Influences of Two FEV1 Reference Equations (GLI-2012 and GIRH-2017) on Airflow Limitation Classification Among COPD Patients.

Objective: To explore the clinical effects of different forced expiratory volume in 1s (FEV1) reference equations on chronic obstructive pulmonary disease (COPD) airflow limitation (AFL) classification. Methods: We conducted a COPD screening program for residents over 40 years old from 2019 to 2021. All residents received the COPD screening questionnaire (COPD-SQ) and spirometry. Postbronchodilator FEV1/FVC (forced vital capacity) <0.7 was used as the diagnostic criterion of COPD and two reference equations of FEV1 predicted values were used for AFL severity classification: the European Respiratory Society Global Lung Function Initiative reference equation in 2012 (GLI-2012) and the Guangzhou Institute of Respiratory Health reference equation in 2017 (GIRH-2017). Clinical characteristics of patients in GOLD (Global Initiative for Chronic Obstructive Pulmonary Disease) 1-4 grades classified by the two reference equations were compared. Results: Among 3524 participants, 659 subjects obtained a COPD-SQ score of 16 or more and 743 participants were found to have AFL. The COPD-SQ showed high sensitivity (59%) and specificity (91%) in primary COPD screening. Great differences in COPD severity classification were found when applying the two equations (p < 0.001). Compared with GIRH-2017, patients with AFL classified by GLI-2012 equations were significantly severer. The relationship between symptom scores, acute exacerbation (AE) history distributions and COPD severities classified by the two equations showed a consistent trend of positive but weak correlation. Group A, B, C and D existed in all GOLD 1 to 3 COPD patients, but in GOLD 4, only Groups B and D existed. However, no clear significant differences were found in symptoms, AE risk assessments, risk factors exposure and even the combined ABCD grouping under the two equations. Conclusion: There were significant differences in COPD AFL severity classification with GLI-2012 and GIRH-2017 FEV1 reference equations. But these severity estimation differences did not affect symptoms, AE risk assessments and ABCD grouping of patients at all GOLD grades.

Non-fasting bioelectrical impedance analysis in cystic fibrosis: Implications for clinical practice and research.

BACKGROUND: Nutritional status affects pulmonary function in cystic fibrosis (CF) patients and can be monitored by using bioelectrical impedance analysis (BIA). BIA measurements are commonly performed in the fasting state, which is burdensome for patients. We investigated whether fasting is necessary for clinical practice and research. METHODS: Fat free mass (FFM) and fat mass (FM) were determined in adult CF patients (n = 84) by whole body single frequency BIA (Bodystat 500) in a fasting and non-fasting state. Fasting and non-fasting BIA outcomes were compared with Bland-Altman plots. Pulmonary function was expressed as Forced Expiratory Volume at 1 s percentage predicted (FEV1%pred). Comparability of the associations between fasting and non-fasting body composition measurements with FEV1%pred was assessed by multiple linear regression. RESULTS: Fasting FFM, its index (FFMI), and phase angle were significantly lower than non-fasting estimates (-0.23 kg, p = 0.006, -0.07 kg/m2, p = 0.002, -0.10°, p = 0.000, respectively). Fasting FM and its index (FMI) were significantly higher than non-fasting estimates (0.22 kg, p = 0.008) 0.32%, p = 0.005, and 0.07 kg/m2, (p = 0.005). Differences between fasting and non-fasting FFM and FM were <1 kg in 86% of the patients. FFMI percentile estimates remained similar in 83% of the patients when measured after nutritional intake. Fasting and non-fasting FFMI showed similar associations with FEV1%pred (ß: 4.3%, 95% CL: 0.98, 7.70 and ß: 4.6%, 95% CI: 1.22, 8.00, respectively). CONCLUSION: Differences between fasting and non-fasting FFM and FM were not clinically relevant, and associations with pulmonary function remained similar. Therefore, BIA measurements can be performed in a non-fasting state.

