Decline In Spirometry Research Articles

Screening for post-TB lung disease at TB treatment completion: Are symptoms sufficient?

Pulmonary TB survivors face a high burden of post-TB lung disease (PTLD) after TB treatment completion. In this secondary data analysis we investigate the performance of parameters measured at TB treatment completion in predicting morbidity over the subsequent year, to inform programmatic approaches to PTLD screening in low-resource settings. Cohort data from urban Blantyre, Malawi were used to construct regression models for five morbidity outcomes (chronic respiratory symptoms or functional limitation, ongoing health seeking, spirometry decline, self-reported financial impact of TB disease, and death) in the year after PTB treatment, using three modelling approaches: logistic regression; penalised regression with pre-selected predictors; elastic net penalised regression using the full parent dataset. Predictors included demographic, clinical, symptom, spirometry and chest x-ray variables. The predictive performance of models were examined using the area under the receiver-operator curve (ROC AUC) values. Key predictors were identified, and their positive and negative predictive values (NPV) determined. The presence of respiratory symptoms at TB treatment completion was the strongest predictor of morbidity outcomes. TB survivors reporting breathlessness had higher odds of spirometry decline (aOR 20.5, 95%CI:3-199.1), health seeking (aOR 10.2, 2.4-50), and symptoms or functional limitation at 1-year (aOR 16.7, 3.3-133.4). Those reporting activity limitation were more likely to report symptoms or functional limitation at 1-year (aOR 4.2, 1.8-10.3), or severe financial impact of TB disease (aOR2.3, 1.0-5.0). Models were not significantly improved by including spirometry or imaging parameters. ROC AUCs were between 0.65-0.77 for the morbidity outcomes. Activity limitation at treatment completion had a NPV value of 78-98% for adverse outcomes. Our data suggest that whilst challenging to predict the development of post-TB morbidity, the use of symptom screening tools at TB treatment completion to prioritise post-TB care should be explored. We identified little benefit from the additional use of spirometry or CXR imaging.

Single-arm, open-labelled, safety and tolerability of intrabronchial and nebulised bacteriophage treatment in children with cystic fibrosis and Pseudomonas aeruginosa

IntroductionCystic fibrosis (CF) is a multisystem condition that is complicated by recurrent pulmonary infections requiring aggressive antibiotic treatment. This predisposes the patient to complications such as sensorineural hearing loss, renal...

Prevalence of Overweight and Obesity and Their Impact on Spirometry Parameters in Patients with Asthma: A Multicentre, Retrospective Study.

Obesity is a common comorbidity in patients with asthma and has a significant impact on health and prognoses. However, the extent to which overweight and obesity impact asthma, particularly lung function, remains unclear. This study aimed to report on the prevalence of overweight and obesity and assess their impacts on spirometry parameters in asthmatic patients. In this multicentre, retrospective study, we reviewed the demographic data and spirometry results of all adult patients with confirmed diagnoses of asthma who visited the studied hospitals' pulmonary clinics between January 2016 and October 2022. In total, 684 patients with confirmed diagnoses of asthma were included in the final analysis, of whom 74% were female, with a mean ± SD age of 47 ± 16 years. The prevalence of overweight and obesity among patients with asthma was 31.1% and 46.0%, respectively. There was a significant decline in spirometry results in obese patients with asthma compared with patients with healthy weights. Furthermore, body mass index (BMI) was negatively correlated with forced vital capacity (FVC) (L), forced expiratory volume in one second (FEV1), forced expiratory flow at 25-75% (FEF 25-75%) L/s and peak expiratory flow (PEF) L/s (r = -0.22, p < 0.001; r = -0.17, p < 0.001; r = -0.15, p < 0.001; r = -0.12, p < 0.01, respectively). Following adjustments for confounders, a higher BMI was independently associated with lower FVC (B -0.02 [95% CI -0.028, -0.01, p < 0.001] and lower FEV1 (B -0.01 [95% CI -0.01, -0.001, p < 0.05]. Overweight and obesity are highly prevalent in asthma patients, and more importantly, they can reduce lung function, characterised mainly by reduced FEV1 and FVC. These observations highlight the importance of implementing a nonpharmacological approach (i.e., weight loss) as part of the treatment plan for patients with asthma to improve lung function.

Body mass index across adulthood and the development of airflow obstruction and emphysema.

