General Overview of Nontuberculous Mycobacteria Opportunistic Pathogens: Mycobacterium avium and Mycobacterium abscessus.

J Clin Med. 2020 Aug 06;9(8):

Authors: To K, Cao R, Yegiazaryan A, Owens J, Venketaraman V

Abstract
Nontuberculous mycobacteria (NTM) are emerging human pathogens, causing a wide range of clinical diseases affecting individuals who are immunocompromised and who have underlying health conditions. NTM are ubiquitous in the environment, with certain species causing opportunistic infection in humans, including Mycobacterium avium and Mycobacterium abscessus. The incidence and prevalence of NTM infections are rising globally, especially in developed countries with declining incidence rates of M. tuberculosis infection. Mycobacterium avium, a slow-growing mycobacterium, is associated with Mycobacterium avium complex (MAC) infections that can cause chronic pulmonary disease, disseminated disease, as well as lymphadenitis. M. abscessus infections are considered one of the most antibiotic-resistant mycobacteria and are associated with pulmonary disease, especially cystic fibrosis, as well as contaminated traumatic skin wounds, postsurgical soft tissue infections, and healthcare-associated infections (HAI). Clinical manifestations of diseases depend on the interaction of the host's immune response and the specific mycobacterial species. This review will give a general overview of the general characteristics, vulnerable populations most at risk, pathogenesis, treatment, and prevention for infections caused by Mycobacterium avium, in the context of MAC, and M. abscessus.

PMID: 32781595 [PubMed]

Influence of early extubation on post-operative outcomes after pediatric lung transplantation.

Pediatr Transplant. 2020 Aug 11;:e13776

Authors: Labarinas S, Coss-Bu JA, Onyearugbulem C, Heinle JS, Mallory GB, Gazzaneo MC

Abstract
Lung transplantation has become an accepted therapeutic option for a select group of children with end-stage lung disease. We evaluated the impact of early extubation in a pediatric lung transplant population and its post-operative outcomes. Single-center retrospective study. PICU within a tertiary academic pediatric hospital. Patients <22 years after pulmonary transplant between January 2011 and December 2016. A total of 74 patients underwent lung transplantation. The primary pretransplantation diagnoses included cystic fibrosis (58%), pulmonary fibrosis (9%), and surfactant dysfunction disorders (10%). Of 60 patients, 36 (60%) were extubated within 24 hours and 24 patients after 24 hours (40%). A total of seven patients (11.6%) required reintubation within 24 hours. Median length of stay for the early extubation group was shorter at 3 days ([(IQR) 2.2-4.7]) compared to 5 days (IQR, 3-7) (P = .02) in the late extubation group. Median costs were lower for the early extubation group with 13,833 US dollars (IQR, 9980-22,822) vs 23 671 US dollars (IQR, 16 673-39 267) (P = .043). Fourteen patients were in the PICU prior to their transplantation; this did not affect their early extubation success. Neither did the fact of requiring invasive or non-invasive mechanical ventilation before transplantation. Early extubation appears to be safe in a pediatric population after lung transplantation and is associated with a shorter LOS and decreased hospital costs. It may prevent known complications associated with mechanical ventilation.

PMID: 32780552 [PubMed - as supplied by publisher]

Discovery and biosynthesis of bolagladins: unusual lipodepsipeptides from Burkholderia gladioli clinical isolates.

Angew Chem Int Ed Engl. 2020 Aug 11;:

Authors: Dashti Y, Nakou I, Mullins A, Webster G, Jian X, Mahenthiralingam E, Challis G

Abstract
Two Burkholderia gladioli strains isolated from the lungs of cystic fibrosis patients were found to produce unusual lipodepsipeptides containing a unique citrate-derived fatty acid and a rare dehydro-β-alanine residue. The gene cluster responsible for their biosynthesis was identified by bioinformatics and insertional mutagenesis. In-frame deletions and enzyme activity assays were used to investigate the functions of several proteins encoded by the biosynthetic gene cluster, which was found in the genomes of about 50% of B. gladioli isolates, suggesting that its metabolic products play an important role in the growth and/or survival of the species. The Chrome Azurol S (CAS) assay indicated that these metabolites bind ferric iron, which suppresses their production when added to the growth medium. Moreover, a gene encoding a TonB-dependent ferric-siderophore receptor is adjacent to the biosynthetic genes, suggesting that these metabolites may function as siderophores in B. gladioli .

