Year in Review 2020: Multisystemic impact of cystic fibrosis.

Clinical care in cystic fibrosis (CF) has continued to advance over the last several years, particularly with the widespread eligibility and use of highly effective modulator therapy. Improved outcomes and longevity of persons with CF (PwCF) have increased recognition of the multisystem impact of the disease on the daily lives of PwCF. This review will cover a broad array of topics, from diagnosis to multisystem effects related to mental health, endocrine, palliative care, reproductive health, otolaryngology, and cardiac issues. Additionally, worldwide care delivery will be reviewed, demonstrating variation in outcomes based on resources and populations served. This review is part of the CF Year in Review 2020 series, focusing on the multi-system effects of CF. This review focuses on articles from Pediatric Pulmonology but also includes articles published in 2020 from other journals that are of particular interest to clinicians.

Year in review 2020: Nutrition and gastrointestinal disease in cystic fibrosis.

The multisystemic manifestations of cystic fibrosis (CF) involve all parts of the gastrointestinal (GI) system, including the pancreas, intestine, and liver. As providers who care for people with CF, knowledge of the manifestations, treatment, and research related to nutrition and GI disease are important. This review is the last installment of the CF year in review 2020 series, focusing on nutritional, GI, and hepatobiliary articles from Pediatric Pulmonology and other journals of particular interest to clinicians.

Predictors of sleep hypoxemia in children with cystic fibrosis.

OBJECTIVES: To identify the determinants of nocturnal hypoxemia in children with CF using clinical parameters, polysomnography (PSG), and lung function. HYPOTHESIS: Sleep hypoxemia in children with CF is predicted by both apnea hypopnea index (AHI) and percent predicted forced expiratory volume in one second (pFEV1). DESIGN: Retrospective case series. METHODS: Children aged 5-18 years were included based on (i) a diagnosis of CF; and (ii) availability of concurrent PSG and pFEV1 data. The impact of (i) demographic and clinical parameters; and (ii) PSG and pFEV1, on the total sleep time spent with arterial oxygen saturation below 90% (TSpO2 < 90) was measured using regression analysis. P-value <0.05 was considered significant. RESULTS: The mean age was 11.6 years (95% confidence interval: 9.5, 13.1). Twenty of 35 (57%) were boys and the mean body mass index percentile was 42.1 (31.5, 52.6). The most common ethnicity was white (66%). OSA was diagnosed in 50%. Neither demographic predictors nor clinical variables predicted the severity of hypoxemia (R2 = 0.23, P = 0.09). While pFEV1 and PSG variables accounted for significant proportion of the overall variance in TSpO2 < 90 (R2 = 0.53, P < 0.001), pFEV1 was identified as the single best predictor of sleep hypoxemia. A pFEV1 cut-off of 53% indicated a sensitivity of 0.80 and a specificity of 0.87 in predicting sleep hypoxemia. CONCLUSIONS: pFEV1 is the best predictor of sleep hypoxemia in children with CF and referred for PSG. No demographic or clinical predictors of hypoxemia were identified in this population.

Obstructive sleep apnea in the formerly preterm infant: an overlooked diagnosis.

BACKGROUND: Obstructive sleep apnea syndrome (OSA) is a frequent disorder in children. The clinical characteristics of OSA in very young children under 2 years of age, and more particularly, in those born prematurely, and who have respiratory complications such as bronchopulmonary dysplasia (BPD), are not well defined. We therefore retrospectively reviewed our experience in a group of preterm infants with OSAS. METHODS: The records of premature infants with BPD followed in the Pediatric Pulmonary Clinic at the University of Chicago who were diagnosed with OSA from 2004 to 2009 were reviewed and analyzed. RESULTS: Twelve children, eight males, and four females with a mean gestational age of 27 weeks were found to have OSA. Mean age at diagnosis was 19 months. Inability to wean nighttime oxygen, the need to resume oxygen after intercurrent respiratory illness, and snoring were the most common presenting symptoms. The apnea-hypopnea index ranged from 1 to 120/h total sleep time (TST; mean: 29). SpO(2) nadir ranged from 50 to 91%. Despite adenotonsillectomy (AT), all children had persistent sleep disordered breathing. CONCLUSION: In preterm infants, while snoring is a frequent symptom, poor weight gain, and inability to wean nighttime oxygen may indicate the need for further investigation for OSA. In the former preterm infant structural changes in the airway may play an important role along with adenotonsillar hypertrophy. A high level of clinical awareness is required to identify OSA in the formerly preterm infant.