The characterisation of pulmonary function in patients with mucopolysaccharidoses IVA: A longitudinal analysis.

INTRODUCTION: Mucopolysaccharidosis (MPS) type IVA is a rare, autosomal recessive lysosomal storage disease causing substrate accumulation in various organs and tissues. MPS IVA is associated with both obstructive and restrictive airway disease, with the former often resulting in sleep disordered breathing (SDB). Respiratory failure is a primary cause of death in this condition. The aim of this study was to characterise and catalogue the long-term respiratory changes in patients with MPS IVA treated with, or without, enzyme replacement therapy (ERT). METHODS: In this retrospective, longitudinal, repeated-measures cohort study, descriptive statistics and non-parametric correlation were performed for demographic, respiratory function and oximetry variables over a study period from January 2009 to December 2018. Composite clinical endpoints used in this study for evaluating pulmonary function included spirometry variables (FEV1, FEV1 [%Pred] FVC, FVC [%Pred] and FEV1/FVC), oximetry variables (median %Spo2, ODI 3%, mean nadir 3%, ODI 4%, mean nadir 4% and min dip SpO2 [%]) and 6MWT to assess functional exercise capacity and thus integrated cardiopulmonary function. RESULTS: Sequential spirometry and oximetry values were collected from 16 patients, of which 13/16 were ERT treated. In general, during the study period there was a global reduction in static spirometry values in all subjects, as well as cardiorespiratory function as assessed by the 6MWT, with the decline being delayed in the ERT group. Oximetry changed to a minor degree over time in the ERT group, whereas it declined in the non-ERT group. FEV1, FVC [%predicted] and ODI 3% exhibited a strong, combined positive correlation (r 0.74-95% CI 0.61 to 0.83; p < .0001). Non-invasive ventilation (NIV) and adenotonsillectomy appeared more effective in the ERT group, either improving pulmonary function or attenuating deterioration. CONCLUSIONS: Whilst spirometry values showed a gradual decline across all groups, oximetry showed modest improvement in respiratory function. The amalgamation of FEV1, FVC [%predicted] and ODI 3% appeared predictive of changes in respiratory function in this study, suggestive as being composite endpoints for monitoring disease progression as well as guiding response to ERT in MPS IVA patients.

Individualized vitamin A supplementation for patients with cystic fibrosis.

BACKGROUND & AIMS: To determine the vitamin A status and appropriate supplementation dosage of cystic fibrosis (CF) patients who received vitamin A supplementation based on annual serum retinol measurements. METHODS: Vitamin A food intake, supplementation dosage, and serum retinol levels were obtained for 32 CF patients >4 years of age (4.3-27.3 years old) who had pancreatic insufficiency and mild-to-moderate lung disease (percent predicted of forced expiratory volume in 1 s > 40%). These measurements were compared with the dietary reference intake for healthy children and adults (D-A-CH dietary recommendations), US and German CF recommendations, and serum retinol concentrations from National Health and Nutrition Examination Survey (NHANES) data. RESULTS: Total vitamin A intake from food and supplementation was 315% ± 182% of D-A-CH recommendations, with 65% from supplements. The range of the prescribed vitamin A supplementation dosage was 0-20,000 IU/day (median 5500 IU), and it was consistent with CF recommendations in 28% of participants. A quarter of all patients did not need any vitamin A supplementation. The total vitamin A intake exceeded the recommended upper limit of intake in 69% of subjects. The mean (range) serum retinol was 38.6 µg/dl (22.1-59.1 µg/dl). All subjects had serum retinol levels above 20 µg/dl and below 72 µg/dl (95th percentile of NHANES reference range). CONCLUSION: Individualized vitamin A supplementation of 0-20,000 IU/day based on annual serum retinol measurements may prevent deficiency and high serum retinol levels, but it may lead to vitamin A intake above the tolerable upper intake level.