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Hemolytic uremic syndrome in a developing country: Consensus guidelines

  • Consensus Conference
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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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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

Author information

Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India

    Arvind Bagga, Priyanka Khandelwal, Aditi Sinha & Pankaj Hari

  2. Department of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India

    Kirtisudha Mishra

  3. Department of Pediatrics, Indian Naval Hospital Ship, Kalyani, Visakhapatnam, India

    Ranjeet Thergaonkar

  4. Department of Pediatric Nephrology, St. Johns Medical College and Hospital, Bengaluru, India

    Anil Vasudevan

  5. Department of Pediatrics, KEM Hospital, Pune, India

    Jyoti Sharma

  6. Department of Pediatrics, Army Hospital Research & Referral, New Delhi, India

    Saroj Kumar Patnaik

  7. Department of Nephrology, Medanta Hospital, New Delhi, India

    Sidharth Sethi

  8. Laboratory of Immunology, Hopital Europeen Georges Pompidou, INSERM UMRS 1138, Paris Descartes University, Paris, France

    Marie-Agnes Dragon-Durey

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  1. Arvind Bagga
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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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