association between the DASH-like diet with current asthma and asthma symptoms., Results: Our findings revealed that higher adherence to a DASH-like diet resulted in lower odds of asthma confirmed by a doctor among the whole population (OR = 0.53; 95%CI: 0.36-0.76) and also in females (OR = 0.47; 95%CI: 0.29-0.78). Moreover, the higher adherence to the DASH-like diet was inversely associated with the chance of wheezing in the past 12 months in all subjects (OR = 0.67; 95%CI: 0.51-0.86) and in boys (OR = 0.57; 95%CI: 0.38-0.85)., Conclusion: The findings of the present study showed that following the DASH diet can be associated with the improvement of asthma symptoms in children and adolescents. However, more research is needed to improve dietary recommendations for asthma prevention., (© 2024. The Author(s).)
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- 2024
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26. Association between a western diet and asthma among children and adolescents.
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Emrani AS, Sasanfar B, Jowshan MR, Behniafard N, Nafei Z, and Salehi-Abargouei A
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- Humans, Male, Child, Adolescent, Female, Cross-Sectional Studies, Risk Factors, Iran epidemiology, Surveys and Questionnaires, Respiratory Sounds etiology, Asthma epidemiology, Asthma etiology, Diet, Western adverse effects
- Abstract
Several risk factors including environmental exposures, socioeconomic status, and dietary factors including dietary patterns have been considered for childhood Asthma. The present study tried to examine the association between a western-style pattern and the likelihood of asthma and its symptoms in Yazd, Iran. In the present cross-sectional study, dietary intakes of elementary and high-school children were obtained through a validated GAN questionnaire. The GAN questionnaire, derived from the ISAAC questionnaire was used to assess the symptoms of allergic diseases and their related risk factors. A western dietary pattern score considered 9 food groups including chicken eggs, margarine, butter, sugar, fast foods, soft drinks, snacks, sauce, and chocolate. In total 7667 children aged 10.9 ± 3.35 years were included in the current investigation. Boys with higher adherence to western dietary pattern had a higher risk of wheezing in the past 12 months (OR 1.37, 5% CI 1.01-1.87, P = 0.04) and this association was also observed in the whole population (OR 1.30, 5% CI 1.05-1.60, P = 0.01). However, after adjustment for confounders this relation did not remain significant in boys. Our results support the hypothesis that a western dietary pattern is associated with an increased risk of wheezing in the past 12 months in children with asthma. Future prospective studies are needed to confirm this finding., (© 2024. The Author(s).)
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- 2024
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27. Dairy intake in association with asthma symptoms among a large sample of children and adolescents: a cross-sectional study.
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Jamalvandi M, Sasanfar B, Nafei Z, Behniafard N, Jafari M, and Salehi-Abargouei A
- Abstract
Background and Objective: Dairy products may be associated with an increased risk of asthma, although there is little scientific evidence to support this association. The goal of this study was to explore the association between dairy consumption and asthma symptoms., Materials and Methods: This cross-sectional study was conducted on children and adolescents aged 6-8 and 13-14 years living in central Iran. Dietary food consumption was assessed using a multiple-choice questionnaire. Logistic regression was used to obtain odds ratios for the association between milk, other dairy products, and total dairy consumption with the risk of asthma symptoms., Results: In total, 7,667 participants (3,414 boys and 4,253 girls) were included in the current study. Milk intake and total dairy consumption were not associated with the likelihood of wheezing, asthma confirmed by a doctor, current asthma, and asthma medication use. In addition, there was no association between other dairy product intake and the odds of wheezing in the past 12 months in the crude model. However, after adjusting for several confounders, those in the top category had lower odds of wheezing in the past 12 months than those in the bottom category (OR: 0.58; 95% CI: 0.40-0.85)., Conclusion: The consumption of dairy products other than milk, including cheese and yogurt, might reduce the likelihood of wheezing in children and adolescents., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Jamalvandi, Sasanfar, Nafei, Behniafard, Jafari and Salehi-Abargouei.)
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- 2024
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28. Clinical heterogeneity in families with multiple cases of inborn errors of immunity.
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Delavari S, Rasouli SE, Fekrvand S, Chavoshzade Z, Mahdaviani SA, Shirmast P, Sharafian S, Sherkat R, Momen T, Aleyasin S, Ahanchian H, Sadeghi-Shabestari M, Esmaeilzadeh H, Barzamini S, Tarighatmonfared F, Salehi H, Esmaeili M, Marzani Z, Fathi N, Abolnezhadian F, Rad MK, Saeedi-Boroujeni A, Shirkani A, Bagheri Z, Salami F, Shad TM, Marzbali MY, Mojtahedi H, Razavi A, Tavakolinia N, Cheraghi T, Tavakol M, Shafiei A, Behniafard N, Ebrahimi SS, Sepahi N, Ghaneimoghadam A, Rezaei A, Kalantari A, Abolhassani H, and Rezaei N
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- Child, Humans, Adaptor Proteins, Signal Transducing, Antigens, CD19, Autoimmunity, Protein Serine-Threonine Kinases, Intracellular Signaling Peptides and Proteins, Delayed Diagnosis, Quality of Life
- Abstract
Background: Inborn errors of immunity (IEI) are a diverse range of genetic immune system illnesses affecting the innate and/or adaptive immune systems. Variable expressivity and incomplete penetrance have been reported in IEI patients with similar clinical diagnoses or even the same genetic mutation., Methods: Among all recorded patients in the national IEI registry, 193 families with multiple cases have been recognized. Clinical, laboratory and genetic variability were compared between 451 patients with different IEI entities., Results: The diagnosis of the first children led to the earlier diagnosis, lower diagnostic delay, timely treatment and improved survival in the second children in the majority of IEI. The highest discordance in familial lymphoproliferation, autoimmunity and malignancy were respectively observed in STK4 deficiency, DNMT3B deficiency and ATM deficiency. Regarding immunological heterogeneity within a unique family with multiple cases of IEI, the highest discordance in CD3
+ , CD4+ , CD19+ , IgM and IgA levels was observed in syndromic combined immunodeficiencies (CID), while non-syndromic CID particularly severe combined immunodeficiency (SCID) manifested the highest discordance in IgG levels. Identification of the first ATM-deficient patient can lead to improved care and better survival in the next IEI children from the same family., Conclusion: Intrafamilial heterogeneity in immunological and/or clinical features could be observed in families with multiple cases of IEI indicating the indisputable role of appropriate treatment and preventive environmental factors besides specific gene mutations in the variable observed penetrance or expressivity of the disease. This also emphasizes the importance of implementing genetic evaluation in all members of a family with a history of IEI even if there is no suspicion of an underlying IEI as other factors besides the underlying genetic defects might cause a milder phenotype or delay in presentation of clinical features. Thus, affected patients could be timely diagnosed and treated, and their quality of life and survival would improve., Competing Interests: Declaration of competing interest The authors declare that they have no relevant conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)- Published
- 2024
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29. Which Groups of Children Are at More Risk of Fatality during COVID-19 Pandemic? A Case-Control Study in Yazd, Iran.
