The Cystic Fibrosis Center(CF) at the Children's Medical Center(CMC), Pediatric Center of Excellence, as the largest children's hospital in Iran has started its activities in 2012. So far, more than 840 CF patients have registered and visited by a team of experts at the center. As a largest referral children hospital there are 25 to 30 new CF cases are diagnosed monthly. At our reference CF center, we have recently performed an initial screening of ~27,000 potential CF patients and 5-7% of these children (1650) were diagnosed with CF based on the sweat test and further characterized.
Cystic Fibrosis Center
The center serves all the CF patients from all over the country by diagnostic and treatment services. They are visited by eight teams of experts and specialists simultaneously. The members of the team are as follows:
1. Pediatric Lung specialist
2. Pediatric Gastroenterologist
3. Pediatric Endocrinologist
4. Pediatric Psychologist and Psychiatrist
5. Physiotherapist of the Chest
6. Clinical Pharmacy Specialist
7. Nutritionist
8. Social worker        

The Registry of CF patients
All the CF patients that referred to the CMC are registered at the CF center by filling a data base registry questionnaire based on the American CF Foundation. For now, the genetic confirmation test through DNA sequencing is performed for patients. The preliminary data shows the F508dl mutation in 50% of the patients. More surveys are in need for exact statistical data.
CF Pulmonary Function Tests
CF pulmonary function tests are performed in the Infant and Pediatric Pulmonary Function Laboratory (PFT Lab) located at the CMC.:
PFT Laboratory Testing:
  1. Multiple breath inert gas washout (Exhalyzer d - Lung Clearance Index: LCI)
    1. FRC / LCI Infant (SF6-washout)
    2. FRC / LCI Pre-School to Adult
  2. Tidal breathing flow volume analysis for non-cooperative infants(TBFVL)
  3. Spirometry
  4. Spirometry Pre/Post Bronchodilator
  5. Bronchial challenge with methacholine
  6. Lung volume measurements (Plethysmography & Gas dilution techniques)
  7. Lung diffusion capacity (DLCO)
  8. Impulse Oscillometery (IOS)
  9. CPAP/BIPAP Testing
  10. Exhaled Nitric Oxide (FENO )
  11. Nasal nitric oxide
  12. Respiratory muscle Strength (PEmax, PI max)
  13. Cardiopulmonary Exercise Test (Ergo spirometry)
  14. End tidal CO2 measurement
  15. Oximetry
  16. Six-minute walk test
Bronchoscopy Suite
For the high risk infants and/or neonates with CF, if it is necessary the bronchoscopy will be done in the bronchoscopy suite as a diagnostic or a treatment procedure.
Home Ventilation
Home Mechanical Ventilation Center (HMV Center) of the Pediatric Pulmonary and Sleep Medicine Department of the CMC, supports patients who are in needs for the chronic mechanical ventilation. This kind of ventilation is used for advanced forms of CF to improve the quality of life and also before the lung transplantation.
Sleep Center
The center for Sleep disorders in the CMCs lung department helps the CF center to diagnose or treat sleep disorders in CF patients.
 

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