Publications by authors named "Maryam Khoshkhui"

29 Publications

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How are patients with chronic urticaria interested in using information and communication technologies to guide their healthcare? A UCARE study.

World Allergy Organ J 2021 Jun 4;14(6):100542. Epub 2021 Jun 4.

Allergy Department, Clinica San Felipe, Lima, Peru.

Background: Patients with chronic urticaria (CU) are increasingly using information and communication technologies (ICTs) to manage their health. What CU patients expect from ICTs and which ICTs they prefer remains unknown. We assessed why CU patients use ICTs, which ones they prefer, and what drives their expectations and choices.

Methods: In this cross-sectional study, 1841 patients across 17 countries were recruited at UCAREs (Urticaria Centers of Reference and Excellence). Patients with CU who were >12 years old completed a 23-item questionnaire.

Results: Most patients were interested in receiving disease information (87.3%), asking physicians about CU (84.1%), and communicating with other patients through ICTs (65.6%). For receiving disease information, patients preferred one-to-one and one-to-many ICTs, especially web browsers. One-to-one ICTs were also the ICTs of choice for asking physicians about urticaria and for communicating with other patients, and e-mail and WhatsApp were the preferred ICTs, respectively. Many-to-many ICTs such as Facebook, Instagram, LinkedIn, and Twitter were least preferred for all 3 purposes. Living in rural areas and higher education were linked to higher odds of being interested in receiving disease information, asking physicians, and communicating with patients through ICTs.

Conclusions: Most patients and especially patients with higher education who live in rural areas are interested in using ICTs for their healthcare, but prefer different ICTs for different purposes, ie, web browsers for obtaining information, e-mail for asking physicians, and WhatsApp for communicating with other patients. Our findings may help to improve ICTs for CU.
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http://dx.doi.org/10.1016/j.waojou.2021.100542DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8190491PMC
June 2021

Significant association between Taq1 gene polymorphism in vitamin D receptor and chronic spontaneous urticaria in the Northeast of Iran.

Clin Mol Allergy 2021 May 27;19(1). Epub 2021 May 27.

Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Objective: Chronic spontaneous urticaria (CSU) is defined as urticaria with an unknown etiology which persists for more than 6 weeks. CSU is an uncomfortable cutaneous condition that occurs due to an immune-mediated inflammatory reaction. Many studies have demonstrated that vitamin D deficiency and single-nucleotide polymorphisms in the vitamin D receptor (VDR) impact the immune response. In the current study, the frequency of the Taq1 polymorphism in the VDR gene were compared between patients with CSU and individuals without CSU.

Methods: In a case-control study, a group of CSU patients (n = 100) was compared with a group of healthy age- and gender-matched individuals as a control group (n =100) who visited our center between 2015 and 2017. After DNA extraction from EDTA-containing blood, polymerase chain reaction (PCR-RFLP) was used to determine the presence of the Taq1 polymorphism. Serum vitamin D levels were measured using ELISA method (Abcam, Cambridge, USA).

Results: Genotyping for Taq1 polymorphism showed that TT, Tt and tt genes frequency in the CSU group were 36%, 54%, and 10% respectively. The TT, Tt and tt genotypes had a distribution of 50%, 47% and 3% respectively in the control group. The mean serum vitamin D level in the CSU group was 19.88 ± 8.14 ng/ml, which was not significantly correlated with the Taq1 polymorphism (P = 0.841). There was a significant relationship between Taq1 gene polymorphism (tt genotype) and CSU (P = 0.038). Tt genotype increased the risk of CSU (odds ratio = 1.596), and inheritance of tt genotype increased the risk even further (odds ratio = 4.630).

Conclusion: The frequency of Taq1 genotype polymorphism in the VDR gene was significantly higher in patients with CSU compared to the control group. The tt genotype polymorphism may be a risk factor for CSU.
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http://dx.doi.org/10.1186/s12948-021-00145-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8161666PMC
May 2021

Effects of pregnancy on chronic urticaria: Results of the PREG-CU UCARE study.

Allergy 2021 May 22. Epub 2021 May 22.

Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Urticaria Center of Reference and Excellence (UCARE), Charité - Universitätsmedizin Berlin, Berlin, Germany.

Background: Chronic urticaria (CU) predominantly affects women, and sex hormones can modulate disease activity in female CU patients. As of now, the impact of pregnancy on CU is largely unknown.

Aim: To analyze the course and features of CU during and after pregnancy.

Patients And Methods: PREG-CU is an international, multicenter study of the Urticaria Centers of Reference and Excellence (UCARE) network. Data were collected via a 47-item questionnaire completed by CU patients, who became pregnant within the last 3 years.