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Shafaei B, Nafei Z, Karimi M, Behniafard N, Shamsi F, Faisal M, Amel Shahbaz AP, and Akbarian E
- Abstract
Introduction: The study aims to investigate the characteristics, comorbidities, laboratory findings, and clinical manifestations of under 18-year-old patients who died with the diagnosis of COVID-19 and determination of the most prevalent risk factors., Method: This case-control study was performed at a referral hospital in Yazd from March 2020 to August 2021. All patients under 18 years who were diagnosed through real-time RT-PCR, chest computed tomography, and the World Health Organization definition were divided into deceased and survived groups. The characteristics (age and sex), disease severity, comorbidities, laboratory findings, and clinical manifestations of the two groups were compared and analyzed using SPSS, version 18 (SPSS Inc., Chicago, III., USA)., Results: A total of 24 patients in the deceased group and 167 patients in the survived group were compared. The highest mortality rate was observed in the age group of 1 month to 5 years, although no statistically significant relationship was found between age groups and the risk of mortality. Disease severity, dyspnea, low oxygen saturation on admission, length of hospital stays, and hospitalization history before the last admission were significantly correlated with mortality ( P < 0.05). Lymphopenia increased the probability of mortality by more than two times (OR: 2.568; 95% CI (0.962-6.852)), but this was not the case for D-dimer and C-reactive protein. Furthermore, 27.5% of survived patients had normal chest CT scans, which was a statistically significant difference compared to the deceased patients ( P : 0.031)., Conclusion: Based on the findings of this study, dyspnea, low oxygen saturation, and lymphopenia are critical indicators for identifying high-risk children with COVID-19 and triaging them for better care and treatment., Competing Interests: The authors declare that there are no conflicts of interest in the publication of this paper., (Copyright © 2023 Behnam Shafaei et al.)
- Published
- 2023
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30. Association Between Pet Keeping and Current Asthma Among Adolescents Living in Yazd; Evidence from Global Asthma Network (GAN) 2020 Cross-sectional Study.
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Behniafard N, Modarresi SZ, Nafei Z, and Vakili M
- Subjects
- Child, Humans, Adolescent, Animals, Dogs, Cross-Sectional Studies, Cough, Health Personnel, Respiratory Sounds etiology, Asthma epidemiology
- Abstract
Background: The relationship between current pet keeping and allergic diseases, including bronchial asthma in adolescents, is controversial. This study was conducted to evaluate these associations among children aged 13-14 years in Yazd., Methods: This study is part of a multicenter cross-sectional study of the Global Asthma Network (GAN) in Yazd, Iran, in 2020, in which 5141adolescents enrolled. Information on respiratory symptoms and pet-keeping (dog/cat/birds) was obtained by a questionnaire derived from the GAN standard questionnaire., Results: Of 5141 participants who completed the study, 1800 (35%) children kept pets during the last year. Birds were the most common pet kept by adolescents (88%). Severe asthma was more common in bird and cat keepers ( P =0.003 and P =0.034, respectively) than dog keepers. Furthermore, there was a statistically significant association between study-defined current asthma and cat keeping, but not bird or dog ownership ( P =0.02). Moreover, we found that current any pet-keeping (birds, cats, dogs) was associated with a higher prevalence of asthma-related symptoms, including wheezing, night dry cough, and exercise-induced wheezing in the past year ( P =0.002, P =0.000 and P =0.000 respectively)., Conclusion: Current any pet-keeping is associated with asthma-related symptoms. Additionally, cat keeping had a significant association with study-defined current asthma. The current keeping of birds, as the most common pet in our area, or cat keeping increases the risk of severe asthma in adolescents. Therefore, as an important health tip, this needs to be reminded to families by health care providers., (© 2023 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)
- Published
- 2023
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31. Clinical, Immunological, and Genetic Findings in Iranian Patients with MHC-II Deficiency: Confirmation of c.162delG RFXANK Founder Mutation in the Iranian Population.
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Mousavi Khorshidi MS, Seeleuthner Y, Chavoshzadeh Z, Behfar M, Hamidieh AA, Alimadadi H, Sherkat R, Momen T, Behniafard N, Eskandarzadeh S, Mansouri M, Behnam M, Mahdavi M, Heydarazad Zadeh M, Shokri M, Alizadeh F, Movahedi M, Momenilandi M, Keramatipour M, Casanova JL, Cobat A, Abel L, Shahrooei M, and Parvaneh N
- Subjects
- Humans, Infant, Newborn, Histocompatibility Antigens Class II genetics, Iran, Mutation genetics, DNA-Binding Proteins genetics, Severe Combined Immunodeficiency genetics, Transcription Factors genetics
- Abstract
Purpose: Major histocompatibility complex class II (MHC-II) deficiency is a rare inborn error of immunity (IEI). Impaired antigen presentation to CD4 + T cells results in combined immunodeficiency (CID). Patients typically present with severe respiratory and gastrointestinal tract infections at early ages. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy., Methods: We describe the clinical, immunologic, and genetic features of eighteen unrelated Iranian patients with MHC-II deficiency., Results: Consanguinity was present in all affected families. The median age at the initial presentation was 5.5 months (range 7 days to 18 years). The main symptoms included failure to thrive, persistent diarrhea, and pneumonia. Autoimmune and neurologic features were also documented in about one-third of the patients, respectively. Thirteen patients carried RFXANK gene mutations, two carried RFX5 gene mutations, and three carried a RFXAP gene mutation. Six patients shared the same RFXANK founder mutation (c.162delG); limited to the Iranian population and dated to approximately 1296 years ago. Four of the patients underwent HSCT; three of them are alive. On the other hand, nine of the fourteen patients who did not undergo HSCT had a poor prognosis and died., Conclusion: MHC-II deficiency is not rare in Iran, with a high rate of consanguinity. It should be considered in the differential diagnosis of CID at any age. With the limited access to HSCT and its variable results in MHC-II deficiency, implementing genetic counseling and family planning for the affected families are mandatory. We are better determined to study the c.162delG RFXANK heterozygous mutation frequency in the Iranian population., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2023
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32. Association between Butter, Margarine, and Olive Oil Intake and Asthma Symptoms among School Children: Result from a Large-Scale Cross-Sectional Study.
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Emrani AS, Sasanfar B, Nafei Z, Behniafard N, and Salehi-Abargouei A
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- Adolescent, Humans, Child, Olive Oil, Butter adverse effects, Cross-Sectional Studies, Prospective Studies, Respiratory Sounds, Dietary Fats adverse effects, Margarine, Asthma epidemiology, Asthma etiology
- Abstract
Background: There are conflicting results about the association between dietary fat intake and asthma symptoms. Since few studies in the Middle East have been explored the relation between dietary fat consumption and risk of asthma, the present study was conducted to investigate the association between the consumption of butter, margarine, and olive oil and asthma risk in school children living in central Iran., Method: In this cross-sectional study, out of 10,240 participants, asthma and its symptoms and dietary intake of butter, margarine, and olive oil of 7,667 children and adolescents were assessed using a validated International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. The relationship between fat subtypes and asthma was assessed using logistic regression., Results: The prevalence of asthma confirmed by a doctor in the study population was 4.22%. An inverse association was found between butter and margarine consumption once or twice a week and odds of current asthma and wheezing in the past 12 months (OR = 0.52, 95% CI: 0.28-0.96; OR = 0.7, 95% CI: 0.55-0.88, respectively); however, those with higher consumption did not have a higher chance for developing wheezing or asthma., Conclusion: We found that margarine and butter intake one or two times a week might have an inverse association with asthma and its symptoms among children. Prospective cohort studies are recommended to confirm these findings., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2023 Arezoo Sadat Emrani et al.)
