Pulmonary Function Test Abnormalities in Pediatric Inflammatory Bowel Disease.

BACKGROUND: Pulmonary involvement in adult patients with inflammatory bowel disease (IBD) seems more common than previously appreciated. Its prevalence and development over time in pediatric IBD patients are largely unknown. OBJECTIVES: The aim was to study lung function including fraction of exhaled nitric oxide (FeNO) and transfer capacity for carbon monoxide (TLCO) in pediatric IBD patients and to describe the longitudinal development in a subset of patients with lung function abnormalities. METHODS: Sixty-six measurements were made in 48 IBD patients (30 patients with Crohn's disease and 18 with ulcerative colitis) and 108 matched controls. Patients with abnormal TLCO or elevated residual volume/total lung capacity (RV/TLC) ratios were invited for a follow-up. Statistical comparisons were made by nonparametric tests and ANOVA. RESULTS: TLCO was decreased in IBD patients [median: 88% predicted (interquartile range, IQR, 22) vs. 99% predicted (IQR 19) in controls]. RV/TLC ratios were mildly elevated in patients with ulcerative colitis [32% (IQR 9) vs. 27% (IQR 8) in controls], and maximum expiratory flows at 50 and 25% of vital capacity were mildly reduced in patients with Crohn's disease. FeNO and disease activity did not correlate with lung function abnormalities. Abnormalities did not consistently persist over a median follow-up period of 34 months. CONCLUSIONS: This study supports evidence that variable and fluctuating pulmonary involvement also occurs in pediatric IBD patients. Its clinical significance is unclear.

Cystic Fibrosis Newborn Screening in Austria Using PAP and the Numeric Product of PAP and IRT Concentrations as Second-Tier Parameters.

In Austria, newborns have been screened for cystic fibrosis (CF) by analyzing immunoreactive trypsinogen (IRT) from dried blood spots (DBS)s for nearly 20 years. Recently, pancreatitis-associated protein (PAP) analysis was introduced as a second-tier test with the aim of reducing recalls for second DBS cards while keeping sensitivity high. For 28 months, when IRT was elevated (65-130 ng/mL), PAP was measured from the first DBS (n = 198,927) with a two-step cut-off applied. For the last 12 months of the observation period (n = 85,421), an additional IRT×PAP cut-off was introduced. If PAP or IRT×PAP were above cut-off, a second card was analyzed for IRT and in case of elevated values identified as screen-positive. Above 130 ng/mL IRT in the first DBS, newborns were classified as screen-positive. IRT analysis of first DBS resulted in 1961 (1%) tests for PAP. In the first 16 months, 26 of 93 screen-positive were confirmed to have CF. Two false-negatives have been reported (sensitivity = 92.8%). Importantly, less than 30% of families compared to the previous IRT-IRT screening scheme had to be contacted causing distress. Adding IRT×PAP caused a marginally increased number of second cards and sweat tests to be requested during this period (15 and 3, respectively) compared to the initial IRT-PAP scheme. One case of confirmed CF was found due to IRT×PAP, demonstrating an increase in sensitivity. Thus, the relatively simple and economical algorithm presented here performs effectively and may be a useful model for inclusion of CF into NBS panels or modification of existing schemes.

The pulmonary outcome of long-term survivors after congenital diaphragmatic hernia repair.

BACKGROUND: Congenital diaphragmatic hernia (CDH) represents a chronic condition with significant pulmonary and non-pulmonary complications. The main aim of the present study was to determine the pulmonary outcome in a group of long-term survivors of CDH. METHODS: Clinical records of 46 patients with CDH admitted to the University Children's Hospital Zurich between 1991 and 2001 were reviewed retrospectively. Survivors underwent clinical examination, lung function tests and measurements of exhaled nitric oxide. RESULTS: 30 of 46 (65%) patients survived after repair of CDH and 19 children participated in a follow-up study at the mean age of 7.9 (2.8) years. At least one wheezy episode requiring inhaled bronchodilators was reported by 9/19 (47%) children and 4/19 (21%) children complained of recurrent wheezy episodes. Nine children showed lung function impairment in spirometry as well as in oscillatory resistance. Neither duration of assisted ventilation nor the length of hospitalisation appeared to correlate with lung function. Exhaled nitric oxide was within normal range in our group of CDH survivors. Measurement of respiratory system resistance using a forced oscillation technique detected those CDH survivors, who showed abnormal pattern in spirometry. However, no correlation between oscillatory resistance and specific airway resistance measured by whole body plethysmography was found. CONCLUSIONS: Despite the presence of rather insignificant symptoms, we found mild to moderate pulmonary functional impairment in children surviving CDH repair.

Correlation of spirometry and symptom scores in childhood asthma and the usefulness of curvature assessment in expiratory flow-volume curves.

BACKGROUND: Spirometry, and in particular forced expiratory volume in the first second (FEV(1)), are standard tools for objective evaluation of asthma. However, FEV(1) does not correlate with symptom scores, and hence its value in the assessment of childhood asthma may be limited. Therefore, some clinicians subjectively assess the presence of curvature in the maximum expiratory flow-volume (MEFV) curves obtained from spirometry, where concave patterns are observable despite normal FEV(1) values. OBJECTIVE: To evaluate the usefulness of subjective and objective measures of the curvature in the descending phase of the MEFV curve for the assessment of asthma. METHODS: We obtained symptom scores and performed spirometry in 48 patients with asthma (21 females, mean +/- SD age 10.8 +/- 2.4 y). We measured FEV(1), the ratio of FEV(1) to forced vital capacity (FEV(1)/FVC), maximum expiratory flow at one quarter of the way, and at halfway, through the forced expiratory maneuver (MEF(25) and MEF(50), respectively), and maximum expiratory flow in the middle half of the forced expiratory maneuver (MEF(25-75)). Expiratory obstruction was ranked independently by 3 pediatric pulmonologists, by subjective assessment of the MEFV curve. In addition, the curvature of the descending limb of the MEFV curve was quantitatively estimated by introducing an "average curvature index." RESULTS: No significant correlations were found between FEV(1), MEF(50), MEF(25), and MEF(25-75,) respectively, and symptom score (r = -0.22, p = 0.14; r = -0.23, p = 0.11; r = -0.28, p = 0.057; r = -0.27, p = 0.06). A weak correlation was found for FEV(1)/FVC and symptom score (r = -0.33, p = 0.021). However, quantitatively determined average curvature index (ACI) correlated significantly better with measured symptom scores (r = 0.53, p < 0.001) and were in good agreement with the assessment of expiratory obstruction from subjective curvature assessment. CONCLUSIONS: Our general findings show that individual lung function variables do not correlate well with symptoms, whereas subjective curvature assessment is thought to be helpful. With the average curvature index we have illustrated a potential clinical usefulness of quantifying the curvatures of MEFV curves.