Extra-Renal manifestations of atypical hemolytic uremic syndrome in children

  • Kibriya Fidan
  • Nilüfer Göknar
  • Bora Gülhan
  • Engin Melek
  • Zeynep Y. Yıldırım
  • Esra Baskın
  • Mutlu Hayran
  • Kaan Gülleroglu
  • Zeynep B. Özçakar
  • Fatih Ozaltin
  • Oguz Soylemezoglu
Original Article

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.

Keywords

Atypical hemolytic uremic syndrome Children Neurological involvement Extrarenal involvement Eculizumab 

Abbreviations

aHUS

Atypical hemolytic uremic syndrome

TMA

Thrombotic microangiopathy

DGKE

Diacylglycerol kinase-ε

THBD

Thrombomodulin

CNS

Central nervous system

PRES

Posterior reversible encephalopathy syndrome

Notes

Acknowledgements

This study was supported by the Hacettepe University Scientific Research and Development Office (grant no. 010A101009).

Author contribution

Kibriya Fidan, Nilufer Goknar, and Oguz Soylemezoglu participated in conception, design, analysis and interpretation of data, drafting the article, and revising. Kaan Gülleroğlu, Esra Baskın, Engin Melek, Zeynep Y. Yıldırım, Bora Gülhan, and Zeynep Birsin Özçakar participated in data collection. Fatih Özaltın participated in study design and evaluation of genetic results. All authors contributed to manuscript development and approved the final manuscript.

Compliance with ethical standards

The study protocol was approved by the Hacettepe University Ethics Committee (FON10/03-22) and written informed consent was obtained from the parents of each patient.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© IPNA 2018

Authors and Affiliations

  • Kibriya Fidan
    • 1
  • Nilüfer Göknar
    • 2
  • Bora Gülhan
    • 3
  • Engin Melek
    • 4
  • Zeynep Y. Yıldırım
    • 5
  • Esra Baskın
    • 6
  • Mutlu Hayran
    • 3
  • Kaan Gülleroglu
    • 6
  • Zeynep B. Özçakar
    • 7
  • Fatih Ozaltin
    • 3
    • 8
  • Oguz Soylemezoglu
    • 1
  1. 1.Gazi University, School of Medicine, Department of Pediatric NephrologyIstanbulTurkey
  2. 2.Bezmialem Vakif University, School of Medicine, Department of Pediatric NephrologyIstanbulTurkey
  3. 3.Hacettepe University, School of Medicine, Department of Pediatric NephrologyAnkaraTurkey
  4. 4.Cukurova University, School of Medicine, Department of Pediatric NephrologyAnkaraTurkey
  5. 5.Istanbul University, School of Medicine, Department of Pediatric NephrologyIstanbulTurkey
  6. 6.Baskent University, School of Medicine, Department of Pediatric NephrologyAnkaraTurkey
  7. 7.Ankara University, School of Medicine, Department of Pediatric NephrologyAnkaraTurkey
  8. 8.Nephrogenetics Laboratory, Hacettepe University Faculty of MedicineAnkaraTurkey

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