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Management of cystic fibrosis

  1. Chest:
    1. Physiotherapy regularly (postural drainage, active cycle techniques or forced expiratory techniques).
    2. Antibiotics are given for acute infective exacerbations (for Staph. aureus, IV for P. aeruginosa) and prophylactically (flucloxacillin) or nebulized (colomycin or tobramycin).
    3. Mucolytic may be useful
    4. Bronchodilator 
  2. Gastrointestinal:
    1. Pancreatic enzyme replacement
    2. Fat soluble vitamin supplements (A, D, E, K)
    3. Ursodeoxycholic acid for impaired liver function
    4. Cirrhosis may require liver transplantation. 
  3. Other:
    Treatment of
    1. CF-related diabetes                      
    2. Screening for
    3. Treatment of osteoporosis            
    4. Treatment of arthritis
    5. Sinusitis, and vasculitis        
    6. Fertility and genetic counseling.


A child presents raised sweat Chloride levels & suspicion of Cystic Fibrosis, Which other test would you do to exclude the diagnosis of cystic fibrosis: (AIPG 09)
A. Repeat sweat chloride measurements
B. Nasal electrode potential difference
C. Fat in stool for next 72 hours
D. DNA analysis for delta F-508 mutation


Ans: (B) Nasal electrode potential difference


  1. Pulmonary Alveolar Proteinosis 
    1. Intra-alveolar accumulation of pulmonary surfactant lipoproteins.
    2. Histo: distal air spaces are filled with a granular,PAS+ Diastase resistant eosinophilic material.
    3. Two forms of PAP have been described in children: a fulminant, often fatal form presenting shortly after birth (termed congenital PAP); and a gradually progressive type presenting in older infants and children that is similar to PAP observed in adults.
    4. Single gene disorders that result in the neonatal form of PAP include mutations in the surfactant protein B (SP-B), surfactant protein C (SP-C), the β chain of the granulocyte-macrophage colony–stimulating factor (GM-CSF) receptor, and the adenosine triphosphate (ATP)–binding cassette transporter (ABCA3) genes (see Chapter 404 ).
    5. Primary (idiopathic) PAP in adults is an autoimmune disease mediated by autoantibodies against GM-CSF.
    6. Congenital PAP is clinically and radiographically indistinguishable from more common disorders of the newborn that lead to respiratory failure, including pneumonia, generalized bacterial infection, respiratory distress syndrome, and total anomalous pulmonary venous return with obstruction


  • Alveolar proteinosis in newborns : no successful medical therapy
  • Subcutaneous or inhaled administration of recombinant GM-CSF may improve adults

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