Hemolitik üremik sendrom tanısıyla izlenen çocuk hastaların geriye dönük değerlendirilmesi

Öz

Amaç: Hemolitik Üremik Sendrom (HÜS), mikroanjiyopatik hemolitik anemi, trombositopeni ve akut böbrek hasarı ile karakterizedir. Bu çalışmada, merkezimizde, Çocuk Nefroloji Polikliniği’nde HÜS tanısıyla takip edilen hastaların etiyolojik faktörleri, takip ve tedavileri açısından değerlendirilmesi amaçlanmıştır. Yöntem: Çocuk Nefrolojisi ve Çocuk Acil Polikliniği’ne Aralık 2010 ve Aralık 2020 tarihleri arasında başvuran ve HÜS tanısı alan çocuk hastalar dahil edildi. Hasta grubunun demografik ve klinik özellikleri, aldıkları tedaviler ve HÜS’e bağlı gelişen böbrek dışı tutulumlar geriye dönük olarak kayıtlardan elde edildi. Bulgular: Hastaların ortalama tanı yaşı 28.8±33.6 aydı. Hastaların 10’una (%52.6) atipik HÜS tanısı konulurken, dokuzuna STEC-HÜS tanısı konuldu (%47,4). Hastaların 16’sında (%84.2) hipertansiyon gelişti. Taze donmuş plazma infüzyonu hastaların 18’ine (18/19, %94.7), ekulizumab ise atipik HÜS tanısıyla izlenen sekiz hastaya (8/10, %80) verilirken üç hastaya plazma değişimi (3/19, %15.8) uygulandı. Renal replasman tedavisi, STEC-HÜS’lü hastaların üçüne (3/9, %33.3), atipik HÜS’lü hastaların ise tamamına uygulandı. Böbrek dışı organ tutulumu, STEC-HÜS tanısı alan hastaların sadece birinde gelişirken (1/9, %11.1), aHÜS’lü hastaların tamamında gelişti. Atipik HÜS tanısıyla takip edilen ve ekulizumab tedavisi alan hastalardan biri gastrointestinal kanama sebebiyle, diğeri ise kalp yetmezliği sebebiyle eksitus oldu. Atipik HÜS tanısıyla izlenen üç hastada (3/19, %15.8) kronik böbrek hastalığı gelişti. Sonuç: Hemolitik Üremik Sendrom, çocuklarda akut böbrek hasarının önemli bir sebebi olup HÜS’e hipertansiyon sıklıkla eşlik etmektedir. Özellikle atipik HÜS’te böbrek dışı tutulum, renal replasman tedavisi ihtiyacı ve mortalite oranı yüksektir.

Anahtar Kelimeler

• Akut böbrek hasarı
• atipik hemolitik üremik sendrom
• trombositopeni

Evaluatıon of the pedıatrıc patıents wıth hemolytıc uremıc syndrome retrospectıvely

Öz

Aim: Hemolytic Uremic Syndrome (HUS) is characterized as microangiopathic hemolytic anemia, trombocytopenia and acute kidney injury. This study aimed to evaluate etiological factors, follow-up and treatment results of the patients with HUS who were followed-up at Pediatric Nephrology Department of our center. Method: Nineteen patients who were admitted to Department of Pediatric Emergency and Pediatric Nephrology Units between December 2010 and December 2020 with HUS diagnosis were included in the study. Demographic and clinical features, treatments and extra-renal involvement of the patients were collected retrospectively from the records. Results: Mean age of the patients was 28.8±33.6 months. Atypic and STEC-HUS was diagnosed in 10 (52.6%) and nine (47.4%) of the patients, respectively. Hypertension developed in 16 (84.2%) of the patients. Fresh frozen plasma infusions were given to 18 (94.7%) patients. Eculizumab were given to three patients with atypic HUS (8/10, 80%). Plasma exchange were applied to three patients (3/19, 15.8%). Renal replecament therapy was applied to three of the patients with STEC-HUS (3/9, 33.3%) and to all of the patients with atypic HUS. Extra-renal involvement was observed in one patient with STEC-HUS (1/19, 11.1%) and in all patients with atypic HUS. Two patients with atypic HUS died due to gastrointestinal bleeding and heart failure, respectively. Chronic kidney disease was developed in three patients with atypic HUS. Conclusion: Hemolytic uremic syndrome is an important etiology for acute kidney injury in children. Hypertension is commonly accompanied with HUS. The rate of extra-renal involvement, requirement of renal replacement therapy and mortality were high in patients with atypic HUS.

Anahtar Kelimeler

• Acute kidney disease
• atypic hemolytic uremic syndrome
• thrombocytopenia

Kaynakça

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