Publications by authors named "Alireza Mahdaviani"
The spectrum of inborn errors of immunity: a single tertiary center retrospective study in Alborz, Iran.
Eur Ann Allergy Clin Immunol
January 2023
Inborn errors of immunity (IEIs) are a group of heterogeneous disorders with inherited faults in the immune system that increase susceptibility to infections, malignancies, lymphoproliferation, and autoimmune/autoinflammatory disorders. We retrospectively studied the demographic characteristics, clinical features, and immunological profiles of the 90 IEIs patients, who were diagnosed and classified according to the European Society for Immunodeficiencies (ESID) and International Union of Immunological Societies (IUIS) criteria from July 2010 to June 2021. The study was carried out in the Non-communicable Diseases Research Center, Imam Ali Hospital, Alborz, Iran.
View Article and Find Full Text PDFBackground: It is known that inflammatory responses occur in the airways of patients with non-cystic fibrosis bronchiectasis during respiratory exacerbations but the role of these cytokines is not clear in this condition. Herein we evaluated the levels of interleukin-1β (IL-1β), IL-8 and tumor necrosis factor α (TNF-α) in the serum and bronchoalveolar lavage among children with non-cystic fibrosis bronchiectasis.
Materials And Methods: This cross-sectional study was performed on all children with non-cystic fibrosis bronchiectasis who were admitted with respiratory exacerbation in the pediatric pulmonology ward of Masih Daneshvari Hospital, Tehran-Iran.
Article Synopsis
- A study of 25 Iranian patients with infantile inflammatory bowel disease (IBD) revealed that 56% had identifiable monogenic defects through whole exome sequencing.
- Four patients had specific missense mutations and one had a large deletion, while others had mutations affecting host:microbiome balance or combined immunodeficiencies.
- The majority of patients with monogenic defects resulted from consanguineous relationships, with only 4 of the 14 patients still alive.
Purpose: Inborn errors of IFN-γ immunity underlie Mendelian susceptibility to mycobacterial disease (MSMD). Autosomal recessive complete IL-12Rβ1 deficiency is the most frequent genetic etiology of MSMD. Only two of the 84 known mutations are copy number variations (CNVs), identified in two of the 213 IL-12Rβ1-deficient patients and two of the 164 kindreds reported.
View Article and Find Full Text PDFBackground: Elevations in the number of immunocompromised patients in the past decade has lead to progressive increase in the incidence of Invasive Pulmonary Aspergillosis (IPA) among children; however, early diagnosis remains a challenge. Detection of galactomannan (GM) in the bronchoalveolar lavage (BAL) fluid appears to possess higher sensitivity and specificity than serum in immunocompromised adult patients but, it rarely has been investigated in pediatric patients.
Methods: We performed a prospective case-control study to evaluate the efficacy of BAL GM in immunocompromised pediatric patients.
Background: Asthma is the most common, chronic, childhood disease. Its chronic nature and long-term treatment decrease the quality of life of children and significantly affect the family function. This study was conducted to assess the impact of family empowerment on the quality of life of school-aged children with asthma.
View Article and Find Full Text PDFBackground: Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers.
Methods: This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006.
Background: The prevalence of allergic diseases has risen in the last decades. The objective of this study was to determine the common allergens in children via the skin prick test.
Methods: This cross-sectional study recruited 313 allergic children (4 months to 18 years old) referred to the Asthma and Allergy Clinic of Children's Medical Center in Tehran.
Background: This study aimed at evaluating HRCT pulmonary manifestations in children with Common Variable Immunodeficiency (CVID) hospitalized in the Pediatric Ward of Masih Daneshvari Hospital during a 10-year period.
Materials And Methods: This retrospective study evaluated 25 children hospitalized with the diagnosis of CVID in the Pediatric Ward of Masih Daneshvari Hospital from 2001 to 2011 and their pulmonary HRCT scans were evaluated.
Results: The most common pulmonary HRCT findings were lymphadenopathy (66.
Article Synopsis
- Recent diagnoses of IL-12Rβ1 deficiency in children with severe tuberculosis from Morocco, Spain, and Turkey suggest that genetic factors can lead to tuberculosis in otherwise healthy kids.
- A study of 50 children with severe tuberculosis in Iran, Morocco, and Turkey found two cases with mutations causing complete IL-12Rβ1 deficiency, indicating that this genetic issue is not uncommon in these regions.
- The research highlights the potential for using recombinant IFN-γ as an effective treatment for mycobacterial infections in children with this deficiency, supporting the idea that severe pediatric tuberculosis may stem from genetic immune disorders.