Differential diagnosis of thrombotic microangiopathy in nephrology

Show full item record



Permalink

http://hdl.handle.net/10138/228660

Citation

Jokiranta , T S , Viklicky , O , Al Shorafa , S , Coppo , R , Gasteyger , C , Macia , M , Pankratenko , T , Shenoy , M , Soylemezoglu , O , Tsimaratos , M , Wetzels , J & Haller , H 2017 , ' Differential diagnosis of thrombotic microangiopathy in nephrology ' , BMC Nephrology , vol. 18 , 324 . https://doi.org/10.1186/s12882-017-0727-y

Title: Differential diagnosis of thrombotic microangiopathy in nephrology
Author: Jokiranta, T. Sakari; Viklicky, Ondrej; Al Shorafa, Saleh; Coppo, Rosanna; Gasteyger, Christoph; Macia, Manuel; Pankratenko, Tatiana; Shenoy, Mohan; Soylemezoglu, Oguz; Tsimaratos, Michel; Wetzels, Jack; Haller, Hermann
Contributor: University of Helsinki, Research Programs Unit
Date: 2017-10-28
Language: eng
Number of pages: 8
Belongs to series: BMC Nephrology
ISSN: 1471-2369
URI: http://hdl.handle.net/10138/228660
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.
Subject: aHUS
AKI
Complement
Haemolytic anaemia
Kidney diseases
Survey
Thrombocytopenia
TMA
TTP
HEMOLYTIC-UREMIC SYNDROME
THROMBOCYTOPENIC PURPURA
RESPONSE RATES
ECULIZUMAB
MANAGEMENT
ADAMTS13
AHUS
3126 Surgery, anesthesiology, intensive care, radiology
Rights:


Files in this item

Total number of downloads: Loading...

Files Size Format View
s12882_017_0727_y.pdf 557.8Kb PDF View/Open

This item appears in the following Collection(s)

Show full item record