- Published
- 2023
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33. Clinical and immunological characteristics of 69 leukocyte adhesion deficiency-I patients.
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Fazlollahi MR, Hamidieh AA, Moradi L, Shokouhi Shoormati R, Sabetkish N, Esmaeili B, Badalzadeh M, Alizadeh Z, Shamlou S, Movahedi M, Mahloujirad M, Razaghian A, Arshi S, Gharagozlou M, Kalantari A, Bemanian MH, Safari M, Heidarzadeh Arani M, Nabavi M, Parvaneh N, Sadeghi-Shabestari M, Behfar M, Behniafard N, Sherkat R, Ahmadian Heris J, Shariat M, Radmehr R, Houshmand M, Kazemnejad A, Molitor A, Carapito R, Bahram S, Pourpak Z, and Moin M
- Subjects
- Male, Pregnancy, Female, Humans, Delayed Diagnosis, Iran, Leukocytes metabolism, CD18 Antigens genetics, Leukocyte-Adhesion Deficiency Syndrome diagnosis, Leukocyte-Adhesion Deficiency Syndrome genetics
- Abstract
Background: In order to support the comprehensive classification of Leukocyte Adhesion Deficiency-I (LAD-I) severity by simultaneous screening of CD11a/CD18, this study assessed clinical, laboratory, and genetic findings along with outcomes of 69 LAD-I patients during the last 15 years., Methods: Sixty-nine patients (40 females and 29 males) with a clinical phenotype suspected of LAD-I were referred to Immunology, Asthma, and Allergy research institute, Tehran, Iran between 2007 and 2022 for further advanced immunological screening and genetic evaluations as well as treatment, were enrolled in this study., Results: The diagnosis median age of the patients was 6 months. Delayed umbilical cord separation was found in 25 patients (36.2%). The median diagnostic delay time was 4 months (min-max: 0-82 months). Forty-six patients (66.7%) were categorized as severe (CD18 and/or CD11a: below 2%); while 23 children (33.3%) were in moderate category (CD18 and/or CD11a: 2%-30%). During the follow-ups, 55.1% of children were alive with a mortality rate of 44.9%. Skin ulcers (75.4%), omphalitis (65.2%), and gingivitis (37.7%) were the most frequent complaints. Genetic analysis of the patients revealed 14 previously reported and three novel pathogenic mutations in the ITGB2 gene. The overall survival of patients with and without hematopoietic stem cell transplantation was 79.3% and 55.6%, respectively., Conclusion: Physicians' awareness of LAD-I considering delayed separation of umbilical cord marked neutrophilic leukocytosis, and variability in CD11 and CD18 expression levels, and genetic analysis leads to early diagnosis and defining disease severity. Moreover, the prenatal diagnosis would benefit families with a history of LAD-I., (© 2023 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)
- Published
- 2023
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34. Dietary approaches to stop hypertension (DASH)-style diet in association with gastroesophageal reflux disease in adolescents.
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Beigrezaei S, Sasanfar B, Nafei Z, Behniafard N, Aflatoonian M, and Salehi-Abargouei A
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- Male, Humans, Adolescent, Cross-Sectional Studies, Diet, Surveys and Questionnaires, Pain, Dietary Approaches To Stop Hypertension methods, Hypertension epidemiology, Hypertension prevention & control, Gastroesophageal Reflux epidemiology, Gastroesophageal Reflux etiology, Gastroesophageal Reflux prevention & control
- Abstract
Background: Dietary patterns and food items have been associated with gastroesophageal reflux disease (GERD) risk and they have led to conflicting findings. The aim of this study was to determine the association between a dietary approach to stop hypertension (DASH)-style diet with the risk of GERD and its symptoms in adolescents., Study Design: Cross-sectional., Methods: This study was performed on 5,141 adolescents aged between 13 and 14 years. Dietary intake was evaluated using a food frequency method. The diagnosis of GERD was done by using a six-item GERD questionnaire that asked about GERD symptoms. A binary logistic regression was used to assess the association between the DASH-style diet score and GERD and its symptoms in crude and multivariable-adjusted models., Results: Our findings revealed that after adjustment for all confounding variables, the adolescents with the highest adherence to the DASH-style diet had a lower chance of developing GERD [odds ratio (OR) = 0.50; 95%CI 0.33-0.75, P
trend < 0.001)], reflux (OR = 0.42; 95%CI 0.25-0.71, Ptrend =0.001), nausea (OR = 0.59; 95% CI:0.32-1.08, Ptrend =0.05) and stomach pain (OR = 0.69; 95%CI 0.49-0.98, Ptrend =0.03) compared to those with the lowest adherence. Similar results were found for odds of GERD among boys, and the total population (OR = 0.37; 95%CI: 0.18-0.73, Ptrend =0.002, OR = 0.51; 95%CI: 0.34-0.77, Ptrend <0.0, respectively)., Conclusion: The current study revealed that adherence to a DASH-style diet might protect against GERD and its symptoms including, reflux, nausea, and stomach pain in adolescents. Further prospective research is needed to confirm these findings., (© 2023. The Author(s).)- Published
- 2023
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35. Protein Kinase C-Delta Defect in Autoimmune Lymphoproliferative Syndrome-Like Disease: First Case from the National Iranian Registry and Review of the Literature.
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Sharifinejad N, Azizi G, Behniafard N, Zaki-Dizaji M, Jamee M, Yazdani R, Abolhassani H, and Aghamohammadi A
- Subjects
- Adolescent, CD8-Positive T-Lymphocytes, Herpesvirus 4, Human, Humans, Iran, Male, Mutation, Protein Kinase C-delta, Registries, Autoimmune Diseases, Autoimmune Lymphoproliferative Syndrome diagnosis, Autoimmune Lymphoproliferative Syndrome genetics, Epstein-Barr Virus Infections
- Abstract
Background: Protein kinase C is a family of serine/threonine kinases that play a key role in the adaptive immune cell signaling, as well as regulation of growth, apoptosis, and differentiation of a variety of cell types. Patients homozygous for a null mutation of the Protein Kinase C Delta (PRKCD) gene, present clinical feature of immune dysregulation with susceptibility to Epstein-Barr virus infection. However, a minority of patients present the autoimmune lymphoproliferative syndrome (ALPS)., Methods: The data were collected by direct interview and examining the patient's clinical record. Whole-exome sequencing was performed to detect the underlying genetic mutation in the patient. We also conducted electronic searches for ALPS-like reported patients in PubMed, Web of Science, and Scopus databases., Results: In this study, we reported a 13-year-old boy who presented with autoimmunity, lymphoproliferation, recurrent pneumonia, cardiomyopathy, and dermatological manifestations. An elevation of double-negative T cells, CD8
+ T cells, serum IgG level, as well as a reduction in NK cells, was observed in the patient. A homozygous frameshift mutation (c.1293_1294insA) in exon 13 of the PRKCD gene was confirmed. The literature search showed 39 ALPS-like patients with monogenic defects which only six (15.3%) of them were due to PRKCD genes., Conclusion: PRKCD should be considered in the context of ALPS clinical manifestations with prominent dermatological involvements.- Published
- 2022
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36. New onset of diabetes in a child infected with COVID-19: a case report.
