Comparison of the Lung Clearance Index in Preschool Children With Primary Ciliary Dyskinesia and Cystic Fibrosis.

BACKGROUND: Previous studies showed that the lung clearance index (LCI) determined by multiple-breath washout (MBW) is sensitive to detecting early lung disease in preschool children with cystic fibrosis (CF). In preschool children with primary ciliary dyskinesia (PCD), data on the onset and severity of lung disease and on the sensitivity of the LCI as a noninvasive quantitative outcome measure remain limited. RESEARCH AND STUDY QUESTION: Is MBW feasible and sensitive to detect ventilation inhomogeneity in preschool children with PCD? STUDY DESIGN AND METHODS: This was a prospective, cross-sectional, multicenter study and included preschoolers with PCD, preschoolers with CF, and healthy control (HC) participants. LCI was determined using nitrogen MBW and was compared among the three groups. RESULTS: LCI was determined in 27 children with PCD, 34 children with CF, and 30 HC participants (mean age, 4.8 years; range, 2.2-6.9 years). The LCI in preschool children with PCD was increased (median, 9.1; 95% CI, 8.6-10.3) compared with HC participants (median, 7.0; 95% CI, 6.7-7.1; P < .0001), but did not differ from preschool children with CF (median, 8.6; 95% CI, 8.4-9.7; P = .71). The feasibility in the PCD group was 93.1% and was similar to that in the CF group (91.9%) and in HC participants (85.7%; P = .55). INTERPRETATION: This study demonstrated early onset of lung disease in preschool children with PCD and indicated that lung disease severity in PCD may be similar to that in CF during preschool years. These data support a need for early diagnostic monitoring and therapy and suggest the LCI as a noninvasive diagnostic tool and as a potential end point in clinical trials testing early interventions in children with PCD.

Tracheobronchial anomalies and stenoses: detection with low-dose multidetector CT with virtual tracheobronchoscopy--comparison with flexible tracheobronchoscopy.

PURPOSE: To prospectively assess the sensitivity and specificity of low-dose multidetector computed tomography (CT) with virtual tracheobronchoscopy (VT) for evaluation of suspected airway stenoses and/or abnormalities by using flexible tracheobronchoscopy (FT) as the reference standard. MATERIALS AND METHODS: The study was approved by the local ethics committee; parental consent was obtained. Forty-five patients with clinically and/or radiographically suspected tracheobronchial stenosis and/or anomaly underwent FT and contrast material-enhanced single-phase multidetector CT with VT. CT was performed with an age- and weight-adjusted low-dose protocol: 120 or 80 kV; 120 or 60 mA; collimation, 1.5 or 0.75 mm; gantry rotation, 0.5 second. Mean effective dose was calculated for all examinations. Postprocessing was performed with surface rendering of VT images and multiplanar reformations. CT images were analyzed in consensus by two radiologists who were blinded to FT results. Statistical analysis was performed with 2 x 2 contingency tables; 95% confidence intervals (CIs) were calculated with the Blyth-Still-Casella procedure. RESULTS: Mean patient age was 4.4 years (range, 2 months to 16 years; 53% male patients). Tracheobronchial narrowing and/or abnormality were depicted at FT in 38 of 45 patients. In 33 of 38 patients, multidetector CT with VT depicted a tracheobronchial narrowing and/or anomaly. In 10 of 38 patients, tracheobronchial stenosis was induced by vascular anomalies. Five patients with normal findings at multidetector CT with VT had tracheobronchomalacia with inspiratory airway stenosis at FT. Sensitivity and specificity of CT with VT were 86.8% (95% CI: 73.3%, 94.7%) and 85.7% (95% CI: 44.6%, 99.3%), respectively. Positive and negative predictive values were 97.1% (95% CI: 84.9%, 99.9%) and 54.5% (95% CI: 25.0%, 80.0%), respectively. Overall accuracy was 86.7% (95% CI: 74.3%, 94.0%). Mean effective dose was 1.1 mSv (range, 0.5-1.8 mSv). CONCLUSION: Multidetector CT with VT with a low-dose protocol had high sensitivity and specificity for depiction of tracheobronchial narrowings and/or anomalies. However, tracheal narrowing due to tracheobronchomalacia was difficult to diagnose at single-phase multidetector CT with VT.

Evaluation of clinical, radiologic, and laboratory prebronchoscopy findings in children with suspected foreign body aspiration.

