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<strong>KCE</strong> reports vol. 6A Prenatale zorg 216 What are the clinical problems of CF and what is the prognosis and treatment of CF? Study Ref Populatio Interventio Outcomes Results Comments Study type EL n n Richards and 92 The frequencies ORGAN TYPE OF PROPORTIO RV 4 Haddow, 2003 of important PROBLEM N WITH clinical problems PROBLEM among Lung Respiratory Nearly all individuals with failure CF Progressive infection and inflammation Most Allergic aspergillosis 10% Pneumothorax 5-8% Massive hemoptysis 5% Pancreas Insufficiency leading to maldigestion and malabsorption of fats and proteins: Newborns 59% By age of 1 year 90% CF related diabetes mellitus at 30 years or older 50% Pancreatitis A few Intestine Meconium ileus, first 48 hours of life 10-20%
<strong>KCE</strong> reports vol. 6A Prenatale zorg 217 Study Ref Populatio n Richards and Haddow, 2003 Richards and Haddow, 2003 Interventio n Outcomes Results Comments Study type EL Distal small bowel obstruction, late childhood or later 10% Rectal prolapse 1% Hepatobiliary tract Cholelithiasis 5% Cholestasis in newborn period 5% Multilobular biliary cirrhosis A few Esophagus Gastroesophag al reflux disease at age 5 years or older 25% 92 Prognosis In the year 2000: Median age at death = 24 years Median predicted survival = 34 years The two major causes of death = Cardiorespiratory failure (77.6%) and transplant complications (11.2%) 92 Treatment ORGAN SYSTEM TREATMENT STRATEGY Respiratory tract Antibiotics Vaccination Anti-inflammatory agents Dnase Physical therapy Lung transplant RV 4 RV 4
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KCE reports 1. Effectiviteit en kos
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