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National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2017 Oct 25. (NICE Guideline, No. 78.)

Cystic Fibrosis: Diagnosis and management.

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Appendix JGRADE Tables

J.1. Diagnosis of cystic fibrosis

Not applicable to this review.

J.2. Information and support

Not applicable to this review.

J.3. Service delivery

J.3.1. Service configuration

J.3.2. Multidisciplinary teams

Not applicable, as no evidence was found for this review.

J.4. Transition

Not applicable to this review.

J.5. Complications of cystic fibrosis

Not applicable to this review.

J.6. Pulmonary monitoring

J.6.1. Review 1. Monitoring for pulmonary disease onset in people with CF without clinical signs or symptoms of lung disease

Monitoring technique 1. Non-invasive microbiological investigation

No evidence was found.

Monitoring technique 2. Invasive microbiological investigation

No evidence was found.

Table 10. Clinical evidence profile: Monitoring technique 3. Lung physiological function test (FEV₁% predicted at baseline) for prognosis of pulmonary exacerbations and FEV₁ percent predicted at 10 years (PDF, 399K)

Table 11. Clinical evidence profile: Monitoring technique 4. Chest CT scan for prognosis of pulmonary exacerbations and FEV₁% predicted at 10 years (PDF, 399K)

Table 12. Clinical evidence profile: Comparison 1. FEV₁% predicted versus chest CT scan for prognosis of pulmonary exacerbations and FEV₁% predicted at 10 years (PDF, 235K)

J.6.2. Review 2. Monitoring for evolving pulmonary disease in people with CF with established lung disease

Not applicable, as evidence was found for this review.

J.6.3. Review 3. Monitoring for evolving pulmonary disease in people with CF following an acute pulmonary exacerbation

Monitoring strategy 1. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS non-invasive microbiological investigations

Table 13. Clinical evidence profile: Comparison 1. BAL monitoring versus standard monitoring (PDF, 320K)

Monitoring strategy 2. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS lung function test

No evidence was found for this strategy.

Monitoring strategy 3. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS non-invasive microbiological investigations and lung function test

No evidence was found for this strategy.

J.7. Airway clearance techniques

Comparison 1. Manual physiotherapy versus no airway clearance techniques

No evidence was found for this comparison.

Table 14. Clinical evidence profile: Comparison 2. Manual physiotherapy techniques versus oscillating devices (PDF, 266K)

Table 15. Clinical evidence profile: Comparison 3. Manual physiotherapy versus high frequency chest wall oscillation (HFCWO) (PDF, 270K)

Table 16. Clinical evidence profile: Comparison 4. Positive expiratory pressure (PEP) versus no airway clearance technique (PDF, 298K)

Comparison 5. Positive expiratory pressure (PEP) versus active cycle of breathing techniques (ACBT)

No evidence was found for this comparison.

Table 17. Clinical evidence profile: Comparison 6. Positive expiratory pressure (PEP) versus oscillating devices (PDF, 318K)

Table 18. Clinical evidence profile: Comparison 7. Positive expiratory pressure (PEP) compared to High Frequency Chest Wall Oscillation (HFCWO) (PDF, 312K)

Comparison 8. Active cycle of breathing technique (ACBT) versus no airway clearance technique

No evidence was retrieved for this comparison.

Comparison 9. Active cycle breathing technique (ACBT) versus autogenic drainage (AD)

No evidence was retrieved for this comparison.

Comparison 10. Autogenic drainage (AD) versus no airway clearance technique

No evidence was retrieved for this comparison.

Comparison 11. Oscillating device versus no airway clearance technique

No evidence was retrieved for this comparison.

Table 19. Clinical evidence profile: Comparison 12. Oscillating device versus High Frequency Chest Wall Oscillation (HFCWO) (PDF, 230K)

Comparison 13. High Frequency Chest Wall Oscillation (HFCWO) versus no clearance technique

No evidence was retrieved for this comparison.

