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Wir berichten uber einen 8-jahrigen Knaben, der wegen rezidivierender Hustenattacken und stechender Thoraxschmerzen an die Klinik kam. Das Kind zeigte Zeichen eines respiratorischen Infekts; ein Thoraxrontgen ergab einen linksseitigen Pneumothorax, der primar keiner invasiven Intervention bedurfte. Der Knabe wurde intensiv uberwacht, und es erfolgten wiederholte radiologische Kontrollen. Bei weiterbestehenden Hustenattacken kam es in der Folge zusatzlich zum Auftreten eines Pneumomediastinums sowie eines Hautemphysems im Halsbereich. Im Anschluss an eine spontane klinische und radiologische Besserung entwickelte sich dann eine Atelektase der linken Lunge. In einer daraufhin durchgefuhrten flexiblen Bronchoskopie wurde die Diagnose einer Kaugummiaspiration in den linken Hauptbronchus diagnostiziert. Nach Entfernung des Fremdkorpers resorbierte sich die freie Luft im Thorax rasch. Bei einem Spontanpneumothorax im Kindesalter sollte auch an die Moglichkeit einer Fremdkorperaspiration gedacht werden.

Congenital malformations of the lower respiratory tract are usually diagnosed and managed in the newborn period, in infancy, or in childhood. To what extent should the adult pulmonologist be experienced in this predominantly paediatric field? There are three ways in which an adult physician may be confronted with this spectrum of disorders. The most frequent type of encounter will be a former paediatric patient, now reaching adulthood, with the history of a surgically treated respiratory malformation; in some of these patients the early loss of lung tissue raises questions of residual damage and compensatory growth. Secondly, there is an increasing number of children in whom paediatric pulmonologists treat respiratory malformations expectantly; these patients eventually become adults with their malformation still in place. Thirdly, there is a small group of patients in whom the malformation goes unrecognised throughout childhood; in these a late complication or the coincidental discovery of a radiographic anomaly may demand a late diagnosis and management in adulthood. All three of these adult presentations will require some expertise on the part of the managing physician. The subsequent text reviews the most common “classical” congenital malformations of the lower respiratory tract from the above three perspectives. ### Paediatric presentation TOF usually occurs in association with oesophageal atresia; thus, the outcome is invariably fatal unless the anomaly is surgically repaired in the first days of life. The rare “H type” TOF without oesophageal atresia is an exception to this rule and may persist into later childhood or even adulthood before the diagnosis is established.1 The malformation is explained by the defective progression of a complex system of folds that separate the foregut into trachea and oesophagus.2 Approximately 50% of infants with TOF have associated anomalies, most often involving the urinary, gastrointestinal, and cardiac systems.3,4 The first successful repair of …

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.

Congenital malformations of the lower respiratory tract are usually diagnosed and managed in the newborn period, in infancy, or in childhood. To what extent should the adult pulmonologist be experienced in this predominantly paediatric field? There are three ways in which an adult physician may be confronted with this spectrum of disorders. The most frequent type of encounter will be a former paediatric patient, now reaching adulthood, with the history of a surgically treated respiratory malformation; in some of these patients the early loss of lung tissue raises questions of residual damage and compensatory growth. Secondly, there is an increasing number of children in whom paediatric pulmonologists treat respiratory malformations expectantly; these patients eventually become adults with their malformation still in place. Thirdly, there is a small group of patients in whom the malformation goes unrecognised throughout childhood; in these a late complication or the coincidental discovery of a radiographic anomaly may demand a late diagnosis and management in adulthood. All three of these adult presentations will require some expertise on the part of the managing physician. The subsequent text reviews the most common “classical” congenital malformations of the lower respiratory tract from the above three perspectives. ### PAEDIATRIC PRESENTATION TOF usually occurs in association with oesophageal atresia; thus, the outcome is invariably fatal unless the anomaly is surgically repaired in the first days of life. The rare “H type” TOF without oesophageal atresia is an exception to this rule and may persist into later childhood or even adulthood before the diagnosis is established.1 The malformation is explained by the defective progression of a complex system of folds that separate the foregut into trachea and oesophagus.2 Approximately 50% of infants with TOF have associated anomalies, most often involving the urinary, gastrointestinal, and cardiac systems.3 4 The first successful repair of …

Da der Husten im Rahmen einer Erkaltungskrankheit zumeist nur von kurzer Dauer ist, wird diese rasche Besserung von vielen Patienten und auch Arzten auf die Wirksamkeit der angewendeten Praparate zuruckgefuhrt. Diese Ansicht durfte einer der Hauptgrunde fur den weitverbreiteten Gebrauch dieser Medikamente sein. Die Aussagekraft solcher Anwendungsbeobachtungen uber die untersuchte Patientengruppe hinaus ist jedoch aufgrund des Fehlens einer Kontrollgruppe fragwurdig. Durch kontrollierte klinische Studien kann die Wirksamkeit einer Behandlung von anderen moglichen therapeutischen Faktoren, die sich aus der Arzt-Patient-Beziehung oder aus der Erwartungshaltung des Patienten ergeben, getrennt werden.

