Evidence map›Paper›PMID 30302726›Full record

ArticleJournal of clinical immunology2018

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

Hassan Abolhassani, Fatemeh Kiaee, Marzieh Tavakol, Zahra Chavoshzadeh, Seyed Alireza Mahdaviani, Tooba Momen, Reza Yazdani, Gholamreza Azizi, Sima Habibi, Mohammad Gharagozlou and 72 more

Abstract read
PubMed Publisher
In one paragraph

Article in Journal of clinical immunology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
51citing papers in PubMed, 2 pooled it
5.6field-weighted citation impact, top 3% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

51 citing papers in PubMed, 2 syntheses or guidelines pooled it, 104 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Trial
  4. Article
  5. Article
  6. ALPS-like Disorder Linked with STAT3 Mutation:Endocrine, metabolic & immune disorders drug targets · 2026
    Review
  7. Dual cancers in Ataxia-Telangiectasia: a case report and literature review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025
    Review
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Article
  14. Article
  15. Severity of SARS-CoV-2 infection in children with inborn errors of immunity (primary immunodeficiencies): a systematic review.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023
    Article
  16. Article
  17. Article
  18. B cells and T cells abnormalities in patients with selective IgA deficiency.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023
    Article
  19. Demographic, clinical, immunological, and molecular features of iranian national cohort of patients with defect in DCLRE1C gene.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023
    Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

82 authors at 20 institutions in 2 countries.

Hassan AbolhassaniResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Fatemeh KiaeeResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Marzieh TavakolNon-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
Zahra ChavoshzadehPediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Seyed Alireza MahdavianiPediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Tooba MomenDepartment of Allergy and Clinical Immunology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran.
Reza YazdaniResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Gholamreza AziziDepartment of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran.
Sima HabibiResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Mohammad GharagozlouDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Masoud MovahediDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Amir Ali HamidiehHematology, Oncology and Stem Cell Transplantation Research Centre, Tehran University of Medical Sciences, Tehran, Iran.
Nasrin BehniafardDepartment of Allergy and Clinical Immunology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
Mohammamd NabaviDepartment of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Mohammad Hassan BemanianDepartment of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Saba ArshiDepartment of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Rasol MolatefiDepartment of Pediatrics, Bo-Ali children's Hospital of Ardabil University of Medical Sciences, Ardabil, Iran.
Roya SherkatAcquired Immunodeficiency Research Center, lsfahan University of Medical Sciences, Isfahan, Iran.
Afshin ShirkaniAllergy and Clinical Immunology Department, Bushehr University of Medical Science, School of Medicine, Bushehr, Iran.
Reza AminAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Soheila AleyasinAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Reza FaridhosseiniAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Farahzad Jabbari-AzadAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Iraj MohammadzadehNoncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran.
Javad GhaffariDepartment of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran.
Alireza ShafieiDepartment of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Arash KalantariDepartment of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Mahboubeh MansouriImmunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran.
Mehrnaz MesdaghiImmunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran.
Delara BabaiePediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Hamid AhanchianAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Maryam KhoshkhuiAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Habib SoheiliDepartment of Pediatrics, School of Medicine, Arak University of Medical Sciences, Arak, Iran.
Mohammad Hossein EslamianDepartment of Pediatrics, Hamadan University of Medical Sciences, Hamadan, Iran.
Taher CheraghiDepartment of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran.
Abbas DabbaghzadehNoncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran.
Mahmoud TavassoliDepartment of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Rasoul Nasiri KalmarziCellular & Molecular Research Center, Kurdistan University of Medical Sciences, Sanandaj, Iran.
Seyed Hamidreza MortazaviDepartment of Pediatrics, Kermanshah University of Medical Sciences, Kermanshah, Iran.
Sara KashefAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Hossein EsmaeilzadehAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Javad TafarojiDepartment of Pediatrics, Qom University of Medical Sciences, Qom, Iran.
Abbas KhaliliDepartment of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
Fariborz ZandiehDepartment of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Mahnaz Sadeghi-ShabestariDepartment of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran.
Sepideh DarougarPediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Fatemeh BehmaneshAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Hedayat AkbariAllergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Mohammadreza ZandkarimiAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Farhad AbolnezhadianDepartment of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran.
Abbas FayeziDepartment of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran.
Mojgan MoghtaderiAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Akefeh AhmadiafsharMousavi Hospital, Zanjan University of Meical Sciences, Zanjan, Iran.
Behzad ShakerianDepartment of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Vahid SajediDepartment of Immunology and Allergy, Hormozgan University of Medical Sciences, Bandar Abbas, Iran.
Behrang TaghvaeiDepartment of Immunology and Allergy, Semnan University of Medical Sciences, Semnan, Iran.
Mojgan SafariDepartment of Pediatrics, Hamadan University of Medical Sciences, Hamadan, Iran.
Marzieh HeidarzadehDepartment of Immunology and Allergy, Kashan University of Medical Sciences, Kashan, Iran.
Babak GhalebaghiDepartment of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran.
Seyed Mohammad FathiDepartment of Immunology and Allergy, Qazvin University of Medical Sciences, Qazvin, Iran.
Behzad DarabiDepartment of Immunology and Allergy, Ilam University of Medical Sciences, Ilam, Iran.
Saeed BazregariAllergy and Clinical Immunology Department, Bushehr University of Medical Science, School of Medicine, Bushehr, Iran.
Nasrin BazarganDepartment of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran.
Morteza FallahpourDepartment of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Alireza KhayatzadehResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Naser JavahertrashDepartment of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Bahram BashardoustPediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Mohammadali ZamaniDepartment of Immunology and Allergy, Shahrekord University of Medical Sciences, Shahrekord, Iran.
Azam MohsenzadehDepartment of Pediatrics, Lorestan University of Medical Sciences, Khorramabad, Iran.
Sarehsadat EbrahimiDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Samin SharafianDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Ahmad VosughimotlaghDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Mitra TafakoridelbariDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Maziar RahimiDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Parisa AshourniaDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Anahita RazaghianDivision of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran.
Arezou RezaeiResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Setareh MamishiPediatric Infectious Diseases Research Center, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran.
Nima ParvanehResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Nima RezaeiResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Lennart HammarströmDivision of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
Asghar AghamohammadiResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@tums.ac.ir.
Tehran University of Medical Sciences · IRChildren's Medical Center · IRMashhad University of Medical Sciences · IRShiraz University of Medical Sciences · IRIsfahan University of Medical Sciences · IRUniversity of Tehran · IRIran University of Medical Sciences · IRShahid Beheshti University of Medical Sciences · IRAhvaz Jundishapur University of Medical Sciences · IRBabol University of Medical Sciences · IRBushehr University of Medical Sciences · IRGuilan University of Medical Sciences · IRHamedan University of Medical Sciences · IRMasih Daneshvari Hospital · IRRasool Akram Hospital · IRShahid Beheshti University · IRShahid Sadoughi University of Medical Sciences and Health Services · IRArak University of Medical Sciences · IRArdabil University of Medical Sciences · IRHormozgan University of Medical Sciences · IR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe 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.

methodThe 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.

resultsConsidering 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.

conclusionsDuring 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.

Indexed as

AdolescentAdultAge FactorsChildChild, PreschoolDisease SusceptibilityFemaleFollow-Up StudiesGenetic Predisposition to DiseaseGenetic TestingGeography, MedicalHumansImmunologic Deficiency SyndromesInfantInfant, NewbornIranEpidemiologyIranmolecular diagnosisprimary immunodeficiency

Identifiers

PMID30302726
OpenAlexW2897055301

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.