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Background: Immunotherapy for bee venom allergy is effective and provides long-term protection. Venom-specific IgG4 levels are increased but with no correlation with clinical improvement. Following grass pollen immunotherapy, elevation of antigen-specific IgG4 is accompanied by increases in IgG-dependent serum inhibitory activity for IgE-facilitated binding of allergen-IgE complexes to B cells. As this 'functional' assay of inhibitory antibodies may be more predictive of clinical efficacy, we investigated the time course of serum inhibitory activity for IgE-facilitated antigen binding during venom immunotherapy (VIT) in children and following 2 years of VIT withdrawal., Methods: Ten bee venom-allergic children (mean age: 9.3 years; m/f, 7/3) with moderate to severe allergic reactions to bee stings received VIT. A separate group of seven children (mean age: 14 years; m/f, 5/2) were investigated 2 years after VIT withdrawal. Ten age- and gender-matched children served as non-allergic controls. Allergen-specific serum IgG4 and IgE levels were measured by ELISA at baseline, after 2 years of VIT and 2 years after VIT withdrawal. Serum inhibitory activity was assessed using the facilitated-allergen binding (FAB) assay., Results: Sera obtained during VIT significantly inhibited allergen-IgE binding to B-cells (pre-treatment=104+/-23%; 2 years=46+/-15%; P<0.001) when compared with sera obtained after treatment withdrawal and sera from normal controls. In parallel to FAB inhibition during VIT, significantly higher IgG4 levels were noted after immunotherapy (pre-treatment=8.6+/-2.3 AU; 2 years=26.7+/-3.5 AU; P<0.001) compared with those observed after withdrawal and in the controls. In contrast, progressively lower IgE concentrations were observed compared with pre-treatment (44+/-7 AU) in sera obtained after 2 years of VIT (25+/-5 AU; P<0.01) and 2 years following the withdrawal of VIT (10+/-3 AU; P<0.05)., Conclusions: In contrast to grass pollen immunotherapy, the persistent decline in venom-specific IgE levels, rather than serum inhibitory activity for FAB, may be more relevant for long-term clinical efficacy of VIT.

Unlabelled: This case report describes a 13-year-old boy with diffuse microvascular pulmonary arteriovenous malformations, in whom total anomalous pulmonary venous drainage had been corrected surgically in the newborn period. Contrast transesophageal echocardiography, cardiac catheterization and a lung perfusion scan suggested, and lung biopsy confirmed the diagnosis in our patient. Treatment with nifedipin was commenced., Conclusion: This case illustrates the diagnostic difficulties with this rare malformation., (Copyright (c) 2007 S. Karger AG, Basel.)

Gorham-Stout syndrome is a rare disease characterized by vascular proliferation in the bones, effecting osteolysis. When it is complicated by chylothorax, the prognosis is poor. The present case illustrates successful management of chylothorax by a combination of surgery (thoracic duct ligation and excision of lymphangiomatous tissue combined with pleurodesis) and treatment with alpha-2b interferon. Treatment in 38 published cases is reviewed.

Objective: To assess the relation between age and bronchodilator responsiveness in infants with bronchiolitis., Study Design: In 41 infants (age, 2 to 18 months) with bronchiolitis, lung function was measured with the raised volume rapid thoracoabdominal compression technique before and after salbutamol inhalation. Lung function was quantified in terms of timed volumes (FEV(0.5), FEV(0.75), and FEV(1.0)). A significant change was defined as a postbronchodilator value that differed from baseline by more than twice the within-subject coefficient of variation., Results: For the group, postbronchodilator values did not differ significantly from baseline (DeltaFEV(0.5), 3.8% +/- 9.3%; DeltaFEV(0.75), 3.5% +/- 9.5%; and DeltaFEV(1.0), 4.0 +/- 9.8%). Eleven subjects showed significantly increased timed volumes; 3 presented with a decreased lung function; the remaining patients failed to show a significant change. The mean age of subjects with improved lung function did not differ significantly from the mean age of those with no or paradoxical responses (9.7 +/- 4.7 vs 8.1 +/- 4.1 months); there was no correlation of age with the size of the bronchodilator response., Conclusions: The results of the current study indicate that bronchodilator responsiveness in infants with bronchiolitis is not age-dependent.