Table 20. Clinical evidence profile: Comparison 14. Non-invasive ventilation (NIV) versus no airway clearance technique (PDF, 270K)

J.8. Mucoactive agents

J.8.1. Mannitol

Table 21. Clinical evidence profile: Comparison 1.1. Mannitol versus placebo (PDF, 676K)

Table 22. Clinical evidence profile: Comparison 1.2.1. Mannitol versus Dornase alfa (PDF, 236K)

Table 23. Clinical evidence profile: Comparison 1.2.2. Mannitol + Dornase alfa versus Dornase alfa alone (PDF, 261K)

Comparison 1.3: Mannitol versus nebulised sodium chloride

No evidence was found for this comparison.

Comparison 1.4. Mannitol versus acetylcysteine

No evidence was found for this comparison.

J.8.2. Dornase alfa

Table 24. Clinical evidence profile: Comparison 2.1. Dornase alfa versus placebo (PDF, 409K)

Table 25. Clinical evidence profile: Comparison 2.2. Dornase alfa versus nebulized sodium chloride (PDF, 238K)

Comparison 2.3. Dornase alfa versus acetylcysteine

No evidence was found for this comparison.

J.8.3. Nebulised sodium chloride

Table 26. Clinical evidence profile: Comparison 3.1. Nebulised sodium chloride (> 3% concentration) versus placebo (0.9% to 0.12%) or low-concentration (≤ 3%) (PDF, 445K)

Comparison 3.2. Nebulised sodium chloride versus acetylcysteine

No evidence was found for this comparison.

J.8.4. Acetylcysteine

Table 27. Clinical evidence profile: Comparison 4. Acetylcysteine versus placebo (PDF, 267K)

J.9. Pulmonary infection – prophylaxis

Table 28. Clinical evidence profile: Comparison 1. Continuous oral Flucloxacillin versus antibiotics ‘as required’ (PDF, 373K)

Table 29. Clinical evidence profile: Comparison 2. Continuous oral Cephalexin versus antibiotics ‘as required’ (PDF, 499K)

J.10. Pulmonary infection – acute

J.10.1. Pseudomonas aeruginosa

J.10.1.1. Antimicrobial treatment for pulmonary exacerbations due to P aeruginosa

Table 30. Clinical evidence profile: Comparison 1. Single IV agents compared for pulmonary exacerbations with P aeruginosa (PDF, 276K)

Table 31. Clinical evidence profile: Comparison 2. Single IV antibiotic (with placebo) vs combination IV antibiotic for pulmonary exacerbations with P aeruginosa (PDF, 368K)

Table 32. Clinical evidence profile: Comparison 3. Single IV antibiotic versus combination IV antibiotic for pulmonary exacerbations with P aeruginosa (PDF, 440K)

Table 33. Clinical evidence profile: Comparison 4. Combination IV antibiotics versus combination IV antibiotics for pulmonary exacerbations with P aeruginosa (PDF, 342K)

Table 34. Clinical evidence profile: Comparison 5. Combination of 2 IV antibiotics + inhaled antibiotic versus 2 IV antibiotics without inhaled antibiotic for pulmonary exacerbations with P aeruginosa (PDF, 299K)

Table 35. Clinical evidence profile: Comparison 6. Combination of IV ceftazidime + IV tobramycin versus oral ciprofloxacin for pulmonary exacerbations with P aeruginosa (PDF, 282K)

J.10.1.2. Antimicrobial treatment for acute infection with P aeruginosa

Table 36. Clinical evidence profile: Comparison 7. Oral ciprofloxacin + inhaled colistin versus inhaled tobramycin for acute infection with P aeruginosa (PDF, 273K)

Table 37. Clinical evidence profile: Comparison 8. Inhaled colistin + oral ciprofloxacin versus inhaled tobramycin + oral ciprofloxacin for acute infection with P aeruginosa (PDF, 330K)

J.10.2. Staphylococcus aureus

Not applicable, as studies were identified for inclusion.

J.10.3. Burkholderia cepacia complex

Not applicable, as studies were identified for inclusion.

J.10.4. Non-tuberculous mycobacteria

Not applicable, as studies were identified for inclusion.

