Five years of experience in transient hypogammaglobulinemia of infancy

Abstract

ÖZAmaç: Süt çocuğunun geçici hipogamaglobulinemisi (SÇGH), immunoglobulin sentezindeki gecikmeden kaynaklanan, yaşla birlikte tamamen düzelen geçici primer immün yetmezliktir. Bu çalışmada, hipogamaglobulinemi ön tanısı ile izlenen ve SÇGH tanısı alan olgular retrospektif olarak değerlendirildi. 'sı ( %3,1) her ikisini almıştı. Olgular düzelme yaş gruplarına göre gruplandırıldığında, enfeksiyon sıklığı, klinik prezentasyon, aşı yanıtı, İV gamaglobülin replasmanı ve antibiyotik profilaksisi açısından gruplar arasında istatistiksel anlamlı fark saptanmadı (p>0.05). Sonuç: SÇGH çoğunlukla üç yaş civarında kendiliğinden düzelir. Düzelme yaşı ile klinik prezantasyon, aşı yanıtları, IV gamaglobülin replasmanı ve antibiyotik profilaksisi arasında ilişkisi yoktur. Doğru tanı için olguların düzenli izlenmesi gerekir.Anahtar kelimeler: Geçici hipogamaglobulinemi, intravenöz immünoglobülin, sütçocuğu ABSTRACT Objective: Transient hypogammaglobulinemia of infancy (THI) is a transient primary immune deficiency stemming from delay in immunoglobulin synthesis which spontaneously, and completely resolves with age. In this study, cases which were followed with the initial diagnosis of hypogammaglobulinemia and received the diagnosis of THI were retrospectively evaluated. Methods: A total of 193 patients, who received the diagnosis of THI in the Department of Immunology of Dr. Behçet Uz Children's Hospital, and Pediatric Surgery between January 2011 and December 2015, were included in the study. Clinical, laboratory and demographic data, prophylactic treatments they received, their follow-up periods, and age of onset of disease resolution were retrospectively analyzed. Results: In the study, 70.5% of 193 cases were male. The age of onset of symptoms was 11.7±7.0 month, the age of hypogammaglobulinemia recovery was 30.6±10.5 month. The cases were most frequently admitted (40.9%) for the recurrent upper respiratory tract infection. Only one case had immunodeficiency in the family history. At the admission, the immunoglobulin levels were as follows; IgG: 420.2±110.6 mg/dL, IgA: 29.8±23.3 mg/dL, IgM: 71±3.1 mg/dL. In vaccine responses; anti tetanus 75.9% (60/79), anti HBs 91.6% (141/154) and anti hemophilus influenza type B was 86.4% (51/59) positive. Twenty five (13%) of the patients received only antibiotic prophylaxis, 19 of them (9.8%) received only IV gamma globulin replacement, and 6 of them ( 3.1%) received both. There was no statistically significant difference between age of recovery and infection frequency, clinical presentation, vaccination response, IV gamma globulin replacement and antibiotic prophylaxis (p>0.05). Conclusion: THI is usually recovers spontaneously around 3 years of age. There is no relationship between age of recovery and clinical presentation, vaccine responses, IV gamma globulin replacement and antibiotic prophylaxis. The cases must be monitored regularly for correct diagnosis.

Investigation of transient hypogammaglobulinemia of infancy in the etiology of chronic cough in children under five years old

“…In the study of Yorulmaz et al, the age at diagnosis of THI varied between 8 months and 48 months, with a mean of 24.6±11.3 months; Karaman et al found the mean age at diagnosis to be 11.7±7.0 months, with a median of 10 months. In their study involving 30 patients, Dogu et al reported the age of diagnosis as 6-46 months (15 below 24 months, 12 24-36 months, 3 36-45 months), and the mean age was 22.5 months [17][18][19] . In our study, the mean age of THI detection in children presenting with chronic cough was 21.73 ± 11.50 months; min -max: 9 -47 months, which was consistent with the literature.…”