• It is important to assess <strong>the</strong> clinical response after itraconazole withdrawal to assess whe<strong>the</strong>r it isstill beneficial (e.g., prevents relapse and is corticosteroid-sparing) [C].• For patients receiving itraconazole, liver function tests should be obtained before <strong>the</strong>rapy andshould be repeated whenever <strong>the</strong>re is any suspicion <strong>of</strong> liver dysfunction. Routine liver functiontesting after 1 month and <strong>the</strong>n every 3–6 months if <strong>the</strong>rapy continues should be considered [C].• Concomitant medications should be meticulously reviewed to avoid a drug-drug interaction anddoses <strong>of</strong> concomitant medications and itraconazole should be adjusted accordingly. This mayrequire determination <strong>of</strong> serum concentrations <strong>of</strong> concomitant drugs and/or itraconazole [C].• Determination <strong>of</strong> itraconazole concentrations should also be considered when <strong>the</strong>re is a lack <strong>of</strong>clinical response or if <strong>the</strong>re is concern about adequate drug absorption or patient compliance.Blood should be drawn 4 hours after a dose; at steady state, achieved during <strong>the</strong> second week <strong>of</strong><strong>the</strong>rapy, random samples may be useful [C].• For those whom antifungal <strong>the</strong>rapy is indicated and <strong>the</strong>re is evidence <strong>of</strong> poor absorption <strong>of</strong>itraconazole, oral voriconazole could be considered as an alternative. The oral dosage schedule isas follows:• Children
7.9.6 Recommendations for invasive pulmonary aspergillosis, aspergillomas, andaspergillus bronchitis.• The optimum <strong>the</strong>rapy for non-ABPA presentations <strong>of</strong> Aspergillus sp. in persons with CF remainsuncertain. The options for systemic antifungal <strong>the</strong>rapy include amphotericin B (non-lipid orlipid preparations), voriconazole or casp<strong>of</strong>ungin. In some presentations e.g., TBA, surgicaldebidement may also be <strong>of</strong> benefit [C].7.9.7 O<strong>the</strong>r fungiO<strong>the</strong>r fungi are an increasingly recognised complication <strong>of</strong> CF. Scedosporium apiospermum isfrequently isolated from persons with CF and has been associated with a symptom complex similar toABPA. 174 Unlike Aspergillus sp. it has been difficult to isolate from <strong>the</strong> environment. Patients canbecome chronically colonised with <strong>the</strong> same strain 175 [3] which can persist in spite <strong>of</strong> antifungal<strong>the</strong>rapy. It is also capable <strong>of</strong> causing invasive disease with high mortality post lung-transplant. 176 [3]Therapy is compromised by its resistance to many antifungal agents, including itraconazole andamphotericin B. 177 [3] Many isolates appear susceptible in vitro to voriconazole 178;179 [3] but this hasbeen associated with clinical failure in patients 180 and in animal models. 181 [3] In vitro data suggeststhat posaconazole may also be a possible treatment. 182 [3] Ano<strong>the</strong>r fungus increasingly observed isExophiala dermatitidis. However, its significance in CF remains uncertain. 1837.9.8 Recommendations for unusual fungal infection• If considered clinically significant, Scedosporium apiospermum should be treated withvoriconazole or posaconazole [C].7.10 References1. Govan JR, Hughes JE, Vandamme P. Burkholderia cepacia: medical, taxonomic and ecological issues. J Med Micro1996;45:395–407.2. LiPuma JJ. Burkholderia cepacia. Management issues and new insights. Clin Chest Med 1998;19:473–86.3. Jones AM, Dodd ME, Webb AK. Burkholderia cepacia: current clinical issues, environmental controversies andethical dilemmas. Eur Respir J 2001;17:295–301.4. Coenye T, Vandamme P, Govan JR, LiPuma JJ. Taxonomy and identification <strong>of</strong> <strong>the</strong> Burkholderia cepacia complex.J Clin Microbiol 2001;39:3427–36.5. Whiteford ML, Wilkinson JD, McColl JH, Conlon FM, Michie JR, Evans TJ et al. Outcome <strong>of</strong> Burkholderia(Pseudomonas) cepacia colonisation in children with cystic fibrosis following a hospital outbreak. Thorax1995;50:1194–8.6. Muhdi K, Edenborough FP, Gumery L, O’Hickey S, Smith EG, Smith DL et al. Outcome for patients colonisedwith Burkholderia cepacia in a Birmingham adult cystic fibrosis clinic and <strong>the</strong> end <strong>of</strong> an epidemic. Thorax1996;51:374–7.7. Hutchison ML,.Govan JR. Pathogenicity <strong>of</strong> microbes associated with cystic fibrosis. Microbes & Infection1999;1:1005–14.8. McCloskey M, McCaughan J, Redmond AO, Elborn JS. Clinical outcome after acquisition <strong>of</strong> Burkholderia cepaciain patients with cystic fibrosis. Ir J Med Sci 2001;170:28–31.9. Mahenthiralingam E, Vandamme P, Campbell ME, Henry DA, Gravelle AM, Wong LT et al. Infection withBurkholderia cepacia complex genomovars in patients with cystic fibrosis: virulent transmissible strains <strong>of</strong>genomovar III can replace Burkholderia multivorans. Clin Infect Dis 2001;33:1469–75.10. Jones AM, Dodd ME, Govan JR, Barcus V, Doherty CJ, Morris J et al. Burkholderia cenocepacia and Burkholderiamultivorans: influence on survival in cystic fibrosis. Thorax 2004;59:948–51.11. Manno G, Dalmastri C, Tabacchioni S, Vandamme P, Lorini R, Minicucci L et al. Epidemiology and clinicalcourse <strong>of</strong> Burkholderia cepacia complex infections, particularly those caused by different Burkholderia cenocepaciastrains, among patients attending an Italian <strong>Cystic</strong> <strong>Fibrosis</strong> Center. J Clin Microbiol 2004;42:1491–7.<strong>Cystic</strong> <strong>Fibrosis</strong> <strong>Trust</strong> 7.9.6March 2009
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4. ORAL ANTIBIOTICS IN CYSTIC FIBRO
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7. OTHER INFECTIONS7.1 Management o
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GRADING SCHEME FOR LEVELS OF EVIDEN
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SUMMARY•All young children with c
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In 1989 the Copenhagen centre recom
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These bacteria can be harmless comm
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2.7 Clinical relevance of in vitro
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