Mol Cell Biochem. 2021 Sep 16. doi: 10.1007/s11010-021-04263-6. Online ahead of print.

ABSTRACT

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, responsible for the synthesis of the CFTR protein, a chloride channel. The gene has approximately 2000 known mutations and all of them affect in some degree the protein function, which makes the pathophysiological manifestations to be multisystemic, mainly affecting the respiratory, gastrointestinal, endocrine, and reproductive tracts. Currently, the treatment of the disease is restricted to controlling symptoms and, more recently, a group of drugs that act directly on the defective protein, known as CFTR modulators, was developed. However, their high cost and difficult access mean that their use is still very restricted. It is important to search for safe and low-cost alternative therapies for CF and, in this context, natural compounds and, mainly, caffeic acid phenethyl ester (CAPE) appear as promising strategies to assist in the treatment of the disease. CAPE is a compound derived from propolis extracts that has antioxidant and anti-inflammatory activities, covering important aspects of the pathophysiology of CF, which points to the possible benefit of its use in the disease treatment. To date, no studies have effectively tested CAPE for CF and, therefore, we intend with this review to elucidate the role of inflammation and oxidative stress for tissue damage seen in CF, associating them with CAPE actions and its pharmacologically active derivatives. In this way, we offer a theoretical basis for conducting preclinical and clinical studies relating the use of this molecule to CF.

PMID:34529223 | DOI:10.1007/s11010-021-04263-6

Clin Pediatr (Phila). 2021 Sep 16:99228211044567. doi: 10.1177/00099228211044567. Online ahead of print.

NO ABSTRACT

PMID:34528464 | DOI:10.1177/00099228211044567

J Appl Physiol (1985). 2021 Sep 16. doi: 10.1152/japplphysiol.00581.2021. Online ahead of print.

NO ABSTRACT

PMID:34528460 | DOI:10.1152/japplphysiol.00581.2021

ERJ Open Res. 2021 Sep 13;7(3):00365-2021. doi: 10.1183/23120541.00365-2021. eCollection 2021 Jul.

ABSTRACT

Respiratory syncytial virus (RSV) infections in early life predispose children with cystic fibrosis (CF) to more severe lung function decline in later life. The mechanisms explaining the associations between RSV and progression of CF lung disease are not clear. In this study, a human bronchial epithelial cell line and primary human nasal epithelial cells (PNECs) from individuals with CF and healthy control donors were infected with RSV. Real-time PCR, plaque assay, cytokine detection, immunofluorescence and Western blot analyses were performed. RSV is replicated to a higher degree in CF epithelial cells as compared to control cells; however, no defects in innate immune pathways were identified in CF cells. Rather, primary p.Phe508del cystic fibrosis transmembrane conductance regulator PNECs produced more cytokines after RSV infection than control cells. Moreover, interleukin-8 and tumour necrosis factor-α production post RSV negatively correlated with lung function (% predicted forced expiratory volume in 1 s) in the individuals who donated the cells. These data suggest that CF epithelium has a dysfunctional response to RSV allowing for enhanced viral replication and an exaggerated inflammatory response that ultimately may predispose to greater airway inflammation and reduced lung function.

PMID:34527729 | PMC:PMC8435810 | DOI:10.1183/23120541.00365-2021

Oxf Med Case Reports. 2021 Sep 13;2021(9):omab080. doi: 10.1093/omcr/omab080. eCollection 2021 Sep.

ABSTRACT

Guillain-Barré syndrome (GBS) is an immune-mediated polyneuropathy, which is characterized by areflexia and ascending paresthesia which can progress to a respiratory failure. Certain conditions, such as vasculitis and heavy metal and drug toxicity, may have misleadingly similar clinical presentation to GBS. We describe a case of a patient with cystic fibrosis and intravenous colistin-induced neurotoxicity mimicking GBS. The patient had used inhaled colistin on five occasions with no adverse effects, however, developed symptoms on the second day of intravenous treatment. Overlapping findings between immune-mediated polyneuropathy and drug-induced neurotoxicity include limb paresthesia and decreased reflexes. Perioral tingling, however, is a common presentation of colistin-induced neurotoxicity, and therefore, is an important differentiating factor. Early diagnosis prevents further neurologic decline, extensive unnecessary workup and potentially harmful incorrect management.

