Extra-Renal manifestations of atypical hemolytic uremic syndrome in children

Autor: Nilüfer Göknar, Kibriya Fidan, Oguz Soylemezoglu, Mutlu Hayran, Fatih Ozaltin, Engin Melek, Bora Gülhan, Esra Baskin, Kaan Gulleroglu, Zeynep Yildirim, Zeynep Birsin Özçakar
Přispěvatelé: Çukurova Üniversitesi
Jazyk: angličtina
Rok vydání: 2018
Předmět:
0301 basic medicine
Nephrology
Male
Turkey
Atypical hemolytic uremic syndrome
Gastrointestinal Diseases
Respiratory Tract Diseases
030232 urology & nephrology
urologic and male genital diseases
Extrarenal involvement
Gastroenterology
0302 clinical medicine
Central Nervous System Diseases
hemic and lymphatic diseases
Medicine
Registries
Respiratory system
Child
Children
Plasma Exchange
Mortality rate
Eculizumab
Prognosis
Survival Rate
Treatment Outcome
Cardiovascular Diseases
Child
Preschool
Female
medicine.drug
Glomerular Filtration Rate
medicine.medical_specialty
Thrombotic microangiopathy
Adolescent
Renal function
Antibodies
Monoclonal
Humanized
03 medical and health sciences
Internal medicine
Humans
Retrospective Studies
Neurological involvement
business.industry
Fidan K.
Goknar N.
GÜLHAN B.
MELEK E.
Yildirim Z. Y.
Baskin E.
HAYRAN K. M.
Gulleroglu K.
ÖZÇAKAR Z. B.
ÖZALTIN F.
et al.
-Extra-Renal manifestations of atypical hemolytic uremic syndrome in children-
PEDIATRIC NEPHROLOGY
cilt.33
ss.1395-1403
2018
Infant
medicine.disease
030104 developmental biology
Pediatrics
Perinatology and Child Health
business
Kidney disease
Follow-Up Studies
Popis: PubMedID: 29610995 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. © 2018, IPNA.
Databáze: OpenAIRE
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