Accidental ingestion and aspiration of hydrocarbons in children are common. Among the various clinical and pathological manifestations of hydrocarbon (HC) poisoning, pneumonitis is the most significant and occurs in up to 40% of children, whereas formation of pneumatoceles is believed to be a rare event. We report two children with HC pneumonitis and pneumatoceles as a reversible complication after ingestion and aspiration of lamp oil with very low viscosity. Patient 1, a 21-month-old boy, started to cough and developed tachypnea, sternal retractions and mild cyanosis immediately after aspiration. Patient 2, a 24-month-old girl, was asymptomatic during the first days after the accident; subsequently, she started to cough and developed fever, dyspnea and chest pain. Chest x-ray and computed tomography revealed multiple patchy infiltrates in both cases; after several days, these confluent infiltrates developed into pneumatoceles. Both children were treated with antibiotics and steroids. They recovered within three and four weeks, respectively, with complete remission of the radiologic abnormalities and had an uneventful follow-up after discharge.

Arthralgia in childhood is an unspecific symptom. One rare cause of arthralgia is hypertrophic osteoarthropathy associated with digital clubbing. We present a child where hypertrophic osteoarthropathy led to the rare diagnosis of an inflammatory pseudotumour of the lung. In a 12-year-old girl with arthralgia and digital clubbing, a chest radiograph disclosed a large round mass in the right upper lobe, and the following chest computed tomography scan showed a large solid homogenous, round, well marginated lesion with little contrast enhancement. A lobectomy of the right upper lobe was performed, and histological examination showed an inflammatory pseudotumour. The postoperative course was without problems: arthralgia and digital clubbing disappeared.

We report three cystic fibrosis (CF) patients with hypersplenism who underwent partial splenectomy. The postoperative course was uneventful in two patients; one patient developed a complication necessitating resection of the rest of the spleen. Haematological parameters improved and oesophageal varices regressed in all patients. On follow up, one patient showed a normal spleen, the other a normally functioning accessory spleen; the third patient again developed splenomegaly with hypersplenism. Partial splenectomy is a promising therapeutic option for CF patients with hypersplenism.

Nedocromil and salbutamol are effective drugs in preventing exercise-induced asthma (EIA). The aim of this study was to compare the protective effects of both drugs and a combination of both drugs against cold dry air-induced bronchoconstriction, using cold dry air challenges (CACh) as a surrogate for exercise. Twenty-five atopic children (mean age 13.7, range 8-18 yrs) with EIA participated in the study. Lung function tests were performed before medication, 30 min after medication and just before CACh, and 3 and 15 min after the challenge on four consecutive days. CACh consisted of a 4-min isocapnic hyperpnoea of -10 degrees C, absolutely dry air. Treatment consisted of nedocromil (two puffs of 2 mg) plus placebo, salbutamol (two puffs of 100 microg) plus placebo, the combination of both drugs, and placebo alone, in a random order. Both active drugs were significantly more protective than placebo and the combination showed an additive effect. Mean maximum postchallenge decrease in forced expiratory volume in one second after placebo was 27+/-8.1%, 12+/-9.5% after nedocromil, 8+/-10.4% after salbutamol, and 4.5+/-6.71% after the combination of both drugs, respectively. These results suggest that both drugs protect against exercise-induced asthma. Although not as effective as salbutamol and combined medication, nedocromil can give sufficient protection for many patients.