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.

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.

Einige der am haufigsten eingesetzten alternativen Behandlungsmethoden des Asthma bronchiale werden diskutiert. Die relevante Literatur und insbesondere die zur Wirksamkeit der einzelnen Methoden durchgefuhrten klinischen Studien werden gesichtet. Nach kurzer Behandlung der Definitionen, der soziodemographischen Daten sowie der zur Evaluierung von Asthmatherapie gangigen Strategien, werden folgende alternativmedizinische Methoden besprochen: Akupunktur, Homoopathie, Yoga, Hypnose, autogenes Training, Muskelentspannung nach Jacobson, manuelle Medizin, Ionisatoren, Bioresonanz. Zusammenfassend ist das wissenschaftlich seriose Literaturgut zu diesen Methoden sparlich und widerspruchlich; eine Ausnahme ergibt sich nur fur Yoga und evtl. auch Hypnose, wo die bisherigen Daten die Moglichkeit einer therapeutischen Wirkung andeuten.

In der vorliegenden Arbeit werden unter Betonung padiatrischer Aspekte die wichtigsten Daten zur Wirksamkeit und Sicherheit der spezifischen Immuntherapie mit Hausstaubmilbenextrakten erortert. Asthmapatienten mit Hausstaubmilbenallergie steht, neben der Allergenkarenz und der antiinflammatorischen Pharmakotherapie, in der spezifischen Immuntherapie eine weitere Behandlungsmoglichkeit zur Verfugung. Kontrollierte Studien zur Wirksamkeit dieser Immuntherapie zeigen eine Reduktion der Asthmasymptome und des Medikamentenverbrauchs sowie positive Effekte auf verschiedene atemphysiologische Parameter. Gute Behandlungserfolge konnten v. a. an padiatrischen Patienten erzielt werden. Schwerwiegende Nebenwirkungen dieser Behandlung sind selten, werden aber haufiger bei Asthmapatienten mit manifester Atemwegsobstruktion, im Kleinkindesalter und unter einem Rush-Protokoll beobachtet.

With special emphasis on the paediatric aspects, the importance of peak expiratory flow rate measurements in asthma patients is reviewed. Portable peak flow meters allow for regular home monitoring of lung function. Repeated peak expiratory flow recordings by patients at home are of great value in the diagnosis and the therapeutic management of bronchial asthma. Monitoring of peak expiratory flow rates may improve the perception of compromised airway function and thereby enhance compliance in asthma patients. As a relatively simple form of pulmonary function testing, the measurement of peak expiratory flow rates, however, cannot substitute for pulmonary function testing by spirometry, flow-volume-curves or bodyplethysmography.

Pseudomonas aeruginosa is the major pathogen in the cystic fibrosis (CF) lung, the predominant source of its acquisition, however, is under discussion. In order to study the molecular epidemiology, we evaluated 86 P. aeruginosa isolates from 43 CF patients from southeast Austria. The DiversiLab system was used to identify genetic relationships among the isolates. Antibiotic susceptibilities were tested with a broth microdilution method (Micronaut Merlin). A total of 39 unrelated P. aeruginosa genotypes were found of which 34 were unique to a single patient and one was unique to a sibling pair. We found low rates of resistance for β-lactams with resistance to piperacillin/tazobactam and ceftazidime ranging from 4 to 6%. Resistance rates for meropenem and ciprofloxacin were 11% and 15%, respectively. The prevalence of multidrug-resistant isolates was 2%. We conclude that the majority of P. aeruginosa isolates from CF patients originate from environmental sources and patient-to-patient spread is very uncommon in our centre.