J.10.5. Non-identified pathogen

Not applicable, as studies were identified for inclusion.

J.11. Pulmonary infection – chronic

J.11.1. P Aeruginosa

Table 38. Clinical evidence profile: Comparison 1. Aztreonam lysine versus placebo (PDF, 479K)

Table 39. Clinical evidence profile: Comparison 2. Ciprofloxacin versus placebo (PDF, 299K)

Table 40. Clinical evidence profile: Comparison 3.1. Colistin versus placebo (PDF, 321K)

Table 41. Clinical evidence profile: Comparison 3.2. Colistin inhalation powder versus colistin inhalation solution (PDF, 305K)

Table 42. Clinical evidence profile: Comparison 3.3. Colistin versus tobramycin (PDF, 547K)

Table 43. Clinical evidence profile: Comparison 4.1. Tobramycin versus placebo (PDF, 550K)

Table 44. Clinical evidence profile: Comparison 4.2. Tobramycin inhalation powder versus Tobramycin inhalation solution (PDF, 364K)

Table 45. Clinical evidence profile: Comparison 4.3 Tobramycin versus Aztreonam lysine (PDF, 373K)

Table 46. Clinical evidence profile: Comparison 5. Combination of fosfomycin + tobramycin versus placebo (PDF, 313K)

Table 47. Clinical evidence profile: Comparison 6. Continuous alternating therapy versus intermittent treatment: aztreonam lysine + tobramycin or placebo + tobramycin (PDF, 324K)

J.11.2. S Aureus

Not applicable, as no relevant studies were identified for this pathogen.

J.11.3. B Cepacia Complex

Not applicable, as no relevant studies were identified for this pathogen.

J.11.4. Aspergillus Fumigatus

Table 48. Clinical evidence profile: Comparison 7. Itraconazole versus placebo (PDF, 452K)

J.12. Immunomodulatory agents

Table 49. Pairwise comparison from NMA. Macrolide antibiotics versus placebo (PDF, 283K)

Table 50. Clinical evidence profile: Comparison 1. Fluticasone versus placebo (PDF, 275K)

Table 51. Clinical evidence profile: Comparison 2. Prednisolone/Prednisone versus placebo (PDF, 422K)

Table 52. Clinical evidence profile: Comparison 3. Azithromycin versus placebo (PDF, 382K)

Table 53. Clinical evidence profile: Comparison 4. Ibuprofen versus placebo (PDF, 360K)

J.13. Nutrition

J.13.1. Oral calorie supplementation

Table 54. Clinical evidence profile: Comparison 1.1. Oral calorie supplementation versus usual care (PDF, 421K)

Table 55. Clinical evidence profile: Comparison 1.2. Oral calorie supplementation versus nutritional advice (PDF, 379K)

J.13.2. Enteral tube feeding

Table 56. Clinical evidence profile: Comparison 2. Enteral tube feeding versus usual care (PDF, 461K)

J.13.3. Appetite stimulants

Table 57. Clinical evidence profile: Comparison 3. Appetite stimulants versus placebo (PDF, 447K)

J.13.4. Nutritional education/dietary advice

Table 58. Clinical evidence profile: Comparison 4. Nutrition education versus usual care (PDF, 454K)

J.13.5. Psychological and behavioural interventions

Table 59. Clinical evidence profile: Comparison 5.1 Behavioural intervention versus usual care (PDF, 325K)

Table 60. Clinical evidence profile: Comparison 5.2 Behavioural intervention versus education and attention control treatment (PDF, 339K)

Table 61. Clinical evidence profile: Comparison 5.3 Behavioural management training + educational intervention versus educational intervention alone (PDF, 394K)

J.14. Exocrine pancreatic insufficiency

J.14.1. Comparison 1. Acid suppressing agents as adjuvant therapy to PERT

Table 62. Clinical evidence profile: Comparison 1.1. PERT + Cimetidine versus. PERT alone in children (PDF, 232K)

Table 63. Clinical evidence profile: Comparison 1.2. PERT + Ranitidine versus. PERT alone in children (PDF, 220K)