Results: A total of 288 pregnancies of 288 CU patients from 13 countries were analyzed (mean age at pregnancy: 32.1 ± 6.1 years, duration of CU: 84.9 ± 74.5 months; CSU 66.9%, CSU + CIndU 20.3%, CIndU 12.8%).During pregnancy, 51.1% of patients rated their CU as improved, 28.9% as worse, and 20.0% as unchanged.CU exacerbations most commonly occurred exclusively during the third trimester (in 34 of 124 patients; 27.6%) or the first (28 of 124; 22.8%). The risk factors for worsening of CU during pregnancy were having mild disease and no angioedema before pregnancy, not taking treatment before pregnancy, CIndU, CU worsening during a previous pregnancy, treatment during pregnancy, and stress as a driver of exacerbations. After giving birth, urticaria disease activity remained unchanged in 43.8% of CU patients, whereas 37.4% and 18.1% experienced worsening and improvement, respectively.

Conclusions: These results demonstrate the complex impact of pregnancy on the course of CU and help to better counsel patients who want to become pregnant and to manage CU during pregnancy.
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http://dx.doi.org/10.1111/all.14950DOI Listing
May 2021

Autoimmune manifestations among 461 patients with monogenic inborn errors of immunity.

Pediatr Allergy Immunol 2021 Mar 27. Epub 2021 Mar 27.

Department of Pediatrics, Hamedan University of Medical Sciences, Hamedan, Iran.

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.
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http://dx.doi.org/10.1111/pai.13510DOI Listing
March 2021

COVID-19 in a child with primary antibody deficiency.

Clin Case Rep 2021 Feb 8;9(2):755-758. Epub 2020 Dec 8.

Research Center for Immunodeficiencies, Children's Medical Center Tehran University of Medical Sciences Tehran Iran.

Although presentation of COVID-19 in patients with immunodeficiency could be mild, it should not be missed, while early diagnosis and appropriate treatment can survive infected patients. Because even severe infections in PID patients may be presented with few symptoms and signs, this diagnosis should be considered in those immunocompromised patients who have exacerbating preexisting symptoms.
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http://dx.doi.org/10.1002/ccr3.3643DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7869323PMC
February 2021

Rhinitis as the Presenting Symptom of Pineal Region Epidermoid Tumor: A Case Report.

Iran J Allergy Asthma Immunol 2020 Aug 25;19(4):452-455. Epub 2020 Aug 25.

Department of Immunology and Allergy, Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Brain tumors are the rarest cause of cerebrospinal fluid rhinorrhea. Non-traumatic cerebrospinal fluid rhinorrhea is also a relatively rare condition. It may be misdiagnosed as allergic rhinitis or chronic sinusitis and lead to unsuitable treatment. We described a 34-year-old man who came to our allergy clinic with a chief complaint of clear rhinorrhea from his left nostril with more than four years of duration. Onlyhypertrophy of left inferior concha was found in the clinical examination. His rhinorrhea aggravated when bending forward. So we were suspicious of CSF rhinorrhea. MRI was done for him and demonstrated a large tumor in the pineal region. The patient underwent surgery with resection of the mass via an infratentorial-supracerebellar approach. This case showed the role of maintaining differential diagnosis for a common complaint; rhinitis which is seen as usual.
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http://dx.doi.org/10.18502/ijaai.v19i4.4121DOI Listing
August 2020

The usage, quality and relevance of information and communications technologies in patients with chronic urticaria: A UCARE study.

World Allergy Organ J 2020 Nov 30;13(11):100475. Epub 2020 Oct 30.

Allergy Department, Clinica San Felipe, Lima, Peru.

Background: Chronic urticaria (CU) is characterized by itchy recurrent wheals, angioedema, or both for 6 weeks or longer. CU can greatly impact patients' physical and emotional quality of life. Patients with chronic conditions are increasingly seeking information from information and communications technologies (ICTs) to manage their health. The objective of this study was to assess the frequency of usage and preference of ICTs from the perspective of patients with CU.

Methods: In this cross-sectional study, 1800 patients were recruited from primary healthcare centers, university hospitals or specialized clinics that form part of the UCARE (Urticaria Centers of Reference and Excellence) network throughout 16 countries. Patients were >12 years old and had physician-diagnosed chronic spontaneous urticaria (CSU) or chronic inducible urticaria (CIndU). Patients completed a 23-item questionnaire containing questions about ICT usage, including the type, frequency, preference, and quality, answers to which were recorded in a standardized database at each center. For analysis, ICTs were categorized into 3 groups as follows: one-to-one: SMS, WhatsApp, Skype, and email; one-to-many: YouTube, web browsers, and blogs or forums; many-to-many: Instagram, Twitter, Facebook, and LinkedIn.