Background: Laser acupuncture, a painless technique, is a widely used alternative treatment method for childhood asthma, although its efficacy has not been proved in controlled clinical studies., Methods: A double blind, placebo controlled, crossover study was performed to investigate the possible protective effect of a single laser acupuncture treatment on cold dry air hyperventilation induced bronchoconstriction in 44 children and adolescents of mean age 11.9 years (range 7.5-16.7) with exercise induced asthma. Laser acupuncture was performed on real and placebo points in random order on two consecutive days. Lung function was measured before laser acupuncture, immediately after laser acupuncture (just before cold dry air challenge (CACh)), and 3 and 15 minutes after CACh. CACh consisted of a 4 minute isocapnic hyperventilation of -10 degrees C absolute dry air., Results: Comparison of real acupuncture with placebo acupuncture showed no significant differences in the mean maximum CACh induced decrease in forced expiratory volume in 1 second (27.2 (18.2)% v 23.8 (16.2)%) and maximal expiratory flow at 25% remaining vital capacity (51.6 (20.8)% v 44.4 (22.3)%)., Conclusions: A single laser acupuncture treatment offers no protection against exercise induced bronchoconstriction in paediatric and adolescent patients.

Inhaled antibiotics are an established treatment for chronic Pseudomonas aeruginosa (PA) infection in patients with cystic fibrosis (CF). However, inhaled antibiotics might also have prophylactic potential to delay acquisition of PA in early stages of the disease. From 1986-1999, all CF patients at this center who experienced defined risk situations for acquisition of PA (28 patients) received inhaled gentamicin (80 mg BID for those < 12 months; 120 mg BID for those > 12 months) for a minimum of 3 years. Twelve patients had repeated risk situations and continued this prophylaxis without interruption during the entire study period (group 1). In the remaining 16 patients, inhaled antibiotics were discontinued at various times for a variety of reasons (group 2). None of the patients in group 1, but 7 in group 2, became chronically infected with PA (P = 0.01). Lung function and chest X-ray scores were significantly worse in those 7 infected patients, when compared to the noninfected ones in both groups. This suggests that long-term-prophylaxis with inhaled gentamicin can effectively delay acquisition of PA and decrease disease progression in children with CF., (Copyright 2002 Wiley-Liss, Inc.)

Unlabelled: We report on two Turkish children who presented with progressive airway obstruction. Open lung biopsy revealed follicular bronchiolitis. The children were treated with systemic steroids and various topical medications. Whereas the respiratory situation of patient 1 required immunosuppressive therapy, the condition of patient 2 stabilised without systemic medication., Conclusion: Diagnosis of follicular bronchiolitis should be considered when children present with recurrent respiratory tract infections, progressive dyspnoea, and chronic bronchial obstruction. Children in whom follicular bronchiolitis is suspected should undergo open lung biopsy for confirmation of diagnosis.

Tracheobronchomegaly (Mounier-Kuhn syndrome) is characterized by dilatation of the central airways, tracheobronchial outpouchings, and chronic tracheobronchitis. Most cases are diagnosed in adulthood. We report the clinical, radiographic, and bronchoscopic findings in a 14-year-old boy with tracheobronchomegaly, ptosis of the right eyelid, and redundant skin and mucosa of the upper lip, who presented with a 2-year history of recurrent lower respiratory tract infections. Pediatricians should be aware of the possibility that tracheobronchomegaly may cause clinical symptoms in childhood and adolescence. Pediatr Pulmonol. 2000; 29: 476-479., (Copyright 2000 Wiley-Liss, Inc.)

Unlabelled: We report on the clinical, laboratory, and molecular genetic findings in a 15-month-old boy who initially presented with disseminated cytomegalovirus and concomitant para-influenza virus infection. Hyper-IgM syndrome, suspected on clinical grounds, was confirmed by immunological investigations. In addition, a previously unreported potentially disease-causing mutation in the CD40 ligand gene was detected in this patient., Conclusion: The present case illustrates that disseminated cytomegalovirus infection with atypical clinical features should be included in the spectrum of the hyper-IgM syndrome.