Low body mass index (BMI) is associated with COPD, but temporal relationships between airflow obstruction (AO) development and emphysematous change are unclear. We investigated longitudinal changes in BMI, AO, and lung density throughout adulthood using data from the Framingham Offspring Cohort (FOC). BMI trajectories were modelled throughout adulthood in 4587 FOC participants from Exam 2 (mean age = 44), through Exam 9 (mean age = 71), in AO participants and non-AO participants (AO n = 1036), determined by spirometry, using fractional polynomial growth curves. This process was repeated for low lung density (LLD) and non LLD participants (LLD n = 225) determined by Computed Tomography. Spirometry decline was compared separately between tertiles of BMI in those aged <40years and associations between fat and lean mass (measured using Dual Energy X-ray Absorptiometry, DEXA) and development of AO and LLD were also assessed. Additional analyses were performed with adjustment for smoking volume. The BMI trajectory from 30years of age was visually lower in the AO group than both non-AO smokers (non-<AO-S) and non-AO non-smokers (non-AO-N). Similarly, BMI trajectories were visually lower in participants with LLD throughout adulthood compared to normal lung density smokers and non-smokers. Differences remained after adjustment for smoking volume. The lowest BMI tertile in ages <40years was associated with the steepest subsequent decline in FEV1/FVC ratio in both sexes. Mean BMI is lower throughout adulthood in AO and LLD participants. Lower BMI is associated with a steeper decline in the ratio of FEV1/FVC. These findings suggest body mass may precede and potentially have a role in the development of COPD lung pathophysiology.

Plasma CAF22 Levels as a Useful Predictor of Muscle Health in Patients with Chronic Obstructive Pulmonary Disease.

Skeletal muscle dysfunction and reduced physical capacity are characteristic features of chronic obstructive pulmonary disease (COPD). However, the search for a reliable biomarker to assess muscle health in CODP remains elusive. We analyzed the course of hand-grip strength (HGS) and appendicular skeletal mass index (ASMI) in COPD in relation to spirometry decline and plasma extracellular heat shock protein-72 (eHSP72) and c-terminal fragment of agrin-22 (CAF22) levels. We evaluated male, 62–73 years old patients of COPD (N = 265) and healthy controls (N = 252) at baseline and after 12 and 24 months for plasma biomarkers, spirometry and HGS measurements. HGS declined significantly over time and plasma CAF22, but not eHSP72 levels, had a significant negative association with HGS and ASMI in COPD. Plasma CAF22 also had an association with walking speed and daily steps count in advanced COPD. Lower ASMI was associated with reduced HGS at all time-point. Narrow age-span of the study cohort and exclusion of lower-limb muscles from the analysis are limitations of this study. Taken together, we report that the plasma CAF22 may be a useful tool to assess muscle weakness and atrophy in COPD patients.

Clarifying the Risk of Lung Disease in SZ Alpha-1 Antitrypsin Deficiency.

Rationale: The ZZ genotype of alpha-1 antitrypsin deficiency (AATD) is associated with chronic obstructive pulmonary disease (COPD), even among never-smokers. The SZ genotype is also considered severe; yet, its effect on lung health remains unclear.Objectives: To determine the effect of SZ-AATD on spirometry compared with a normal-risk population and to determine the effect of smoking cessation in this genotype.Methods: We prospectively enrolled 166 related individuals, removing lung index cases to reduce bias, and compared spirometry between 70 SZ and 46 MM/MS individuals (control subjects). The effect of AAT concentrations on outcomes was assessed in 82 SZ individuals (including lung index cases). Subsequently, we analyzed retrospective SZ registry data to determine the effect of smoking cessation on spirometry decline (n = 60) and plasma anti-neutrophil elastase capacity (n = 20).Measurements and Main Results: No difference between SZ and control never-smokers was seen. Ever smoking was associated with a lower FEV1% predicted (-14.3%; P = 0.0092) and a lower FEV1/FVC ratio (-0.075; P = 0.0041) in SZ-AATD. No association was found between AAT concentration and outcomes for SZ-AATD. Longitudinal analysis of 60 SZ individuals demonstrated that COPD at baseline, but not former smoking or AAT concentrations, predicted greater spirometry decline. Finally, anti-neutrophil elastase capacity did not differ between former smokers and never-smokers (P = 0.67).Conclusions: SZ never-smokers demonstrated no increased risk of COPD, regardless of AAT concentration. Smoking interacts with SZ-AATD to significantly increase airflow obstruction. Former smoking alone is not associated with greater spirometry decline in SZ-AATD, suggesting that cessation attenuates the obstructive process. We found no evidence that the putative protective threshold or AAT concentrations predict risk within the SZ genotype, raising further doubts over the need for intravenous AAT augmentation in this cohort.