PMID: 32780452 [PubMed - as supplied by publisher]

Drug efficacy and toxicity prediction: an innovative application of transcriptomic data.

Cell Biol Toxicol. 2020 Aug 11;:

Authors: Xia X

Abstract
Drug toxicity and efficacy are difficult to predict partly because they are both poorly defined, which I aim to remedy here from a transcriptomic perspective. There are two major categories of drugs: (1) restorative drugs aiming to restore an abnormal cell, tissue, or organ to normal function (e.g., restoring normal membrane function of epithelial cells in cystic fibrosis), and (2) disruptive drugs aiming to kill pathogens or malignant cells. These two types of drugs require different definition of efficacy and toxicity. I outlined rationales for defining transcriptomic efficacy and toxicity and illustrated numerically their application with two sets of transcriptomic data, one for restorative drugs (treating cystic fibrosis with lumacaftor/ivacaftor aiming to restore the cellular function of epithelial cells) and the other for disruptive drugs (treating acute myeloid leukemia with prexasertib). The conceptual framework presented will help and sensitize researchers to collect data required for determining drug toxicity.

PMID: 32780246 [PubMed - as supplied by publisher]

Giant ethmoidal mucocele leading to proptosis and hypertelorism in a pediatric patient with cystic fibrosis: a case report.

Turk J Pediatr. 2020;62(4):673-676

Authors: Tunç O, Gönüldaş B, Aytaç İ, Mumbuç S, Kanlıkama M

Abstract
BACKGROUND: Chronic sinusitis and its complications are common in patients with cystic fibrosis. Mucoceles are one of these complications and can have life-threatening consequences if left untreated.
CASE: We present the case of a giant ethmoid mucocele leading to proptosis and hypertelorism in a 5-year-old child with cystic fibrosis.
CONCLUSION: Chronic sinusitis and its complications are common in patients with CF. Mucoceles are a rare complication of sinusitis that can be treated surgically. As seen in this case if left untreated mucoceles can lead to orbital pathologies such as proptosis, hypertelorism. To the best of our knowledge, we report the first case report of giant ethmoidal mucocele leading to proptosis and hypertelorism in a patient with cystic fibrosis.

PMID: 32779423 [PubMed - in process]

Diverse clinical characteristics of Aspergillus growth in patients with cystic fibrosis.

Turk J Pediatr. 2020;62(4):560-568

Authors: Emiralioğlu N, Doğru D, Doğan Ö, Gülmez D, Akdağlı SA, Polat SE, Tuğcu G, Hızal MG, Yalçın E, Özçelik U, Şener B, Kiper N

