Title |
Extra-Renal manifestations of atypical hemolytic uremic syndrome in children
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Published in |
Pediatric Nephrology, April 2018
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DOI | 10.1007/s00467-018-3933-3 |
Pubmed ID | |
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 |
Abstract |
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. 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. 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. The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
India | 1 | 10% |
Saudi Arabia | 1 | 10% |
Netherlands | 1 | 10% |
Canada | 1 | 10% |
United States | 1 | 10% |
Brazil | 1 | 10% |
Unknown | 4 | 40% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 9 | 90% |
Science communicators (journalists, bloggers, editors) | 1 | 10% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 55 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Researcher | 10 | 18% |
Student > Master | 7 | 13% |
Student > Ph. D. Student | 5 | 9% |
Other | 5 | 9% |
Student > Postgraduate | 4 | 7% |
Other | 9 | 16% |
Unknown | 15 | 27% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 24 | 44% |
Biochemistry, Genetics and Molecular Biology | 4 | 7% |
Nursing and Health Professions | 3 | 5% |
Pharmacology, Toxicology and Pharmaceutical Science | 2 | 4% |
Psychology | 2 | 4% |
Other | 6 | 11% |
Unknown | 14 | 25% |