Chest physiotherapy for mucus clearance may be seen as a therapeutic application of respiratory physiology; the basic mechanical principles do not differ between the treatment of paediatric and adult patients. However, children present with a developmentally defined, age-specific situation regarding respiratory structure and function that calls for a specialised therapeutic approach. Inspiration dilates bronchi and thus brings air behind obstructing mucus plugs. Newborns and infants have the special problem of an overly compliant, instable thorax and an easily obstructed upper airway; hence they require special help with lung volume management. Forced expirations mobilise and transport secretions by expiratory airflow in combination with a wave of moving choke points. Early in life, low stability of airways threatens to hamper this mechanism by complete closure; this calls for special therapeutic assistance. Positioning regionally modifies ventilatory excursions and is thus used to target gas-liquid pumping. However, lateral decubitus position does not affect ventilation in children in the same way as in adults. Disease-inflicted changes of structure and function call for further modification of the therapeutic approach. Finally, the psychology of therapist-patient interaction differs substantially between different age groups.

This case study reports on two infants, 5 and 6 months of age, respectively, with chronic interstitial lung disease who presented with failure to thrive, tachypnoea, rales and mild hypoxaemia. Epstein-Barr virus (EBV) was detected by in situ hybridization in lung biopsy specimens and by EBV-deoxyribonucleic acid-polymerase chain reaction (PCR) in bronchoalveolar lavage (BAL) fluid in one patient and by in situ hybridization and PCR analysis in BAL fluid in the second patient. There was serological evidence of immunoglobulin G antibodies to EBV capsid antigen by indirect immunofluorescence in both patients. After 7 months of respiratory symptoms one patient was successfully treated with a 10 day course of intravenous ganciclovir followed by oral acyclovir for 20 days. The other patient became symptom free after 3.5 months of respiratory symptoms, without any specific antiviral medication. During a follow-up of 2 and 1.5 yrs, respectively, both infants remained symptom free and showed normal physical development.

Insight into the physiology of a forced expiration is the most important prerequisite for understanding and correctly interpreting the most common pulmonary function tests like spirometry and recording of a maximum expiratory flow-volume curve. Along the airway, intraluminal pressure falls progressively from alveolar pressure in the periphery to atmospheric pressure at the airway opening. Downstream of the equal pressure point, where intraluminal equals transthoracic pressure, the airway is dynamically compressed. Once the forced expiration is flow limited, more transthoracic pressure will rather effect more compression than a further increase in flow. It follows that the achieved maximum expiratory flow rates are then exclusively defined by the resistance of the intrathoracic airways and no longer by muscular effort. The end of a forced expiration is determined by the elastic resistance of the thoracic cage and by airway closure.

Whether bronchodilators should be used for the treatment of infants with bronchiolitis is subject to debate, partly because of the low sensitivity of the methods for assessing lung function changes in infants. In the present study, we compared the recently introduced raised volume (RVRTC) with the conventional end-tidal rapid thoracoabdominal compression (ETRTC) technique in infants with acute viral bronchiolitis. In 17 infants lung function was assessed by both methods, at baseline values and after salbutamol inhalation. Forced expiratory volumes (FEV(0.5), FEV(0.75), FEV(1.0)) were used for the quantification of RVRTC measurement, and maximal expiratory flow at functional residual capacity (Vmax (FRC)) for ETRTC measurements. A significant individual change was defined by a mean postbronchodilator value that differed from baseline value by more than twice the within-subject coefficient of variation (CV). Group mean intrasubject CVs ranged from 4.7% to 5.3% for FEV parameters; it was 14.0% for Vmax (FRC). For the group, post-bronchodilator measurements did not differ significantly from baseline measurements. For the majority of infants, however, the within-subject comparison of responses revealed substantial differences between both techniques; while no infant demonstrated a significant increase in Vmax (FRC), eight (47%) infants responded with significantly improved timed volumes. The RVRTC technique provides the investigator with a more sensitive diagnostic tool for documenting the effectiveness of therapeutic interventions on an individual basis. Furthermore, the findings of the present study provide a rationale for the application of bronchodilators in a subgroup of infants with acute bronchiolitis.

