Tülin GÜNGÖR, Deniz KARAKAYA, Evrim KARGIN ÇAKICI, Fatma YAZILITAŞ, Mutlu UYSAL YAZICI, Evra ÇELİKKAYA, Ebru AZAPAĞASI, Mehmet BÜLBÜL

Tipik Hemolitik Üremik Sendromlu Çocuklarda Klinik Özelliklerin ve Renal Replasman Tedavisinin Değerlendirilmesi

Amaç: Tipik hemolitik üremik sendrom (HÜS) hastalarının bazılarında, agresif tıbbi müdahaleye rağmen renal replasman tedavisi (RRT) gerekebilir. Bu çalışmanın amacı, tipik HÜS tanısı alan hastaların klinik ve laboratuvar bulgularının değerlendirilmesi, HÜS tanılı hastalarda periton diyalizi (PD) ile hemodiyalizin (HD) etkinlik ve güvenilirliğinin karşılaştırılmasıdır. Gereç ve Yöntemler: Tipik HÜS tanısı olan hastalar retrospektif olarak belirlendi ve RRT modalitelerinin renal sağ kalım üzerindeki etkisi değerlendirildi. Bulgular: Çalışmaya tipik HÜS tanılı 49 hasta dahil edildi ve ortalama yaşları 2.99 ± 1.88 yıldı. Nörolojik tutulum 15 (%30.6) hastada saptandı. Nötrofil/lenfosit oranı nörolojik tutulum olan grupta anlamlı olarak yüksek bulundu [olasılık oranı (OO) 15.42, %95 güven aralığı (GA) (3.39-70.1), p = 0.005]. Toplamda 43 (%87.7) hastaya RRT uygulandı ve ilk kaydedilen semptomdan RRT’ye kadar geçen ortanca süre 6 gündü (aralık 3-11). RRT boyunca hastaların %10.2’sinde mekanik ventilasyon desteği gerekti. Yirmi altı (%53.1) hastada PD, 17 (%34.6) hastada HD yapılmıştı. Takipte tüm hastaların %10.2’si kronik böbrek hastalığına (KBH) ilerledi, %6.1’inde proteinüri ve %4.08’inde hipertansiyon saptandı. PD yapılan hastalarda 3 aylık dönemde KBH riski HD yapılan hastalara göre anlamlı olarak daha düşüktü [ OO 7.69, %95 GA (0.77-76.07), p = 0.04]. Sonuç: Böbrek iyileşmesi açısından PD’in HD kadar etkili hatta daha güvenli olabileceği sonucuna vardık. Tipik HÜS vakalarında klinik özellikler ve inflamatuar belirteçler sayesinde nörolojik tutulum tahmin edilebilir.

Anahtar Kelimeler:

Tipik Hemolitik üremik sendrom, Çocuklar, Renal replasman tedavisi, Biyomarkerlar

Evaluation of Clinical Features and Renal Replacement Therapy in Children with Typical Hemolytic Uremic Syndrome

Objective: A subset of typical Hemolytic-uremic syndrome (HUS) patients requires initiation of renal replacement therapy (RRT) despite aggressive medical intervention. This study aims to evaluate the clinical and laboratory findings of patients diagnosed with typical HUS and efficacy and safety of peritoneal dialysis (PD) and hemodialysis (HD) were compared in managing these cases. Material and Methods: Patients having a diagnosis of typical HUS were retrospectively determined, and assessment the effects of RRT modalities on renal survival. Results: The present study included 49 children with typical HUS, and the mean age was 2.99 ± 1.88 years. Neurological involvement was detected in 15 (30.6%) patients. The neutrophil/lymphocyte ratio (NLR) was significantly higher in the neurological involvement group [odds ratio (OR) 15.42, 95% CI (3.39-70.1), p = 0.005]. In total, 43 (87.7%) patients received RRT and the median time from the first recorded symptom to RRT was six days (range 3-11). While throughout RRT, 10.2% of patients required mechanical ventilation. In this study, 26 (53.1%) patients were managed with PD, and 17 (34.6%) patients were managed with HD. During follow-up, 10.2% of all patients progressed to chronic kidney disease (CKD), proteinuria was detected in 6.1% and hypertension in 4.08%. The risk of CKD was significantly lower in the 3-month period in PD patients than in HD patients (odds ratio (OR) 7.69, 95% CI (0.77-76.07), p = 0.04). Conclusion: We concluded that PD might be as effective and safer as HD concerning kidney recovery. Clinical features and inflammatory markers can predict neurological involvement in typical HUS cases.

Keywords:

Children, Biomarkers, Typical Hemolytic uremic syndrome, Renal replacement therapy,

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