Publications by authors named "Shariat Mansoureh"
How Hospitalizations Can Be Effective in Subsequent Care of Children with Asthma?
Iran J Allergy Asthma Immunol
December 2024
Asthma is a prevalent chronic inflammatory disorder in children, and poor therapeutic response in asthmatic children could result from various factors related to the doctor, patient, disease, and treatment. This study aimed to evaluate the most important causes of failure in asthma control. One hundred three children referred to the Children's Medical Center in Tehran, Iran, participated in this study in 2017.
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- Primary immunodeficiency diseases with partial albinism, such as Chediak Higashi Syndrome (CHS) and Griscelli Syndrome type 2 (GS2), are autosomal recessive disorders characterized by immune deficiencies and abnormal pigmentation.
- The study evaluated 25 patients over the past decade, using genetic analyses and examinations of leukocyte granules and hair shafts to identify these conditions, along with control groups of patients with albinism and healthy individuals.
- The findings indicated specific genetic variants associated with CHS and HPS2, highlighting the importance of timely hematopoietic stem cell transplantation and genetic testing for accurate diagnosis and management of these syndromes.
Clinical, immunological and molecular findings of 8 patients with typical and atypical severe combined immunodeficiency: identification of 7 novel mutations by whole exome sequencing.
Genes Immun
August 2023
Article Synopsis
- - Severe combined immunodeficiency (SCID) is a serious genetic disorder affecting the immune system, leading to critical infections, with variances in disease presentation complicating timely diagnosis.
- - A study described four patients, including both typical and atypical SCID cases, who showed symptoms within six months of birth with various genetic mutations identified, such as in the RAG2, IL7R, ADA, JAK3, LIG4, and LAT genes.
- - The findings provide insights into the genetic diversity of SCID and emphasize the importance of early diagnosis and management for affected individuals, including the confirmation of mutations through Sanger sequencing in both patients and their parents.
Article Synopsis
- This study analyzed 69 patients suspected of having Leukocyte Adhesion Deficiency-I (LAD-I) over a 15-year period to better classify the severity based on clinical, laboratory, and genetic findings.
- The majority of patients were diagnosed at a median age of 6 months, with a notable delayed diagnosis and high rates of severe cases, leading to a 44.9% mortality rate.
- Genetic testing revealed several mutations associated with the disease, highlighting the importance of early detection and awareness among physicians to improve patient outcomes and support families with a history of LAD-I.
Article Synopsis
- Oral immunotherapy (OIT) is a new method for treating food allergies, specifically designed to help patients with wheat allergies develop tolerance.
- A study conducted from 2015 to 2017 involved 26 patients with confirmed wheat allergies, where they underwent OIT treatment and had their specific IgE levels and regulatory T cells monitored.
- Results indicated a high success rate of 100% for desensitization and 93.3% for tolerance, along with a significant drop in specific IgE levels after 12 months, suggesting OIT is an effective approach for wheat allergy management.
Clinical features of children with atopic dermatitis according to variants.
Allergol Immunopathol (Madr)
December 2021
Article Synopsis
- Filaggrin is a crucial protein for skin protection, and mutations in its gene are linked to atopic dermatitis (AD), affecting about 50% of affected individuals.
- The study involved comparing 25 Iranian children with AD to 25 healthy controls, analyzing demographic data and specific genetic variations (polymorphisms) related to filaggrin.
- Results showed that certain polymorphisms were associated with the age and early onset of AD, but no significant links were found regarding disease severity or other allergy-related factors; further research is needed for broader insights.
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.
Serum sickness-like reactions in Iranian children: a registry-based study in a referral center.
Allergol Immunopathol (Madr)
July 2021
Introduction And Objectives: Considering that no studies have been done on a comprehensive review of Serum sickness-like reactions patients (SSLRs) at a referral center in Iran so far, this study aimed to determine the clinical and laboratory characteristics of children with SSRL in Tehran Children's Medical Center.
Patients: The present study was a registry-based study in which the data of 94 SSLRs patients registered in a two-year period were investigated. Confirmation of fever, rash, urticaria, arthralgia / arthritis and history of antibiotic consumption up to three weeks before were the criteria for the diagnosis.
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.
Background: Congenital agammaglobulinemia is the first primary immunodeficiency disorder characterized by a defect in B lymphocyte development and subsequently decreased immunoglobulin levels. These patients are prone to suffer from recurrent infections mostly involving the respiratory tract. In this study, we aimed to describe in detail respiratory tract complications as the most prominent clinical feature among agammaglobulinemic patients.
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- - Severe combined immunodeficiency (SCID) is a serious immune disorder linked to mutations in over 20 genes, leading to ineffective T cell development; this study focused on Iranian SCID patients, analyzing both clinical and genetic data from 242 individuals.
- - The research found a high mortality rate of 78.9% with T-NK-B- SCID showing the worst outcomes; genetic analysis identified mutations in 80.6% of cases, notably in recombination-activating genes (RAG1 and RAG2) affecting a significant portion of patients.
- - The study suggests that tailoring genetic testing based on the patient's specific immunophenotype could enhance SCID diagnosis, advocating for various sequencing methods for und
Associations of Behavioral Disorders with Asthma in Iranian Children.
Iran J Allergy Asthma Immunol
June 2019
Article Synopsis
- * Conducted in 2017 at a pediatric center in Tehran, the study included 80 children with asthma and 92 controls, assessing asthma control and psychiatric symptoms using validated methods.
- * The findings revealed higher rates of ADHD (42.5% vs. 25%) and ODD (25% vs. 5.4%) in children with asthma compared to controls, while conduct disorder rates showed no significant difference; these results suggest the need for comprehensive evaluation and treatment for affected children.
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.
Background: Filaggrin is a key structural epidermal protein in terminal differentiation and formation of skin barrier. The important role of filaggrin and its effects in various cutaneous and noncutaneous disorders initiated a cascade of considerable research in recent years. Loss-of-function mutations in FLG, the human gene encoding profilaggrin/filaggrin, is the cause of the common skin condition ichthyosis vulgaris (IV) and major genetic predisposing factor for atopic dermatitis (AD).
View Article and Find Full Text PDFA Review on Defects of Dendritic Cells in Common Variable Immunodeficiency.
Endocr Metab Immune Disord Drug Targets
June 2018
Background And Objectives: Common variable immunodeficiency (CVID) is the most important primary disorder that is associated with clinical complications including recurrent infections, malignancy and autoimmune diseases. The genetic cause of CVID is mostly unknown and only a few genetic causes are identified. The various options are proposed for determining the etiology of CVID patients, such as T- and B-cell defects, Toll-like receptors (TLRs) impairments, altered cytokine production as well as blemished dendritic cells (DCs).
View Article and Find Full Text PDFBackground: To evaluate quality of life (QoL) and psychological score in adults with allergic rhinitis (AR) and to assess the possible risk factors.
Materials And Methods: A total of 110 adult patients with a define diagnosis of AR, who were referred to an outpatient clinic of allergy and immunology, were included in this study. The modified Rhinoconjunctivitis Quality of Life Questionnaire (RQLQ) was applied for the evaluation of QoL in these patients.
Impact of IgE-mediated Food Allergy on Parental Quality of Life in Iranian Patients.
Iran J Allergy Asthma Immunol
October 2016
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).
View Article and Find Full Text PDFQuality of life in the Iranian adults with allergic rhinitis.
Iran J Allergy Asthma Immunol
December 2012
Allergic rhinitis (AR) is a common global health problem with approximately one quarter of the world population affected. The quality of life (QOL) of sufferers with AR is significantly affected. The aim of this study was to evaluate the QOL of adults with AR.
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