PMID:34527253 | PMC:PMC8436279 | DOI:10.1093/omcr/omab080

Eur Respir Rev. 2021 Sep 15;30(161):210055. doi: 10.1183/16000617.0055-2021. Print 2021 Sep 30.

ABSTRACT

Chronic airway colonisation by Pseudomonas aeruginosa, a hallmark of cystic fibrosis (CF) lung disease, is associated with increased morbidity and mortality and despite aggressive antibiotic treatment, P. aeruginosa is able to persist in CF airways. In vitro antibiotic susceptibility assays are poor predictors of antibiotic efficacy to treat respiratory tract infections in the CF patient population and the selection of the antibiotic(s) is often made on an empirical base. In the current review, we discuss the factors that are responsible for the discrepancies between antibiotic activity in vitro and clinical efficacy in vivo We describe how the CF lung microenvironment, shaped by host factors (such as iron, mucus, immune mediators and oxygen availability) and the microbiota, influences antibiotic activity and varies widely between patients. A better understanding of the CF microenvironment and population diversity may thus help improve in vitro antibiotic susceptibility testing and clinical decision making, in turn increasing the success rate of antibiotic treatment.

PMID:34526313 | DOI:10.1183/16000617.0055-2021

J Cyst Fibros. 2021 Sep 12:S1569-1993(21)01374-6. doi: 10.1016/j.jcf.2021.08.033. Online ahead of print.

NO ABSTRACT

PMID:34526221 | DOI:10.1016/j.jcf.2021.08.033

BMC Microbiol. 2021 Sep 15;21(1):247. doi: 10.1186/s12866-021-02305-z.

ABSTRACT

BACKGROUND: Infants with cystic fibrosis (CF) suffer from gastrointestinal (GI) complications, including pancreatic insufficiency and intestinal inflammation, which have been associated with impaired nutrition and growth. Recent evidence identified altered fecal microbiota taxonomic compositions in infants with CF relative to healthy infants that were characterized by differences in the abundances of taxa associated with GI health and nutrition. Furthermore, these taxonomic differences were more pronounced in low length infants with CF, suggesting a potential link to linear growth failure. We hypothesized that these differences would entail shifts in the microbiome's functional capacities that could contribute to inflammation and nutritional failure in infants with CF.

RESULTS: To test this hypothesis, we compared fecal microbial metagenomic content between healthy infants and infants with CF, supplemented with an analysis of fecal metabolomes in infants with CF. We identified notable differences in CF fecal microbial functional capacities, including metabolic and environmental response functions, compared to healthy infants that intensified during the first year of life. A machine learning-based longitudinal metagenomic age analysis of healthy and CF fecal metagenomic functional profiles further demonstrated that these differences are characterized by a CF-associated delay in the development of these functional capacities. Moreover, we found metagenomic differences in functions related to metabolism among infants with CF that were associated with diet and antibiotic exposure, and identified several taxa as potential drivers of these functional differences. An integrated metagenomic and metabolomic analysis further revealed that abundances of several fecal GI metabolites important for nutrient absorption, including three bile acids, correlated with specific microbes in infants with CF.

CONCLUSIONS: Our results highlight several metagenomic and metabolomic factors, including bile acids and other microbial metabolites, that may impact nutrition, growth, and GI health in infants with CF. These factors could serve as promising avenues for novel microbiome-based therapeutics to improve health outcomes in these infants.

PMID:34525965 | DOI:10.1186/s12866-021-02305-z

Int J Clin Pract. 2021 Sep 15:e14879. doi: 10.1111/ijcp.14879. Online ahead of print.

ABSTRACT

INTRODUCTION: The aim of this study was to investigate the immediate effect of coughing episodes and diaphragmatic breathing exercise (DBE) on respiratory mechanics of children/adolescents with cystic fibrosis (CF).

METHODS: It is a cross-sectional analytical study that occurred in a reference center for children with CF. Forty-five children/adolescents with CF [60% male; mean age 10,22±2,84 years old; mean forced expiratory volume in 1 second (FEV1 ) 73,74±21,38 % predicted] were divided in 3 groups according to the R5 parameter response to the DBE: G1 (increased R5), G2 (no change R5) and G3 (decreased R5). The children/adolescents performed 5 successive coughs and 10 DBE. The main outcome measures were the IOS parameters evaluated before, during and after the interventions.

RESULTS: In the total sample, the IOS parameters (Z5, R5 and R20) were worse after coughing and they did not change after the DBE. In the G1, the parameters were progressively worsening during the interventions. In the G2, they worsened after coughing and after the DBE. And, in the G3, they worsened after coughing; however, after the DBE the IOS parameters have improved and returned close to the baseline.

