ArticleYonsei medical journal2008
Analysis of clinical presentations of Bruton disease: a review of 20 years of accumulated data from pediatric patients at Severance Hospital.
Article in Yonsei medical journal, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 68 citations in OpenAlex.
- Next-generation Sequencing and Other Second Tier Tests in Newborn Screening for (X-linked) Agammaglobulinemia.Journal of clinical immunology · 2025Article
- Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis.Journal of clinical immunology · 2025Article
- Advancing Newborn Screening in Washington State: A Novel Multiplexed LC-MS/MS Proteomic Assay for Wilson Disease and Inborn Errors of Immunity.International journal of neonatal screening · 2025Article
- Future Perspectives of Newborn Screening for Inborn Errors of Immunity.International journal of neonatal screening · 2021Review
- Streptococcal pneumonia meningitis as an initial presentation of X-linked agammaglobulinemia: A case report and discussion.Journal of the American College of Emergency Physicians open · 2021Article
- Infection Phenotypes Among Patients with Primary Antibody Deficiency Mined from a US Patient Registry.Journal of clinical immunology · 2021Article
- Granulomatous-Lymphocytic Interstitial Lung Disease Mimicking Sarcoidosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2021Article
- Clinical characteristics and prenatal diagnosis for 22 families in Henan Province of China with X-linked agammaglobulinemia (XLA) related to Bruton's tyrosine kinase (BTK) gene mutations.BMC medical genetics · 2020Article
- Case Report: A Case of X-Linked Agammaglobulinemia With High Serum IgE Levels and Allergic Rhinitis.Frontiers in immunology · 2020Article
- Primary Immunodeficiency Disorders Among North Indian Children.Indian journal of pediatrics · 2019Article
- X-linked agammaglobulinemia (XLA):Phenotype, diagnosis, and therapeutic challenges around the world.The World Allergy Organization journal · 2019Article
- Role of the Btk-PLCMediators of inflammation · 2019Article
- Uncommon Infections in Children Suggest Underlying Immunodeficiency: A Case of Infective Endocarditis in a 3-Year-Old Male.Case reports in infectious diseases · 2018Article
- The Toll-like receptor 9 signalling pathway regulates MR1-mediated bacterial antigen presentation in B cells.Immunology · 2017Article
- DisseminatedBMJ case reports · 2017Article
- Gastrointestinal disorders next to respiratory infections as leading symptoms of X-linked agammaglobulinemia in children - 34-year experience of a single center.Archives of medical science : AMS · 2017Article
- A novelKorean journal of pediatrics · 2016Article
- Successful hematopoietic cell transplantation in a patient with X-linked agammaglobulinemia and acute myeloid leukemia.Pediatric blood & cancer · 2015Article
- X linked agammaglobulinemia: a single centre experience from India.Indian journal of pediatrics · 2014Article
- Distribution and clinical features of primary immunodeficiency diseases in Chinese children (2004-2009).Journal of clinical immunology · 2011Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeX-linked agammaglobulinemia (XLA) is a humoral immunodeficiency disease caused by a mutation in the Bruton tyrosine kinase (BTK) gene resulting in defective B cell differentiation. Because it is a relatively rare disorder, it is difficult for clinicians to have a comprehensive understanding of XLA due to a lack of exposure to the disease. Clinical presentations of patients with XLA were analyzed and discussed to improve care plans. MATERIALS AND
methodsDuring a 20 year period, from January 1987 to June 2006, a total of 19 patients were diagnosed as XLA in the Department of Pediatrics at Severance Hospital, Seoul, Korea. A retrospective analysis of the clinical presentations of those patients was performed.
resultsThe mean age of the XLA patients included in the study was 4.89 years, with a range of 6 months to 13 years. Twelve patients were diagnosed before age 5, while the other 7 patients were diagnosed after age 5. Recurrent infections observed in the patients included pneumonia, acute otitis media, septic arthritis, skin infection, sepsis, sinusitis, acute gastroenteritis, cervical lymphadenitis, epididymitis, meningitis, osteomyelitis, urinary tract infection and encephalitis. Frequency of admissions was variable from 0 to 12 times, depending on the time at which immunoglobulin therapy was started. Six cases had family histories positive for XLA. BTK gene mutations were found in 8 cases.
conclusionThe overall prognosis of XLA is good as long as patients are diagnosed and treated early with regular intra venous gamma globulin therapy before the sequelae of recurrent infections appear.
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