Table 64. Clinical evidence profile: Comparison 1.3. PERT + Omeprazole versus. PERT alone in adults (PDF, 264K)

Table 65. Clinical evidence profile: Comparison 1.4. PERT + Ranitidine versus. PERT alone in adults (PDF, 270K)

J.14.2. Comparison 2. High-dose PERT versus low-dose of PERT

Table 66. Clinical evidence profile: Comparison 2.1. High dose PERT versus low dose PERT in children (PDF, 300K)

Table 67. Clinical evidence profile: Comparison 2.2. High dose PERT versus low dose PERT in adults (PDF, 222K)

J.15. Distal intestinal obstruction syndrome

Not applicable, as no studies were included in this review.

J.16. Liver disease

J.16.1. Review question 1. What is the diagnostic accuracy of tests to detect/strategies to detect early and late CF liver disease?

J.16.1.1. Target condition: cystic fibrosis liver disease (CFLD) (including cirrhosis)

Table 68. Test 16. Index test (Transient elastography) versus practice guideline CFLD definition^† to detect CFLD (PDF, 317K)

Table 69. Tests 8 & 13. Index tests (Ultrasound and Transient elastography) versus Clinical CFLD definition† to detect CFLD (PDF, 235K)

Table 70. Tests 9 & 14. Index tests (Ultrasound and Transient elastography) versus Biochemical CFLD† definition to detect CFLD (PDF, 241K)

Table 71. Tests 10 & 15. Index test (Ultrasound) versus Clinical and/or biochemical definition^† to detect CFLD (PDF, 263K)

Table 72. Test 2. Index tests (ALT, AST, GGT) versus Ultrasound definition† to detect CFLD (PDF, 226K)

Table 73. Tests 5–7 & 17. Index tests (ALP, APRI, Forns score and Transient Elastography) versus practice guideline CFLD definitions^† to detect CFLD (PDF, 329K)

Table 74. Tests 1, 3, 4, 11, 19 & 20. Index tests (Clinical examination, biochemical testing and/or ultrasound) versus Biopsy CLFD definitions† to detect CFLD (PDF, 355K)

Table 75. Tests 12 & 18. Index tests (Transient Elastography or MRI) versus liver function tests or ultrasound abnormalities† to detect CFLD (PDF, 236K)

J.16.1.2. Target condition: Cirrhosis

Table 76. Tests 1, 2 and 4. Index tests (APRI, Forn’s score and Transient Elastography) versus clinical and ultrasound cirrhosis definition to detect cirrhosis in a population with CFLD (practice guideline defined) † (PDF, 242K)

Table 77. Test 3. Index test (Ultrasound) versus biopsy definition to detect cirrhosis (PDF, 236K)

J.16.1.3. Target condition: portal hypertension

Table 78. Tests 1 to 3. Index tests (APRI, Forn’s score, transient elatography) versus clinical definition to detect portal hypertension† (PDF, 356K)

Table 79. Test 4. Index test (Transient elastography) versus biochemical and imaging defined portal hypertension † (PDF, 325K)

J.16.1.4. Target condition: Oesophageal varices

Table 80. Tests 1 to 3. Index tests (APRI, Forn’s score, Transient elastography) versus published definition of oesophageal varices † (PDF, 339K)

J.16.2. Review question 2. What is the diagnostic and prognostic value of different strategies to detect CF liver disease and predict progression (including progression to cirrhosis and portal hypertension with (out) oesophageal varices)?

Table 13. Index tests (transient elastography and biopsy) for prognosis of CFLD and portal hypertension (PDF, 300K)

J.17. Ursodeoxycholic acid

Table 81. Clinical evidence profile: Comparison 1. UDCA versus placebo or control (PDF, 337K)

J.18. Cystic fibrosis related diabetes

Not applicable, as no studies were identified for this review.

J.19. Bone mineral density

Not applicable to this review.