Molecular phenotyping of rejection-related changes in mucosal biopsies from lung transplants.

Diagnosing lung transplant rejection currently depends on histologic assessment of transbronchial biopsies (TBB) with limited reproducibility and considerable risk of complications. Mucosal biopsies are safer but not histologically interpretable. Microarray-based diagnostic systems for TBBs and other transplants suggest such systems could assess mucosal biopsies as well. We studied 243 mucosal biopsies from the third bronchial bifurcation (3BMBs) collected from seven centers and classified them using unsupervised machine learning algorithms. Using the expression of a set of rejection-associated transcripts annotated in kidneys and validated in hearts and lung transplant TBBs, the algorithms identified and scored major rejection and injury-related phenotypes in 3BMBs without need for labeled training data. No rejection or injury, rejection, late inflammation, and recent injury phenotypes were thus scored in new 3BMBs. The rejection phenotype correlated with IFNG-inducible transcripts, the hallmarks of rejection. Progressive atrophy-related changes reflected by the late inflammation phenotype in 3BMBs suggest widespread time-dependent airway deterioration, which was especially pronounced after two years posttransplant. Thus molecular assessment of 3BMBs can detect rejection in a previously unusable biopsy format with potential utility in patients with severe lung dysfunction where TBB is not possible and provide unique insights into airway deterioration. ClinicalTrials.gov NCT02812290.

Radiographic lung volumes predict progression to COPD in smokers with preserved spirometry in SPIROMICS.

The characteristics that predict progression to overt chronic obstructive pulmonary disease (COPD) in smokers without spirometric airflow obstruction are not clearly defined.We conducted a post hoc analysis of 849 current and former smokers (≥20 pack-years) with preserved spirometry from the Subpopulations and Intermediate Outcome Measures in COPD Study (SPIROMICS) cohort who had baseline computed tomography (CT) scans of lungs and serial spirometry. We examined whether CT-derived lung volumes representing air trapping could predict adverse respiratory outcomes and more rapid decline in spirometry to overt COPD using mixed-effect linear modelling.Among these subjects with normal forced expiratory volume in 1 s (FEV1) to forced vital capacity (FVC) ratio, CT-measured residual volume (RVCT) to total lung capacity (TLCCT) ratio varied widely, from 21% to 59%. Over 2.5±0.7 years of follow-up, subjects with higher RVCT/TLCCT had a greater differential rate of decline in FEV1/FVC; those in the upper RVCT/TLCCT tertile had a 0.66% (95% CI 0.06%-1.27%) faster rate of decline per year compared with those in the lower tertile (p=0.015) regardless of demographics, baseline spirometry, respiratory symptoms score, smoking status (former versus current) or smoking burden (pack-years). Accordingly, subjects with higher RVCT/TLCCT were more likely to develop spirometric COPD (OR 5.7 (95% CI 2.4-13.2) in upper versus lower RVCT/TLCCT tertile; p<0.001). Other CT indices of air trapping showed similar patterns of association with lung function decline; however, when all CT indices of air trapping, emphysema, and airway disease were included in the same model, only RVCT/TLCCT retained its significance.Increased air trapping based on radiographic lung volumes predicts accelerated spirometry decline and progression to COPD in smokers without obstruction.

Late manifestation of alloantibody-associated injury and clinical pulmonary antibody-mediated rejection: Evidence from cell-free DNA analysis

