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Table 3 Results of AGREE II appraisal for all the identified guidelines and recommendations

From: Review and evaluation of the methodological quality of the existing guidelines and recommendations for inherited neurometabolic disorders

Authors OR D1 D2 D3 D4 D5 D6 OQA GR
Disorders of amino acid and other organic acid metabolism  
Arnold GL R 67 44 52 61 29 0 50 2
Baumgartner MR SR 94 86 95 94 46 50 92 3
Frazier D SR 78 92 83 97 65 58 92 2.5
Haberle J SR 83 89 90 89 58 96 92 3
Kölker S-2011 SR 56 58 83 92 63 100 75 2.5
Kölker S-2007 R 67 36 91 89 29 100 75 2.5
NIH CDP SR 89 86 86 81 73 4 58 2.5
Vockley J R 89 31 60 78 48 54 75 2
Disorders of carbohydrate metabolism  
Barba-Romero MA R 80 61 53 83 31 50 75 2
Cochat P R 72 28 27 92 31 33 58 2.5
Cupler EJ R 64 64 46 67 41 63 75 2
Kishnani PS-2014 SR 86 61 47 100 58 63 75 2.5
Kishnani PS-2010 R 89 61 50 83 48 33 67 2
Kishnani PS-2006 SR 83 56 44 56 50 75 67 2
Rake JP R 94 17 27 92 25 0 58 2
Visser G R 83 19 23 83 23 0 50 2
Winchester B NR 72 36 14 53 25 67 42 1.5
Disorders of vitamin and non protein cofactor metabolism and transport  
BCMSC R 94 31 51 61 27 0 67 2.5
Devalia V R 83 33 71 94 67 83 83 2
Disorders of porphyrin and hem metabolism  
Stein P SR 89 81 56 89 56 50 75 2.5
Disorders of mineral absorption and transport  
Bacon BR R 56 44 75 83 46 17 67 2
BCMA R 94 8 13 81 27 0 58 2
EASL R 67 28 49 86 35 38 67 2
Qaseem A NR 100 36 27 50 23 42 33 1.5
Roberts EA R 67 17 32 78 33 0 58 2
Disorders of energy metabolism  
Angelini NR 64 8 36 31 8 21 33 1.5
Arnold GL R 86 67 61 72 38 0 67 2
Finsterer J R 75 53 48 72 19 0 58 2.5
Disorders of lysosomal and lysosomal-related organelles  
Andersson R 83 33 29 67 25 0 58 2.5
Bennett RL SR 92 86 69 78 52 46 83 3
Biegstraaten M SR 64 81 65 64 42 71 67 2
Charrow J R 81 56 43 69 33 13 58 2
de Ru MH SR 89 50 63 89 54 58 75 2.5
Desnick RJ NR 64 22 40 53 48 46 42 2
Eng CM R 92 42 45 89 46 0 67 2.5
Fahnehjelm KT R 75 58 32 81 38 50 75 2.5
Giugliani R R 75 56 56 67 29 38 58 2
Grabowski GA R 72 33 27 56 33 0 50 2
Kaplan P R 78 39 29 78 38 100 83 2
Kes VB R 61 33 29 56 21 0 50 2
Laney DA R 94 92 46 78 46 83 67 2.5
Langereis EJ R 86 72 58 53 23 46 58 2
Muenzer J-2012 R 92 53 33 69 40 92 50 2.5
Muenzer J-2009 R 69 36 28 69 42 50 58 2
Ortiz A R 58 31 40 61 23 92 50 2.5
Patterson MC R 78 28 38 78 42 4 67 2
Peters C NR 64 39 25 33 13 0 25 1.5
Scarpa M SR 61 75 61 89 42 58 75 2.5
Solanki GA R 72 39 24 61 54 100 50 1.5
Terryn W SR 81 44 67 78 58 54 67 2.5
Vellodi A NR 83 61 30 72 17 0 42 1.5
Vom Dahl S R 89 36 54 56 31 83 50 2
Wang RY SR 92 64 53 92 67 92 83 2.5
Weinreb NJ R 67 39 30 67 17 4 50 2
Wraith JE R 83 28 46 83 42 0 83 3
  1. OR Overall recommendation, D Domain, OQA Overall quality assessment, GR Grade of recommendation, NIH CDP National institutes of health consensus development panel, BCMSC British Columbia medical services commission, BCMA British Columbia medical association, EASL European association for study of liver, SR Strongly recommended, R Recommended, NR Not recommended domain scores were calculated as described in Methods