1. Step 6
1.1. Review session 1
1.1.1. Summary of step 5
1.2. Report new knowledge
1.2.1. cystic fibrosis
1.2.1.1. epidemiology
1.2.1.2. mechanism
1.2.1.3. risk factors
1.2.1.4. presentation
1.2.1.5. diagnosis
1.2.2. bronchiactesis
1.2.2.1. causes
1.2.2.2. mechanism
1.2.3. why infections are common in winters
1.3. 40 minutes
2. Step 8
2.1. Diagnostic decision
2.1.1. cystic fibrosis
2.1.2. bronchiactesis
2.1.3. pseudomonas aeurgenosa infection
2.2. Mechanism
2.2.1. mutations lead to gene abnormality lead to cystic fibrosis then recurrent infections and bronchiactesis after that
2.3. Presentation
2.3.1. chronic persistent cough with sputum during winters and green sputum since last year, along with history of asthma (wheezing and dysnea) and weight loss
2.4. Supporting data
2.4.1. cyanosis, clubbing, + sweat cl test, CT and x-ray, vitA deficiency, elevated liver enzymes and the presence of pseudomonas auregenosa
2.5. 10 minutes
3. Step 7
3.1. Inquiry plan and info gathering
3.1.1. History of presenting complain
3.1.1.1. chronic persistent cough and half a cup green sputum with smell previously and no hemoptysis
3.1.1.2. occasional fever during the attaks
3.1.1.3. wheezing and dysnea during exacerbations
3.1.1.4. weight loss
3.1.2. Previous medical / surgical history
3.1.2.1. clean surgical history
3.1.2.2. no diabetes or hypertension
3.1.2.3. no nasal polyps or sinusitis
3.1.2.4. no vit deficiency
3.1.2.5. history of ashma when 2 y o
3.1.2.6. negative chloride sweat test when 6 months old
3.1.3. Drug history / allergy
3.1.3.1. oral prednisolone, ventolin and antibiotics during exacerbations
3.1.3.2. no allergies
3.1.4. Family history
3.1.4.1. clean family history of respiratory diseases
3.1.5. Social / occupational history
3.1.5.1. not a smoker, step father is a smoker
3.1.5.2. living with his family
3.1.5.3. no pits
3.1.5.4. no alcohol consumption
3.1.5.5. high school degree
3.1.6. Systemic review
3.1.6.1. normal thrive
3.1.6.2. clean other systems history
3.1.7. Physical examination
3.1.7.1. 37 temp
3.1.7.2. 120 pulse rate
3.1.7.3. 120\60 bp
3.1.7.4. 18 rr
3.1.7.5. 18.6 bmi
3.1.7.6. 86% O sat
3.1.7.7. marked clubbing
3.1.7.8. no tremors and no muscle wasting
3.1.7.9. not raised JVP
3.1.7.10. cebtral cyanosis
3.1.7.11. mucopurulent discharge in the nasopharyns and crusted secretions in the nostrils
3.1.7.12. no burrel chest and no enlarged lymph nodes
3.1.7.13. normal percussion
3.1.7.14. occasional crepitations (crackles) in both lungs at the bases
3.1.7.15. polyphoning expiratory wheezing in forced
3.1.7.16. normal apex beat and not elevated pulmonary valve
3.1.7.17. normal heart sound (1st and 2nd)
3.1.7.18. normal liver and spleen spans
3.1.7.19. normal bowel movement and no rectal prolapse
3.1.7.20. normal genitourinary, neorological systems
3.1.8. tests results
3.1.8.1. patchy peripheral ling inflitrates
3.1.8.2. ring-like arrowheads (dilated bronchi)
3.1.8.3. elevated liver enzymes
3.1.8.4. elevated ESR
3.1.8.5. pseudomonas auregenosa detection with mucopurulent sputum
3.1.8.6. vitA difeciency
3.1.8.7. spirometry no results
3.1.8.8. + sweat chloride test )61)
3.1.8.9. D508 mutation (1 copy)
3.1.8.10. nasal potential difference no results
3.1.8.11. elevated protein and alk phosph