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Hemolytic uremic syndrome in a developing country: Consensus guidelines
Arvind Bagga1, Priyanka Khandelwal2, Kirtisudha Mishra3
1Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India. arvindbagga@hotmail.com.
Insights
Developing countries face challenges in treating hemolytic uremic syndrome (HUS) due to limited resources. This study proposes tailored guidelines for HUS diagnosis and management in India, addressing Shiga toxin-associated and atypical HUS.
Area of Science:
- Pediatric Nephrology
- Hematology
- Immunology
Background:
- Hemolytic uremic syndrome (HUS) is a primary cause of acute kidney injury in children.
- International HUS guidelines are difficult to implement in developing nations due to limited access to diagnostics and eculizumab.
- The prevalence of Shiga toxin-associated HUS and anti-factor H antibodies in Indian children requires specific management strategies.
Purpose of the Study:
- To formulate country-specific guidelines for the diagnosis and management of HUS in children.
- To address the unique challenges faced in developing countries regarding HUS care.
- To adapt international best practices for HUS treatment to the Indian context.
Main Methods:
- Literature review and grading of research studies by four workgroups.
- Consensus statements developed by workgroups were discussed in a meeting.
- Development of a classification and evaluation algorithm for HUS.
Main Results:
- Supportive management is recommended for Shiga toxin-associated HUS.
- Plasma exchange (PEX) is the primary therapy for atypical HUS.
- A combination of PEX and immunosuppressants is advised for anti-factor H antibody-associated HUS; eculizumab indications were defined.
Conclusions:
- Limited resources in developing countries hinder adherence to global HUS guidelines.
- Proposed practice guidelines for India may be applicable to other developing nations.
- Capacity building in laboratories is crucial for effective HUS management.
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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