CONCLUSIONS: The children/adolescents with CF airway resistance got worse after coughing episodes, and 10 DBE repetitions did not affect the respiratory mechanics in most of the sample. Meanwhile, in the group with older children the DBE worsened the respiratory mechanics, yet in the younger group it improved.

PMID:34525264 | DOI:10.1111/ijcp.14879

Pediatr Pulmonol. 2021 Sep 15. doi: 10.1002/ppul.25647. Online ahead of print.

ABSTRACT

In this study, the spectrum and frequency of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations previously reported among Iranian cystic fibrosis (CF) patients have been reviewed and discussed. Using the keywords of Cystic Fibrosis, CF, CFTR, and Iran, along with their Persian equivalents, a comprehensive search was performed on the online databases. After applying the inclusion and exclusion criteria, 16 articles with an overall sample of 735 Iranian patients with CF, were included in this systematic review. A total of 101 different CFTR gene variants had been reported. The mutation of p.Phe508del (c.1521_1523delCTT) (21.22%) was the most frequent one among Iranian patients with CF. In conclusion, due to the fact that in many provinces of Iran no specific study has been done so far, it seems that the CFTR gene mutation spectrum in patients with CF from Iran is much wider.

PMID:34525262 | DOI:10.1002/ppul.25647

Acta Physiol (Oxf). 2021 Sep 15:e13729. doi: 10.1111/apha.13729. Online ahead of print.

ABSTRACT

AIM: Slc26a9 is a member of the Slc26 multifunctional anion transporter family. Polymorphisms in Slc26a9 are associated with an increased incidence of meconium ileus and diabetes in cystic fibrosis patients. We investigated the expression of Slc26a9 in the murine pancreatic ducts, islets and parenchyma, and elucidated its role in pancreatic ductal electrolyte and fluid secretion and endocrine function.

METHODS: Pancreatic Slc26a9 and CFTR mRNA expression, fluid and bicarbonate secretion were assessed in slc26a9-/- mice and their age- and sex-matched wild-type (wt) littermates. Glucose and insulin tolerance tests were performed.

RESULTS: Compared with stomach, the mRNA expression of Slc26a9 was low in pancreatic parenchyma, 20-fold higher in microdissected pancreatic ducts than parenchyma, and very low in islets. CFTR mRNA was ∼ 10fold higher than Slc26a9 mRNA expression in each pancreatic cell type. Significantly reduced pancreatic fluid secretory rates and impaired glucose tolerance were observed in female slc26a9-/- mice, whereas alterations in male mice did not reach statistical significance. No significant difference was observed in peripheral insulin resistance in slc26a9-/- compared to sex- and aged-matched wt controls. In contrast, isolated slc26a9-/- islets in short term culture displayed no difference in insulin content, but a significantly reduced glucose-stimulated insulin secretion compared to age- and sex-matched wt islets, suggesting that the impaired glucose tolerance is a pancreatic defect.

CONCLUSIONS: Deletion of Slc26a9 is associated with a reduction in pancreatic fluid secretion and impaired glucose tolerance in female mice. The results underline the importance of Slc26a9 in pancreatic physiology.

PMID:34525257 | DOI:10.1111/apha.13729

Am J Physiol Cell Physiol. 2021 Sep 15. doi: 10.1152/ajpcell.00295.2021. Online ahead of print.

ABSTRACT

Altered esophageal ion transport mechanisms play a key role in inflammatory and cancerous diseases of the esophagus, but epithelial ion processes have been less studied in the esophagus because of the lack of a suitable experimental model. In this study, we generated 3D esophageal organoids (EOs) from two different mouse strains and characterized the ion transport processes of the EOs. EOs form a cell-filled structure with a diameter of 250-300 µm and generated from epithelial stem cells as shown by FACS analysis. Using conventional PCR and immunostaining, the presence of Slc26a6 Cl-/HCO3- anion exchanger (AE), Na+/H+ exchanger (NHE), Na+/HCO3- cotransporter (NBC), cystic fibrosis transmembrane conductance regulator (CFTR) and anoctamin 1 Cl- channels were detected in EOs. Microfluorimetric techniques revealed high NHE, AE, and NBC activities, whereas that of CFTR was relatively low. In addition, inhibition of CFTR led to functional interactions between the major acid-base transporters and CFTR. We conclude that EOs provide a relevant and suitable model system for studying the ion transport mechanisms of esophageal epithelial cells, and they can be also used as preclinical tools to assess the effectiveness of novel therapeutic compounds in esophageal diseases associated with altered ion transport processes.

