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Unlabelled: Maldigestion in cystic fibrosis (CF) affects approximately 90% of patients. As soon as pancreatic insufficiency is identified, enzyme supplementation is prescribed even with breast fed infants. A pancreatic enzyme preparation developed particularly for infants, Creon for children (CfC), contains smaller granules to be administered with a dosing spoon (5000 lipase units per scoop)., Patients and Methods: In a prospective, randomised, multi-centre study, 40 infants and toddlers received both CfC and Creon 10000 (C10) for two weeks each in a cross-over design. Dosing of pancreatic enzymes was continued as applied before the study. The primary endpoint was the parents' treatment preference. Secondary endpoints included coefficient of fat absorption (CFA), clinical symptoms and safety parameters., Results: 20 parents (51%) from the N=39 intent to treat sample preferred CfC, 9 (23%) preferred C10, and 10 (26%) had no preference The applied doses led to a mean CFA with similar results for both treatments (77.8% vs. 78.7%). Gastrointestinal symptoms were reported on a number of study days, and some children had abnormal results for laboratory parameters of malabsorption. Safety and tolerability of the preparations were good and all these parameters were comparable for both treatments., Conclusion: Those parents who had a preference favoured CfC over C10. Both enzyme preparations improved malabsorption to a similar degree, although the applied dosages could have been too low in some children reflected in a suboptimal CFA. These data support the use of CfC for young patients with cystic fibrosis improving the daily care of this cohort detected mainly now through neonatal screening programmes.

Introduction: Aspergillosis colonisation in cystic fibrosis (CF), usually due to Aspergillus fumigatus (AF), classically presents as allergic bronchopulmonary aspergillosis. However, aspergillus infection can produce a range of manifestations: from minor colonization to an invasive infection., Case Report: A 14-year-old CF patient, chronically colonized with Staphylococcus aureus and Pseudomonas aeruginosa, presented with acute right-sided chest pain, a moderate fever and no modification of the sputum. The chest X-ray showed a 5 cm round opacity. Laboratory parameters were WBC 24,500 G/l, CRP 27 mg/l, Total IgE 1527 UI/l, Specific Aspergillus fumigatus IgE 31 UI/l. Bronchoscopy revealed external compression of the middle lobe bronchus with mucopurulent secretions coming from apical segment of the lower lobe. Bronchoalveolar lavage revealed the presence of pseudomonas aeruginosa 103 CFA/ml, staph aureus 107 CFA/ml and one colony of AF. Chest CT scan showed a large necrotic mass with an air-fluid level located in the apical segment of the right lower lobe. Initial therapy with itraconazole and corticosteroid was replaced by voriconazole, caspofungin, metronidazole and linezolide. Treatment was well tolerated and after 8 weeks the chest X-ray appearances had returned to normal., Conclusions: The possible diagnoses and therapeutic options are discussed. Conventional antifungal treatment with amphotericin B is limited by nephrotoxicity. These newer antifungal agents appear to be effective and well-tolerated.

Poor clearance of airway secretions contributes to the pulmonary disease in cystic fibrosis (CF). Bronchodilator therapy might benefit in CF, but the efficacy is controversial. Effects of mucolytic agents have not been demonstrated conclusively. Only, efficacy of recombinant human deoxyribonuclease (rhDNase) is established with a rapid onset of benefit.