The end-tidal rapid thoracoabdominal compression (ETRTC) technique is an established method for lung function testing in infancy. Previous work in healthy infants, however, has shown that measurements with the newly developed raised volume rapid thoracoabdominal compression (RVRTC) technique are more reproducible than those with the ETRTC technique. So far, reproducibility of the two techniques has not been compared in infants with acute airway disease. Twenty-three infants with acute viral bronchiolitis underwent lung function assessment with both the ETRTC and the RVRTC technique. A series of 8-10 measurements with each technique was done in randomized order. Forced expired volumes at 0.5, 0.75, and 1 sec after chest compression (FEV(0.5), FEV(0.75), and FEV(1.0)) were measured with the RVRTC technique; maximum expiratory flow at functional residual capacity (V'(maxFRC)) was measured with the ETRTC technique. Group mean intrasubject coefficients of variation (CV) were 4.84% for FEV(0.5), 5.01% for FEV(0.75), 5.43% for FEV(1. 0), and 13.79% for V'(maxFRC), respectively. Differences between FEV parameters were statistically insignificant, whereas the difference between each FEV parameter and V'(maxFRC) was highly significant (P < 0.001). In infants with acute viral bronchiolitis, RVRTC measurements have significantly less intraindividual variability than flow rates assessed with the conventional ETRTC technique. This finding provides the basis for assessing disease course and effects of therapeutic interventions on an individual basis., (Copyright 1999 Wiley-Liss, Inc.)

Background: Serum eosinophil cationic protein (ECP) has been promoted as a marker of inflammatory activity in bronchial asthma. Bronchial responsiveness, measured either by inhaling pharmacologically active substances such as histamine or methacholine, or by applying physical stimuli such as the hyperventilation of cold dry air, is also considered to be an indirect marker of bronchial inflammation., Objectives: In this study, we investigated the possible relationship between serum ECP and bronchial responsiveness to both cold dry air and histamine in presently symptom- and medication-free pediatric and adolescent asthma patients., Subjects: Thirty-six children and adolescents with atopic asthma were studied., Methods: On 2 consecutive days, bronchial responsiveness was assessed nonpharmacologically by cold dry air and pharmacologically by histamine in random order. Blood samples for determination of ECP were collected before each challenge., Results: Serum ECP levels correlated with neither cold dry air-induced changes in FEV1 nor the provocation concentrations of histamine causing a 20% fall in FEV1. Subjects with bronchial hyperresponsiveness to cold dry air and histamine had somewhat higher levels of serum ECP than subjects with normal responses, but these differences were insignificant., Conclusions: Our results indicate a lack of relationship both between serum ECP and bronchial responsiveness to cold dry air and between serum ECP and bronchial responsiveness to histamine.

The measurement of passive respiratory mechanics by the single-breath occlusion technique is one of the more frequently used tests of infant lung function. Measurements are routinely done under chloral hydrate sedation, and a possible influence of sleep stages on these measurements has not been evaluated so far. We combined the assessment of passive respiratory mechanics with sleep stage monitoring in 44 infants and toddlers with mild to moderately severe bronchiolitis. In 31 infants, only nonrapid eye movement (NREM) sleep was recorded. In 13 patients who showed both NREM and rapid eye movement (REM) sleep, compliance of the respiratory system was significantly lower during REM than NREM sleep (73.2 +/- 19.7 vs. 81.2 +/- 21.3 mL/kPa, P = 0.0007), while resistance remained essentially unchanged. This finding was explained by an unchanged airway opening pressure in combination with a significantly decreased extrapolated volume. As tidal volume did not change from NREM to REM, this indicates reduced dynamic elevation of lung volume during REM sleep and thus supports previous observations of decreased lung volume in this sleep stage. From a practical perspective, these findings argue for the monitoring of sleep stage during measurements of passive respiratory mechanics, thereby increasing the complexity of these measurements significantly.

