Clinical and laboratory evaluation of Turkish children with IgG subclass deficiency

MakaleWOS:000922080700001PubMed ID: 36089538Background: IgG subclass deficiency is a laboratory diagnosis and becomes important with recurrent infections. This study aimed to examine the demographic, clinical, and laboratory results of pediatric cases with IgG subclass deficiency and to improve the understanding of the clinical significance of IgG subclass deficiency. Methods: In this study, the clinical and laboratory features of 111 pediatric patients, with at least one whose serum IgG subclasses was measured as lower than 2 standard deviation of healthy aged-matched control values, were evaluated. The clinical and laboratory features of the cases with isolated IgG subclass deficiency (Group 1) and those with low serum levels of any of IgG, IgA, and IgM in addition to the IgG subclass deficiency (Group 2) were compared. Results: A total of 55 (49.54%) and 56 (50.45 %) patients were included in Groups 1 and 2, respectively. Among our studied cases, 20 (18. 1%) had a history of hospitalization in the neonatal period, 61 (54.95 %) had at least one hospitalization due to infection, and 55 (49.54%) had a history of recurrent infection. The frequencies of these three conditions were statistically significantly higher in Group 2 (p < 0.05). The frequencies of infections in the last year in Groups 1 and 2 were 4.4 +/- 1.2 and 5.4 +/- 1.9, respectively (p < 0.05). As a result of recurrent infections, 43.24% (n = 48) of our patients received antibiotic prophylaxis, and 21.62% (n = 24) had immunoglobulin replacement therapy. Furthermore, the numbers of pa-tients who needed these treatments were higher in Group 2 (p < 0.05).Conclusion: In cases with IgG subclass deficiencies, concomitant main-group immunoglobulin deficiencies may increase the number and severity of infections, leading to hospitalizations, antibiotic prophylaxis, and immunoglobulin therapy. More attention should be paid to cases of immunoglobulin main-group deficiencies in the follow-up of these cases.Copyright (c) 2022, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/ by-nc-nd/4.0/)

Evaluation of pediatric patients diagnosed with acute poststreptococcal glomerulonephritis

