Abstract
Sık Değişken İmmünyetmezlik (SDİ) çok farklı klinik prezantasyona ve immünolojik özelliğe sahip nadir bir primer immünyetmezlik hastalığıdır. Tüm hastalarda serum immünglobulin düzeyleri azalmış veya tamamen eksiktir. Bu çalışmada 2000-2007 yılları arasında S.B. Ankara Dışkapı Çocuk Hastalıkları Eğitim ve Araştırma Hastanesi çocuk İmmünoloji Bölümünde yerleşmiş kriterlere göre SDİ tanısı alan ve takip edilen 34 hastanın ana klinik ve immünolojik bulguları değerlendirildi. Dokuz hasta agammaglobulinemik, 25 hasta ise hipogammagobulinemikti. On iki hastada kronik sinopul- moner enfeksiyonlar ve bronşektazi (% 35), 10 hastada kronik diare ve malabsorbsiyon (% 29), 8 hastada çeşitli otoimmün hastalıklar (% 23), ve 3 hastada lenfoid maligniteler (% 9) saptandı. Çalışmadan elde edilen veriler bronşektazinin agammaglobulinemik hastalarda daha sık, otoimmün patolojilerin ise hipogammaglobulinemik hastalarda daha sık olduğunu gösterdi. Ayrıca akraba evliliği bulunan ailelerden gelen hastaların, sporadik olgulara göre klinik ve laboratuvar bulgularının daha ciddi seyirli olduğu gözlendi.
References
- and interleukin-6 in patients with common variable immuno- deficiency (CVI) are associated with chronic immune activation and low numbers of CD4+ lymphocytes. Clin Immunol Immunopathol 1994;70:217-224.
- Eisenstein EM, Chua K, Strober W. B cell differentiation defects in common variable immunodeficiency are ameliorated after stimu- lation with anti-CD40 antibody and IL-10. J Immunol 1994;152: 5957-5968.
- Farrant J, Spickett G, Matamoros N, Copas D, Hernandez M, North M, Chapel H, Webster AD. Study of B and T cell phenotypes in blood from patients with common variable immunodeficiency (CVID). Immunodeficiency 1994; 5:159-169.
Abstract
Common Variable Immunodeficiency (CVID) is a rare primary immunodeficiency disease with heterogeneous clinical and immunological features. In all patients levels of serum immunoglobulins are absent or reduced, We presented the main clinical and immunological problems of 34 CVID patients who were followed in S.B. Ankara Dıskapı Children's Hospital, Pediatric Immunology Division, between the years of 2000-2007. Nine patients had agammaglobulinemia while 25 patients had hypogammaglobulinemia. Chronic respiratory infection (bronchiectasis) was present in 12 (35 %), chronic diarrhea with malabsorbtion in 10 (29 %), various autoimmune diseases in 8 (23 %) and lymphoid malignancies in 3 (9 %) patients in our series. Data received from this study showed that bronchiectasis was more prevalent in agammaglobulinemic patients while autoimmunities were more frequently developed in hypogammaglobulinemic patients. It was also seen that the clinical and laboratory features of CVID patients whose parents are consanguineous were more severe than sporadic cases
References
- and interleukin-6 in patients with common variable immuno- deficiency (CVI) are associated with chronic immune activation and low numbers of CD4+ lymphocytes. Clin Immunol Immunopathol 1994;70:217-224.
- Eisenstein EM, Chua K, Strober W. B cell differentiation defects in common variable immunodeficiency are ameliorated after stimu- lation with anti-CD40 antibody and IL-10. J Immunol 1994;152: 5957-5968.
- Farrant J, Spickett G, Matamoros N, Copas D, Hernandez M, North M, Chapel H, Webster AD. Study of B and T cell phenotypes in blood from patients with common variable immunodeficiency (CVID). Immunodeficiency 1994; 5:159-169.
Details
| Other ID | JA32UA99MT |
|---|---|
| Journal Section | Research Article |
| Authors | |
| Publication Date | August 1, 2008 |
| Submission Date | August 1, 2008 |
| Published in Issue | Year 2008 Volume: 2 Issue: 1 |
Cite
The publication language of Turkish Journal of Pediatric Disease is English.
Manuscripts submitted to the Turkish Journal of Pediatric Disease will go through a double-blind peer-review process. Each submission will be reviewed by at least two external, independent peer reviewers who are experts in the field, in order to ensure an unbiased evaluation process. The editorial board will invite an external and independent editor to manage the evaluation processes of manuscripts submitted by editors or by the editorial board members of the journal. The Editor in Chief is the final authority in the decision-making process for all submissions. Articles accepted for publication in the Turkish Journal of Pediatrics are put in the order of publication taking into account the acceptance dates. If the articles sent to the reviewers for evaluation are assessed as a senior for publication by the reviewers, the section editor and the editor considering all aspects (originality, high scientific quality and citation potential), it receives publication priority in addition to the articles assigned for the next issue.
The aim of the Turkish Journal of Pediatrics is to publish high-quality original research articles that will contribute to the international literature in the field of general pediatric health and diseases and its sub-branches. It also publishes editorial opinions, letters to the editor, reviews, case reports, book reviews, comments on previously published articles, meeting and conference proceedings, announcements, and biography. In addition to the field of child health and diseases, the journal also includes articles prepared in fields such as surgery, dentistry, public health, nutrition and dietetics, social services, human genetics, basic sciences, psychology, psychiatry, educational sciences, sociology and nursing, provided that they are related to this field. can be published.