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Ordooei M, Behniafard N, Soheilipour F, and Akbarian E
- Abstract
Background: Diabetic ketoacidosis (DKA) is a serious complication of type 1 diabetes. A few studies have reported that COVID-19 is associated with the development of new-onset diabetes. Here, we present an infected child with new onset diabetes leading to DKA., Case Presentation: A 10-year-old patient with respiratory distress admitted to the Emergency Department of our center. The patient's COVID-19 Polymerase Chain Reaction (PCR) test was positive and also biochemical analyses confirmed that he had DKA. Despite standard initial treatments, ketoacidosis remained resistant; hence we prescribed oral bicarbonate (40 cc every 8 h) to treat the patient's refractory acidosis. Due to the patient's improvement, he was discharged after 10 days (7 days in the PICU), receiving outpatient enoxaparin (for a week) and ongoing subcutaneous insulin., Conclusion: We report an interesting case of a child with COVID-19 infection precipitating presentation with new onset diabetes. Due to refractory acidosis, starting oral bicarbonate treatment after 2 days improved acidosis and tachypnea in the patient. The patient's medical team suggest close biochemical monitoring, prescribing enoxaparin for high level of D-dimer, and ordering oral bicarbonate acidosis persists., Competing Interests: Conflicts of interest/Competing interestsThe authors declare that they have no conflict of interest., (© Springer Nature Switzerland AG 2021.)
- Published
- 2021
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37. Autoimmune manifestations among 461 patients with monogenic inborn errors of immunity.
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Azizi G, Tavakol M, Yazdani R, Delavari S, Moeini Shad T, Rasouli SE, Jamee M, Pashangzadeh S, Kalantari A, Shariat M, Shafiei A, Mohammadi J, Hassanpour G, Chavoshzadeh Z, Mahdaviani SA, Momen T, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Alyasin S, Jabbari-Azad F, Ghaffari J, Mesdaghi M, Ahanchian H, Khoshkhui M, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Nasiri Kalmarzi R, Esmaeilzadeh H, Tafaroji J, Khalili A, Sadeghi-Shabestari M, Darougar S, Moghtaderi M, Ahmadiafshar A, Shakerian B, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Fallahpour M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahimi Haji-Abadi M, Ashournia P, Razaghian A, Rezaei A, Salami F, Shirmast P, Bazargan N, Mamishi S, Khazaei HA, Negahdari B, Shokri S, Nabavizadeh SH, Bazregari S, Ghasemi R, Bayat S, Eshaghi H, Rezaei N, Abolhassani H, and Aghamohammadi A
- Subjects
- Adaptor Proteins, Signal Transducing genetics, Adolescent, Adult, Autoimmunity genetics, Child, Female, High-Throughput Nucleotide Sequencing, Humans, Iran epidemiology, Male, Retrospective Studies, Young Adult, Autoimmune Diseases epidemiology, Autoimmune Diseases genetics, Common Variable Immunodeficiency
- Abstract
Background: The inborn errors of immunity (IEIs) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections besides other complications including autoimmune and inflammatory diseases. In this study, we aim to evaluate clinical, immunologic, and molecular data of monogenic IEI patients with and without autoimmune manifestations., Methods: We have retrospectively screened cases of monogenic IEI in the Iranian PID registry for the occurrence of autoimmunity and immune dysregulation. A questionnaire was filled for all qualified patients with monogenic defects to evaluate demographic, laboratory, clinical, and molecular data., Results: A total of 461 monogenic IEI patients (290 male and 171 female) with a median (IQR) age of 11.0 (6.0-20.0) years were enrolled in this study. Overall, 331 patients (72.1%) were born to consanguineous parents. At the time of the study, 330 individuals (75.7%) were alive and 106 (24.3%) were deceased. Autoimmunity was reported in 92 (20.0%) patients with a median (IQR) age at autoimmune diagnosis of 4.0 (2.0-7.0) years. Sixteen patients (3.5%) showed autoimmune complications (mostly autoimmune cytopenia) as the first presentation of the disease. Most of the patients with autoimmunity were diagnosed clinically with common variable immunodeficiency (42.4%). The frequency of sinusitis and splenomegaly was significantly higher in patients with autoimmunity than patients without autoimmunity. In patients with autoimmunity, the most common pathogenic variants were identified in LRBA (in 21 patients, 23.0%), ATM (in 13 patients, 14.0%), and BTK (in 9 patients, 10.0%) genes. In the evaluation of autoimmunity by different genes, 4 of 4 IL10RB (100%), 3 of 3 AIRE (100%), and 21 of 30 LRBA (70.0%) mutated genes had the highest prevalence of autoimmunity., Conclusions: Autoimmune phenomena are common features among patients with monogenic IEI and are associated with a more complicated course of the disease. Therefore, when encountering autoimmune disorders, especially in the setting of dysgammaglobulinemia, it would be appropriate to conduct next-generation sequencing to discover responsible genes for the immune dysregulation at an early stage of the disease., (© 2021 The Authors. Pediatric Allergy and Immunology published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)
- Published
- 2021
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38. Prevalence of Allergic Rhinitis and Eczema in Adolescents Living in Yazd City: Part of Global Asthma Network Survey.
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Nafei Z, Behniafard N, Mirzaei M, Karimi M, and Akbarian E
- Subjects
- Adolescent, Age Distribution, Cross-Sectional Studies, Eczema diagnosis, Eczema therapy, Female, Health Surveys, Humans, Iran epidemiology, Male, Prevalence, Rhinitis, Allergic diagnosis, Rhinitis, Allergic therapy, Severity of Illness Index, Sex Distribution, Symptom Assessment, Eczema epidemiology, Rhinitis, Allergic epidemiology
- Abstract
Allergic rhinitis and eczema are two common global diseases that can lead to impaired quality of life. Determining the prevalence of these allergic disorders can be useful in planning prevention and treatment. This study aimed to investigate the prevalence and severity of allergic rhinitis and eczema in adolescents living in Yazd city. Using an electronic questionnaire based on the Global Asthma Network (GAN) core questionnaire, 5141 adolescents aged 13-14 years were cross-sectionally surveyed. The prevalence of current symptoms of rhinitis turned out to be 36.3%, proving significantly higher in boys (p=0.009). Moreover, the prevalence of allergic rhinitis and rhinoconjunctivitis in the past year leveled at 12.4% and 10.5%, respectively; however, the former was significantly higher in females (p=0.014). Additionally, severe rhinoconjunctivitis was observed in 0.2% of the participants with no gender preference (p=0.09). Confirmed hay fever by a doctor was reported in 13.2% of adolescents, significantly higher in males (p<0.001). The prevalence of current itchy rash and current eczema was found to be 5.5%, and 2.9%, respectively, with no difference in terms of gender. Severe atopic eczema and eczema confirmed by a doctor were seen in 0.4% and 5% of the participants, no gender preference was identified. Concurrent prevalence of current rhinoconjunctivitis and eczema was detected in 1% of the participants. Despite the increasing trend of allergic diseases in most parts of the world, the prevalence of rhinoconjunctivitis and eczema in adolescents has not increased in Yazd in the last two decades, and this city is located in a low to moderate prevalence area.
