AI Summary & Key Takeaways
AI InsightFunctional impairment in T lymphocyte in cellular immun system and impairments such as IgA, IgG2 and IgG4 deficiency in humoral immünity were determined in our patients with WS.
📌 3 Key Takeaways
Abstract
West Syndrome (WS) is the most common intractable convulsions of childhood, and etyopathogenesis of disease is unknown. Recent studies suggest that the pathology of immun system may play a role on the etiology of WS. In this study, we aimed to determine the role and importance of immune system on the WS. The study included 31 patients, 18 boys (54%) and 13 girls (46%) diagnosed as WS. Their ages ranged from 6 months to 13 months and the mean age was 10.5±1.7 months. Patients and healty control groups were compared in cellular immunity (absolute lymphocyte count, CD3, CD4, CD8, CD16, CD19, CD4/CD8 ratio) and humoral immunity (serum Immünoglobulins and IgG subgroups). PPD test was applied on the patients. Patients' absolute Iymphocyte counts, total T-Lymphocyte, B-Lymphocyte, CD4, CD8, NK counts and CD4/CD8 ratios were not different from healty children's values (p>0.05). PPD test was found to be negative of 32.2% of the patients. Patients serum IgA levels were found significantly lower than those of the cont rol group (p<0.05), there was no difference between the serum IgG and IgM levels in the two groups (p>0.05). In our study, low levels of IgA were detected in four cases (12.9%) with WS. Serum IgG4 levels of the patients were also found significantly lower compared with control group's (p<0.05), there was no difference between two groups in serum IgG1, IgG2 and IgG3 levels (p>0.05). Low levels of IgG4, IgG2, IgG3 were detected as 19.3%, 9.7%, 6.4% in cases with WS, respectively. Our findings show that there may be different inmunolojical impairments. Functional impairment in T lymphocyte in cellular immun system and impairments such as IgA, IgG2 and IgG4 deficiency in humoral immünity were determined in our patients with WS.
Özet
West Sendromu (WS), çocukluk çağının dirençli konvülsiv hastalıklarından olup, etiyopatogenezi tam olarak bilinmemektedir. Son yıllardaki çalışmalarda etiyolojide immün sistemdeki patolojilerin de rol oynayabileceği iddia edilmektedir. Çalışma, WS'ında hücresel ve hümoral immuniteyi araştırmak amacıyla planlandı. Çalışmaya, WS'lu 31 olgu alındı. Olguların yaş dağılımı 6-13 ay arasında olup, yaş ortalaması 10.5±1.7 ay, 18'i (%54) erkek, 13'ü (%46) kız idi. Olgular, sağlıklı kontrol grubu ile hücresel immün sistem (Absolut lenfosit sayısı, CD3, CD4, CD8, CD16, CD19 ve CD4/CD8) ve humoral immün sistem (Serum Ig'leri ve IgG alt grupları) yönünden karşılaştırıldı. Olguların hepsine Tüberkülin cilt testi (PPD) yapıldı. Absolut lenfosit sayısı, total T lenfosit, B lenfosit, CD4, CD8, Natural Killer (NK) sayıları ve CD4/CD8 oranlarının sağlıklı çocuklardan farklı olmadığı saptandı (p>0.05). Olguların %32.2'sinde Tüberkülin cilt testi negatif bulundu. Serum IgA düzeyleri kontrol grubuna göre anlaml ı düşük bulundu (p 0.05). Dört olguda (%12.9) IgA eksikliği saptandı. Serum IgG4 düzeyleri kontrol grubuna göre anlamlı düşük bulundu (p 0.05). IgG4, IgG2, IgG3 eksikliği sırasıyla %19.3, %9.7, %6.4 oranında bulundu. Bulgularımız, WS'nda farklı immünolojik bozuklukların olabileceğini gösterdi. Hücresel immün sistemde T-lenfositlerde fonksiyonel bozukluk, hümoral immün sistemde IgA, IgG4 eksikliği saptandı.