Abstract | BACKGROUND: 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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Authors | Kibriya Fidan, Nilüfer Göknar, Bora Gülhan, Engin Melek, Zeynep Y Yıldırım, Esra Baskın, Mutlu Hayran, Kaan Gülleroglu, Zeynep B Özçakar, Fatih Ozaltin, Oguz Soylemezoglu |
Journal | Pediatric nephrology (Berlin, Germany)
(Pediatr Nephrol)
Vol. 33
Issue 8
Pg. 1395-1403
(08 2018)
ISSN: 1432-198X [Electronic] Germany |
PMID | 29610995
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Antibodies, Monoclonal, Humanized
- eculizumab
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Topics |
- Adolescent
- Antibodies, Monoclonal, Humanized
(therapeutic use)
- Atypical Hemolytic Uremic Syndrome
(complications, genetics, therapy)
- Cardiovascular Diseases
(diagnosis, epidemiology, etiology, prevention & control)
- Central Nervous System Diseases
(diagnosis, epidemiology, etiology, prevention & control)
- Child
- Child, Preschool
- Female
- Follow-Up Studies
- Gastrointestinal Diseases
(diagnosis, epidemiology, etiology, prevention & control)
- Glomerular Filtration Rate
- Humans
- Infant
- Male
- Plasma Exchange
- Prognosis
- Registries
(statistics & numerical data)
- Respiratory Tract Diseases
(diagnosis, epidemiology, etiology, prevention & control)
- Retrospective Studies
- Survival Rate
- Treatment Outcome
- Turkey
(epidemiology)
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