Background. Systemic steroids and adjunctive antifungal therapy are the cornerstone in treating allergic bronchopulmonary aspergillosis (ABPA) in the context of CF. Aim. Evaluate the use of inhaled amphotericin B (iAMB) as antifungal agent in this context. Methods. Report of 7 CF patients with recurrent or difficult to treat ABPA and failure to taper systemic corticosteroids treated with AMB deoxycholate (AMB-d) (Fungizone 25 mg 3× a week) or AMB lipid complex (ABLC) (Abelcet 50 mg twice weekly). Successful therapy was defined as steroid withdrawal without ABPA relapse within 12 months. Results. Therapy was successful in 6 of 7 patients treated with iAMB. In 5/6, lung function improved. The patient with treatment failure has concomitant MAC lung infection. Conclusion. Inhaled AMB may be an alternative to commonly used adjunctive antifungal therapy in the treatment of ABPA. More data are needed on safety and efficacy.

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The overall respiratory morbidity in our sample of children with PIMD was lower than anticipated. While a subgroup of children are prone to recurrent severe airway problems, the majority of children did not experience severe airway infections.

Airway clearance techniques are an important part of the respiratory management in children with cystic fibrosis, bronchiectasis and neuromuscular disease. They are also, however, frequently prescribed in previously healthy children with an acute respiratory problem with the aim to speed up recovery. The current review explores the evidence behind this use of airway clearance techniques in children without underlying disease. Few studies have been performed; many different techniques are available and the therapies used are often poorly specified. It is necessary to name the specific airway clearance technique used in treatment rather than to just state "chest physiotherapy," a term that is often confused with chest clapping or vibration plus postural drainage. There is little evidence that airway clearance techniques play a role in the management of children with an acute respiratory problem. Physicians routinely prescribing airway clearance techniques in previously healthy children should question their practice.

The pulmonary innate immune functions are over-active in HIV-associated bronchiectasis, with readily detected sTREM-1 values, which were higher than those in CF. sTREM-1 does not correlate with markers of HIV-disease activity but does correlate with markers of neutrophilic inflammation. In CF sTREM-1 has a negative correlation with pulmonary function parameters.

The Belgian CF Association sent this questionnaire to all Belgian CF patients. Of the 903 questionnaires distributed, 379 (42%) were returned. Fifty-two, 40 and 8% of patients used a vibrating-mesh nebulizer, jet nebulizer, or ultrasonic device, respectively. Ninety-eight percent of patients cleaned their material and soap was used by 35% of patients. Disinfection was performed by 91% of the patients and was daily performed by 57% of patients. Thermal disinfection (boiling water or sterilizer), a hypochlorite-based solution, or acetic acid solution was used by 44, 29, and 25% of patients, respectively, to disinfect their nebulizers. Thermal disinfection was used by 37 and 53% of patients using a classical or vibrating-mesh nebulizer, respectively. After cleaning or disinfection, 91% of the material was dried. The high percentage of Belgian CF patients disinfecting their nebulizer proves that they seem aware of the hygiene problem. The lack of unanimous guidelines in Belgium at the time of the study perhaps explains the variety of methods used. The arrival of vibrating-mesh nebulizers has led to a change in disinfection methods.

Airway clearance therapy (ACT) is one of the cornerstone treatment modalities to improve mucociliary clearance for patients with bronchiectasis. The progression of lung disease in patients with bronchiectasis can be evaluated by spirometry and multiple breath washout (MBW) and it is advised to monitor these on a regular basis. However, the short term effect of ACT on spirometry and MBW parameters is insufficiently clear and this variability may impact standardization. For cystic fibrosis (CF), available literature refutes a short time effect on spirometry and MBW parameters in children, however, for primary ciliary dyskinesia (PCD) no data are available. We performed a single-center, prospective cross-over study to evaluate the short term effect of a single ACT session using positive expiratory pressure mask on forced expiratory volume in 1 s (FEV1) and lung clearance index (LCI), derived from MBW, compared to no ACT (control) in pediatric patients with CF and PCD. A total of 31 children were included: 14 with PCD and 17 with CF. For the whole group, there was no difference in median change of FEV1 pp between the treatment and the control group (p 0.969), nor in median change of LCI (p 0.294). For the CF subgroup, the mean change in FEV1 pp with ACT was −1.4% (range −9 to + 5) versus −0.2% (range −6 to + 5) for no ACT (p 0.271), the mean change in LCI with ACT was + 0.10 (range −0.7 to + 1.2) versus + 0.17 (range −0.5 to + 2.8) for no ACT (p 0.814). In the PCD subgroup, the mean change in FEV1 pp with ACT was + 1.0 (range −7 to + 8) versus −0.3 (range −6 to + 5) for no ACT (p 0.293) and the mean change in LCI with ACT was −0.46 (range −3.7 to + 0.9) versus −0.11 (range −1.4 to + 1.3) for no ACT (p 0.178). There was no difference between PCD and CF for change in FEV1 pp after ACT (p = 0.208), nor for LCI (p = 0.095). In this small group of pediatric patients, no significant short-term effect of chest physiotherapy on FEV1 pp nor LCI in PCD and CF values nor variability was documented.

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