Çalışmamızın amacı APSGN tanılı olgularda, cinsiyet dağılımını, başvuru yaşını, başvuru şikayetlerini, tanı anındaki klinik, laboratuvar ve görüntüleme bulgularını, uygulanan tedavileri, hastaların klinik seyirlerini ve hastanede yatış sürelerini etkileyen faktörleri belirlemektir. YÖNTEM: Çalışmamızda Necmettin Erbakan Üniversitesi Meram Tıp Fakültesi Çocuk Nefroloji kliniğimize 2010-2020 yılları arasında başvuran, APSGN tanısı konan 44 olgunun verileri geriye dönük olarak değerlendirilmiştir. Veriler, tanı ve takip sırasında kaydedilen bilgiler ve laboratuvar sonuçları dikkate alınarak dosyaların incelenmesinden elde edilmiştir. BULGULAR: Çalışmamızda APSGN tanısı alan 24'ü (%54,5) erkek, 20'si (%45,5) kız 44 hasta incelendi. Tüm hastaların başvuru yaşları ortalaması 8,65±3,31 yıldı. Hastaların en sık başvuru şikayeti ve bulguları 44 hastada (%100) hematüri [34'ünde (%77,3) makroskobik, 10'unda (%22,7) mikroskobik hematüri], 28'inde (%63,6) ödem, 22'sinde (%50) hipertansiyondu. Laboratuvar bulgularında hastalarımızın 30'unda (%68,2) CRP, 26'sında (%59,1) sedimantasyon yüksekliği olduğu; 19'unda (%43,1) albumin, hepsinde (%100) C3, 15'inde (%34) C4 düzeyi düşüklüğü olduğu görüldü. GFR<90 mL/dk/1.73m2 olan hasta sayısı 17'ydi (%38,6). GFR'leri düşük olan hastalarda nötrofil değeri, nötrofil/lenfosit oranı, CRP değeri daha yüksek; hemoglobin ve sodyum değeri daha düşük bulunmuştur (p<0,05). Hastalarımızın hepsine başvuru anında ultrason ile üriner sistem değerlendirmesi yapılmış olup 27 hastamızın (%61,3) böbrek parankiminde ekojenite artışı saptandı. Böbrek parankiminde ekojenite artışı olanlar ve olmayanlar arasında hastanede yatış süreleri, mikroskobik hematüri düzelme süresi, CRP değeri ve beyaz küre sayısı arasında anlamlı farklılık olduğu görüldü (p<0,05). Hastanede daha uzun yatanların başvuru sırasındaki GFR'si, albumin ve C3 değeri daha düşük, idrarda protein/kreatinin oranı ve idrar dansitesi daha yüksek bulunmuştur (p<0,05). SONUÇ: APSGN'li hastalarda yüksek nötrofil, nötrofil/lenfosit oranı ve CRP değeri hastalığın şiddeti ile ilişkili bulunmuştur. Böbrek parankiminde ekojenite artışı mikroskobik hematüri düzelme süresinin göstergesi olabilir. Ayrıca böbrek parankiminde ekojenite artışı, düşük GFR, albumin ve C3 değeri, artmış idrar dansitesi ve idrarda protein atılımı gibi etmenlerin de hastanede uzun yatış süresi için risk faktörleri olduğu saptanmıştır.The aim of our study is to determine the gender distribution, age of admission, presenting complaints, as well as clinical, laboratory and imaging findings at the time of diagnosis, treatments applied, clinical course of patients and the factors affecting duration of hospitalization in patients diagnosed with APSGN. METHOD: In our study, the data of 44 cases diagnosed with APSGN and admitted to our Meram Faculty of Medicine Division of Pediatric Nephrology between 2010 and 2020 were evaluated retrospectively. The data were obtained through examination of the medical records, taking into account the information recorded during diagnosis and follow-up, and the laboratory results. RESULTS: In our study, a total of 44 patients, 24 (54.5%) male and 20 (45.5%) female, who were diagnosed with APSGN were examined. Patients' mean age at admission was 8.65±3.31 years. The most common presenting complaints and findings of the patients were hematuria in 44 patients (100%) [macroscopic hematuria in 34 (77.3%), microscopic hematuria in 10 (22.7%)], with 28 (63.6%) patients having edema and 22 (50%) patients having hypertension. In laboratory findings, 30 (68.2%) of our patients had high CRP and 26 (59.1%) high erythrocyte sedimentation rate; It was observed that 19 (43.1%) had low albumin, all (100%) had low C3 levels and 15 (34%) had low C4 levels. The number of patients with GFR<90 mL/min/1.73m2 was 17 (38.6%). Neutrophil count, neutrophil/lymphocyte ratio and CRP level were higher in patients with low GFR, whereas hemoglobin and sodium levels were lower (p<0.05). All of our patients had urinary system evaluated by ultrasound on admission, and an increased echogenicity of renal parenchyma was detected in 27 patients (61.3%). It was determined that there was a significant difference between the patients with and without echogenicity in the kidney parenchyma in terms of duration of hospitalization, recovery period of microscopic hematuria, CRP value and white blood cell count (p<0.05). Those with a longer duration of hospitalization had lower GFR, albumin and C3 levels on admission, and higher urine density (p<0.05). CONCLUSION: In patients with APSGN, elevated neutrophil count, neutrophil/lymphocyte ratio and CRP levels were found to be associated with disease severity. Increased echogenicity of renal parenchyma may be an indicator for recovery time of microscopic hematuria. Furthermore, factors including increased echogenicity of renal parenchyma; decreased GFR, albumin and C3 levels; increased urine density and urinary protein excretion were determined to be risk factors for longer duration of hospitalization