Abstract
BACKGROUND AND OBJECTIVES: Patients with cystic fibrosis (CF) have a varying spectrum of clinically significant Aspergillus disease in addition to allergic bronchopulmonary aspergillosis (ABPA). Here we aimed to review the different clinical phenotypes related with Aspergillus growth on the airway culture of patients with CF, we also aimed to investigate the effect of Aspergillus growth on lung function tests.
METHOD: The medical records of 100 patients with CF who had Aspergillus growth on airway culture within the period of April 2001 and June 2016 were retrospectively analyzed. Age, gender, symptoms, physical examination findings, pulmonary function tests, the diagnosis of ABPA, and airway culture results were recorded for every visit. Patients with Aspergillus growth on airway cultures were classified into different groups as ABPA, Aspergillus sensitization, Aspergillus colonization and Aspergillus bronchitis.
RESULTS: Medical records of 83 patients and 147 sputum cultures were attained from 100 patients. The mean age of the patients was 17.6±7.6 years and the mean age of the first Aspergillus growth in sputum culture was 12.5±6.7 years. At first isolation, Aspergillus fumigatus SC was the most common Aspergillus SC in sputum (76.3%) and 14.5% of these patients required hospitalization. Aspergillus sensitization was diagnosed in 3.6% (n= 3) of the patients. Aspergillus colonization was diagnosed in 18.1% (n= 15) of all patients and led to a decline in FEV1%, FVC% and FEF25-75% which was not statistically significant, furthermore. ABPA was detected in 9.6% (n= 8) of all patients and led to a statistically significant decline in FEV1% (p= 0.02); nonsignificant decline in FVC% and FEF25-75%. Aspergillus bronchitis was detected in 43.4% (n= 36) of all patients and led to nonsignificant decline in FEV1%, FVC% and FEF25-75%.
CONCLUSION: ABPA is recognized as the most common Aspergillus associated disorder in CF patients and is related to deteriorated pulmonary function tests; however Aspergillus colonization and bronchitis may also be associated with worsening lung function.

PMID: 32779408 [PubMed - in process]

Cystic fibrosis carrier screening using next generation sequencing: A cautionary tale.

Fertil Steril. 2020 Aug 07;:

Authors: Coward RM, Davis EL, Young SL

PMID: 32778331 [PubMed - as supplied by publisher]

How to define CRMS/CFSPID conversion to CF.

Pediatr Pulmonol. 2020 07;55(7):1548-1549

Authors: Rock MJ

PMID: 32407573 [PubMed - indexed for MEDLINE]

Generation of induced pluripotent stem cell line (RCMGi001-A) from human skin fibroblasts of a cystic fibrosis patient with p.F508del mutation.

Stem Cell Res. 2020 Aug 02;48:101933

Authors: Kondrateva E, Adilgereeva E, Amelina E, Tabakov V, Demchenko A, Ustinov K, Yasinovsky M, Voronina E, Lavrov A, Smirnikhina S

Abstract
Skin fibroblasts obtained from a 27-year-old man with clinically manifested and genetically proven (F508del/F508del) cystic fibrosis were successfully transformed into induced pluripotent stem cells (iPSCs) by using Sendai virus-based reprogramming vectors including the four Yamanaka factors, OCT3/4, SOX2, KLF4, and c-MYC. The iPSCs showed a normal karyotype, expressed pluripotency markers and exhibited the potential to differentiate into three germ layers in spontaneous differentiation assay. This iPSC line may be subsequently used for development of a personalized etiotropic treatment including genome editing, and for disease modelling and drug screening.

PMID: 32777768 [PubMed - as supplied by publisher]

Mutation analysis of the cystic fibrosis transmembrane conductance regulator gene in Chinese congenital absence of vas deferens patients.

Gene. 2020 Aug 07;:145045

Authors: Luo S, Feng J, Zhang Y, Yang X, Ma G, Hu T, Xi Y, Tu X, Wang C, Zhang H, Zou Z, Zhang Y

Abstract
To find the variant spectrum of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and evaluate its frequent variants in Chinese congenital absence of vas deferens (CAVD) patients. A total of 276 patients with azoospermia and CAVD (aged from 21 to 44 years old) were investigated from May 2013 to September 2019 in the Third Affiliated Hospital of Sun Yat-sen University. Additionally, 50 healthy, unrelated volunteers were recruited as controls (aged from 21 to 46 years old). The 5'-UTR, exons and their flanking side of the CFTR gene were sequenced by high-throughput sequencing technology. The results were compared with those retrieved from the Ensembl Genome Browser. In addition, all 13 novel variants were further confirmed independently by Sanger sequencing and evaluated in the bioinformatics web servers. A schematic of the variant spectrum of the CFTR gene, including 13 novel variants (12 in CAVD patients, one in the control group), is shown, and the frequent variants in Chinese CAVD patients were 5T (27.54%), c.-8G>C (7.25%), p.Q1352H (5.98%), and p.I556V (3.08%). 5T was found to be the most frequent variant. p.Q1352H had a significantly high allelic frequency in CAVD patients (P<0.05). c.-8G>C and p.I556V had high allelic frequencies but showed no difference between patients and controls (P>0.05). p.Q1352H is the most common and important missense variant in Chinese patients with CAVD, while the pathological effects of C.-8G>C and p.I556V may be weak after evaluation.