Results: Overall, 99.6% of CU patients had access to ICT platforms and 96.7% had internet access. Daily, 85.4% patients used one-to-one ICT platforms most often, followed by one-to-many ICTs (75.5%) and many-to-many ICTs (59.2%). The daily ICT usage was highest for web browsers (72.7%) and WhatsApp (70.0%). The general usage of ICT platforms increased in patients with higher levels of education. One-to-many was the preferred ICT category for obtaining general health information (78.3%) and for CU-related information (75.4%). A web browser (77.6%) was by far the most commonly used ICT to obtain general health information, followed by YouTube (25.8%) and Facebook (16.3%). Similarly, for CU-specific information, 3 out of 4 patients (74.6%) used a web browser, 20.9% used YouTube, and 13.6% used Facebook. One in 5 (21.6%) patients did not use any form of ICT for obtaining information on CU. The quality of the information obtained from one-to-many ICTs was rated much more often as very interesting and of good quality for general health information (53.5%) and CU-related information (51.5%) as compared to the other categories.

Conclusions: Usage of ICTs for health and CU-specific information is extremely high in all countries analyzed, with web browsers being the preferred ICT platform.
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http://dx.doi.org/10.1016/j.waojou.2020.100475DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7606865PMC
November 2020

Death due to COVID-19 in an Infant with Combined Immunodeficiencies.

Endocr Metab Immune Disord Drug Targets 2020 Oct 21. Epub 2020 Oct 21.

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad. Iran.

Novel coronavirus disease 2019 (COVID-19) has affected millions of people, which led to death especially in older cases with underlying diseases. Meanwhile pediatric patients with inherited defects of T cell should potentially be prone to viral diseases. Herein, we report an infant with combined immunodeficiency who died because of COVID-19.
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http://dx.doi.org/10.2174/1871530320666201021142313DOI Listing
October 2020

Monogenic Primary Immunodeficiency Disorder Associated with Common Variable Immunodeficiency and Autoimmunity.

Int Arch Allergy Immunol 2020 2;181(9):706-714. Epub 2020 Jul 2.

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

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.
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http://dx.doi.org/10.1159/000508817DOI Listing
February 2021

Evaluation of Serum Substance P Level in Chronic Urticaria and Correlation with Disease Severity.

Iran J Allergy Asthma Immunol 2020 Feb 1;19(1):18-26. Epub 2020 Feb 1.

Department of Immunology, Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Substance P (SP) is a neurotransmitter emitted from neurons that plays a role in the pathogenesis of itching conditions including chronic urticarial (CU). The present research aims to investigate the serum level of S.P among CU patients and compare them with healthy subjects and explore how it correlates with the severity of urticaria. The present research was conducted on 87 CU patients who visited the allergy clinic of Ghaem Hospital, Mashhad, Iran from October 2017 to June 2018. Besides, 86 healthy subjects were recruited as the control group. Background information of patient was collected including age, sex, duration of the disease and the co-occurrence of angioedema. S.P serum level was measured in two groups by ELISA method. In the patients group, the autologous serum skin test (ASST) was performed along with the urticaria evaluation questionnaire include Urticaria Activity Score 7 (UAS7), Urticaria Control Test (UCT) and Chronic Urticaria Quality of Life (CU-Q2OL). Among the patients, the SP serum level showed to be about two times higher than the healthy subjects (p˂0.001). SP showed to be increased as patients' age grew (p=0.010). In patients with a positive ASST, SP level was higher (p=0.012). No correlation was found between SP and the presence of angioedema among patients. There was no correlation between the SP serum level and the scores obtained from urticaria evaluation questionnaires. SP among CU patients was higher than healthy subjects. SP was also higher among female, older and positive ASST patients. The SP value was not correlated with the severity of urticaria, angioedema. In conclusion, Using SP antagonist drugs could be a potential treatment for chronic urticaria.
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http://dx.doi.org/10.18502/ijaai.v19i1.2414DOI Listing
February 2020

Effects of Serum Vitamin D and Efficacy of Subcutaneous Immunotherapy in Adult Patients With Allergic Rhinitis.

Allergy Asthma Immunol Res 2019 Nov;11(6):885-893

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Immunotherapy is the standard of treatment for long-life relief of symptoms of allergic rhinitis. Vitamin D may affect the outcomes of treatment. This study evaluated the clinical efficacy of subcutaneous allergen immunotherapy in adult patients with allergic rhinitis based on the serum level of vitamin D. Patients with persistent allergic rhinitis and positivity for skin prick test were evaluated by Sino-nasal Outcome Test (SNOT-22) and Mini Rhinoconjunctivitis Quality of Life Questionnaire (MiniRQLQ) before subcutaneous allergen immunotherapy and during the maintenance phase to assess the relation of the serum level of vitamin D and the clinical efficacy of immunotherapy. After immunotherapy, the greatest reduction in SNOT-22 scores were reported in patients with vitamin D sufficiency (39.0 ± 9.2), followed by vitamin D suboptimal provision (35.1 ± 12.1), insufficiency (25.0 ± 7.5), and deficiency (18.3 ± 6.0) ( < 0.001). The MiniRQLQ reduction in patients with vitamin D sufficiency, suboptimal provision, insufficiency, or deficiency was 30.7 ± 8.7, 27.1 ± 8.7, 20.0 ± 8.6, or 17.4 ± 7.1, respectively ( < 0.001). Both of SNOT-22 and MiniRQLQ scores decreased significantly following immunotherapy in patients with different levels of vitamin D. However, these effects were more pronounced when the level of vitamin D was sufficient.
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http://dx.doi.org/10.4168/aair.2019.11.6.885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6761068PMC
November 2019

The expression of serotonin transporter protein in the skin of patients with chronic spontaneous urticaria and its relation with depression and anxiety.

