Clinical and laboratory features in pediatric patients with common variable immunodeficiency Original Investigation

Year 2007, Volume: 42 Issue: 1, 24 - 28, 01.03.2007

Hasibe Artaç Sevgi Keleş Reyhan Kara İsmail Reisli

Abstract

Aim: Common variable immunodeficiency CVID is a primary immunodeficiency disease characterized by defective antibody production and heterogeneous clinical features We aimed to describe clinical and laboratory features of our patients with CVID Materail and Method: We restrospectively evaluated the records of children diagnosed with CVID from February 2002 to June 2006 and followed up for a mean of 29 7±17 4 2 51 months Results: A total of 10 patients 7 females and 3 males mean age: 9 4±5 1 ranging from 3 to 18 were included in this study All of them were admitted to our clinic due to recurrent bronchopneumonia Nine patients had chronic pulmonary disease at the time of CVID diagnosis and lobectomy had been performed in three patients Serum IgG concentrations were less than 200 mg dl in four patients There was a reduction of at least two serum immunoglobulin levels in six patients Four patients had normal nbsp; IgG levels but two of them had decreased IgM levels and the other two had decreased IgA levels The percentages of B cells was found to be low 1 6 8 9; 5 3±2 8 in all of the patients The inversion in the CD4 CD8 ratio was observed in the peripheral blood lymphocytes of eight patients nbsp; Conclusions: We suggested that CVID should be especially investigated in patients with chronic pulmonary disease Turk Arch Ped 2007; 42: 24 8 Key words: Children chronic pulmonary disease common variable immunodeficiency hypogammaglobulinemia

Keywords

-

Değişken immün yetersizlikli çocuk hastalarda klinik ve laboratuvar özellikler Orijinal Araştırma

Abstract

Amaç: Değişken immün yetersizlik DİY bozulmuş antikor yanıtı ile belirgin nedeni bilinmeyen birincil bir immün yetersizliktir Bu çalışmanın amacı DİY’li çocuk hastaların klinik ve laboratuvar özelliklerinin değerlendirilmesidir Gereç ve Yöntem: Şubat 2002 Haziran 2006 tarihleri arasında ortalama 29 7±17 4 2 51 ay DİY tanısıyla takip ve tedavi edilen 10 çocuk hastanın dosya kayıtları gözden geçirildi Bugular: Hastaların yedisi kız üçü erkek olup tanı yaşları ortalama 9 4±5 1 3 18 yıldı Hastaların hepsi yineleyen alt solunum yolu enfeksiyonu nedeniyle başvurmuşlardı Dokuz olguda kronik akciğer hastalığı mevcuttu ve bunların üçü lobektomi geçirmişti Dört hastanın IgG düzeyi 200 mg dl nin altındaydı Altı hastada en az iki immünglobulin düzeyinde azalma vardı IgG düzeyi normal olan dört olgunun ikisinde IgA düşüklüğü ve diğer ikisinde ise IgM düşüklüğü saptandı Tüm hastaların tanı anında B lenfosit oranı düşük bulundu 1 6 8 9; 5 3±2 8 Sekiz olguda CD4 CD8 oranı tersine dönmüştü Çıkarımlar: “Heterojen” bir immün yetersizlik olan bu hastalığın kronik akciğer hastalığı olan olgularda araştırılması gerektiğini düşünüyoruz Türk Ped Arş 2007; 42: 24 8 Anahtar kelimeler: Çocuk değişken immün yetersizlik hipogamaglobulinemi kronik akciğer hastalığı

Keywords

-

References

• Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol 1999: 93: 190-7. 8. Tezcan I, Berkel AI, Ersoy F, Sanal O. Sağlıklı Türk çocukları ve eriskinlerde turbidimetrik yöntemle bakılan serum immünoglobülin düzeyleri. Çocuk Sağlığı ve Hastalıkları Dergisi 1996: 39: 649-56. 9. Cunningham-Rundles C. Clinical and immunologic analy- ses of 103 patients with common variable immunodefici- ency. J Clin Immunol 1989; 9: 22-3.
• Cunningham-Rundles C, Bodian C. Common variable im- munodeficiency: Clinical and immunological features of 248 patients. Clin Immunol 1999; 92: 34-48.
• Blore J, Haeney MR. Primary antibody deficiency and diag- nostic delay. BMJ 1989; 298: 516-7.
• Di Renzo M, Pasqui AL, Auteri A. Common variable immu- nodeficiency: a review. Clin Exp Med 2004: 3: 211-7.
• Slyper AH, Pietryga D. Conversion of selective IgA defici- ency to common variable immunodeficiency in an adoles- cent female with 18q deletion syndrome. Eur J Pediatr 1997; 156: 155-6.
• Castigli E, Geha RS. Molecular basis of common variable im- munodeficiency. J Allergy Clin Immunol 2006; 117: 740-6.
• Aghamohammadi A, Farhoudi A, Moin M, et al. Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol 2005; 12: 825-32.
• Kondratenko I, Amlot PL, Webster AD, Farrant J. Lack of spesific antibody response in common variable immunode- ficiency (CVID) associated with failure in production of anti- gen-spesific memory T cells. MRC Immunodeficiency Group. Clin Exp Immunol 1997; 108: 9-13.
• Kokron CM, Errante PR, Barros MT. Clinical and laboratory aspects of common variable immunodeficiency. An Acad Bras Cienc 2004; 76: 707-26.