PMID: 32777524 [PubMed - as supplied by publisher]

Desensitization for inhaled vancomycin.

Ann Allergy Asthma Immunol. 2020 Aug 07;:

Authors: Al-Kaabi A, Pandya A, Martinez J, Love M

PMID: 32777433 [PubMed - as supplied by publisher]

The benefits of mechanical insufflator-exsufflator (MI-E) compared to autogenic drainage (AD) in adults with Cystic Fibrosis (CF).

Pediatr Pulmonol. 2020 Aug 10;:

Authors: Helper N, Kodesh E, Sokol G, Hakimi R, Vilozni D, Efrati O

Abstract
BACKGROUND: Autogenic drainage (AD) is used for airway clearance in individuals with Cystic fibrosis (CF). The Mechanical Insufflator-Exsufflator (MI-E) device is commonly used in patients with neuromuscular diseases for airway clearance by increasing inspiratory volume and expiratory flow. MI-E use has not been studied extensively in CF patients.
OBJECTIVES: To examine whether there are advantages to using the MI-E device in patients with CF.
SUBJECTS: 14 males and 8 females from the national center for CF, with an average FEV1 of 54% were recruited DESIGN: Prospective cross-over trial (IRB 3009-16-SMC).
INTERVENTION: Subjects received either AD or MI-E in a random order. Sputum was collected and weighed immediately after treatment. Subjects performed lung function tests at baseline, 20 minutes after and 1 hour after treatment; additionally, a two-minute walk test was performed at the end of all lung function tests. Saturation, dyspnea scores while resting and after a 2-minute walk and subjective fatigue were recorded.
RESULTS: 36% more sputum was collected following MI-E than AD treatment (P<0.0001). A significant difference in saturation in response to the 2MWT was noted in both treatments significantly less desaturation was recorded after the 2MWT in the MI-E treatment (P<0.01).
CONCLUSIONS: Treatment with the MI-E was more effective for clearing sputum in CF subjects, initial evidence suggests that the MI-E may be successfully incorporated into treatment protocols. Further studies are needed to assess the long-term benefits of MI-E in patients with CF. This article is protected by copyright. All rights reserved.

PMID: 32776649 [PubMed - as supplied by publisher]

[Assessment of lung impairment in patients with cystic fibrosis : Novel magnetic resonance imaging methods].

Radiologe. 2020 Aug 10;:

Authors: Nyilas S, Bauman G, Sommer G

Abstract
CLINICAL/METHODOLOGICAL ISSUE: The differentiated assessment of respiratory mechanics, gas exchange and pulmonary circulation, as well as structural impairment of the lung are essential for the treatment of patients with cystic fibrosis (CF). Clinical lung function measurements are often not sufficiently specific and are often difficult to perform.
STANDARD RADIOLOGICAL METHODS: The standard procedures for pulmonary imaging are chest X‑ray and computed tomography (CT) for assessing lung morphology. In more recent studies, an increasing number of centers are using magnetic resonance imaging (MRI) to assess lung structure and function. However, functional imaging is currently limited to specialized centers.
METHODOLOGICAL INNOVATIONS: In patients with CF, studies showed that MRI with hyperpolarized gases and Fourier decomposition/matrix pencil MRI (FD/MP-MRI) are feasible for assessing pulmonary ventilation. For pulmonary perfusion, dynamic contrast-enhanced MRI (DCE-MRI) or contrast-free methods, e.g., FD-MRI, can be used.
PERFORMANCE: Functional MRI provides more accurate insight into the pathophysiology of pulmonary function at the regional level. Advantages of MRI over X‑ray are its lack of ionizing radiation, the large number of lung function parameters that can be extracted using different contrast mechanisms, and ability to be used repeatedly over time.
ACHIEVEMENTS: Early assessment of lung function impairment is needed as the structural changes usually occur later in the course of the disease. However, sufficient experience in clinical application exist only for certain functional lung MRI procedures.
PRACTICAL RECOMMENDATIONS: Clinical application of the aforementioned techniques, except for DCE-MRI, should be restricted to scientific studies.