Arch Dermatol Res 2019 Dec 13;311(10):825-831. Epub 2019 Sep 13.

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Studies have indicated a possible role for serotonin transporter protein (SERT) in the pathophysiology of inflammatory skin disorders. This study was aimed to determine the expression of SERT in the skin of patients with chronic spontaneous urticaria (CSU) and its relation to depression and anxiety. In this case-control study, 30 CSU patients and 30 healthy controls were evaluated with skin biopsies to evaluate the expression of the SERT protein based on histopathologic findings. Beck depression and anxiety inventories were used to investigate depression and anxiety in the case group. Data were analyzed by SPSS software. P values < 0.05 were considered significant. The case group showed significantly higher percentage of stained cells (P < 0.0001) and intensity of SERT expression (P < 0.0001) compared with the control group. The patients with uncontrolled CSU showed significantly higher percentage (P < 0.002) and intensity (P < 0.006) of SERT expression, compared with those with controlled CSU. The intensity of SERT expression in CSU patients had no significant correlation with the severity of depression, but was significantly correlated with the severity of anxiety (r = 0.555; P = 0.001). The percentage of stained cells was significantly correlated with the severities of depression (r = - 0.433; P = 0.017) and anxiety (r = 0.528; P = 0.003). The SERT expression in patients with CSU was higher compared with controls, which can demonstrate the role of serotonin in the pathogenesis of this disease. This higher SERT expression is correlated with the severity of the disease.
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http://dx.doi.org/10.1007/s00403-019-01969-yDOI Listing
December 2019

Investigation of the relationship between atopy and psoriasis.

Postepy Dermatol Alergol 2019 Jun 18;36(3):276-281. Epub 2019 Jun 18.

Allergy Research Center, Mashhad University of Medical sciences, Mashhad, Iran.

Introduction: The relationship between allergic and autoimmune diseases is an important issue, which has recently attracted the researchers' interest.

Aim: To determine the relationship between atopy and psoriasis.

Material And Methods: This case-control study was conducted on 102 patients referred to the Ghaem Hospital, Mashhad, Iran, in 2016. The participants were assigned into two groups: experimental and control groups, including the patients suffering from psoriasis and those with no history of cutaneous or other systemic diseases, respectively. Both groups filled in the ISAAC questionnaire and had skin prick tests. In addition, the serum levels of immunoglobulin E (IgE) and blood eosinophil cell count were measured. The data were analysed using the regression test through SPSS version 16.

Results: According to the results of the ISAAC questionnaire, there was a significant difference between the control and experimental groups in terms of asthma ( = 0.04). The mean serum concentrations of IgE and eosinophil cell count were not significantly different between the experimental (153.93 IU/ml and 187.77 cells/μl, respectively) and control groups (152.19 IU/ml and 187.68 cells/μl, respectively) ( = 0.057 and = 0.886, respectively). In addition, there was an indirect correlation between the eosinophil cell count and psoriasis severity ( = 0.032, = -0.297). Furthermore, the comparison of the skin prick test results revealed no significant difference between the two groups regarding the number of positive and negative cases ( = 0.436).

Conclusions: The findings suggested that atopy was not common in the patients with psoriasis and supported the concept that atopy protects against such autoimmune diseases such as psoriasis.
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http://dx.doi.org/10.5114/ada.2019.85639DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6640011PMC
June 2019

Evaluation of Sub cutaneousRush Immunotherapy Effectiveness in Perennial Allergic Rhinitis after a Year from Treatment.

Iran J Otorhinolaryngol 2019 May;31(104):135-139

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Introduction: Allergen immunotherapy is an effective treatment for allergic rhinitis. Conventional immunotherapy takes at least 5 to 6 months to reach the maintenance dosage; nonetheless, rush immunotherapy accelerates to reach the maintenance dose several months earlier. However, the safety and efficacy of this treatment has not been widely investigated. The objective of the present study was to determine the efficacy of subcutaneous rush immunotherapy in the patients with perennial allergic rhinitis after a year from treatment.

