Table 1 Grading of recommendations for the percentage of agreement or disagreement (R1–R7)
From: International consensus on initial screening and follow-up of asymptomatic SDHx mutation carriers
Recommendation | Likert scale (%) | Grade | ||||
|---|---|---|---|---|---|---|
1, strongly agree | 2, agree | 3, neutral | 4, disagree | 5, strongly disagree | ||
R1: Tumour screening should be performed after identification of an SDHA, SDHB, SDHC and SDHD-pi mutation in an asymptomatic carrier | 65.5 | 27.6 | 3.4 | 0 | 3.4 | A |
R2: During childhood, genetic screening should only be performed if tumour screening would be considered if a mutation was discovered | 34.5 | 41.4 | 13.8 | 6.9 | 3.4 | B |
R3: During childhood, tumour screening should only be performed following the discovery of a mutation | 41.4 | 44.8 | 3.4 | 6.9 | 3.4 | A |
R4 for SDHB: First tumour screening should be performed between 6 and 10 years of age for asymptomatic SDHB mutation carriers | 51.7 | 37.9 | 6.9 | 0 | 3.4 | A |
R4 for other genes: First tumour screening should be performed between 10 and 15 years of age in asymptomatic SDHA, SDHC and SDHD-pi mutation carriers | NA | NA | NA | NA | NA | NA |
R4 for SDHA | 13.8 | 69.0 | 10.3 | 6.9 | 0 | A |
R4 for SDHC | 10.3 | 72.4 | 6.9 | 6.9 | 3.4 | A |
R4 for SDHD | 13.8 | 65.5 | 10.3 | 6.9 | 3.4 | A |
R5: Tumour screening in asymptomatic SDHA, SDHB, SDHC and SDHD-pi mutation carriers should include clinical examination: blood pressure measurement and a symptoms and signs questionnaire | NA | NA | NA | NA | NA | NA |
R5 during childhood | 51.7 | 34.5 | 6.9 | 6.9 | 0 | A |
R5 during adulthood | 69.0 | 27.6 | 0 | 3.4 | 0 | A |
R6: Biochemical testing for tumour screening in asymptomatic SDHA, SDHB, SDHC and SDHD-pi mutation carriers should include measurements of either plasma or urinary metanephrine and normetanephrine | NA | NA | NA | NA | NA | NA |
R6a: During childhood, the choice between plasma or urinary tests should be left to the clinician and local laboratory availability and expertise | 41.4 | 44.8 | 13.8 | 0 | 0 | A |
R6b: During adulthood, measurements of plasma-free metanephrine and normetanephrine should be preferred over urinary measurements | 72.4 | 17.2 | 10.3 | 0 | 0 | A |
R7: Biochemical testing should not include either vanillylmandelic acid or catecholamines in addition to metanephrine and normetanephrine | 48.3 | 31.0 | 10.3 | 3.4 | 6.9 | A |