- Published
- 2021
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39. Prevalence and Severity of Adolescent Asthma in Yazd, Iran: Based on the 2020 Global Asthma Network (GAN) Survey.
- Author
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Behniafard N, Nafei Z, Mirzaei M, Karimi M, and Vakili M
- Subjects
- Adolescent, Asthma diagnosis, Asthma etiology, Asthma therapy, Disease Management, Disease Susceptibility, Female, Humans, Iran epidemiology, Male, Prevalence, Public Health Surveillance, Severity of Illness Index, Asthma epidemiology
- Abstract
Asthma, as the most common chronic disease in children, encompasses substantial health and socioeconomic burden worldwide. This study aimed to evaluate the prevalence, severity, and management of asthma in adolescents aged 13-14 years. This cross-sectional study, which was part of the Global Asthma Network (GAN) 2020 survey, was conducted in early 2020 in Yazd, Iran. A total of 48 schools in Yazd city were selected by cluster sampling. In this study, 5141 students, including 3069 (59.7%) females and 2072 (40.3%) males participated. Prevalence of ever and current wheezing was 19% and 9% respectively. The former was significantly higher in males than females (p<0.001). About 2% of the participants, which was 22.1% of students with current wheezing, had severe asthma. The prevalence of severe asthma was significantly higher in males (p=0.026). Our results showed that 4.8% of students had confirmed asthma by a doctor, significantly predominant in males (p<0.001). The prevalence of using inhaled medicines in the past 12 months was 3.8%, which was significantly higher in males than females (p<0.001). The prevalence of using short-acting β-agonists (SABA) and long-acting β-agonists (LABA) was 2.3% and 1.4% among the students, respectively, with a male predominance (p=0.019). About 1.2% and 0.8% of participants were using inhaled corticosteroids (ICS) and the combination of ICS and LABA, respectively, which was significantly higher in male participants (p<0.001). According to our findings and in comparison to the previous ISAAC study in Yazd, the prevalence of severe asthma has decreased which indicates better management of asthma during the past two decades.
- Published
- 2021
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40. Monogenic Primary Immunodeficiency Disorder Associated with Common Variable Immunodeficiency and Autoimmunity.
- Author
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Asgardoon MH, Azizi G, Yazdani R, Sohani M, Pashangzadeh S, Kalantari A, Shariat M, Shafiei A, Salami F, Jamee M, Rasouli SE, Mohammadi J, Hassanpour G, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Alyasin S, Jabbari-Azad F, Ghaffari J, Mesdaghi M, Ahanchian H, Khoshkhui M, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Nasiri Kalmarzi R, Esmaeilzadeh H, Tafaroji J, Khalili A, Sadeghi-Shabestari M, Darougar S, Moghtaderi M, Ahmadiafshar A, Shakerian B, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Fallahpour M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahimi Haji-Abadi M, Ashournia P, Razaghian A, Rezaei A, Delavari S, Shirmast P, Babaha F, Samavat A, Mamishi S, Khazaei HA, Negahdari B, Rezaei N, Abolhassani H, and Aghamohammadi A
- Subjects
- Adolescent, Adult, Autoimmune Diseases diagnosis, Autoimmune Diseases epidemiology, Autoimmunity genetics, Child, Cohort Studies, Common Variable Immunodeficiency diagnosis, Common Variable Immunodeficiency epidemiology, Delayed Diagnosis, Female, Humans, Immunologic Deficiency Syndromes diagnosis, Immunologic Deficiency Syndromes epidemiology, Iran epidemiology, Male, Exome Sequencing, Young Adult, Adaptor Proteins, Signal Transducing genetics, Autoimmune Diseases genetics, Common Variable Immunodeficiency genetics, Immunologic Deficiency Syndromes genetics, Mutation genetics
- Abstract
Background: Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency disorder mainly characterized by recurrent bacterial infections besides other immunological defects including loss of or dysfunction of B cells and decreased immunoglobulin levels. In this study, our aim is to evaluate clinical, immunological, and molecular data of patients with a primary clinical diagnosis of CVID and autoimmune phenotype with a confirmed genetic diagnosis., Methods: Among 297 patients with CVID, who were registered in the Iranian Primary Immunodeficiency Registry at Children's Medical Center Hospital in Iran, 83 patients have been genetically examined and 27 patients with autoimmunity and confirmed genetic mutations were selected for analysis. Whole-exome sequencing and confirmatory Sanger sequencing methods were used for the study population. A questionnaire was retrospectively filled for all patients to evaluate demographic, laboratory, clinical, and genetic data., Results: In the 27 studied patients, 11 different genetic defects were identified, and the most common mutated gene was LRBA, reported in 17 (63.0%) patients. Two patients (7.7%) showed autoimmune complications as the first presentation of immunodeficiency. Eleven patients (40.7%) developed one type of autoimmunity, and 16 patients (59.3%) progressed to poly-autoimmunity. Most of the patients with mono-autoimmunity (n = 9, 90.0%) primarily developed infectious complications, while in patients with poly-autoimmunity, the most common first presentation was enteropathy (n = 6, 37.6%). In 13 patients (61.9%), the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency. The most frequent autoimmune manifestations were hematologic (40.7%), gastrointestinal (48.1%), rheumatologic (25.9%), and dermatologic (22.2%) disorders. Patients with poly-autoimmunity had lower regulatory T cells than patients with mono-autoimmunity., Conclusion: In our cohort, the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency in most patients. This association highlights the fact that patients referring with autoimmune manifestations should be evaluated for humoral immunity., (© 2020 S. Karger AG, Basel.)
- Published
- 2020
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41. Antiepileptic Hypersensitivity Syndrome to Phenobarbital: A Case Report.
- Author
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Nafei Z, Behniafard N, and Shefai F
- Subjects
- Child, Preschool, Drug Hypersensitivity Syndrome drug therapy, Female, Humans, Immunoglobulins, Intravenous therapeutic use, Methylprednisolone therapeutic use, Anticonvulsants adverse effects, Drug Hypersensitivity Syndrome etiology, Phenobarbital adverse effects
- Abstract
Phenobarbital is still one of the most commonly used medical treatments for different types of seizures. It has numerous different side-effects. Antiepileptic hypersensitivity syndrome (AHS) is a rare and potentially life-threatening adverse reaction to aromatic anticonvulsants such as phenobarbital. Its characteristic features are fever, rash, and lymphadenopathy with different severity of hematologic abnormalities. This case report presents a 26-month-old girl that developed fever, disseminated maculopapular rash, petechiae and thrombocytopenia two weeks after the initiation of phenobarbital prescribed for febrile seizure prophylaxis. The patient was admitted in our center with the impression of hypersensitivity syndrome, so phenobarbital was discontinued and her treatment was resumed with methylprednisolone and intravenous immunoglobulin. After a few days, all symptoms improved and the platelet count was normalized. Thrombocytopenia is a rare complication of hypersensitivity syndrome to phenobarbital in children. Paying attention to this point can prevent the life-threatening adverse effects of this highly consumed medicine.