PMID: 32776240 [PubMed - as supplied by publisher]

[Abdominal manifestations in cystic fibrosis : Clinical review].

Radiologe. 2020 Aug 10;:

Authors: Sommerburg O, Schenk JP

Abstract
Cystic fibrosis (CF) is the most common fatal autosomal recessive disease in the Caucasian population. A mutation in the cystic fibrosis transmembrane regulator protein (CFTR) gene leads to the production of abnormally viscous mucus and secretions in the lungs of these patients. A similar pathology also occurs in other organs. In the abdomen, among others the gastrointestinal tract, the pancreas, and the hepatobiliary system are affected. The involvement of the pancreas leads to its exocrine and endocrine insufficiency. Hepatic manifestations include hepatic steatosis, focal biliary and multilobular cirrhosis, and portal hypertension. Biliary complications include cholelithiasis, microgallbladder, and sclerosing cholangitis. In the gastrointestinal tract, complications such as the distal intestinal obstruction syndrome, invaginations, chronic constipation, wall thickening, and fibrosis in the colon may occur. An important renal manifestation is nephrolithiasis. With currently rapidly increasing life expectancy of patients with cystic fibrosis, complications of extrapulmonary cystic fibrosis manifestations including hepatic and gastrointestinal malignancy could be an increasing cause of morbidity and mortality of these patients. It is therefore important for radiologists to know and recognize these clinical patterns and to monitor these manifestations in follow-up exams. Previous therapy of extrapulmonary manifestations has been largely symptomatic. Fortunately, the new CFTR modulators seem to represent an effective causal therapeutic approach here.

PMID: 32776239 [PubMed - as supplied by publisher]

Reduced exertion high-intensity interval training (REHIT) in an adult with Cystic Fibrosis: A mixed-methods case study.

Respir Med Case Rep. 2020;31:101173

Authors: Aspinall SP, Hill DM, Mackintosh KA, McNarry MA

Abstract
Although aerobic capacity has been identified as an important predictor of mortality in Cystic Fibrosis (CF) individuals, many remain insufficiently active. As a 'lack of time' is a commonly cited barrier to exercise, reduced-exertion high-intensity interval training (REHIT) may provide a truly time-effective method to increase aerobic capacity. Six-weeks of REHIT in a CF individual was assessed by a cardiopulmonary exercise test (CPET) and individual perceptions described using a self-report narrative. Peak oxygen uptake ( V ˙ O2peak) increased by 6% whilst pulmonary function remained unchanged. Qualitative data indicated social support and low-time commitment positively influenced adherence with fatigue and lack of enjoyment noted as a significant barriers. REHIT was demonstrated as a viable, manageable option for a CF individual with moderate-severe pulmonary limitation. Further research is needed to determine the efficacy of REHIT in a large representative sample to ascertain whether it represents an alternative treatment strategy.

PMID: 32775190 [PubMed - as supplied by publisher]

Pancreatic complications in children with cystic fibrosis.