PMID:34524930 | DOI:10.1152/ajpcell.00295.2021

J Clin Microbiol. 2021 Sep 15:JCM0144721. doi: 10.1128/JCM.01447-21. Online ahead of print.

ABSTRACT

The Burkholderia cepacia complex (BCC) is known for causing serious lung infections in people with cystic fibrosis (CF). These infections can require lung transplantation, eligibility for which may be guided by antimicrobial susceptibility testing (AST). While the Clinical and Laboratory Standards Institute recommends AST for BCC, the European Committee on Antimicrobial Susceptibility Testing (EUCAST) does not due to poor method performance and correlation with clinical outcomes. Furthermore, limited data exists on the performance of automated AST methods for BCC. To address these issues, reproducibility and accuracy were evaluated for disk diffusion (DD), broth microdilution (BMD), and MicroScan WalkAway using 50 B. cenocepacia and 50 B. multivorans isolates collected from people with CF. The following drugs were evaluated in triplicate: chloramphenicol (CAM), ceftazidime (CAZ), meropenem (MEM), trimethoprim-sulfamethoxazole (TMP-SMX), minocycline (MIN), levofloxacin (LVX), ciprofloxacin (CIP) and piperacillin-tazobactam (PIP-TAZ). BMD reproducibility was ≥ 95% for MEM and MIN only, and MicroScan WalkAway reproducibility was similar to BMD. DD reproducibility was < 90% for all drugs tested when a 3 mm cut-off was applied. When comparing the accuracy of DD to BMD, only MEM met all acceptance criteria. TMP-SMX and LVX had high minor errors, CAZ had unacceptable very major errors (VME), and MIN, PIP-TAZ, and CIP had both unacceptable minor errors and VMEs. For MicroScan WalkAway, no drugs met acceptance criteria. Analyses also showed that errors were not attributed to one species. In general, our data agree with EUCAST recommendations that routine AST should not be performed for BCC CF isolates.

PMID:34524889 | DOI:10.1128/JCM.01447-21

Mol Hum Reprod. 2021 Sep 15:gaab060. doi: 10.1093/molehr/gaab060. Online ahead of print.

ABSTRACT

Embryo implantation failure is a major cause of infertility in women of reproductive age and a better understanding of uterine factors that regulate implantation is required for developing effective treatments for female infertility. This study investigated the role of the uterine kisspeptin receptor (KISS1R) in the molecular regulation of implantation in a mouse model. To conduct this study, a conditional uterine knockout (KO) of Kiss1r was created using the Pgr-Cre (progesterone receptor-CRE recombinase) driver. Reproductive profiling revealed that while knockout females exhibited normal ovarian function and mated successfully to stud males, they exhibited significantly fewer implantation sites, reduced litter size, and increased neonatal mortality demonstrating that uterine KISS1R is required for embryo implantation and a healthy pregnancy. Strikingly, in the uterus of Kiss1r KO mice on day 4 (D4) of pregnancy, the day of embryo implantation, KO females exhibited aberrantly elevated epithelial ERα (estrogen receptor α) transcriptional activity. This led to the temporal misexpression of several epithelial genes [Cftr (Cystic fibrosis transmembrane conductance regulator), Aqp5 (aquaporin 5), Aqp8 (aquaporin 8) and Cldn7 (claudin 7)] that mediate luminal fluid secretion and luminal opening. As a result, on D4 of pregnancy, the lumen remained open disrupting the final acquisition of endometrial receptivity and likely accounting for the reduction in implantation events. Our data clearly show that uterine KISS1R negatively regulates ERα signaling at the time of implantation, in part by inhibiting ERα overexpression and preventing detrimentally high ERα activity. To date, there are no reports on the regulation of ERα by KISS1R, therefore this study has uncovered an important and powerful regulator of uterine ERα during early pregnancy.

PMID:34524460 | DOI:10.1093/molehr/gaab060

Cureus. 2021 Aug 10;13(8):e17045. doi: 10.7759/cureus.17045. eCollection 2021 Aug.