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- 2019
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42. Candidiasis associated with very early onset inflammatory bowel disease: First IL10RB deficient case from the National Iranian Registry and review of the literature.
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Yazdani R, Moazzami B, Madani SP, Behniafard N, Azizi G, Aflatoonian M, Abolhassani H, and Aghamohammadi A
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- Age of Onset, Child, Preschool, Colonoscopy, Humans, Inflammatory Bowel Diseases pathology, Iran, Male, Mutation, Recurrence, Candidiasis, Oral genetics, Inflammatory Bowel Diseases genetics, Interleukin-10 Receptor beta Subunit genetics
- Abstract
Defects in interleukin-10 (IL10) and interleukin-10 receptors (IL10R) are closely related to very early onset (infantile) inflammatory bowel disease (VEO-IBD). In the present study, we report a novel homozygous null mutation within interleukin-10 receptor B (IL10RB) gene in a child presenting with severe VEO-IBD. In accordance with previous reports, our patient manifested with chronic diarrhea, failure to thrive, intermittent fever and multiple anal ulcers associated with Candidiasis. Homozygous null mutation within IL10RB gene (c.92C > T, p.S31P) affecting the extracellular domain of protein was discovered in this patient. In conclusion, the diagnosis of IL-10R gene mutations should always be considered as a possible cause of refractory diarrhea and failure to thrive. Mutation analysis could help detect the genetic defects associated with these clinical manifestations and to determine the most appropriate treatment option for patients affected by this disease., (Copyright © 2019 Elsevier Inc. All rights reserved.)
- Published
- 2019
- Full Text
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43. The Effect of Gelfoam Impregnated With Botulinum Toxin on Allergic Rhinitis.
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Zand V, Baradaranfar M, Dadgarnia M, Meybodian M, Vaziribozorg S, Mandegari M, Behniafard N, and Dehghani A
- Abstract
Introduction: This study evaluated the effect of gelfoam impregnated with botulinum toxin on the symptoms induced by allergic rhinitis., Materials and Methods: In total, 30 patients with allergic rhinitis who did not respond to common therapies were included in this clinical trial study. All patients were treated with intranasal gelfoam impregnated with botulinum toxin type a (40 unit in each side) placed in the middle meatus of each nostril. The main symptoms of allergic rhinitis were scored from zero to three by the patients. Symptoms recorded and compared before and two months after the treatment., Result: The mean age of patients was 31.03±6.9 years. The mean score for sneezing was 2.23 before the treatment which significantly decreased to 1.06 after the treatment (P<0.05). The mean scores of rhinorrhea, nasal congestion, and nasal itching were 2.53, 2.03, and 1.93, respectively, before the treatment which significantly decreased to 0.93, 1, and 0.8 after the treatment (P<0.05). No reported side effects was observed in this study., Conclusion: According to the results, treatment with gelfoam impregnated with botulinum toxin is an effective and safe method in patients who have not responded to common therapies for allergic rhinitis. Accordingly, it is recommended to relieve symptoms in patients with seasonal allergic rhinitis in order to maintain the effectiveness of this treatment at least 8 weeks.
- Published
- 2019
44. Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort.
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Yazdani R, Abolhassani H, Kiaee F, Habibi S, Azizi G, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, Gharagozlou M, Movahedi M, Hamidieh AA, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Amin R, Aleyasin S, Faridhosseini R, Jabbari-Azad F, Mohammadzadeh I, Ghaffari J, Shafiei A, Kalantari A, Mansouri M, Mesdaghi M, Babaie D, Ahanchian H, Khoshkhui M, Soheili H, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Tavassoli M, Kalmarzi RN, Mortazavi SH, Kashef S, Esmaeilzadeh H, Tafaroji J, Khalili A, Zandieh F, Sadeghi-Shabestari M, Darougar S, Behmanesh F, Akbari H, Zandkarimi M, Abolnezhadian F, Fayezi A, Moghtaderi M, Ahmadiafshar A, Shakerian B, Sajedi V, Taghvaei B, Safari M, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Bazregari S, Bazargan N, Fallahpour M, Khayatzadeh A, Javahertrash N, Bashardoust B, Zamani M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahim M, Ashournia P, Razaghian A, Rezaei A, Samavat A, Mamishi S, Khazaei HA, Mohammadi J, Negahdari B, Parvaneh N, Rezaei N, Lougaris V, Giliani S, Plebani A, Ochs HD, Hammarström L, and Aghamohammadi A
- Subjects
- Adolescent, Adult, Agammaglobulinaemia Tyrosine Kinase genetics, CD40 Ligand genetics, Child, Child, Preschool, Diarrhea genetics, Diarrhea mortality, Female, Genetic Association Studies, Humans, Immunoglobulin mu-Chains genetics, Male, Meningitis genetics, Meningitis mortality, Mutation, Poliomyelitis genetics, Poliomyelitis mortality, Severity of Illness Index, Young Adult, Agammaglobulinemia genetics, Agammaglobulinemia mortality, Common Variable Immunodeficiency genetics, Common Variable Immunodeficiency mortality, Hyper-IgM Immunodeficiency Syndrome genetics, Hyper-IgM Immunodeficiency Syndrome mortality
- Abstract
Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses., Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings., Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID., Results: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 Bruton's tyrosine kinase [BTK] and 6 μ heavy chain deficiencies), HIgM syndrome (21 CD40 ligand and 7 activation-induced cytidine deaminase deficiencies), and CVID (17 lipopolysaccharides-responsive beige-like anchor deficiency and 12 atypical Immunodeficiency, Centromeric instability, and Facial dysmorphism syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40 ligand mutations compared with patients with BTK (P = .003) and activation-induced cytidine deaminase (P = .009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P < .001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in most patients with Immunodeficiency, Centromeric instability, and Facial dysmorphism were respiratory complications (P = .008), whereas first presentations in patients with lipopolysaccharides-responsive beige-like anchor deficiency were nonrespiratory complications (P = .008)., Conclusions: This study highlights similarities and differences in the clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment., (Copyright © 2018 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)
- Published
- 2019
- Full Text
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45. Fourth Update on the Iranian National Registry of Primary Immunodeficiencies: Integration of Molecular Diagnosis.