Curr Opin Pediatr. 2020 Aug 06;:

Authors: Sellers ZM

Abstract
PURPOSE OF REVIEW: The pancreas is highly affected in cystic fibrosis, with complications occurring early in childhood. This review highlights recent research in exocrine pancreatic function in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies and discusses how these are affecting pancreatitis and exocrine pancreatic insufficiency (EPI) in children. Additionally, new research into exocrine--endocrine interactions sheds light on how CFTR dysfunction in ductal cells may affect beta cells.
RECENT FINDINGS: Ivacaftor has disproved the hypothesis that EPI in children with cystic fibrosis is irreversible. Improvements in pancreatic function have increased pancreatitis episodes in some children and reduced them in others. Imaging advances are providing complementary methods for exocrine pancreatic function testing. New research into the interplay between the exocrine and endocrine components of the pancreas are elucidating the intertwined and complex relationship between the exocrine and endocrine pancreas.
SUMMARY: Pancreatic complications contribute to the morbidity and mortality of children with cystic fibrosis. Increasing use of highly effective CFTR modulators will not only abrogate these but will also advance our understanding of pancreatic pathophysiology in cystic fibrosis. New frontiers into pancreatic gene therapy and exocrine--endocrine research will help provide new therapeutic opportunities for pancreatitis, EPI, and diabetes in cystic fibrosis.

PMID: 32773577 [PubMed - as supplied by publisher]

Carriers of cystic fibrosis among sperm donors: complete CFTR gene analysis versus CFTR genotyping.

Fertil Steril. 2020 Aug 06;:

Authors: Molina M, Yoldi A, Navas P, Gañán M, Vaquero Á, Del Pico JL, Ramírez JP, Castilla JA

Abstract
OBJECTIVE: To determine the frequency of cystic fibrosis (CF) carriers among sperm donors in Spain studied through a complete analysis of the CFTR gene and to compare the results with those that would have been obtained by the 4 genotyping panels of the CFTR gene most commonly used as a carrier test in the context of assisted reproduction in our country.
DESIGN: Descriptive observational study.
SETTING: Private center.
PATIENTS: Nine hundred thirty-five sperm donors, from January 2014 to June 2019.
INTERVENTION: None.
MAIN OUTCOME MEASURE: Presence of pathogenic variants in the CFTR gene.
RESULTS: 17% of the donors were carriers of at least 1 pathogenic variant in CFTR, with 39 different pathogenic variants detected. Only 4 of these 39 variants (10.27%) would have been detected by the 4 genotyping tests considered, and 22 variants (56.41%) would not have been detected by any of the genotyping tests. The pathogenic variants of the CFTR gene included in the different genotyping tests analyzed vary widely, and <50% are common to all of them.
CONCLUSIONS: Although the was not based in the general population, these results show that the use of genotyping tests is associated with a high reproductive risk, because the rate of detection of CF carriers was lower when these panels were applied, in comparison with the complete study of the CFTR gene. We recommend that complete sequencing of the CFTR gene by next-generation sequencing be performed as a screening method for CF in sperm donors.

PMID: 32773111 [PubMed - as supplied by publisher]

A mutation in NOTCH2 gene first associated with Hajdu-Cheney syndrome in a Greek family: diversity in phenotype and response to treatment.

Endocrine. 2020 Aug 09;:

Authors: Efstathiadou ZA, Kostoulas C, Polyzos SA, Adamidou F, Georgiou I, Kita M

Abstract
INTRODUCTION: Hajdu-Cheney Syndrome (HCS) is a rare genetic autosomal dominant disorder, characterized by distinctive facial features, acroosteolysis, and severe osteoporosis. Very rarely HCS is associated with polycystic kidney disease, splenomegaly or Crohn's disease (CD). It is caused by gain-of-function mutations in NOTCH2 gene. Treatment with bisphosphonates or denosumab is reported to result in BMD increase.
OBJECTIVE: We report a mutation in exon 34 of NOTCH2 gene, in a Greek pedigree, with diverse phenotypes among members.
DESCRIPTION OF THE PEDIGREE: The 48-year-old mother had a history of a T12 vertebral fracture, postpartum at the age of 21 and two subsequent uneventful full-term pregnancies and never received treatment. Her 29-year-old son, presented with severe osteoporosis and multiple morphological vertebral fractures. Her 21-year-old daughter had recurrent vertebral fractures starting at 10 years of age. At 17 years, she developed severe CD, resistant to treatment with biologic agents, and functional hypothalamic hypogonadism. One male pedigree died of cystic fibrosis. All subjects bore the typical facial characteristics and acroosteolysis, while none had splenomegaly or renal defects. Zoledronate infusion led to BMD increase.
GENETIC TESTING: Mutation in c.6758 G > A (NM_008163.1), leading to a Trp2253Ter replacement. This mutation has been reported as possibly pathogenic (SCV000620308), but not in association with HCS.
CONCLUSIONS: Bone involvement can present with diverse severity in the same pedigree, ranging from low BMD to multiple fragility fractures. Antiresorptive therapy improves BMD, but its anti-fracture efficacy remains to be shown. The presence of CD might indicate the significant role of NOTCH2 signaling in different tissues.