Antibody-mediated rejection (AMR) often progresses to poor health outcomes in lung transplant recipients (LTRs). This, combined with the relatively insensitive clinical tools used for its diagnosis (spirometry, histopathology) led us to determine whether clinical AMR is diagnosed significantly later than its pathologic onset. In this study, we leveraged the high sensitivity of donor-derived cell-free DNA (ddcfDNA), a novel genomic tool, to detect early graft injury after lung transplantation. We adjudicated AMR and acute cellular rejection (ACR) in 157 LTRs using the consensus criteria of the International Society for Heart and Lung Transplantation (ISHLT). We assessed the kinetics of allograft injury in relation to ACR or AMR using both clinical criteria (decline in spirometry from baseline) and molecular criteria (ddcfDNA); percent ddcfDNA was quantitated via shotgun sequencing. We used a mixed-linear model to assess the relationship between and ddcfDNA levels and donor-specific antibodies (DSA) in AMR+ LTRs. Compared with ACR, AMR episodes (n = 42) were associated with significantly greater allograft injury when assessed by both spirometric (0.1 liter vs -0.6 liter, p < 0.01) and molecular (ddcfDNA) analysis (1.1% vs 5.4%, p < 0.001). Allograft injury detected by ddcfDNA preceded clinical AMR diagnosis by a median of 2.8 months. Within the same interval, spirometry or histopathology did not reveal findings of allograft injury or dysfunction. Elevated levels of ddcfDNA before clinical diagnosis of AMR were associated with a concurrent rise in DSA levels. Diagnosis of clinical AMR in LTRs lags behind DSA-associated molecular allograft injury as assessed by ddcfDNA.

조선소 근로자들의 폐쇄성 폐기능 저하와 관련된 요인

Objectives: Alongside smoking, occupational exposure is an important risk factor for chronic obstructive pulmonary disease. The purpose of this study was to evaluate factors associated with occupational pulmonary function decline that can be used to create guidelines for the health management of shipyard workers Materials: This study analyzed spirometry from 10,597 male shipbuilding workers. Functional decline in spirometry was defined as FEV1/FVC <70% and logistic regression for work duration and occupational hazard exposure was performed Results: Among the subjects, 4.2% showed an obstructive pattern in pulmonary function. The odds ratios for hazard exposure were 1.67(indirect) and 3.54(direct), and for work duration 1.97(10-18 years), 2.29(19-27), and 5.02(28+). After adjusting for smoking and work-related factors, the odds ratios for work durations of over 10 years were 1.73(10-18 years), 1.99(19-27), and 4.09(28+), but for hazards exposure was 1.71(direct) alone after adjustment. Conclusions: Occupational COPD is insidious and chronic, and thus long-term hazard exposed(especially over 10 years) shipyard workers with functional decline in spirometry need to prevent and manage COPD. This study is important for establishing guidelines to manage hazard exposure among shipyard workers and prevent COPD.

Post-mortem brain pathology is related to declining respiratory function in community-dwelling older adults.

Damage to brain structures which constitute the distributed neural network that integrates respiratory muscle and pulmonary functions, can impair adequate ventilation and its volitional control. We tested the hypothesis that the level of brain pathology in older adults is associated with declining respiratory function measured during life. 1,409 older adults had annual testing with spirometry (SPI) and respiratory muscle strength (RMS) based on maximal inspiratory and maximal expiratory pressures (MEPs). Those who died underwent structured brain autopsy. On average, during 5 years of follow-up, SPI and RMS showed progressive decline which was moderately correlated (ρ = 0.57, p < 0.001). Among decedents (N = 447), indices of brain neuropathologies showed differential associations with declining SPI and RMS. Nigral neuronal loss was associated with the person-specific decline in SPI (Estimate, −0.016 unit/year, S.E. 0.006, p = 0.009) and reduction of the slope variance was equal to 4%. By contrast, Alzheimer’s disease (AD) pathology (Estimate, −0.030 unit/year, S.E. 0.009, p < 0.001) and macroscopic infarcts (−0.033 unit/year, S.E., 0.011, p = 0.003) were associated with the person-specific decline in RMS and reduction of the slope variance was equal to 7%. These results suggest that brain pathology is associated with the rate of declining respiratory function in older adults.

Longitudinal Forced Vital Capacity Monitoring as a Prognostic Adjunct after Lung Transplantation.