Lung disease in cystic fibrosis (CF) starts early in life. Infection, inflammation, reduced lung function and abnormal chest CT findings are present in a significant proportion of infants with CF at a time when many of these children have no clinically apparent lung disease.1–6 Infection and inflammation are intimately linked early in the course of CF lung disease.2 7–9 The detection of airway inflammation in the absence of apparent infection led to the speculation that CF is associated with an intrinsic abnormality of immune regulation.10 However, more recent data argue against a primary CF-associated dysregulation of local immune function where inflammation might precede infection; they rather support the central role of bacterial pathogens in initiating and sustaining the neutrophil-mediated airway inflammation characteristic of CF lung disease, with a higher inflammatory burden in children infected with Pseudomonas aeruginosa (PA) than in those infected with organisms other than PA or uninfected.6 11 12 Chronic respiratory infection is one of the main characteristics of CF and significantly contributes to morbidity and mortality.12 13 In young children Staphylococcus aureus , Haemophilus influenzae and PA are the major lower airway pathogens.12 13 Chronic infection with PA is known to be associated with a worse prognosis in children, adolescents and adults, and patients with mucoid PA strains do worse than those with non-mucoid strains.14–18 Thus, prevention or early detection and treatment of infection, in particular with PA, may prevent or delay irreversible lung damage from inflammation and consequently may improve prognosis. In order to prevent acquisition of PA, hygienic measures to decontaminate environmental reservoirs of this organism including medical equipment have been stressed, and CF centres have adopted meticulous microbiological surveillance and effective segregation policies to limit cross-infection between patients. Furthermore, several trials have been undertaken to assess the …

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.The present case illustrates that disseminated cytomegalovirus infection with atypical clinical features should be included in the spectrum of the hyper-IgM syndrome.

For pharmacological challenges, a correlation between the induced changes of the transcutaneously measured oxygen tension (Ptc) and of conventional pulmonary function tests (PFTs) has been documented. We performed a 4-minute cold air challenge (CACh) in 17 children with bronchial asthma under continuous monitoring of Ptc, and correlated observed changes with CACh-induced alterations of conventional PFTs. Ptc decreased significantly with CACh (from 86 ± 10 mmHg to 66 ± 14 mmHg, P < 0.001), but returned to near-baseline within the next 30 minutes. PFTs changed with a similar pattern; the closest correlation was found between the CACh-induced fall of Ptc and Delta-FEV1 (r = 0.833, P < 0.001). Changes of FVC, PEF, and Vmax50 correlated significantly as well. Ptc can complement or substitute for conventional PFTs in assessing the response to CACh in children. Pediatr Pulmonol 1991; 10:273–277.

Lung injury caused by intrauterine inflammation has recently been strongly implicated in the pathogenesis of Wilson-Mikity syndrome (WMS). This article supports this theory by suggesting a causative role of intrauterine cytomegalovirus (CMV) infection for the development of WMS. A male premature infant, born at 33 weeks of gestational age, developed chronic lung disease compatible with WMS and diagnostic evaluation was positive for CMV infection. High-resolution computed tomography scan and lung histology revealed typical features of WMS in association with signs of interstitial pneumonia. CMV was found in urine, breastmilk, bronchoalveolar lavage material and lung tissue from open lung biopsy. Follow-up after treatment with ganciclovir and steroids showed resolving lung disease at the age of 6, 10 and 16 months, with lung function signs of mild obstruction. Assuming that a chance coexistence of cytomegalovirus pneumonia and Wilson-Mikity syndrome is rather unlikely, it is possible that intrauterine cytomegalovirus infection caused a pattern of lung injury consistent with Wilson-Mikity syndrome. Further cases of Wilson-Mikity syndrome should be investigated as to a possible role of congenital infection.

To the Editors: We read with interest the recently published article by Taccetti et al . 1 in the European Respiratory Journal and the accompanying editorial by Jones 2. Tacetti et al . 1 report on their experience with early eradication therapy against Pseudomonas aeruginosa (PA) in cystic fibrosis (CF), concluding that this treatment exerts a number of beneficial effects and is also cost-effective. However, as stated in the editorial 2, questions pertaining to many details of eradication therapy, such as the choice and combination of antibiotics, dosages and modes of delivery, and, in particular, the optimal duration of treatment remain as yet unanswered. We recently published the preliminary outcome of another eradication regimen against PA that had been introduced into the clinical routine of our centre in Graz (Austria) in 1999 3. A comparison of these two studies, both in terms of methodology and outcome, may shed some additional light on the previous questions. There are considerable major differences …

In cooperative children, spirometry is the most commonly used pulmonary function test. Because the test is simple and reproducible, it plays a key role in the assessment and management of respirator

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 (ΔFEV 0.5 , 3.8% ± 9.3%; ΔFEV 0.75 , 3.5% ± 9.5%; and ΔFEV 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.

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.

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°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 those12 months; 120 mg BID for those12 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.

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. © 2000 Wiley-Liss, Inc.