ABSTRACT

Ascending cholangitis is a bacterial infection of the extra-hepatic biliary system and presents as a life-threatening systemic condition. Increased bacterial loads and biliary obstruction favor bacterial translocation into the vascular and lymphatic systems. Common organisms isolated are Escherichia Coli, Klebsiella, Enterococcus species, and Enterobacter species. Methicillin-resistant Staphylococcus aureus (MRSA) is a rare isolate in ascending cholangitis. We present a case of a 24-year-old patient with cystic fibrosis who presented with epigastric abdominal pain, low-grade fever, jaundice, dark urine, and nausea for two days. Initial workup revealed elevated liver enzymes, hyperbilirubinemia, leukocytosis, and an ultrasound which showed common bile duct dilation to 14 mm with choledocholithiasis. He underwent endoscopic retrograde cholangiopancreatography (ERCP) with stone extraction and bile fluid culture. Cultures grew out MRSA and the patient was treated with appropriate antibiotic therapy. The mainstay of therapy for ascending cholangitis is adequate hydration, antibiotics, and biliary decompression. Early recognition of the offending organism is critical in guiding therapy. Current guidelines focus on the empiric treatment of Gram-negative and anaerobic bacteria. Clinicians should be aware of the possibility of less common pathogens (such as MRSA), especially in a patient who is decompensating despite antibiotic therapy.

PMID:34522523 | PMC:PMC8427740 | DOI:10.7759/cureus.17045

J Cyst Fibros. 2021 Sep 11:S1569-1993(21)01364-3. doi: 10.1016/j.jcf.2021.08.023. Online ahead of print.

ABSTRACT

The Covid-19 pandemic has accelerated the use of telehealth within the cystic fibrosis (CF) community to deliver CF care. The article by Solomon and colleagues exploring the patient and family experiences of telehealth care delivery, as part of the CF chronic care model in the US, is therefore timely. In this commentary, we discuss how the US experience of telehealth care compares with reports from CF centres in other parts of the world. We highlight the potential challenges, including whether the inverse care law will apply in this new era of CF telehealth.

PMID:34521596 | DOI:10.1016/j.jcf.2021.08.023

J Cyst Fibros. 2021 Sep 11:S1569-1993(21)01307-2. doi: 10.1016/j.jcf.2021.07.009. Online ahead of print.

ABSTRACT

BACKGROUND: Barriers to implementing infection prevention and control (IP&C) practices may be experienced by healthcare workers (HCWs) caring for people with CF (PwCF), PwCF, and their families. We hypothesized that these stakeholders from CF centers with early adoption of the updated 2013 IP&C guideline would experience fewer barriers implementing selected recommendations compared to stakeholders from CF centers with delayed adoption.

METHODS: In 2018-2019 we surveyed HCWs and PwCF/parents from 25 CF centers to identify knowledge, attitude, and practice barriers. Each center recruited five HCWs with different occupations. Pediatric centers recruited five parents of children <18 years old and five young adults 18-21 years old. Adult centers recruited 10 adults ≥18 years old. We determined respondents' knowledge scores, the proportion who agreed with or perceived health benefits from recommendations, and reported adherence to recommendations.

RESULTS: Knowledge scores, perception of health benefits, and adherence to selected practices were similar among participants from centers with early vs. delayed adoption, yet generally lower for inpatient nurses. IP&C practitioners were less likely to perceive health benefits from PwCF wearing masks and HCWs wearing gowns and gloves. Among HCWs, 57% educated >75% of PwCF/parents about IP&C and 43% advised >75% of PwCF/parents to avoid socializing with other PwCF. Among PwCF/parents, 69%, 53%, and 56% reported discussions with their care teams about performing hand hygiene, avoiding socialization, or the 2013 IP&C guideline, respectively.

CONCLUSIONS: Our findings suggest opportunities for targeted education for specific HCW occupations and for PwCF and their families.

PMID:34521595 | DOI:10.1016/j.jcf.2021.07.009

BMC Pediatr. 2021 Sep 14;21(1):404. doi: 10.1186/s12887-021-02869-9.

ABSTRACT

BACKGROUND: Relatively little is known about protective factors and the emergence and maintenance of positive outcomes in the field of adolescents with chronic conditions. Therefore, the primary aim of the study is to acquire a deeper understanding of the dynamic process of resilience factors, coping strategies and psychosocial adjustment of adolescents living with chronic conditions.

