Kibriya Fidan1, Nilüfer Göknar2, Bora Gülhan3, Engin Melek4, Zeynep Y Yıldırım5, Esra Baskın6, Mutlu Hayran3, Kaan Gülleroglu6, Zeynep B Özçakar7, Fatih Ozaltin3,8, Oguz Soylemezoglu9. 1. Gazi University, School of Medicine, Department of Pediatric Nephrology, Istanbul, Turkey. kibriyafidan@yahoo.co.uk. 2. Bezmialem Vakif University, School of Medicine, Department of Pediatric Nephrology, Istanbul, Turkey. 3. Hacettepe University, School of Medicine, Department of Pediatric Nephrology, Ankara, Turkey. 4. Cukurova University, School of Medicine, Department of Pediatric Nephrology, Ankara, Turkey. 5. Istanbul University, School of Medicine, Department of Pediatric Nephrology, Istanbul, Turkey. 6. Baskent University, School of Medicine, Department of Pediatric Nephrology, Ankara, Turkey. 7. Ankara University, School of Medicine, Department of Pediatric Nephrology, Ankara, Turkey. 8. Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Ankara, Turkey. 9. Gazi University, School of Medicine, Department of Pediatric Nephrology, Istanbul, Turkey.
Abstract
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. RESULTS: The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. CONCLUSIONS: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.
BACKGROUND:Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. RESULTS: The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. CONCLUSIONS: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.
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