Publications by authors named "Sima Shokri"

Background: Elevated level of double-negative T (DNT) cells is a historical hallmark of autoimmune lymphoproliferative syndrome (ALPS) diagnosis. However, the peripheral blood level of DNT cells might also be compromised in autoimmune lymphoproliferative immunodeficiencies (ALPID) other than ALPS, inattention to which would increase the delay in diagnosis of the underlying genetic defect and hinder disease-specific treatment.

Materials And Methods: This cross-sectional study recruited patients suffering from ALPID (exclusion of ALPS) with established genetic diagnosis.

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Background: The poly-sensitization to Hymenoptera venom makes it difficult to select genuine allergens for immunotherapy and increases patients' costs. The objective of this study was to determine the culprit allergen in dual or triple-sensitized patients to three Hymenoptera venoms through molecular diagnosis and evaluating the results of incorporating the molecular diagnosis with skin tests.

Methods: Thirty-two patients with anaphylactic reactions and dual or triple-sensitization to Hymenoptera venoms in skin tests entered this study.

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Background: Chronic Rhinosinusitis (CRS) is a paranasal sinus inflammatory disease and is divided into two subgroups defined as CRS with nasal polyps (CRSwNP) and CRS without nasal polyps (CRSsNP). CRSwNP displays a T helper (Th)2 biased phenotype, and based on sensitivity or tolerance to aspirin or non-steroidal anti-inflammatory drugs (NSAID), is further subdivided into Aspirin-exacerbated respiratory disease (AERD) and non-AERD groups. Considering the challenge of diagnosis and treatment in patients with CRSwNP, particularly the AERD subtype, and the significance of endotyping in these patients, we examined the immune profile and endotyping based on gene expression analysis in the AERD and the non-AERD groups of patients with CRSwNP.

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Article Synopsis
  • Severe combined immunodeficiencies (SCIDs) are genetic disorders leading to malfunctioning immune systems due to issues with T and B cells, with many cases linked to RAG mutations.
  • This report details two siblings who both had SCID caused by a new mutation in the RAG2 gene, affecting their immune responses and leading to serious health complications.
  • The findings emphasize the importance of ongoing research to better understand the mutation's effects, as well as improving communication among healthcare professionals to enhance patient care.
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  • NSAID-exacerbated respiratory disease (N-ERD) is a complex condition diagnosed mostly through aspirin challenge tests, which can be risky and time-consuming.
  • This study followed patients undergoing standard medical treatment for three months, measuring symptom severity using the Sinonasal Outcome Test (SNOT22).
  • Results indicated that aspirin sensitivity in patients was not reliably predicted by the severity of their symptoms or by their improvement after treatment, with both aspirin-sensitive and tolerant patients showing similar symptom reductions.
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Background: Clinical trials were conducted on children on side effects after vaccination. We tried to assess the frequency and onset of the main symptoms in children who were vaccinated. We aimed to evaluate early and delayed adverse effects after coronavirus disease 2019 (COVID-19) vaccine among Iranian pediatrics and adolescents in a national survey.

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Background: Chronic rhinosinusitis with nasal polyps (CRSwNP) is a complex disorder and effective treatment remains a major challenge. Some antibiotics with anti-inflammatory properties are reported to have potential to be used as an adjunct therapy in the management of chronic airway inflammation.

Objective: The aim of this study was to evaluate the efficacy of doxycycline in CRSwNP.

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Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 25% and the type of malignancy is highly dependent on the specific mutant gene underlying IEI. We investigated 3056 IEI patients registered in the Iranian national registry between the years 1999 and 2020 in this retrospective cohort study.

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Coronavirus disease 2019 (COVID-19) affects millions of people worldwide. Clinical manifestations range from asymptomatic to severe viral pneumonia. CVID patients with COVID-19 infection are not adequately studied.

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Article Synopsis
  • - Type 2 Griscelli syndrome (Type 2 GS) is a genetic immune disorder characterized by hypopigmentation and varies in severity of neurological and immunodeficiency symptoms across its types, with specific genetic mutations identified for each type.
  • - Hemophagocytic lymphohistiocytosis (HLH) is a serious condition marked by severe inflammation, which can be either primary or secondary, often associated with autoimmune diseases, infections, or cancers.
  • - The text discusses a case of type 2 GS in a newborn who developed HLH due to a prenatal infection, highlighting the link between prenatal infections and severe complications like HLH.
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Background: Most severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-infected individuals are asymptomatic or only exhibit mild disease. In about 10% of cases, the infection leads to hypoxemic pneumonia, although it is much more rare in children.

Objective: We evaluated 31 young patients aged 0.

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Objective: Sesame allergy is the most prevalent allergy to seeds. Oral immunotherapy (OIT) is defined as continuous consumption of an allergen at special doses and time. Omalizumab (Anti-IgE) increases tolerance to allergens used in OIT.