Background and Objective: Allergic asthma is common in children, and house dust mite (HDM) is an important source of perennial allergens. Bronchial hyperresponsiveness is a functional hallmark of asthma. Specific immunotherapy (SIT) with HDM extracts were shown to decrease symptoms, but its effect on bronchial responsiveness, as measured by non-pharmacological challenges, has not been evaluated., Methods: Twenty-six paediatric asthma patients allergic to HDM participated in this study. Fourteen patients received SIT with a HDM extract (Alavac, Bencard) for 2 years, and 12 served as controls. Bronchial responsiveness was assessed non-pharmacologically by cold dry air challenge (CACh) before and 3, 6, 12 and 24 months after SIT, and 12 months after termination of SIT., Results: After 24 months, the SIT group showed a statistically significant reduction of the mean CACh-induced changes of both forced expiratory volume in one second (-21.8+/-2.7% vs. -13.7+/-2.4%; P = 0.03) and maximal expiratory flow at 25% remaining vital capacity (-48.9+/-4.9% vs. -27.9+/-6.2%; P = 0.01). In contrast, no significant changes of bronchial responsiveness were observed in the control group. In the SIT group more patients lost their bronchial hyperresponsiveness than in the control group (6/14 vs. 1/12; P<0.05). One year after terminating SIT, the treatment group showed a tendency towards returning bronchial hyperresponsiveness., Conclusion: These results demonstrate that during 2 years of SIT there was a reduction of bronchial hyperresponsiveness in HDM-allergic paediatric asthma patients.

Objective: To investigate possible renal toxicity of long-term gentamicin inhalation in patients with cystic fibrosis., Methods: Urinary N-acetyl-beta-D-glucosaminidase (NAG) activity was measured during routine respiratory clinic visits. Outpatient records were reviewed for data on long-term gentamicin inhalation, and parents and patients were interviewed for compliance. Exclusion criteria were irregular gentamicin inhalation, urinary infection or other febrile illness, intravenous aminoglycoside treatment during the previous three months, and diabetes mellitus. Patients were assigned to three groups: group 1, current gentamicin inhalation; group 2, previous gentamicin inhalation that had been stopped at least three months ago; and group 3, never any gentamicin inhalation., Results: 52 patients (34 girls, 18 boys), mean (SD) age 11.5 (5.7) years, entered the study. Patients currently on gentamicin inhalation (n = 20) were significantly younger and had higher urinary NAG activity (0.83 (0.57) U/mmol creatinine) than the 23 patients without gentamicin inhalation (0.26 (0.10) (p = 0.0001) and the nine patients with previous gentamicin inhalation (0.32 (0.15) (p = 0.0125). Twelve patients on current gentamicin inhalation had raised NAG values but all those in groups 2 and 3 had NAG values within the normal range. In patients currently on gentamicin inhalation, there was a positive correlation between urinary NAG activity and cumulative dose of nebulised gentamicin (r = 0.60, p = 0.0049)., Conclusions: Long-term gentamicin inhalation in patients with cystic fibrosis poses a risk of renal toxicity. It is not known whether further treatment might result in more severe renal damage.