BACKGROUND: Foreign body aspiration (FBA) in infants and young children is a common and potentially life-threatening event. Although studies have extensively described the signs and symptoms of suspected FBA (sFBA), only few systematically compared their value for predicting bronchoscopy results. OBJECTIVES: The objectives of this study were to describe the clinical and radiologic signs and symptoms of sFBA and to identify predictors of bronchoscopically proven FBA (pFBA). SETTING: This study was conducted at a referral tertiary university hospital with an outpatient clinic and a 90-bed pediatric unit. METHODS: Signs and symptoms were retrospectively analyzed for all children who had received bronchoscopy between July 1992 and April 2000 because of sFBA. Radiologic signs of FBA were reviewed and scored by 2 independent radiologists. RESULTS: One hundred sixty children (mean age, 2.8 years; range, 11 months to 16.8 years) were enrolled in the study. Foreign body aspiration, mostly affecting the right main bronchus, was proven bronchoscopically in 122 (76%) of these children. Independent predictors of pFBA in multivariable analyses were focal hyperinflation on chest radiograph (beta = 45.4; 95% confidence interval [CI] = 5.3-390.5; P = .001), witnessed choking crisis (beta = 18.6; 95% CI = 4.7-73.0; P < .001), and white blood cell count greater than 10,000/muL (beta = 4.2; 95% CI = 1.2-14.7; P = .026). The cumulative proportion of pFBA cases increased with the number of risk factors (0, 16%; 1, 47%; 2, 96%; 3, 100%). CONCLUSIONS: Clinical judgment to perform bronchoscopy for sFBA was correct in 76% of the children investigated. Focal hyperinflation, witnessed choking crisis, and elevated white blood cell count were strongly associated with pFBA; bronchoscopy can be strongly recommended in the presence of at least 2 risk factors when FBA is suspected.

Pulmonary haemosiderosis in infants and children.

Pulmonary haemosiderosis (PH) results from recurrent bleeding into alveolar spaces and interstitial lung tissue. If untreated, fibrosis and restrictive lung disease will develop and may lead to death. A distinction can be made between primary and secondary pulmonary haemosiderosis as a manifestation of auto-immune small vessel vasculitides, coagulation disorders or diseases with increased venous pressure. Interestingly, in some cases of 'idiopathic' PH, exposure to moulds and van Willebrand's disease may contribute to the pathogenesis. Haemosiderosis usually begins with haematemesis. Anaemia, alveolar infiltrates on chest x ray and siderophages in broncho-alveolar lavage fluid are diagnostic factors. Immunosuppressive treatment has improved the prognosis in recent years.

Evaluation of chronic infectious interstitial pulmonary disease in children by low-dose CT-guided transthoracic lung biopsy.

BACKGROUND: Children with chronic infectious interstitial lung disease often have to undergo open lung biopsy to establish a final diagnosis. Open lung biopsy is an invasive procedure with major potential complications. Transthoracic lung biopsy (TLB) guided by computed tomography (CT) is a less-invasive well-established procedure in adults. OBJECTIVE: Detailing the role of low-dose CT-guided TLB in the enhanced diagnosis of chronic lung diseases related to infection in children. MATERIALS AND METHODS: A group of 11 children (age 8 months to 16 years) underwent CT-guided TLB with a 20-gauge biopsy device. All investigations were done under general anaesthesia on a multidetector CT scanner (SOMATOM Volume Zoom, Siemens, Erlangen, Germany) using a low-dose protocol (single slices, 120 kV, 20 mAs). Specimens were processed by histopathological, bacteriological, and virological techniques. RESULTS: All biopsies were performed without major complications; one child developed a small pneumothorax that resolved spontaneously. A diagnosis could be obtained in 10 of the 11 patients. Biopsy specimens revealed chronic interstitial alveolitis in ten patients. In five patients Chlamydia pneumoniae PCR was positive, in three Mycoplasma pneumoniae PCR was positive, and in two Cytomegalovirus PCR was positive. The average effective dose was 0.83 mSv. CONCLUSION: Low-dose CT-guided TLB can be a helpful tool in investigating chronic infectious inflammatory processes in children with minimal radiation exposure. It should be considered prior to any open surgical procedure performed for biopsy alone. In our patient group no significant complication occurred. A disadvantage of the method is that it does not allow smaller airways and vessels to be assessed.