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To determine the safety and efficacy profile of teenager COVID-19 vaccination. In this retrospective cohort study, contact numbers of parents of teenagers under 18 years of age referred to a teenager vaccination centers in Tehran-Iran to receive the corona vaccine were collected, and the following information was obtained via the phones: demographic information, type of vaccine, and the number of doses received, as well as additional information like complications and required treatments. Eleven thousand forty-two subjects aged 10-18 years, mean age 14.

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Children and adolescents form a large proportion of societies and play an important role in the transmission of COVID-19. On the other hand, their education, mental and physical wellness, and safety are compromised which makes vaccination a crucial step to return to normal life. In the current systematic review, the COVID-19 vaccination was evaluated in a total of 50,148 children and adolescents in 22 published studies and 5,279 participants in two ongoing clinical trials.

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Background: Progressive multifocal leukoencephalopathy is a rare demyelinating disease that is often secondary to lytic destruction of oligodendrocytes and, to a lesser extent, to astrocytes' response to human neurotrophic John Cunningham polyomavirus. Any underlying congenital disorder of primary or secondary immunodeficiency may predispose to virus infection and possible invasion of the brain. We present the first reported case of progressive multifocal leukoencephalopathy due to a mutation in the RAC2 gene.

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Article Synopsis
  • Severe viral skin infections can occur in people with genetic immune disorders, known as inborn errors of immunity (IEI).
  • Researchers have developed a method using RNA sequencing (RNA-Seq) on skin biopsies to identify both viral infections and genetic variants associated with IEI simultaneously.
  • The study found pathogenic variants in 6 genes linked to IEI in the patients, and the new computational tool called VirPy helps in detecting viral infections and genetic issues in affected individuals.
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Recalcitrant warts, caused by human papillomaviruses (HPVs), can be a cutaneous manifestation of inborn error of immunity. This study investigated the clinical manifestations, immunodeficiency, single-gene susceptibility, and HPV repertoire in a consanguineous family with severe sinopulmonary infections and recalcitrant warts. Clinical and immunologic evaluations, including FACS and lymphocyte transformation test, provided evidence for immunodeficiency.

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The COVID-19 infection is a novel virus without any specific targeted therapies; thus, focusing on primary epidemiologic concerns, preventive strategies, risk factors, exacerbation factors, and mortality-related factors are of great importance to better control this disorder. There are some controversies about the factors associated with COVID-19 in different theories, and addiction is no exception. We conducted a large cross-sectional study of 513 hospitalized Iranian patients with COVID-19 infection to evaluate the severity of disease courses in patients with or without history of opium addiction.

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Allergic proctocolitis is a cell-dependent food allergy that is present in both breast and formula-fed infants. The presence of blood with different amounts in the stool is the main manifestation of the disease. Different results have been published on the accuracy and specificity of the atopic patch test (APT).

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Background: Intravenous immunoglobulins (IVIg) are the major treatment in inborn errors of immunity (IEI) disorders; However, IVIg infusions show some adverse effects. We aimed to assess the adverse reactions of IVIg infusions.

Methods: Data of IVIg infusions in IEI patients were collected from 2011 to 2021.

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Immunoglobulin G4-Related Disease (IgG4-RD) is a systemic fibro-inflammatory disease that has been proposed as a separate entity since the beginning of this century. The disease is often manifested by increased serum IgG4 levels and certain histopathological manifestations. The patient mentioned in this article is a 29-year-old man from Tajikistan, who has had a chronic cough since the beginning of 2018 without a previous history of the disease.

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Asthmatic patients may have aspirin-exacerbated respiratory disease and experience acute dyspnea and nasal symptoms within 3 hours after the ingestion of aspirin. This study aimed to evaluate the effect and outcome of daily low-dose aspirin in the treatment of moderate to severe asthma in patients with concomitant aspirin hypersensitivity and chronic rhinosinusitis with nasal polyposis (CRSwNP). This clinical trial was conducted from February 2014 to February 2015 on 46 adult patients with moderate to severe asthma accompanied by CRSwNP.

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Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare primary immunodeficiency disorder characterized by recurrent infections and low immunoglobulin levels due to variable combined immunodeficiency, and centromeric region instability, and facial dysmorphism. We describe a 12-year-old boy with recurrent respiratory tract infections, facial anomalies, scoliosis, and psychomotor retardation. He had recurrent pneumonia with low serum IgG and IgM levels during infancy and preschool age.

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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.

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Hereditary angioedema (HAE) is characterized by recurrent attacks of skin and mucosal swelling in any part of the body including the digestive and respiratory tract which generally improve spontaneously within 12-72 hours. The underlying mechanism in HAE is related to bradykinin dysregulation which causes these attacks not to respond to common treatment strategies including epinephrine/corticosteroid or adrenaline. There are several types of HAE with different etiology but with the same clinical picture.

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