Materials And Methods: This study was carried out on a total of 15 patients with allergic rhinitis who received rush immunotherapy and were evaluated for the quality of life and clinical symptoms improvement with Sino-Nasal Outcome Test Questionnaire (SNOT-22) and Mini Rhino conjunctivitis Quality of Life Questionnaire (RQLQ) before and after a year from treatment. Moreover, specific weed mix Immunoglobulin E (IgE) was measured before and after a year from treatment. Statistical analysis was performed using SPSS software (version 16) (P<0.05).

Results: The comparison of specific IgE indicated a significant reduction between before and after a year from treatment (P=0.005for pigweed)(P=0.022 for salsola). There was a significant decrease in clinical symptoms according to SNOT-22 Questionnaire [(mean score: 46.00, before the treatment) and (mean score: 14.06, after the treatment)]. The quality of life for most of the patients was moderate (46.7%) before the treatment and good (80%) after the treatment, which was considered statistically significant (P>0.001).

Conclusion: Rush immunotherapy is an effective treatment in the patients with allergic rhinitis. It seems to be an alternative treatment in cases that need more rapid treatment. However, it is recommended to carry out other studies on the control group.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6556749PMC
May 2019

Clinical Manifestations, Immunological Characteristics and Genetic Analysis of Patients with Hyper-Immunoglobulin M Syndrome in Iran.

Int Arch Allergy Immunol 2019 22;180(1):52-63. Epub 2019 May 22.

Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran,

Background: Hyper-immunoglobulin M (HIGM) syndrome is a rare heterogeneous group of primary immunodeficiency disorders characterized by low or absent serum levels of IgG and IgA along with normal or elevated serum levels of IgM.

Methods: Clinical and immunological data were collected from the 75 patients' medical records diagnosed in Children's Medical Center affiliated to Tehran University Medical Sciences and other Universities of Medical Sciences in Iran. Among 75 selected patients, 48 patients (64%) were analyzed genetically using targeted and whole-exome sequencing.

Results: The ratio of male to female was 2.9:1. The median age at the onset of the disease, time of diagnosis, and diagnostic delay were 10.5, 50, and 24 months, respectively. Pneumonia and lower respiratory tract infections (61.3%) were the most common complications. Responsible genes were identified in 35 patients (72.9%) out 48 genetically analyzed patients. Cluster of differentiation 40 ligand gene was the most mutated gene observed in 24 patients (68.5%) followed by activation-induced cytidine deaminase gene in 7 patients, lipopolysaccharide-responsive and beige-like anchor (1 patient), nuclear factor-kappa-B essential modulator (1 patient), phosphoinositide-3-kinase regulatory subunit 1 (1 patient), and nuclear factor kappa B subunit 1 (1 patient) genes. Nineteen (25.3%) patients died during the study period, and pneumonia was the major cause of death occurred in 6 (31.6%) patients.

Conclusion: Physicians in our country should carefully pay attention to respiratory tract infections and pneumonia, particularly in patients with a positive family history. Further investigations are required for detection of new genes and pathways resulting in HIGM phenotype.
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http://dx.doi.org/10.1159/000500197DOI Listing
September 2019

Short-course administration of a traditional herbal mixture ameliorates asthma symptoms of the common cold in children.

Avicenna J Phytomed 2019 Mar-Apr;9(2):126-133

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Objective: Asthma is an increasing chronic respiratory disease affecting over 300 million people worldwide. Several studies have shown that herbal remedies may improve asthma control and reduce asthma symptoms. In this study, the effects of short-course administration of an herbal mixture (ASMATUS) in asthmatic children during viral respiratory tract infection, were evaluated.

Materials And Methods: Forty-six children (7-12 years old) with intermittent asthma were enrolled in this double-blind randomized clinical trial. At the onset of common cold symptoms, the patients were randomly assigned to daily receive either the herbal mixture (comprised of , , , , , and ) or placebo for 5 days. Primary outcomes included day symptoms, night symptoms, and asthma attacks. Secondary outcomes included Peak Expiratory Flow Rate (PEFR), the need for β-agonist administration, oral prednisolone usage, necessity for re-visit due to uncontrolled or insupportable symptoms, as well as the number of hospital admissions and days absent from school.

Results: the herbal mixture significantly decreased the severity of coughs (p=0.049) and nighttime awakenings (p=0.029) in comparison to placebo. There was no significant reduction in wheezing, tachypnea, respiratory distress, PEF rate, absence from school, outpatient visits, asthma exacerbation, oral prednisolone or β-agonist usage and hospitalization.

Conclusion: Short-course of herbal mixture this traditional herbal mixture, starting at the onset of signs of a viral respiratory tract infection in children with intermittent asthma, reduced cough and nights awakening. Further studies should be done to determine the most effective herbal admixture, as well as dose and duration of treatment.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448545PMC
April 2019

Effects of a Novel Barley-Based Formulation on Allergic Rhinitis: A Randomized Controlled Trial.

