Abstract
Background
Hemolytic uremic syndrome (HUS) is a leading cause of acute kidney injury in children. Although international guidelines emphasize comprehensive evaluation and treatment with eculizumab, access to diagnostic and therapeutic facilities is limited in most developing countries. The burden of Shiga toxin-associated HUS in India is unclear; school-going children show high prevalence of anti-factor H (FH) antibodies. The aim of the consensus meeting was to formulate guidelines for the diagnosis and management of HUS in children, specific to the needs of the country.
Methods
Four workgroups performed literature review and graded research studies addressing (i) investigations, biopsy, genetics, and differential diagnosis; (ii) Shiga toxin, pneumococcal, and infection-associated HUS; (iii) atypical HUS; and (iv) complement blockade. Consensus statements developed by the workgroups were discussed during a consensus meeting in March 2017.
Results
An algorithm for classification and evaluation was developed. The management of Shiga toxin-associated HUS is supportive; prompt plasma exchanges (PEX) is the chief therapy in patients with atypical HUS. Experts recommend that patients with anti-FH-associated HUS be managed with a combination of PEX and immunosuppressive medications. Indications for eculizumab include incomplete remission with plasma therapy, life-threatening features, complications of PEX or vascular access, inherited defects in complement regulation, and recurrence of HUS in allografts. Priorities for capacity building in regional and national laboratories are highlighted.
Conclusions
Limited diagnostic capabilities and lack of access to eculizumab prevent the implementation of international guidelines for HUS in most developing countries. We propose practice guidelines for India, which will perhaps be applicable to other developing countries.
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References
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Acknowledgments
Funding support for collaborative research studies and consensus development by Indo-French Centre for the Promotion of Advanced Research (CEFIPRA) [IFC/A/4703-1/2015/1562; IFC/Network 1/2185]; Department of Biotechnology, Government of India [BT/PR14651/MED/30/566/2010]; Indian Council of Medical Research [Advanced Centre for Research in Pediatric Kidney Diseases; 5/7/1090/2013-RHN]; Department of Science and Technology, Government of India [EMR12016/002781]; and All India Institute of Medical Sciences, New Delhi [A-386].
Working group members
Ranjeet Thergaonkar, Vishakapatnam (Group Chair)
Kirtisudha Mishra, New Delhi (Group Chair)
Sushmita Banerjee, Kolkata
Kamran Afzal, Aligarh
Sriram Krishnamurthy, Puducherry
Girish Bhatt, Bhopal
Manish Kumar, New Delhi
Mamta Puraswani, New Delhi
Anil Vasudevan, Bangalore (Group Chair)
Jyoti Sharma, Pune (Group Chair)
Indira Agarwal, Vellore
OP Mishra, Varanasi
Karalanglin Tiewsoh, Chandigarh
Aditi Sinha, New Delhi (Group Chair)
Saroj K Patnaik, New Delhi (Group Chair)
Amarjeet Mehta, Jaipur (President, ISPN)
Susan Uthup, Thiruvanthapuram
Rajiv Sinha, Kolkata
Sudha Ekambaram, Chennai
Pankaj Hari, New Delhi (Group Chair)
Sidharth Sethi, Gurugram (Group Chair and Secretary, ISPN)
Abhijeet Saha, New Delhi
Swati Bhardwaj, New Delhi
Priyanka Khandelwal, New Delhi (Rapporteur)
Arvind Bagga, New Delhi (Coordinator)
Experts
Marie-Agnes Dragon-Durey, Hopital Europeen Georges Pompidou, INSERM UMRS 1138, Paris, France
Amit K Dinda, All India Institute of Medical Sciences, New Delhi
Neelam Taneja, Postgraduate Institute of Medical Education and Research, Chandigarh
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Bagga, A., Khandelwal, P., Mishra, K. et al. Hemolytic uremic syndrome in a developing country: Consensus guidelines. Pediatr Nephrol 34, 1465–1482 (2019). https://doi.org/10.1007/s00467-019-04233-7
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DOI: https://doi.org/10.1007/s00467-019-04233-7