J.20. Exercise

J.20.1. Aerobic exercise programmes

Table 82. Clinical evidence profile: Comparison 1. Aerobic exercise training programme versus no exercise programme (PDF, 412K)

J.20.2. Strength resistance training/anaerobic training

Table 83. Clinical evidence profile: Comparison 2.1. Strength resistance/anaerobic training programme versus no exercise programme (PDF, 429K)

Table 84. Clinical evidence profile: Comparison 2.2. Strength/anaerobic training programme versus aerobic training programme (PDF, 432K)

J.20.3. High intensity interval training

Table 85. Clinical evidence profile: Comparison 3. High-intensity interval training versus standard aerobic and anaerobic exercise programme (PDF, 340K)

J.20.4. Inspiratory muscle training

Table 86. Clinical evidence profile: Comparison 4. Inspiratory muscle training (80% of maximal effort) versus usual care (PDF, 254K)

J.20.5. Combined programmes

Table 87. Clinical evidence profile: Comparison 5. Combined aerobic and anaerobic training programme versus no exercise programme (PDF, 639K)

Table 88. Clinical evidence profile: Comparison 6. Combined inspiratory muscle training, resistance and aerobic training (PDF, 317K)

J.20.6. Habitual physical activity

Table 89. Clinical evidence profile: Comparison 7. Physical activity for higher amount or longer duration versus lower amount or shorter duration (PDF, 304K)

J.21. Psychological assessment

Not applicable to this review.

J.22. Cross infection

J.22.1. Outpatient care

Table 90. Clinical evidence profile: Comparison 1. Cohort segregation by clinic times versus no cohort segregation (PDF, 341K)

Table 91. Clinical evidence profile: Comparison 2. Cohort segregation by location versus no cohort segregation (PDF, 308K)

Table 92. Clinical evidence profile: Comparison 3. Combination of protective equipment + individual segregation versus incomplete protective equipment + incomplete individual segregation (PDF, 269K)

J.22.2. Inpatient care

Table 93. Clinical evidence profile: Comparison 4. Cohort segregation by location versus no cohort segregation (PDF, 282K)

Table 94. Clinical evidence profile: Comparison 5. Individual segregation by location versus usual care (PDF, 290K)

J.22.3. Combined inpatient and outpatient care

Table 95. Clinical evidence profile: Comparison 6. Cohort segregation versus no cohort segregation (PDF, 407K)

Table 96. Clinical evidence profile: Comparison 7. Complete cohort segregation versus incomplete cohort segregation (PDF, 283K)

Table 97. Clinical evidence profile: Comparison 8. Individual segregation versus usual care (PDF, 312K)

Table 98. Clinical evidence profile: Comparison 9. Cohort segregation + individual segregation versus cohort segregation (PDF, 318K)

Table 99. Clinical evidence profile: Comparison 10. Cohort segregation + individual segregation + protective equipment versus usual care (PDF, 320K)

Table 100. Clinical evidence profile: Comparison 11. Cohort segregation + individual segregation versus usual care (PDF, 250K)

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National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2017 Oct 25. (NICE Guideline, No. 78.) Appendix J, GRADE Tables.
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GRADE Tables - Cystic Fibrosis
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MAG: hypothetical protein CVT47_00280 [Thermoplasmata archaeon HGW-Thermoplasmata-2]
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Triticum turgidum subsp. dicoccoides strain CRB-INRA-MTP-2396 HgA protein (HgA) ...
Triticum turgidum subsp. dicoccoides strain CRB-INRA-MTP-2396 HgA protein (HgA) gene, exons 1, 2 and partial cds
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Nucleotide
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Sp Transcription Factors
A family of DNA-binding proteins that contain three ZINC FINGERS at their carboxy termini. They are transcription factors that have specificity for GC boxes....<br/>Year introduced: 2006
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Cystic Fibrosis: Diagnosis and management.

