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Table 2 Comparison of paediatric and adult nephrologist’s diagnostic practices for TMA

From: Differential diagnosis of thrombotic microangiopathy in nephrology

  Response n (%)a
Paediatric nephrologist
n = 75
Adult nephrologist
n = 132
Apart from renal symptoms, which other organ manifestations would you consider clinically related to a diagnosis of TMA?
 Central nervous system 64 (96) 103 (84)
 Gastrointestinal 45 (67) 62 (50)
 Cardiovascular 36 (54) 52 (42)
 Pulmonary 30 (45) 22 (18)
What other conditions do you usually consider when assessing the differential diagnosis of TMA?
 Antiphospholipid syndrome 41 (60) 96 (79)
 Autoimmune haemolytic anaemia 42 (62) 96 (78)
 Clotting disorder 45 (66) 25 (21)
 Cobalamin metabolism disorder 18 (26) 43 (35)
 Drug induced 44 (65) 87 (71)
 HIV 15 (22) 38 (31)
 Infections 55 (81) 58 (48)
 Malignancy 22 (32) 78 (64)
 Pregnancy (HELLP syndrome) 15 (22) 86 (71)
 Scleroderma 9 (13) 48 (39)
 Sepsis 36 (53) 71 (58)
 Systemic lupus erythematosus 49 (72) 103 (84)
 Otherb 4 (7) 11 (9)
How extensive is your investigation of the patients’ family history?
 Ask the patient 21 (31) 75 (61)
 Investigate immediate family 19 (28) 28 (23)
 Complete a full family tree 28 (41) 19 (16)
Do you routinely perform genetic testing?
 Yes 46 (69) 49 (41)
 No 21 (31) 70 (59)
Are there any guidelines in place for the diagnosis of TMA at your hospital?
 Yes 49 (72) 51 (42)
 No 19 (28) 70 (58)
When the differential diagnosis of TMA was aHUS, what was the most challenging aspect of the diagnosis?c
 Absence of guidelines 2.1 2.5
 Delay in getting some laboratory results 3.7 3.4
 Absence of a single and reliable diagnostic test 3.6 3.5
 Heterogeneity of disease presentation 2.9 3.5
  1. aPercentage obtained from the number of responses received for individual questions
  2. bFifteen respondents also selected “other” considerations as free text; these included malignant hypertension (n = 6), viral infections (n = 3; H1N1, hantavirus and parvovirus), disseminated intravascular coagulation (n = 2), transplantation (n = 2), vasculitis (n = 2), aHUS (n = 1), HUS (n = 1), pre-eclampsia (n = 1), TTP (n = 1), and AKI of unknown aetiology (n = 1). Clinicians could include >1 answer in response to ‘Other’
  3. cClinicians were asked to grade their response from 1 (least challenging) to 5 (most challenging), data are presented as mean score. HELLP, haemolysis, elevated liver enzyme levels, and low platelet levels; HIV, human immune deficiency virus; TMA, thrombotic microangiopathy
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