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Abolhassani H, Kiaee F, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, Yazdani R, Azizi G, Habibi S, Gharagozlou M, Movahedi M, Hamidieh AA, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Amin R, Aleyasin S, Faridhosseini R, Jabbari-Azad F, Mohammadzadeh I, Ghaffari J, Shafiei A, Kalantari A, Mansouri M, Mesdaghi M, Babaie D, Ahanchian H, Khoshkhui M, Soheili H, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Tavassoli M, Kalmarzi RN, Mortazavi SH, Kashef S, Esmaeilzadeh H, Tafaroji J, Khalili A, Zandieh F, Sadeghi-Shabestari M, Darougar S, Behmanesh F, Akbari H, Zandkarimi M, Abolnezhadian F, Fayezi A, Moghtaderi M, Ahmadiafshar A, Shakerian B, Sajedi V, Taghvaei B, Safari M, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Bazregari S, Bazargan N, Fallahpour M, Khayatzadeh A, Javahertrash N, Bashardoust B, Zamani M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahimi M, Ashournia P, Razaghian A, Rezaei A, Mamishi S, Parvaneh N, Rezaei N, Hammarström L, and Aghamohammadi A
- Subjects
- Adolescent, Adult, Age Factors, Child, Child, Preschool, Disease Susceptibility, Female, Follow-Up Studies, Genetic Predisposition to Disease, Genetic Testing, Geography, Medical, Humans, Immunologic Deficiency Syndromes diagnosis, Immunologic Deficiency Syndromes etiology, Infant, Infant, Newborn, Iran epidemiology, Male, Middle Aged, Molecular Diagnostic Techniques, Population Surveillance, Prevalence, Registries, Young Adult, Immunologic Deficiency Syndromes epidemiology
- Abstract
Background: The number of inherited diseases and the spectrum of clinical manifestations of primary immunodeficiency disorders (PIDs) are ever-expanding. Molecular diagnosis using genomic approaches should be performed for all PID patients since it provides a resource to improve the management and to estimate the prognosis of patients with these rare immune disorders., Method: The current update of Iranian PID registry (IPIDR) contains the clinical phenotype of newly registered patients during last 5 years (2013-2018) and the result of molecular diagnosis in patients enrolled for targeted and next-generation sequencing., Results: Considering the newly diagnosed patients (n = 1395), the total number of registered PID patients reached 3056 (1852 male and 1204 female) from 31 medical centers. The predominantly antibody deficiency was the most common subcategory of PID (29.5%). The putative causative genetic defect was identified in 1014 patients (33.1%) and an autosomal recessive pattern was found in 79.3% of these patients. Among the genetically different categories of PID patients, the diagnostic rate was highest in defects in immune dysregulation and lowest in predominantly antibody deficiencies and mutations in the MEFV gene were the most frequent genetic disorder in our cohort., Conclusions: During a 20-year registration of Iranian PID patients, significant changes have been observed by increasing the awareness of the medical community, national PID network establishment, improving therapeutic facilities, and recently by inclusion of the molecular diagnosis. The current collective study of PID phenotypes and genotypes provides a major source for ethnic surveillance, newborn screening, and genetic consultation for prenatal and preimplantation genetic diagnosis.
- Published
- 2018
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46. Clinical, immunologic, and genetic spectrum of 696 patients with combined immunodeficiency.
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Abolhassani H, Chou J, Bainter W, Platt CD, Tavassoli M, Momen T, Tavakol M, Eslamian MH, Gharagozlou M, Movahedi M, Ghadami M, Hamidieh AA, Azizi G, Yazdani R, Afarideh M, Ghajar A, Havaei A, Chavoshzadeh Z, Mahdaviani SA, Cheraghi T, Behniafard N, Amin R, Aleyasin S, Faridhosseini R, Jabbari-Azad F, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Mansouri M, Mesdaghi M, Babaie D, Mohammadzadeh I, Ghaffari J, Shafiei A, Kalantari N, Ahanchian H, Khoshkhui M, Soheili H, Dabbaghzadeh A, Shirkani A, Nasiri Kalmarzi R, Mortazavi SH, Tafaroji J, Khalili A, Mohammadi J, Negahdari B, Joghataei MT, Al-Ramadi BK, Picard C, Parvaneh N, Rezaei N, Chatila TA, Massaad MJ, Keles S, Hammarström L, Geha RS, and Aghamohammadi A
- Subjects
- Adolescent, Child, Child, Preschool, Consanguinity, Female, Genes, Recessive genetics, Genes, Recessive immunology, Genetic Predisposition to Disease genetics, High-Throughput Nucleotide Sequencing methods, Humans, Immunologic Deficiency Syndromes mortality, Infant, Iran, Job Syndrome genetics, Job Syndrome immunology, Job Syndrome mortality, Male, Mutation genetics, Mutation immunology, Phenotype, Retrospective Studies, STAT3 Transcription Factor genetics, STAT3 Transcription Factor immunology, Sequence Analysis, DNA methods, Survival Rate, Immunologic Deficiency Syndromes genetics, Immunologic Deficiency Syndromes immunology
- Abstract
Background: Combined immunodeficiencies (CIDs) are diseases of defective adaptive immunity with diverse clinical phenotypes. Although CIDs are more prevalent in the Middle East than Western countries, the resources for genetic diagnosis are limited., Objectives: This study aims to characterize the categories of patients with CIDs in Iran clinically and genetically., Methods: Clinical and laboratory data were obtained from 696 patients with CIDs. Patients were subdivided into those with syndromic (344 patients) and nonsyndromic (352 patients) CIDs. Targeted DNA sequencing was performed on 243 (34.9%) patients., Results: The overall diagnostic yield of the 243 sequenced patients was 77.8% (189 patients). The clinical diagnosis of hyper-IgE syndrome (P < .001), onset of disease at greater than 5 years (P = .02), and absence of multiple affected family members (P = .04) were significantly more frequent in the patients without a genetic diagnosis. An autosomal recessive disease was found in 62.9% of patients, reflecting the high rate of consanguinity in this cohort. Mutations impairing VDJ recombination and DNA repair were the most common underlying causes of CIDs. However, in patients with syndromic CIDs, autosomal recessive mutations in ataxia-telangiectasia mutated (ATM), autosomal dominant mutations in signal transducer and activator of transcription 3 (STAT3), and microdeletions in 22q11.21 were the most commonly affected genomic loci. Patients with syndromic CIDs had a significantly lower 5-year survival rate rather than those with nonsyndromic CIDs., Conclusions: This study provides proof of principle for the application of targeted next-generation sequencing panels in countries with limited diagnostic resources. The effect of genetic diagnosis on clinical care requires continued improvements in therapeutic resources for these patients., (Copyright © 2017 American Academy of Allergy, Asthma & Immunology. All rights reserved.)
- Published
- 2018
- Full Text
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47. The Effect of Topical Tranexamic Acid on Bleeding Reduction during Functional Endoscopic Sinus Surgery.
