2019ISPN共识指南：发展中国家溶血性尿毒综合征.pdf

Pediatric Nephrology /10.1007/s00467-019-04233-7 CONSENSUS CONFERENCE Hemolytic uremic syndrome in a developing country: Consensus guidelines Arvind Bagga 1 Priyanka Khandelwal1 Kirtisudha Mishra2 Ranjeet Thergaonkar3 Anil Vasudevan4 Jyoti Sharma 5 Saroj Kumar Patnaik6 Aditi Sinha 1 Sidharth Sethi 7 Pankaj Hari 1 Marie-Agnes Dragon-Durey8 on behalf of the Indian Society of Pediatric Nephrology Received: 18 September 2018 /Revised: 6 January 2019 /Accepted: 7 March 2019 # IPNA 2019 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 comp