METHODS/DESIGN: We plan to consecutively recruit N = 450 adolescents (12-21 years) from three German patient registries for chronic conditions (type 1 diabetes, cystic fibrosis, or juvenile idiopathic arthritis). Based on screening for anxiety and depression, adolescents are assigned to two parallel groups - "inconspicuous" (PHQ-9 and GAD-7 < 7) vs. "conspicuous" (PHQ-9 or GAD-7 ≥ 7) - participating in a prospective online survey at baseline and 12-month follow-up. At two time points (T1, T2), we assess (1) intra- and interpersonal resiliency factors, (2) coping strategies, and (3) health-related quality of life, well-being, satisfaction with life, anxiety and depression. Using a cross-lagged panel design, we will examine the bidirectional longitudinal relations between resiliency factors and coping strategies, psychological adaptation, and psychosocial adjustment. To monitor Covid-19 pandemic effects, participants are also invited to take part in an intermediate online survey.

DISCUSSION: The study will provide a deeper understanding of adaptive, potentially modifiable processes and will therefore help to develop novel, tailored interventions supporting a positive adaptation in youths with a chronic condition. These strategies should not only support those at risk but also promote the maintenance of a successful adaptation.

TRIAL REGISTRATION: German Clinical Trials Register (DRKS), no. DRKS00025125 . Registered on May 17, 2021.

PMID:34521358 | DOI:10.1186/s12887-021-02869-9

J Psychosom Res. 2021 Sep 8;150:110607. doi: 10.1016/j.jpsychores.2021.110607. Online ahead of print.

ABSTRACT

Background Adults with cystic fibrosis have unique barriers that may decrease their ability to receive adequate social support and socialization, leading to social isolation. Social isolation has been correlated with negative health outcomes in other populations. In those with cystic fibrosis, social isolation may present additional physiological and psychological challenges, potentially interfering with clinical outcomes and quality of life. However, there is a lack of understanding as to how social isolation presents in this population. Methods The purpose of this integrative review is to identify and critically analyze how social support and social isolation are reported for adults with cystic fibrosis. PubMed, Scopus, and CINAHL Complete were searched for related publications, resulting in an initial yield of 1767 articles. After eligibility screening, 21 studies met the criteria for this review, which were all then critically analyzed and synthesized. Results There is a scarcity of literature focusing on social isolation and social support in this population. Reduced physical and mental health were the most commonly reported variables associated with reduced social functioning and social support. Conclusion Preliminary studies are warranted to understand how adults with cystic fibrosis experience social isolation, as well as its relationship to social support. This knowledge can guide future research focusing on physical and psychological effects of social isolation, along with interventions that facilitate socialization and support.

PMID:34521060 | DOI:10.1016/j.jpsychores.2021.110607

Respir Med. 2021 Sep 8;188:106606. doi: 10.1016/j.rmed.2021.106606. Online ahead of print.

ABSTRACT

BACKGROUND: In this study, we report clinical outcomes in COVID-19 infection in a large cohort of people with cystic fibrosis (pwCF) and compare these outcomes to a propensity score matched cohort of people without CF.

METHODS: Analysis of a multicenter research network TriNETX was performed including patients more than 16 years of age diagnosed with COVID-19. Outcomes in COVID-19 positive pwCF were compared with a propensity-matched cohort of people without CF.

RESULTS: A total of 507,810 patients with COVID-19 were included (422 patients, 0.08% with CF; 507,388 patients, 99.92% without CF. Mean age at COVID-19 diagnosis in CF cohort was 46.6 ± 19.3 years, with female predominance (n = 225, 53.32%). Majority of the participants were Caucasian (n = 309, 73.22%). In the crude, unmatched analysis, mortality, hospitalization, critical care need, mechanical ventilation, acute kidney injury and composite (combination of intubation and mortality) outcome at 30 days was higher in the pwCF. Following robust propensity matching, pwCF had higher hospitalization rate (RR 1.56, 95% CI 1.20-2.04), critical care need (RR 1.78, 95% CI 1.13-2.79), and acute renal injury (RR 1.60, 95% CI 1.07-2.39) as compared to patients without CF.

CONCLUSION: People with CF are at risk of poor outcomes with COVID-19.5.2% of these patients died within one month of COVID-19 diagnosis, and more than one in 10 patients required critical care. Therefore, the relatively young median age of cystic fibrosis patients, and lower prevalence of obesity do not protect these patients from severe disease contrary to prior reports.

PMID:34520894 | DOI:10.1016/j.rmed.2021.106606