- Author
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Baradaranfar MH, Dadgarnia MH, Mahmoudi H, Behniafard N, Atighechi S, Zand V, Baradaranfar A, and Vaziribozorg S
- Abstract
Introduction: Bleeding is a common concern during functional endoscopic sinus surgery (FESS) that can increase the risk of damage to adjacent vital elements by reducing the surgeon's field of view. This study aimed to explore the efficacy of topical tranexamic acid in reducing intraoperative bleeding., Materials and Methods: This double-blind, randomized clinical trial was conducted in 60 patients with chronic rhinosinusitis with polyposis (CRSwP) who underwent FESS. Patients were randomly divided into two groups; tranexamic or saline treatment. During surgery, normal saline (400 mL) or tranexamic acid (2 g) in normal saline with a total volume of 400 mL were used in the saline and tranexamic groups, respectively, for irrigation and suctioning. The surgeons' assessment of field of view during surgery and intraoperative blood loss were recorded., Results: Mean blood loss was 254.13 mL in the saline group and 235.6 mL in the tranexamic group (P=0.31). No statistically significant differences between the two groups were found in terms of other investigated variables, such as surgical field quality based on Boezzart's scale (P=0.30), surgeon satisfaction based on a Likert scale (P=0.54), or duration of surgery (P=0.22)., Conclusion: Use of tranexamic acid (2 g in 400 mL normal saline) through washing of the nasal mucosa during FESS did not significantly reduce blood loss or improve the surgical field of view. Further studies with larger sample sizes and higher drug concentrations, and using other methods of administration, such as spraying or applying pledgets soaked in tranexamic acid, are recommended.
- Published
- 2017
48. Ten years of experience in clinicopathologic characteristics, treatment and outcome of patients with nasopharyngeal pathologies in Yazd, Iran.
- Author
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Binesh F, Shajari A, Abdollahi S, and Behniafard N
- Abstract
Introduction: Although there are some studies regarding the clinical characteristics and outcome of nasopharyngeal tumors, one such study from Iran has not been reported to date. This study aimed to evaluate the clinical features, treatment, and consequences of nasopharyngeal lesions., Methods: In this cross sectional study, we conducted a retrospective review of patients who were diagnosed with nasopharyngeal pathologies and treated at Shahid Sadoughi Hospital and Shahid Ramazanzadeh Radiotherapy Center in Yazd, Iran, over a period of 10 years (from 2005 to 2014). The variables in the study were the patient's hospital registration number, date, name, age, gender, address, topography, clinical symptoms, morphology, stage, types of treatment, and survival for each subject. Survival data were analyzed using Kaplan-Meier estimates, and multivariate analysis was performed using the Cox regression method. Statistical analyses were performed using IBM-SPSS, version 22., Results: In the study, there were 123 patients, 85 males and 38 females, who had nasopharyngeal lesions. The mean age at diagnosis was 41.51 ± 20.33 years. There were 83 patients with malignant neoplasms and 40 patients with non-malignant lesions. The most common malignant tumor was nasopharyngeal carcinoma, and the most common benign lesion was angiofibroma. Survival analysis was performed for the 83 patients who had malignant neoplasms, and survival was found to be 94.5 ± 4.32 months. There were significant differences in survival based on the stage of the disease, histological type, and the type of treatment (p = 0.001, 0.02, and 0.003, respectively)., Conclusion: In our institutional study, malignant tumors were more common than benign lesions. Patients with nasopharyngeal carcinoma presented relatively late and at an advanced stage. Regular follow-up is necessary for early detection, recurrence, or metastases of malignant tumors., Competing Interests: There is no conflict of interest to be declared.
- Published
- 2016
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49. The comparison of bleeding and pain after tonsillectomy in bipolar electrocautery vs cold dissection.
- Author
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Dadgarnia MH, Aghaei MA, Atighechi S, Behniafard N, Vahidi MR, Meybodian M, Zand V, Vajihinejad M, and Ansari A
- Subjects
- Blood Loss, Surgical statistics & numerical data, Child, Child, Preschool, Cold Temperature, Double-Blind Method, Female, Humans, Male, Operative Time, Prospective Studies, Recurrence, Adenoidectomy methods, Dissection methods, Electrocoagulation methods, Pain, Postoperative epidemiology, Postoperative Hemorrhage epidemiology, Sleep Apnea Syndromes surgery, Tonsillectomy methods, Tonsillitis surgery
- Abstract
Objective: Although tonsillectomy is one of the most common surgeries performed in pediatric, it has potential major complications such as pain and bleeding. This study aimed to compare the bleeding and pain after tonsillectomy in bipolar electrocautery tonsillectomy versus cold dissection., Methods: This double blind clinical trial was conducted on 70 pediatric patients who were candidate of tonsillectomy. Patients were divided into two groups of including bipolar cautery (BC) and cold dissection (CD). operation time, intraoperative blood loss, and postoperative bleeding and pain were evaluated in the current study., Results: In both of the CD and BC groups, no significant difference was found in terms of sex and age. The average amount of the intraoperative blood loss in BC group was 14.086 ± 5.013 ml and in CD group was 26.14 ± 4.46 ml (p. v = 0.0001). The mean time of operation in BC group was 19 ± 2.89 min and in CD group was 29.31 ± 5.29 min (p. v = 0.0001). patients were evaluated in terms of pain on the first, third, fifth, and seventh days after the operation. No statistically significant difference was found between two groups. Moreover, Compared pain scores in all times across two groups, no significant difference was found. In terms of postoperative bleeding, none of the patients in both groups had bleeding during follow-up., Conclusion: Our study showed that bipolar electrocautery tonsillectomy can significantly reduce the operation time and intraoperative blood loss; however, postoperative pain and blood loss were similar in both techniques. We recommend bipolar electrocautery as the most suitable alternative method for tonsillectomy, especially in children., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)
- Published
- 2016
- Full Text
- View/download PDF
50. Impact of IgE-mediated Food Allergy on Parental Quality of Life in Iranian Patients.
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Fathi SM, Tavakol M, Rezaei N, Movahedi M, Aghamohammadi A, Shariat M, Sadeghi B, Behniafard N, Darabi B, Hajikhani A, Abdollahpour I, and Gharagozlou M
- Subjects
- Child, Preschool, Female, Humans, Iran, Male, Middle Aged, Parents, Sex Factors, Cost of Illness, Food Hypersensitivity, Immunoglobulin E, Quality of Life, Stress, Psychological
- Abstract
Food allergy is a common disorder especially in the first years of life. Strict avoidance of the responsible food is the most effective therapeutic measure so far. But this continuous vigilance could be stressful for the patient and family and decreases their quality of life (QOL). This survey was designed to evaluate the impact of IgE-mediated food allergy on parental QOL in Iranian patients and to develop a valid Persian version of "Food Allergy Quality Of Life- Parental Burden (FAQL-PB) questionnaire". 90 patients (28 females, 62 males) and their parents who were referred to the clinic of Allergy in Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran were enrolled. The questionnaire was translated with scientific method and its reliability was approved in a pilot study (ICC=0.75, cronbach-α=0.90). Among all patients the most common allergens were wheat (60%) and cow's milk (42%). The patient's age (p=0.02), parent's gender (p=0.004), mother's age (p=0.02), duration of the disease (p=0.048), and allergen multiplicity (p=0.004) were found to have the most significant correlation with family and social activity (FSA) domain. The most meaningful associations were achieved between parent's gender (p<0.001) and emotional issues (EM); as well as meal preparation (MP) with patient's age (p=0.02), parent's gender (p<0.0001) and also allergen multiplicity (p= 0.003); likewise nutrition and health concern (NH) domain with parent's gender (p<0.001). Anaphylaxis's history did not place any burden on each domain. It was concluded that presence of food allergic patients in families could considerably affect all domains of QOL.
- Published
- 2016
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