ArticleFrontiers in immunology2020
Clinical and Genetic Profile of X-Linked Agammaglobulinemia: A Multicenter Experience From India.
Article in Frontiers in immunology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 23 citations in OpenAlex.
- Clinical and Laboratory Features of Pediatric Agammaglobulinemia: A Comparative Analysis of Classical, Common Variable Immunodeficiency, and Syndromic Forms.Indian pediatrics · 2026Article
- Development of Clinical Pathways for Early Diagnosis and Management of SCID, SMA, and XLA Through Newborn Screening in Malaysia.International journal of neonatal screening · 2026Review
- Pre- and peri-hematopoietic cell transplant management of disseminated non-Helicobacter pylori Helicobacter infection in X-linked agammaglobulinemia: Case series and literature review.Clinical immunology (Orlando, Fla.) · 2026Review
- Primary Humoral Immunodeficiencies and Bronchiectasis in Adults.Journal of clinical medicine · 2025Review
- Pulmonary Manifestations of Inborn Errors of Immunity: Diagnostic and Therapeutic Insights.Life (Basel, Switzerland) · 2025Review
- Case Report: Hematopoietic stem cell transplantation in an adult patient with X-linked agammaglobulinemia and severe refractory enteropathy.Frontiers in immunology · 2025Article
- Hematopoietic stem cell gene editing rescues B-cell development in X-linked agammaglobulinemia.The Journal of allergy and clinical immunology · 2024Article
- An International Survey of Allogeneic Hematopoietic Cell Transplantation for X-Linked Agammaglobulinemia.Journal of clinical immunology · 2023Review
- A Registry Study of 240 Patients with X-Linked Agammaglobulinemia Living in the USA.Journal of clinical immunology · 2023Article
- Clinical features and mutational analysis of X-linked agammaglobulinemia patients in Malaysia.Frontiers in immunology · 2023Article
- Novel BTK Mutation in Patient with Late Diagnosis of X-Linked Agammaglobulinemia.Case reports in immunology · 2023Article
- Primary antibody deficiency-associated arthritis shares features with spondyloarthritis and enteropathic arthritis.RMD open · 2022Article
- Utility of targeted next generation sequencing for inborn errors of immunity at a tertiary care centre in North India.Scientific reports · 2022Article
- Targeted Gene Sanger Sequencing Should Remain the First-Tier Genetic Test for Children Suspected to Have the Five Common X-Linked Inborn Errors of Immunity.Frontiers in immunology · 2022Article
- Achromobacter xylosoxidans Pneumonia in a Young Child with Chronic Granulomatous Disease-a Case-Based Review.Journal of clinical immunology · 2021Review
- Hematopoietic Stem Cell Transplantation Cures Chronic Aichi Virus Infection in a Patient with X-linked Agammaglobulinemia.Journal of clinical immunology · 2021Article
- Bruton's Tyrosine Kinase Inhibitors Impair FcγRIIA-Driven Platelet Responses to Bacteria in Chronic Lymphocytic Leukemia.Frontiers in immunology · 2021Article
Corrections and comments
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Authors and funding
42 authors at 16 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: There is paucity of literature on XLA from developing countries. Herein we report the clinical and molecular profile and outcome in a multicenter cohort of patients with XLA from India. Methods: Data on XLA from all regional centers supported by the Foundation for Primary Immunodeficiency Diseases (FPID), USA and other institutions providing care to patients with PIDs were collated. Diagnosis of XLA was based on European Society for Immunodeficiencies (ESID) criteria. Results: We received clinical details of 195 patients with a provisional diagnosis of XLA from 12 centers. At final analysis, 145 patients were included (137 'definite XLA' and eight 'probable/possible XLA'). Median age at onset of symptoms was 12.0 (6.0, 36.0) months and median age at diagnosis was 60.0 (31.5, 108) months. Pneumonia was the commonest clinical manifestation (82.6%) followed by otitis media (50%) and diarrhea (42%). Arthritis was seen in 26% patients while 23% patients developed meningitis. Bronchiectasis was seen in 10% and encephalitis (likely viral) in 4.8% patients. Conclusion: There was a significant delay in the diagnosis and facilities for molecular diagnosis were not available at many centers. Optimal immunoglobulin replacement is still a challenge.
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