After lung transplantation, spirometric values are routinely followed to assess graft function. FEV1 is used to characterize chronic allograft dysfunction, whereas the course of FVC change has been less acknowledged and rarely used. To better understand the temporal relationship and prognostic ability of FEV1 and FVC decline after lung transplantation. Serial FEV1 and FVC values were studied among 205 bilateral lung transplant recipients. Different decline patterns were characterized and evaluated for prognostic value via restricted mean modeling of mortality and times to other pertinent events. Baseline FEV1 was achieved earlier than baseline FVC (median, 296 vs. 378 d; P < 0.0001). Decline in FEV1 or FVC from their respective post-transplant baselines occurred in 85 patients (41%). Fifty-nine of 85 (69%) had an isolated FEV1 decline, with 80% later meeting the FVC decline criterion. This subsequent FVC decline was associated with worsening FEV1 and lower median survival. Twenty-five of 85 patients (29%) demonstrated concurrent FEV1 and FVC decline. Patients with concurrent decline had higher 1- and 5-year mortality rates (1-yr, 53% vs. 18%, P < 0.0001; 5-yr, 61% vs. 48%, P = 0.001). These patients were more likely to have rapid-onset of spirometry decline (P = 0.05) and lower FEV1% predicted (P = 0.04) at presentation. FVC decline from its post-transplant baseline provides valuable prognostic information. Concurrent FEV1 and FVC decline identifies patients with fulminant, rapid deterioration and is the strongest clinical predictor of poor survival. Subsequent FVC decline in patients with an initial isolated FEV1 decline identifies disease progression and portends poor prognosis.

Oxidative Stress Correleates with Survival in Lung Transplantation.

Introduction: Oxidative stress plays a key role in balancing of innate and adaptive immunity in pulmonary inflammation. The roles of two major endogenous thiols and antioxidants, Cys (Cysteine) and GSH (Glutathione), in HIV infections and other diseases have been associated with wasting and immunological dysfunction. We hypothesized that increased levels of oxidative stress as measured directly in lung lavage, and assessed by lung macrophage transcriptional profiling would correlate with poorer outcomes in lung transplant patients. Methods:. During 2007 to 2012 cohort of 86 lung transplant patients were followed to identify predictors using death and graft function as primary outcome. 23 (27%) patients died and 96% lung transplant patients manifest at least one decline in spirometry. BAL from these patients (344 total samples, mean 4 samples per patient) was analyzed by HPLC. The oxidative stress markers GSH, GSSG, CyS, CySS, GSH/CySS, GSSG/CyS and GSH/CyS were measured from each BAL sample. Macrophage expression profiling was performed by RT-PCR on BAL macrophages recovered during BAL after brief adherence to plastic. Our preliminary work had shown a strong correlation of the oxidative stress induced gene prostaglandin-endoperoxide synthase 1 (PTGS1) to levels of oxidized cysteine and glutathione. Hence PTGS1 expression was chosen as a measure for this study. Results: Our analysis identified Glutathione disulfide (GssG) (p=0.0489; HR=1.133) as a predictor of death in this cohort. The other markers of oxidative stress showed weak non-statistically significant correlation with survival. In support of the finding that oxidative stress correlates to outcome we found PTGS1 gene expression in lung macrophages was also associated with death (p=0.0463; HR=2.217). Despite these survival associations, neither PTGS1 of GssG longitudinal measures correleated with lung spirometry. Conculsion: We found that oxidative stress as measured by GssG levels significantly correlated with a risk of death in our cohort. That PTGS 1 gene expression was also increased raises the possibility that oxidative stress results in skewing of lung macrophage polarity. Based on these findings we are assessing the relationship between oxidative stress and macrophage function in a prospective study.

Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach

BackgroundIntravenous augmentation therapy is the only specific treatment available for emphysema associated with alpha-1 antitrypsin deficiency. Despite large observational studies and limited interventional studies there remains controversy about the efficacy of this treatment due to the impracticality of conducting adequately powered studies to evaluate the rate of decline in lung function, due to the low prevalence and the slow progression of the disease. However, measurement of lung density by computed tomography is a more specific and sensitive marker of the evolution of emphysema and two small placebo-controlled clinical trials have provided evidence supporting a reduction in the rate of decline in lung density with augmentation therapy.The problemWhere augmentation therapy has become available there has been little consideration of a structured approach to therapy which is often introduced on the basis of functional impairment at diagnosis. Data from registries have shown a great variability in the evolution of lung disease according to patient acquisition and the presence of recognised risk factors. Avoidance of risk factors may, in many cases, stabilise the disease. Since augmentation therapy itself will at best preserve the presenting level of lung damage yet require intravenous administration for life with associated costs, identification of patients at risk of continued rapid or long term progression is essential to select those for whom this treatment can be most appropriate and hence generally more cost-effective. This represents a major reconsideration of the current practice in order to develop a consistent approach to management world wide.Purpose of this reviewThe current review assesses the evidence for efficacy of augmentation therapy and considers how the combination of age, physiological impairment, exacerbation history and rate of decline in spirometry and other measures of emphysema may be used to improve therapeutic decision making, until a reliable predictive biomarker of the evolution of lung impairment can be identified. In addition, individual pharmacokinetic studies may permit the selection of the best regimen of administration for those who need it.SummaryThe rarity and variable characteristics of the disease imply the need for an individualised approach to therapy in specialised centres with sufficient experience to apply a systematic approach to monitoring and management.

