Publications by authors named "Pourpak Z"

Background: According to the WHO's recommendation for developing countries, Bacillus Calmette-Guerin (BCG) vaccination has been implemented in some countries as part of national vaccination programs at birth. Although it is generally considered safe, some complications may occur; including BCGitis (local) or BCGosis (systemic), ranging from mild like local abscesses to fatal impediments like osteomyelitis and disseminated BCG infection. This study aimed to determine the spectrum of inborn errors of immunity (IEI) in BCG-vaccinated neonates experiencing local or systemic complications.

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The purpose of this study is to investigate the effect of air pollutants and noise on the prevalence of childhood asthma in Tehran, Iran. The standardized questionnaire was completed by one of the parents of children aged 6-7 years or by adolescents aged 13-14 years. The asthma prevalence in ages 6-7 and 13-14 was found to be 8.

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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.
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  • The study aimed to establish reference ranges for T cell proliferation induced by phytohemagglutinin (PHA-SI) and Bacillus Calmette-Guérin (BCG-SI) to assist in clinical diagnosis.
  • Researchers evaluated data from 359 healthy children and 35 patients with cellular immunodeficiency using a colorimetric method to measure cell proliferation and determined reference ranges at the 2.5th and 97.5th percentiles.
  • The results showed significant differences in proliferation between healthy controls and patients with immunodeficiency, providing crucial thresholds for diagnosing congenital immunodeficiency diseases.
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Many studies have evaluated the possible utility of cycle threshold (Ct) values as a predictor of Coronavirus disease 2019 (COVID-19) severity and patient outcome. Given the inconsistent results, we aimed to evaluate the association between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Ct values and disease severity, inflammatory markers, and outcomes in Iranian patients with COVID-19. A retrospective study of 528 patients with COVID-19 hospitalized from September 2020 to October 2021 was conducted.

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Background: Few studies have evaluated COVID-19 vaccine efficacy in patients with inborn errors of immunity (IEI).

Objective: To evaluate the levels of antibody (Ab) production and function after COVID-19 vaccination in IEI patients with phagocytic, complement, and Ab deficiencies and their comparison with healthy controls.

Methods: Serum samples were collected from 41 patients and 32 healthy controls at least one month after the second dose of vaccination, while clinical evaluations continued until the end of the third dose.

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  • - 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.
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  • 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.
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  • - Griscelli syndrome type 2 (GS2) is a rare genetic disorder caused by mutations in the RAB27A gene, leading to symptoms like hair color changes, recurrent infections, and blood issues in affected individuals.
  • - This study focused on 18 Iranian children with GS2, uncovering novel mutations in the RAB27A gene through genetic testing techniques like PCR and whole-exome sequencing.
  • - The research identified a common mutation (c.514_518delCAAGC) found in 10 of the patients, suggesting it may be prevalent in Iran; early diagnosis is essential to improve treatment outcomes, including potential stem cell transplantation.
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Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder more common in autosomal recessive (AR) than X-linked in Iran. This study aimed to assess whether having a child with AR-CGD would increase the likelihood of the next child being affected by CGD. Ninety-one families with at least one child affected by AR-CGD entered this study.

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  • * Researchers analyze neutrophils from patients with known genetic variations, identifying key differences in their protein composition (proteome).
  • * Experiments using human stem cells and zebrafish models show that SRP deficiency disrupts important cellular processes needed for proper neutrophil development.
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Early diagnosis of primary immunodeficiencies is crucial for timely treatment and preventing unwanted complications. Next-generation sequencing (NGS) and detailed clinical and immunological evaluation can help early detect such disorders. This study aimed to confirm the diagnosis of two cases of autosomal recessive hyper-immunoglobulin E (IgE) syndrome (AR-HIES), presenting with irreversible eye involvement.

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Objective: Human neutrophil antigens (HNAs) can be targeted by HNA-allo antibodies and cause a variety of clinical conditions such as transfusion-related acute lung injury (TRALI) and neonatal alloimmune neutropenia (NAIN). The current study is aimed at identifying the genotype and allele frequencies of HNAs in Iranian blood donors.

Methods: A total of 150 blood samples were obtained from healthy blood donors.