Background: It has remained unclear whether bronchial responsiveness as measured by a single-step cold-dry air challenge (CACh) correlates closely to the responsiveness that is assessed by a routine pharmacologic challenge., Methods: On 2 consecutive days, we performed a CACh and a histamine challenge in 128 symptom- and medication-free pediatric and adolescent asthma patients. The CACh consisted of 4 min of isocapnic hyperventilation of -10 degrees C, absolutely dry air; responsiveness was expressed by the induced change in FEV1 (delta FEV1). The histamine challenge consisted of sequential inhalations of incremental increases in concentrations of histamine; responsiveness was expressed by the concentration which caused a 20% fall of FEV1 (PC20)., Results: Five children did not bronchoconstrict sufficiently in the histamine challenge for measuring a PC20 and were excluded from analysis. In the remaining 123, delta FEV1 (CACh) ranged from +5 to -73%, PC20 (histamine) from 0.05 to 7.2 mg/mL. There was a statistically significant correlation between delta FEV1 and PC20 (r = 0.54, p < 0.001), but also a considerable scatter of individual data points around the regression line. Fifty-two subjects were hyperresponsive by CACh and 114 by histamine criteria., Conclusions: There is a relatively weak correlation between the results of these two challenges; thus, one cannot be substituted one for the other. Histamine appears as more sensitive in detecting airway hyperresponsiveness than CACh. The poor correlation between the responses to these two challenges can be explained by differences between the challenge protocols, or, alternatively, by differences between applied stimuli and activated mechanisms.

We report on a now 9-month-old infant with severe tracheobronchomalacia associated with campomelic dysplasia. The diagnosis of tracheobronchomalacia was suggested in the neonatal period by typical changes of the tidal breathing flow-volume loop and was confirmed by tracheobronchoscopy. Tidal breathing flow-volume loop analysis also allowed to titrate the optimal, continuous positive airway pressure for the respiratory management of the patient. Since bedside pulmonary function testing is noninvasive and rapid, it may reduce the need for frequent bronchoscopic evaluations in the management of patients with tracheobronchomalacia.

The passive, single-breath, flow-volume technique is a simple method for measuring the resistance (Rrs) and the compliance (Crs) of the respiratory system in infants. So far, the potential influence of end inspiratory occlusion time on these measurements has not been investigated. We measured Rrs and Crs in 36 infants and toddlers with bronchiolitis; in each child, a spectrum of nine fixed occlusion times, ranging from 90 to 600 ms, was applied in random sequence. Increasing the duration of occlusions from 90 to 275 ms resulted in marked stepwise changes of measured Rrs and Crs; occlusions longer than 275 ms, however, produced highly reproducible measurements, as expressed 1) by minimal absolute differences between measured values at subsequent occlusion times and 2) by minimal percentage changes of measured values from one occlusion time to the next. There was no influence of age on the results; reproducible measurements were made in children as old as 1.5 y. This suggests that, in infants with bronchiolitis, 1) occlusions between 300 and 450 ms might be ideal for obtaining reliable measurements, and 2) the age range for applying this method can be extended into the 2nd y of life.

Fourteen patients with cystic fibrosis were trained in 2 self-administered chest physiotherapy (PT) techniques: high-pressure PEP-mask physiotherapy (PEP), and autogenic drainage (AD). They then visited the clinic on 5 consecutive days, and, in a random order, performed 1 of the following: PEP, AD, PEP followed by AD (PEP-AD), AD followed by PEP (AD-PEP), and, no PT except for spontaneous coughing. Lung function was measured repeatedly before, during, and after PT; time needed for and sputum produced by each form of PT was recorded. PEP produced the highest amount of sputum, followed by PEP-AD, AD-PEP, and AD; all 4 forms of PT produced significantly more sputum than coughing. Lung function improved significantly after PEP, AD, and PEP-AD, but PEP-induced changes did not exceed those after AD. Within the investigated group, the PEP-induced lung function improvement per milliliter of sputum produced was significantly lower for those patients with airway hyperreactivity. The fact that the highest sputum yield with PEP was not reflected in higher PEP-effected lung function changes might thus be explained by PEP-induced bronchospasm in patients with airway hyperreactivity. PEP clears more sputum than AD or combined techniques; patients with airway hyperreactivity, however, should either prefer AD or should take a bronchodilator premedication before PEP.