Association of vitamin A status with lung function in children and adolescents with cystic fibrosis.

IMPORTANCE: Vitamin A (or retinol) has considerable antioxidative and anti-inflammatory attributes and it may have protective effects on the respiratory health of patients with cystic fibrosis (CF). This issue, however, remains controversial. OBJECTIVE: The purpose of the present study was to investigate the relationship between serum retinol levels (SRL) and force expiratory volume in 1 second (FEV1) in patients with CF. METHODS: All patients with pancreatic insufficiency attending the CF Department of "Agia Sofia" Children's Hospital in Athens, Greece, aged 6 to 19 years during the 2012-2016 period, who could perform spirometry effectively, were included in the study. The impact of SRL on FEV1 was examined longitudinally and analyzed with generalized estimating equations. RESULTS: The study included 231 patients and 851 SRL measurements were performed. In 25 (3.2%) cases the SRL were below the 5th percentile of reference distribution; none was above the 95th percentile. The analysis showed that SRL was positively correlated with the FEV1 (P < 0.001). INTERPRETATION: In this sample of children and adolescents with CF, vitamin A deficiency was rare. Our results suggest a positive relationship between SRL and FEV1.

Nutritional Status and Quality of Life in Hospitalised Cancer Patients Who Develop Intestinal Failure and Require Parenteral Nutrition: An Observational Study.

(1) Background: Malnutrition in cancer patients impacts quality of life (QoL) and performance status (PS). When oral/enteral nutrition is not possible and patients develop intestinal failure, parenteral nutrition (PN) is indicated. Our aim was to assess nutritional status, QoL, and PS in hospitalised cancer patients recently initiated on PN for intestinal failure. (2) Methods: The design was a cross-sectional observational study. The following information was captured: demographic, anthropometric, biochemical and medical information, as well as nutritional screening tool (NST), patient-generated subjective global assessment (PG-SGA), functional assessment of cancer therapy-general (FACT-G), and Karnofsky PS (KPS) data. (3) Results: Among 85 PN referrals, 30 oncology patients (56.2 years, 56.7% male) were identified. Mean weight (60.3 ± 16.6 kg) corresponded to normal body mass index values (21.0 ± 5.1 kg/m2). However, weight loss was significant in patients with gastrointestinal tumours (p < 0.01). A high malnutrition risk was present in 53.3-56.7% of patients, depending on the screening tool. Patients had impaired QoL (FACT-G: 26.6 ± 9.8) but PS indicated above average capability with independent daily activities (KPS: 60 ± 10). (4) Conclusions: Future research should assess the impact of impaired NS and QoL on clinical outcomes such as survival, with a view to encompassing nutritional and QoL assessment in the management pathway of this patient group.

Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination.

BACKGROUND: The combination of lumacaftor and ivacaftor (LUM/IVA) is a recently approved CFTR modulator treatment for homozygous F508del CF patients. Our study aimed at evaluating the change in the rate of lung function decline after one-year treatment with LUM/IVA. METHODS: The study evaluated patients homozygous for F508del, 12 to 23 years old. All had been treated for one year with LUM/IVA. The collected data included the percent predicted values of FEV1 (ppFEV1) and FVC (ppFVC), and the FEV1/FVC ratio (FEV1/FVC), that corresponded to 12, 24, and 36 months prior to, and 12 months after the initiation of LUM/IVA; also, the 3 highest values of the ppFEV1 (and the corresponding ppFVC, and FEV1/FVC) for the periods 0-12 months, 12-24 months, and 24-36 months prior to as well as the 12-month period after the initiation of LUM/IVA. The baseline lung function was estimated before the commencement of the drug. Data were analyzed longitudinally with generalized estimating equations models and continuous linear splines. A single knot was used that corresponded to the time point of LUM/IVA initiation. RESULTS: Fifty-two patients were analyzed. The multivariate longitudinal analysis of spirometric indices with linear splines demonstrated a significant change in the slopes of ppFEV1 and ppFVC decline, reflecting a significant improvement after the initiation of LUM/IVA treatment. CONCLUSIONS: In this real-world study, lung function improved over a relatively short time period of only one year, after the commencement of LUM/IVA.

Association of vitamin D status with lung function measurements in children and adolescents with cystic fibrosis.

BACKGROUND: Vitamin D status is considered a potential determinant of lung function in cystic fibrosis (CF). The aim of this retrospective longitudinal study was to investigate the decline of spirometric values in association with vitamin D status. METHODS: The data regarding 25-hydroxy vitamin D (25OHD) serum levels, spirometric measurements (FEV1, FVC, FEF25-75%), and factors known to be associated with the decline of lung function in CF were retrospectively collected over a 5-year period. The spirometric indices were recorded as the best and the average value of each year, as well as the value, recorded concurrently or closely with 25OHD level measurement. RESULTS: A significantly positive relationship was observed between 25OHD serum levels and the best annual value of FEV1 (P = .034), and the values of FEV1 (P = .010) and FVC (P = .018) measured concurrently or closely with serum 25OHD levels. The evolution of the best annual value of FEV1 was worse in patients with a mean 5-year value of 25OHD levels less than 20 ng/mL compared with patients with a mean 5-year value of ≥30 ng/mL (P < .001), or ≥20 to <30 (P < .001). There was no significant difference between patients with mean 5-year 25OHD levels ≥30 ng/mL and ≥20 to <30 ng/mL (P = .76). CONCLUSIONS: Vitamin D status is associated with lung function in patients with CF. Levels of 25OHD above 20 ng/mL were associated with higher best annual FEV1.