Interleukin-2 Causes a Decline in Spirometry

Interleukin-2 Causes a Decline in Spirometry

Do CT Findings Correlate With the Phenomena of “BOS” and “DeBOS/ReBOS” in Lung Transplant Patients?

PURPOSE: A physiologic decline in spirometry meeting criteria for bronchiolitis obliterans syndrome (BOS) is sometimes transient. We sought to evaluate long term consequences of transient BOS physiology, and correlate CT findings.

Chest computed tomography scores of severity are associated with future lung disease progression in children with cystic fibrosis.

Most children with cystic fibrosis (CF) experience a slow decline in spirometry, although some children continue to be at risk for more significant lung disease progression. Chest computed tomography (CT) scans have been shown to be more sensitive to changes in lung disease than spirometry and may provide a means for predicting future lung disease progression. We hypothesized that Brody chest CT scan scores obtained in 2000 in a prospectively monitored cohort of children with CF would be associated with the most recent measures of lung disease severity. Brody chest CT scan scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. Multivariable linear regression was used to determine associations between Brody scores and the most recent (age 21 yr or June 30, 2010, whichever was later) measures of CF lung disease. The mean observation time after the chest CT scan was 7.5 years. Brody chest CT scan scores were significantly associated with the most recent measures of spirometry (P < 0.001) and Wisconsin and Brasfield chest radiograph scores (P < 0.001). The strength of this association was much stronger than spirometry obtained near the time of the chest CT scan (P < 0.01) but not chest radiograph scores. Chest CT scan scores are associated with future lung disease severity, and quantitative chest imaging(chest CT scan and chest radiograph scores) is more strongly associated with future lung disease severity than measures of spirometry. These findings may help clinicians identify patients at risk of future lung disease progression.

P105 Burkholderia latens—a new infection in CF patients

BackgroundBurkholderia latens was first recognised as a novel species of the Burkholderia cepacia complex (Bcc) in 2008. To date, there are no reports of pulmonary infection. Here, we report a...

Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?

It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS).We collected annual lung function data between the ages of 8-15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2-15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period.Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters.We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF.

Cystic fibrosis and pregnancy.

The case records of 11 patients with cystic fibrosis (CF) who had 13 completed pregnancies between 1975 and 1995 were retrospectively reviewed to assess: (1) the changes in spirometry and body mass index (BMI) during pregnancy; and (2) maternal and neonatal complications and outcomes. Prepregnancy the mean age of the group was 24 (range 17-27) years. Two patients were exsmokers, 7 had pancreatic insufficiency and 7 had chest X-ray evidence of bronchiectasis. None of the patients had diabetes mellitus but 3 developed gestational diabetes. The mean +/- SEM (% predicted) forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) prepregnancy were 2.3 +/- 1.0 (83%) litres and 3.0 +/- 0.9 (85%) litres respectively. Five patients had normal spirometry (FEV1 and FVC >80% predicted) prior to 6 pregnancies. The mean body mass index (kg/height(m)2) for the group was 20.5 +/- 2.0. There was a significant decline in spirometry during pregnancy (FEV1 15.5 +/- 6.6% p<0.01; FVC 14.0 +/- 8.3% p<0.5). However, FVC but not FEV1 recovered to prepregnancy values by 12 months postpartum. There was a significant increase in both weight (7.1 kg) and BMI (2.6 kg/height(m)2) at the time of delivery compared with prepregnancy (p=0.0004). However, postpregnancy both weight and BMI had returned to their prepregnancy values (p<0.2). Mothers with an FEV1>80% had less decline in FEV1 related to pregnancy, better outcomes, fewer operative and instrumental deliveries, fewer preterm infants and fewer neonatal complications. Suggestions for the planning and management of pregnancy in women with CF are discussed.