Endocr Metab Immune Disord Drug Targets 2019 ;19(8):1224-1231

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Objective: Current treatment options for Allergic Rhinitis (AR) may have their own limitations and side effects. This study aimed to investigate the effects of Ma-al-Shaeer (MS), a novel natural formulation based on Hordeum vulgare, in the treatment of AR compared with Fexofenadine (FX).

Methods: A total of 77 patients with AR were divided into two groups: MS group (n=38) and FX group (n=39). The first group received 15 g of dried MS powder, and the second group received 60 mg of FX twice daily for 14 days. At baseline (week zero) and after the 14-day treatment period (week two), both groups were evaluated for sneezing, rhinorrhea, nasal congestion, nasal itching, post nasal drip, eye, throat, or ear symptoms, headache, cough, mental function, quality of life scores, blood eosinophil count and total IgE levels. Rhinitis control assessment tests were conducted at week zero and again at one week after cessation of treatment (week three) in both groups.

Results: All symptoms of AR except cough were significantly reduced in both groups; for nasal congestion, post nasal drip, and headache, the MS treatment was found to be superior. Rhinitis control was significantly increased after treatment in both groups (p value < 0.001). Both drugs significantly reduced total IgE levels. There was no significant change in eosinophil count in either group.

Conclusion: MS formulation based on H. vulgare may be an effective treatment for AR. Further studies are needed to confirm the effect of MS as an alternative treatment in AR.
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http://dx.doi.org/10.2174/1871530319666190306100611DOI Listing
April 2020

CD14 as A Serum Immune Biomarker and Genetic Predisposition Factor for Allergic Rhinitis.

Iran J Otorhinolaryngol 2019 Jan;31(102):1-9

Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Introduction: Allergic Rhinitis (AR) is a common inflammatory disease of the nasal mucosa. The CD14 is a receptor for lipopolysaccharide and inhaled endotoxin which can stimulate the production of interleukins by antigen presenting cells. Accordingly, CD14 plays an important role in allergic and atopic diseases, which can be one of the etiological factors for allergic diseases. The present study investigated the association between the CD14 gene polymorphism C-159T and AR and aimed to detect the correlation between serum levels of CD14 and AR.

Materials And Methods: This study was conducted on two groups of participants. The experimental group consisted of 125 patients with AR referring to Ghaem Hospital, Mashhad University of Medical Sciences in Mashhad, Iran, and the control group included 125 healthy subjects from Mashhad National Blood Center, Iran. Serum CD14 levels were measured by enzyme-linked immunosorbent assay. Polymerase chain reaction-restriction fragment length polymorphism was employed to detect C-159T gene polymorphism in the CD14 promoter region.

Results: There was a significant association between CD14 C-159T gene polymorphism and AR (P<0.001). The results of the statistical analysis showed that the TT genotype could significantly increase the risk of AR (P<0.001). Additionally, a significant association was observed between C-159T gene polymorphism and the serum level of CD14 (P<0.001). Regardless of the genotypes, the serum CD14 levels were significantly higher in AR patients than in those of the participants in the controls (P=0.007).

Conclusions: According to the obtained results of this study, CD14 in serum might be a potential marker for the diagnosis of AR, and in genetic levels it might be a predictive factor for the disease.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368989PMC
January 2019

A survey of anaphylaxis etiology and treatment.

Med Gas Res 2018 Oct-Dec;8(4):129-134. Epub 2019 Jan 9.

Department of Pediatric Allergy and Immunology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

Identifying the causes of anaphylaxis which is an acute, potentially fatal systemic reaction is very important in every community. Treatment strategies and pitfalls should also be determined. We sought to determine the most common triggers of anaphylaxis, clinical manifestations and treatment strategies in Mashhad, northeast of Iran. An observational cross-sectional study was conducted to evaluate all patients with a history of anaphylactic reaction who were referred to University Allergy Clinics between 2006 and 2016 in Mashhad Iran. We used a combination of patient's clinical history and allergy diagnostic testing including radioallergosorbant test and skin prick test in order to determine the etiology of anaphylaxis. We identified 172 anaphylactic reactions in 70 patients. Median age was 15 years with a range from 6 months to 48 years. The triggers included: foods, 61.4%; drugs, 15.7%; hymenoptera venom, 8.6%; idiopathic, 5.7%; immunotherapy, 4.3% and other etiologies: 5.7%. Nuts and seeds were the most important triggers of food induced anaphylaxis, especially in school children, adolescents and young adults, followed by fruits. However, Cow's milk and hen's egg were the main triggers of anaphylaxis in children aged under 2 years. The most common symptoms were cutaneous and cardiovascular. Corticosteroids (94.3%) and/or antihistamines (85.7%) were used most frequently for treatment followed by intravenous fluids (54.3%), whereas epinephrine was only used in 17.1% of the cases. Food related anaphylaxis and other typical triggers of anaphylaxis are age dependent and the risks and triggers change with age. Epinephrine injection should be increased by improving the awareness of physician and medical teams. The study was approved by the Ethics Committee of the Faculty of Medicine of Mashhad University of Medical Sciences (approved number: IR.MUMS.REC.1393.960).
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http://dx.doi.org/10.4103/2045-9912.248262DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352571PMC
January 2019

Fourth Update on the Iranian National Registry of Primary Immunodeficiencies: Integration of Molecular Diagnosis.