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  • - Recent advancements in molecular diagnosis of allergies have led to the development of the friendly allergen nano-bead array (FABER), a multiplex assay that tests for specific IgE against 244 different allergens in a routine manner.
  • - A study involving 60 allergic patients assessed their IgE sensitization patterns using the FABER test, which identifies responses to both inhalant and food allergens.
  • - Results indicated that common allergenic sensitizations included certain grasses and their components, with findings showing higher rates of sensitization to specific grass proteins compared to previous extract-based tests.
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  • - Chronic granulomatous disease (CGD) is a serious immune disorder treated primarily through hematopoietic stem cell transplantation (HSCT), which was studied in 15 patients using a combination of specific drugs for better outcomes.
  • - The study showed a three-year overall survival rate of 73.3% and an event-free survival rate of 46.7%, with about 73% of patients achieving full donor chimerism.
  • - The results suggest that HSCT with these treatments is effective for CGD, with recommendations to proceed quickly once a matched donor is identified; however, there is a notable risk of transplant-related mortality, especially in adults.
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Background: Preliminary studies revealed the safety and effectiveness of convalescent plasma (CP) therapy for patients with coronavirus. In this study, we aimed to evaluate and summarize the available evidence on CP therapy, identify the research gap regarding the immunological response to CP therapy and pave the road for future studies.

Methods: This study was conducted according to the Hilary Arksey and Lisa O'Malley framework.

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Autoimmune neutropenia is a type of immune-mediated neutropenia, caused by antibody-induced neutrophil destruction. Here we report two cases (3-year-old boy and 9-year-old girl) with suspected autoimmune neutropenia. The presence of neutrophil antibodies in sera of these patients was investigated using standard neutrophil antibody screening tests such as granulocyte immunofluorescence test (GIFT), granulocyte agglutination test (GAT), and lymphocyte immunofluorescence test (LIFT).

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Noise has been reported as one of the most important risk factors for asthma, but there are some disagreements. This study aimed to investigate the effect of road noise on asthma prevalence in adults. In the current study, 3172 adults were interviewed through the ECRHS standardized questionnaire in Tehran, the capital of Iran.

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Meatal stenosis (MS) is known as one of the most frequent complications of circumcision. In the present study, we aimed to find any possible relationship between MS and allergic disorders. A total of 36 children with a mean±SD age of 5.

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No effective antiviral drugs and vaccines are available for the treatment of patients with severe coronavirus 2019 (COVID-19). Therefore, available, safe, and inexpensive drugs and supplements such as melatonin are among the proposed options for controlling inflammation. We did a randomized, single-blind study in Imam Khomeini Hospital between June 30, 2020, and August 5, 2020.

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T-cell receptor excision circles (TREC)/Kappa-deleting recombination excision circles (KREC) assay has been recently recognized for detecting patients with primary (T- and/or B-cell) immunodeficiency (PID). We aimed to investigate the alterations of these biomarkers in some combined immunodeficiency patients compared to the healthy controls in different age groups. TREC and KREC were assessed in a total of 82 PID patients, most of them with exact genetic diagnosis (3 months to 42 years); using quantitative real-time-polymerase chain reaction (PCR).

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Inflammatory biomarkers in exhaled breath condensate (EBC) are measured to estimate the effects of air pollution on humans. The present study was conducted to investigate the relationship between particulate matter and inflammatory biomarkers in blood plasma and exhaled air in young adults. The obtained results were compared in two periods; i.

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This study aims to investigate the role of prenatal diagnosis (PND) in Iranian couples with a previous history of primary immunodeficiency disorders (PIDD) in their family. All referred couples with a family history of PIDD and a tendency for PND were included in this project. Based on gestational age, chorionic villus sampling (CVS) was performed to analyze the molecular defect of the fetus according to the previous gene defect of the affected case in the family.

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The skin prick test (SPT) could be applied as a useful in vivo method for the detection of sensitization in epidemiological and diagnostic studies if the wheal size is ideally evaluated. We focused on SPT wheal size to identify sensitization pattern to common inhalant and food allergens. In this cross-sectional study, SPT results were obtained from a total of 972 allergic patients.

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