Differential diagnosis of thrombotic microangiopathy in nephrology

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dc.contributor.author Sakari Jokiranta, T.
dc.contributor.author Viklicky, Ondrej
dc.contributor.author Al Shorafa, Saleh
dc.contributor.author Coppo, Rosanna
dc.contributor.author Gasteyger, Christoph
dc.contributor.author Macia, Manuel
dc.contributor.author Pankratenko, Tatiana
dc.contributor.author Shenoy, Mohan
dc.contributor.author Soylemezoglu, Oğuz
dc.contributor.author Tsimaratos, Michel
dc.contributor.author Wetzels, Jack
dc.contributor.author Haller, Hermann
dc.date.accessioned 2017-10-29T13:01:26Z
dc.date.available 2017-10-29T13:01:26Z
dc.date.issued 2017-10-28
dc.identifier.citation BMC Nephrology. 2017 Oct 28;18(1):324
dc.identifier.uri http://hdl.handle.net/10138/228072
dc.description.abstract Abstract Background The differential diagnosis of thrombotic microangiopathy (TMA) is complex however the rapid diagnosis of the underlying condition is vital to inform urgent treatment decisions. A survey was devised with the objective of understanding current practices across Europe and the Middle East, and of challenges when diagnosing the cause of TMA. Methods Over 450 clinicians, from 16 countries were invited to complete an online survey. Results Of 254 respondents, the majority were nephrologists, had >10 years’ experience in their specialty, and had diagnosed a patient with TMA. The triad of thrombocytopenia, haemolytic anaemia and acute kidney injury are the main diagnostic criteria used. Responses indicate that a differential diagnosis of TMA is usually made within 1–2 (53%) or 3–4 days (26%) of presentation. Similarly, therapy is usually initiated within the first 4 days (74%), however 13% report treatment initiation >1-week post-presentation. Extrarenal symptoms and a panoply of other conditions are considered when assessing the differential diagnosis of TMA. While 70 and 78% of respondents stated they always request complement protein levels and ADAMTS13 activity, respectively. Diagnostic considerations of paediatric and adult nephrologists varied. A greater proportion of paediatric than adult nephrologists consider extrarenal manifestations clinically related to a diagnosis of TMA; pulmonary (45% vs. 18%), gastrointestinal (67% vs. 50%), CNS (96% vs. 84%) and cardiovascular (54% vs. 42%), respectively. Variability in the availability of guidelines and extent of family history taken was also evident. Conclusions This survey reveals the variability of current practices and the need for increased urgency among physicians in the differential diagnosis of TMA, despite their experience. Above all, the survey highlights the need for international clinical guidelines to provide systematically developed recommendations for understanding the relevance of complement protein levels, complement abnormalities and ADAMTS13 testing, in making a differential diagnosis of TMA. Such clinical guidelines would enable physicians to make a more rapid and informed diagnosis of TMA, therefore initiate effective treatment earlier, with a consequent improvement in patient outcomes.
dc.publisher BioMed Central
dc.subject aHUS
dc.subject AKI
dc.subject Complement
dc.subject Haemolytic anaemia
dc.subject Kidney diseases
dc.subject Survey
dc.subject Thrombocytopenia
dc.subject TMA
dc.subject TTP
dc.title Differential diagnosis of thrombotic microangiopathy in nephrology
dc.date.updated 2017-10-29T13:01:27Z
dc.language.rfc3066 en
dc.rights.holder The Author(s).
dc.type.uri http://purl.org/eprint/entityType/ScholarlyWork
dc.type.uri http://purl.org/eprint/entityType/Expression
dc.type.uri http://purl.org/eprint/type/JournalArticle

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