PMID: 32772338 [PubMed - as supplied by publisher]

Microbiota-derived metabolites as diagnostic markers for respiratory fungal infections.

J Pharm Biomed Anal. 2020 Jul 15;189:113473

Authors: Hérivaux A, Gonçalves SM, Carvalho A, Cunha C

Abstract
An emerging body of evidence has highlighted the significant role of the pulmonary microbiota during respiratory infections. The individual microbiome is nowadays recognized to supervise the outcome of the host-pathogen interaction by orchestrating mechanisms of immune regulation, inflammation, metabolism, and other physiological processes. A shift in the normal flora of the respiratory tract is associated with several lung inflammatory disorders including asthma, chronic obstructive pulmonary disease, or cystic fibrosis. These diseases are characterized by a lung microenvironment that becomes permissive to infections caused by the opportunistic fungal pathogen Aspergillus fumigatus. Although the role of the lung microbiota in the pathophysiology of respiratory fungal diseases remains elusive, microbiota-derived components have been proposed as important biomarkers to be considered in the diagnosis of these severe infections. Here, we review this emerging area of research and discuss the potential of microbiota-derived products in the diagnosis of respiratory fungal diseases.

PMID: 32771720 [PubMed - as supplied by publisher]

Quality Improvement Initiative to Improve Pulmonary Function in Pediatric Cystic Fibrosis Patients.

Pediatr Pulmonol. 2020 Aug 08;:

Authors: Filbrun AG, Enochs C, Caverly L, Rajala K, Powell C, Merrick E, Nasr SZ

Abstract
BACKGROUND: Our Cystic Fibrosis (CF) Center initiated a Quality Improvement (QI) project in November 2017 with the goal of improving our patients' Forced Expiratory Volume in One Second (FEV1) percent predicted (pp) and continued for 1 year. Our specific aim was to increase the relative mean FEV1 pp by 5% in 12 months for CF patients 6-21 years old with FEV1 ≤80 pp.
METHODS: We identified patients with FEV1 ≤80 pp, developed cause and effect diagrams (fishbones) to identify contributing factors to FEV1 ≤80 pp, and created flowcharts to address barriers. The barriers to adherence that may result in FEV1 ≤80 pp were studied using a fishbone. A standardized approach across providers was implemented to individualize care for each patient. Each discipline developed a flowchart to address barriers to improving FEV1.
RESULTS: Forty patients were identified (43% male). Their mean age was 16.8 years (range 8.2-21.5 years). Mean FEV1 pp at baseline was 58.6 (range 30-80). The fishbone identified needs for continuing education for patients/families, and providing a treatment plan at each clinic visit. After 6 months of implementation, patients had an improvement in mean FEV1 pp by 6.4% (CI 0.4%-12.9%). At 12 months, mean FEV1 pp had improved by 14% (CI 6.5%-21.4%) which exceeded our goal of 5%.
CONCLUSION: Through this ongoing project, team members, patients, and families partnered to improve lung function in pediatric CF patients. Flowcharts facilitated a standardized approach across providers to develop individualized treatment plans for patients, which resulted in improved lung function. This article is protected by copyright. All rights reserved.

PMID: 32770822 [PubMed - as supplied by publisher]