Appendix JGRADE Tables

J.1. Diagnosis of cystic fibrosis

J.2. Information and support

J.3. Service delivery

J.3.1. Service configuration

J.3.1.1. Home-based care

J.3.1.2. CF centre care

J.3.1.3. Shared care

J.3.1.4. Telemedicine

J.3.2. Multidisciplinary teams

J.4. Transition

J.5. Complications of cystic fibrosis

J.6. Pulmonary monitoring

J.6.1. Review 1. Monitoring for pulmonary disease onset in people with CF without clinical signs or symptoms of lung disease

Monitoring technique 1. Non-invasive microbiological investigation

Monitoring technique 2. Invasive microbiological investigation

J.6.2. Review 2. Monitoring for evolving pulmonary disease in people with CF with established lung disease

J.6.3. Review 3. Monitoring for evolving pulmonary disease in people with CF following an acute pulmonary exacerbation

Monitoring strategy 1. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS non-invasive microbiological investigations

Monitoring strategy 2. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS lung function test

Monitoring strategy 3. Invasive microbiological investigations and/or imaging techniques in addition to non-invasive microbiological investigations and/or lung function test VERSUS non-invasive microbiological investigations and lung function test

J.7. Airway clearance techniques

Comparison 1. Manual physiotherapy versus no airway clearance techniques

Comparison 5. Positive expiratory pressure (PEP) versus active cycle of breathing techniques (ACBT)

Comparison 8. Active cycle of breathing technique (ACBT) versus no airway clearance technique

Comparison 9. Active cycle breathing technique (ACBT) versus autogenic drainage (AD)

Comparison 10. Autogenic drainage (AD) versus no airway clearance technique

Comparison 11. Oscillating device versus no airway clearance technique

Comparison 13. High Frequency Chest Wall Oscillation (HFCWO) versus no clearance technique

J.8. Mucoactive agents

J.8.1. Mannitol

Comparison 1.3: Mannitol versus nebulised sodium chloride

Comparison 1.4. Mannitol versus acetylcysteine

J.8.2. Dornase alfa

Comparison 2.3. Dornase alfa versus acetylcysteine

J.8.3. Nebulised sodium chloride

Comparison 3.2. Nebulised sodium chloride versus acetylcysteine

J.8.4. Acetylcysteine

J.9. Pulmonary infection – prophylaxis

J.10. Pulmonary infection – acute

J.10.1. Pseudomonas aeruginosa

J.10.1.1. Antimicrobial treatment for pulmonary exacerbations due to P aeruginosa

J.10.1.2. Antimicrobial treatment for acute infection with P aeruginosa

J.10.2. Staphylococcus aureus

J.10.3. Burkholderia cepacia complex

J.10.4. Non-tuberculous mycobacteria

J.10.5. Non-identified pathogen

J.11. Pulmonary infection – chronic

J.11.1. P Aeruginosa

J.11.2. S Aureus

J.11.3. B Cepacia Complex

J.11.4. Aspergillus Fumigatus

J.12. Immunomodulatory agents

J.13. Nutrition

J.13.1. Oral calorie supplementation

J.13.2. Enteral tube feeding

J.13.3. Appetite stimulants

J.13.4. Nutritional education/dietary advice

J.13.5. Psychological and behavioural interventions

J.14. Exocrine pancreatic insufficiency

J.14.1. Comparison 1. Acid suppressing agents as adjuvant therapy to PERT

J.14.2. Comparison 2. High-dose PERT versus low-dose of PERT

J.15. Distal intestinal obstruction syndrome

J.16. Liver disease

J.16.1. Review question 1. What is the diagnostic accuracy of tests to detect/strategies to detect early and late CF liver disease?

J.16.1.1. Target condition: cystic fibrosis liver disease (CFLD) (including cirrhosis)

J.16.1.2. Target condition: Cirrhosis

J.16.1.3. Target condition: portal hypertension

J.16.1.4. Target condition: Oesophageal varices

J.16.2. Review question 2. What is the diagnostic and prognostic value of different strategies to detect CF liver disease and predict progression (including progression to cirrhosis and portal hypertension with (out) oesophageal varices)?

J.17. Ursodeoxycholic acid

J.18. Cystic fibrosis related diabetes

J.19. Bone mineral density

J.20. Exercise

J.20.1. Aerobic exercise programmes

J.20.2. Strength resistance training/anaerobic training

J.20.3. High intensity interval training

J.20.4. Inspiratory muscle training