J Clin Immunol 2018 10 9;38(7):816-832. Epub 2018 Oct 9.

Pediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

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.
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http://dx.doi.org/10.1007/s10875-018-0556-1DOI Listing
October 2018

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort.

J Allergy Clin Immunol Pract 2019 03 19;7(3):864-878.e9. Epub 2018 Sep 19.

Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.

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.
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http://dx.doi.org/10.1016/j.jaip.2018.09.004DOI Listing
March 2019

Quality of Life in Children with Asthma versus Healthy Children.

Open Access Maced J Med Sci 2018 Aug 16;6(8):1413-1418. Epub 2018 Aug 16.

Allergy Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

Background: Asthma is the most prevalent chronic disease in the pediatric age group. The disease affects different aspects of the children's lives, such as physical, emotional, social and educational aspects. Thus, more focus has been on the quality of life in these patients rather than the duration of their illness in recent years.

Aim: This study examined the different aspects of quality of life in asthmatic children for the first time in this geographic area.

Methods: The study was cross-sectional conducted in 2015-2016. The asthmatic group was 100 patients aged 8 to 12 admitted to the Asthma and Allergy Clinic of Ghaem Hospital (as) in Mashhad with the control group composed of 100 healthy children of the same age and gender. The standard questionnaire pedsQLTM was used for comparing the quality of life of children in the two groups. Statistical analysis was SPSS23 with P-value less than 0.05, which was statistically significant.

Results: In each group, 58 patients were boys, and 42 were girls. In a comparison of the quality of life of children, the asthma group with a mean total score of Peds QL 20.99 ± 12.54 compared to the healthy children with a mean total score of Peds QL of 8.8 ± 5.41 had a lower quality of life (P < 0.001). Moreover, regarding various aspects of quality of life asthma group had a lower quality of life in physical performance, emotional performance and performance in school (P < 0.001). Nonetheless, there was no significant difference between the two groups considering social function (P = 0.267). Examining the relationship between Peds QL score of patients with asthma with various variables was indicative of the fact that Peds QL scores were significantly correlated with the gender of the patients, showing better quality of life in the girls (P = 0.001).

Conclusion: The results indicated that children with asthma have a significantly lower quality of life compared with healthy children of the same age. Also, in examining the different aspects of quality of life, these children had a lower quality of life in physical performance, emotional performance, and performance at school, and were at the level as that of healthy children only in social performance.
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http://dx.doi.org/10.3889/oamjms.2018.287DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6108822PMC
August 2018

Clinical, immunologic, and genetic spectrum of 696 patients with combined immunodeficiency.

J Allergy Clin Immunol 2018 04 12;141(4):1450-1458. Epub 2017 Sep 12.

Acquired Immunodeficiency Research Center, Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.

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.
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http://dx.doi.org/10.1016/j.jaci.2017.06.049DOI Listing
April 2018

Evaluation of serum interleukin- 35 level in children with persistent asthma.

Asian Pac J Allergy Immunol 2017 Jun;35(2):91-95

Allergy Research Center, Mashhad University of Medical Sciences, Shiraz, Iran.

Background: IL-35 is a novel cytokine suppressing the immune response through the expansion of regulatory T cells and suppression of Th17 cell development. Few studies have been done on the effect of this cytokine in a different disease. Asthma is a complex disease that many inflammatory cells and cytokines play a role in.

Objective: We decide to determine the difference between serum level of IL-35 in childhood asthma & healthy children.

Method: We obtained serum samples from 44 asthmatic children between 2 and 15 years as a case group and from healthy children as a control group. IL-35 serum concentration was determined by ELISA method in both groups.

Results: Mean serum level is 30.9 pg/ml in the case group and 30.2 pg/ml in the control group. There is no significant difference between serum level of IL-35 in asthmatic & healthy children.

Conclusions: Our data reveal no relation between childhood asthma and serum level of IL35. So, further study will be needed to clarify effects of this cytokine in human allergic diseases.
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http://dx.doi.org/10.12932/AP0761DOI Listing
June 2017

Level of Agreement between Children with Asthma and their Parents on Quality of Life.

Iran J Med Sci 2016 Mar;41(2):86-93

Allergy Research Center, Department of Pediatrics, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Child-parent agreement is a controversial aspect of measuring health-related quality of life (HRQoL) in children and adolescents. The aim of this study was to assess the agreement between the child self-reports and parent proxy reports of the PedsQL 3.0 Asthma Module in Iranian children with asthma to evaluate HRQoL. Moreover, the psychometric properties of the child and parent reports of the PedsQL 3.0 Asthma Module were assessed in the present study.

