PMID- 31173399
OWN - NLM
STAT- MEDLINE
DCOM- 20200220
LR  - 20200220
IS  - 1098-1101 (Electronic)
IS  - 0733-2459 (Linking)
VI  - 34
IP  - 5
DP  - 2019 Oct
TI  - Membrane-filtration based plasma exchanges for atypical hemolytic uremic 
      syndrome: Audit of efficacy and safety.
PG  - 555-562
LID - 10.1002/jca.21711 [doi]
AB  - BACKGROUND: While complement blockade with eculizumab is recommended as 
      first-line therapy of atypical hemolytic uremic syndrome (aHUS), plasma exchanges 
      (PEX) remain the chief option for anti-factor H (FH) antibody associated disease 
      and when access to eculizumab is limited. METHODS: We reviewed adverse events 
      (AEs) and adverse outcomes (eGFR <30 mL/min/1.73 m(2) or death), in all patients 
      with aHUS managed with membrane-filtration based PEX at one tertiary care center 
      over 5.5 years. RESULTS: During January 2013 to June 2018, 109 patients with aHUS 
      (74 with antibodies to FH), aged median (range) 7.6 (0.5-18) year weighing 22.1 
      (6-90) kg, underwent 2024 sessions of PEX. AE, in 12.1% patients, were usually 
      self-limiting and included chills (5.5%), vomiting/abdominal pain (3.3%), 
      hypotension (1.6%), urticaria (1.5%), seizures (0.2%), hypocalcemia (0.2%), and 
      hemorrhage (0.1%); plasma hypersensitivity and severe reactions were rare. Rate 
      of catheter-related infections was 1.45/1000 catheter-days. Filter reuse (OR 
      1.69; 95% CI 1.26-2.26; P < .001) and >20 sessions of PEX/patient (OR 1.99; 95% 
      CI 1.27-3.10; P = .002) were independently associated with adverse events; 
      infusion of IV calcium gluconate during PEX was protective (OR 0.26; 95% CI 
      0.16-0.43; P < .001). Hematological remission was achieved in 96.3% patients 
      after 6 (5-8) PEX sessions; 80.8% and 89.6% patients were dialysis independent by 
      one and 3 months, respectively. CONCLUSIONS: PEX is safe and associated with 
      satisfactory short-term outcomes in children with aHUS. Prolonged PEX and 
      filter-reuse are associated with complications.
CI  - (c) 2019 Wiley Periodicals, Inc.
FAU - Khandelwal, Priyanka
AU  - Khandelwal P
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Thomas, Christy C
AU  - Thomas CC
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Rathi, Bhim Singh
AU  - Rathi BS
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Hari, Pankaj
AU  - Hari P
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Tiwari, Anand N
AU  - Tiwari AN
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Sinha, Aditi
AU  - Sinha A
AUID- ORCID: 0000-0002-9566-3370
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
FAU - Bagga, Arvind
AU  - Bagga A
AD  - Division of Nephrology, Department of Pediatrics, All India Institute of Medical 
      Sciences, New Delhi, India.
LA  - eng
GR  - 5/7/1090/2013-RHN/Funding support for collaborative research studies on HUS: 
      Indian Council of Medical Research, (Advanced Center for Research in Pediatric 
      Kidney Diseases) Department of Science and Technology, Government of India/
GR  - EMR12016/002781/Funding support for collaborative research studies on HUS: Indian 
      Council of Medical Research, (Advanced Center for Research in Pediatric Kidney 
      Diseases) Department of Science and Technology, Government of India/
PT  - Journal Article
DEP - 20190607
PL  - United States
TA  - J Clin Apher
JT  - Journal of clinical apheresis
JID - 8216305
RN  - 0 (Membranes, Artificial)
SB  - IM
MH  - Adolescent
MH  - Atypical Hemolytic Uremic Syndrome/complications/*therapy
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Filtration/*methods
MH  - Humans
MH  - Infant
MH  - Male
MH  - Medical Audit
MH  - Membranes, Artificial
MH  - Plasma Exchange/adverse effects/*methods
MH  - Risk Factors
MH  - Tertiary Care Centers
MH  - Treatment Outcome
OTO - NOTNLM
OT  - central venous catheter
OT  - clinical audit
OT  - complement factor H
OT  - plasmapheresis
EDAT- 2019/06/08 06:00
MHDA- 2020/02/23 06:00
CRDT- 2019/06/08 06:00
PHST- 2019/01/14 00:00 [received]
PHST- 2019/04/10 00:00 [revised]
PHST- 2019/05/14 00:00 [accepted]
PHST- 2019/06/08 06:00 [pubmed]
PHST- 2020/02/23 06:00 [medline]
PHST- 2019/06/08 06:00 [entrez]
AID - 10.1002/jca.21711 [doi]
PST - ppublish
SO  - J Clin Apher. 2019 Oct;34(5):555-562. doi: 10.1002/jca.21711. Epub 2019 Jun 7.