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 |