Methods: Participants were 112 children with asthma and their parents, who completed the Farsi version of the PedsQL 3.0 Asthma Module. The multitrait-multimethod correlation matrix and factor analysis were used to test whether the child self-reports and the parent proxy reports measured the same construct. Additionally, convergent and discriminant validity and internal consistency were assessed using the Pearson correlation.

Results: The correlation between the child and parent HRQoL perceptions ranged between 0.13 and 0.36 across the same domains. Our factor analysis revealed that the child self-reports and the parent proxy reports measured 2 different constructs of HRQoL. Furthermore, our findings showed that both the child self-reports and the parent proxy reports of the PedsQL 3.0 Asthma Module had excellent internal consistency and acceptable convergent and discriminant validity.

Conclusion: Although the child self-reports and the parent proxy reports of the Farsi version of PedsQL 3.0 Asthma Module showed good psychometric properties, they were not interchangeable. Our children with asthma and their parents evaluated child HRQoL from their own viewpoints.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4764972PMC
March 2016

Familial Churg-Strauss Syndrome in a Sister and Brother.

Iran J Allergy Asthma Immunol 2015 Jun;14(3):338-40

Department of Allergy and Clinical Immunology, Shiraz University of Medical Sciences, Shiraz, Iran.

Churg-Strauss syndrome (CSS) is a granulomatous small vessel vasculitis. It is characterized by asthma, allergic granulomatosis and vasculitis. This syndrome is rare in children. A 5 years old boy was admitted with cough, fever and dyspnea for 2 weeks. On the basis of laboratory data (peripheral eosinophilia), associated with skin biopsy, and history of CSS in his sister, this disease was eventually diagnosed. The patient had good response to corticosteroid. In every asthmatic patient with prolonged fever, eosinophilia and multisystemic involvment, CSS should be considered.
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June 2015

A Case of Probable MHC Class II Deficiency with Disseminated BCGitis.

Iran J Immunol 2015 Sep;12(3):219-25

Clinical Immunology and Allergy, Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran, e-mail:

Major histocompatibility complex (MHC) class II deficiency is a primary immunodeficiency disease characterized by abnormality of MHC class II molecules surface expression on peripheral blood lymphocytes and monocytes. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections but the immunodeficiency is not as severe as SCID (severe combined immunodeficiency), as evidenced by failure to develop disseminated infection after BCG vaccination. Therefore, MHC II deficiency with BCGosis, that is disseminated BCGitis, is not reported commonly. We report an interesting case of BCGosis after vaccination that was diagnosed to have probable MHC II deficiency.
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http://dx.doi.org/IJIv12i3A7DOI Listing
September 2015

Autoimmune hemolytic anemia in a patient with probable ataxia telangiectasia: a case report.

Iran J Immunol 2014 Sep;11(3):217-20

Allergy Research Center, Shiraz university of medical science, Shiraz, Iran,

Background: Ataxia telangiectasia (AT) is one of the combined immunodeficiency syndromes with immunologic, neurologic, endocrinologic, hepatic and cutaneous abnormalities. Regarding the fact that autoimmune disorders; such as autoimmune hemolytic anemia (AIHA), are not generally expected in the course of AT, we present a patient with an unusual presentation of these two conditions.

Case Presentation: An otherwise seemingly normal girl, who had developed limping at the age of 11 months old, referred to Namazi Hospital, Shiraz, Iran, due to pallor and latitude at the age of 3 yrs and was diagnosed of AIHA. After 2 years of therapeutic course she developed ocular telangiectasia and ataxic gate.

Conclusion: This case emphasizes on the possibility of ataxia telangiectasia coexistence with autoimmune disorders that must be taken into consideration by physicians.
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http://dx.doi.org/IJIv11i3A8DOI Listing
September 2014

Etiology of Portal Hypertension in Children:A Single Center's Experiences.

Middle East J Dig Dis 2012 Oct;4(4):206-10

Researcher, Gastroenterohepatology Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. In previous studies, extra-hepatic disorders have been reported as the main cause of portal hypertension in children. In this study, we investigate the underlying causes of portal hypertension in children.

Methods: This prospective, descriptive study investigated the etiology of 45 children with portal hypertension who referred to Nemazee Hospital Pediatric Gastroenterology Ward from 2005 to 2007. The underlying causes of portal hypertension were determined by liver biopsy, abdominal sonography, abdominal computed tomography scan, and liver Doppler sonography.

Results: In this study, 42 of 45 patients (93.3%) developed portal hypertension due to intrahepatic diseases. Extra-hepatic portal hypertension was detected in 3 (6.7%) patients with portal vein thrombosis.

Conclusion: Intrahepatic diseases were the most common etiology of portal hypertension among children who referred to our center.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3990125PMC
October 2012