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.

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.

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 FEV 1 nor the provocation concentrations of histamine causing a 20% fall in FEV 1 . 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.

In adolescence, some paediatric asthma patients will become symptom-free and require no further treatment. There is little information on the atopic status, lung function and bronchial responsiveness of these patients. Symptom-free asthma patients (n=118) aged 7.7-19.2 yrs, were evaluated 1 year after termination of therapy. Bronchial asthma had previously been diagnosed on the basis of recurrent wheezing episodes. Atopic status was assessed by skin-prick testing. Baseline lung function was measured by spirometry, flow-volume curve and plethysmography. Bronchial responsiveness was assessed nonpharmacologically by cold dry air challenge. Eighty one patients had at least one positive skin test result, and the remaining 37 were defined as nonatopic. In atopic subjects, the prevalence of bronchial hyperresponsiveness was significantly higher than in nonatopic patients (41 out of 81 versus 7 out of 37; p=0.001). Atopic subjects showed a significantly lower maximal expiratory flow at 25% remaining vital capacity (p

Cold air challenge (CACh) can be applied by either a single step (SSCACh) or a multiple step (MSCACh) protocol. The interrelationship of the responses of the different protocols has not yet been studied. Furthermore, there is contradictory information on the correlation of cold air challenge responses to the outcome of pharmacological provocations. A single and a multiple step cold air challenge and a histamine provocation were performed in random order on three consecutive days on 28 children and adolescents with bronchial asthma, who were currently symptom- and medication-free. Single step cold air challenge consisted of a 4 min isocapnic hyperventilation of dry, -10 degrees C air; the subjects's response was quantified by the induced change in forced expiratory volume in one second (FEV1). Multiple step cold air challenge consisted of a series of 3 min, cold dry air hyperventilation steps from 20 to 80% of maximal voluntary ventilation (MVV); response was expressed as the provocative dose causing a 10% fall in FEV1 (PD10). Histamine provocation consisted of a series of 2 min inhalations of stepwise increasing histamine concentrations from 0.03 to 8.0 mg.mL-1; response was expressed as the provocative concentration of histamine causing a 20% fall in FEV1 (PC20). Change in FEV1 (delta FEV1) (SSCACh) correlated closely with PD10 (MSCACh); scatter around the regression line was minimal. With one exception, both types of CACh identified the same subjects as hyper- and normoresponsive. delta FEV1 (SSCACh) correlated significantly to PC20 (histamine), but scatter around the regression line was substantial. The correlation of PD10 (MSCACh) to PC20 (histamine) failed to reach statistical significance. These results indicate that the stimulus applied and the bronchoconstrictor mechanism activated, and not the challenge protocol, determine the outcome of a cold air challenge. In clinical practice, a brief single step cold air challenge can substitute for a more time-consuming multiple step cold air challenge. As nonpharmacological challenges seem to measure a different type of bronchial responsiveness, neither a single step nor a multiple step cold air challenge can substitute for a pharmacological provocation.

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.

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.

Cold air challenge (CACh) for measuring airway reactivity uses respiratory heat or water loss as a bronchoconstrictor stimulus; this stimulus is also important for the development of exercise-induced asthma, for which late asthmatic reactions (LARs) have been described. At 1200 hr on day 1, 22 children with asthma started to record their peak expiratory flow rate (PEFR) in 3 hr intervals until 0900 hr on day 2. At 1000 hr, they underwent a standardized 4 min CACh. Children then inhaled salbutamol and continued to record PEFR until 0900 hr on day 3. As a bronchodilator effect, PEFR measurements were significantly higher 2, 5, and 8 hr after CACh, but subsequently did not differ significantly from pre-CACh values. At 5 hr after CACh, individual values ranged from 95 to 137% of the corresponding pre-CACh measurements, at 8 hr from 94 to 150%, and at 11 hr from 80 to 121%. This random sample of children with asthma demonstrated no LAR after CACh.

Our recently published article on partial splenectomy in cystic fibrosis (CF) patients with hypersplenism1 appeared with a commentary by colleagues from the Birmingham Children’s Hospital.2 The authors of this commentary rightfully point out that liver disease in CF may have a widely varying symptomatology ranging from portal hypertension, bleeding oesophageal varices, ascites, to splenomegaly with hypersplenism. While the quoted clinical experience of 200 patients with CF liver disease might be considered as substantial, it nevertheless appears unjustified to rush from that experience to the statement that severe hypersplenism, requiring a specific surgical approach, is not a feature of the discussed disease spectrum. In the equally substantial clinical experience …