AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesDisciplines and OccupationsAnthropology, Education, Sociology and Social PhenomenaInformation ScienceNamed GroupsHealth CareGeographicals
Cystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorFibrosisMortalityPseudomonas InfectionsPseudomonas aeruginosaHospital MortalitySweatSputumInfant MortalityMice, Inbred CFTRBurkholderia InfectionsBurkholderia cepaciaCause of DeathLungChloridesIdiopathic Pulmonary FibrosisEndomyocardial FibrosisLiver CirrhosisExocrine Pancreatic InsufficiencyTime FactorsRetrospective StudiesChloride ChannelsChild MortalityRespiratory MucosaCohort StudiesTobramycinTreatment OutcomeForced Expiratory VolumeInfant, NewbornNasal MucosaNeonatal ScreeningProspective StudiesPancreatinFollow-Up StudiesLung DiseasesMaternal MortalityRetroperitoneal FibrosisRespiratory Function TestsBurkholderia cepacia complexRespiratory SystemBronchiectasisAnti-Bacterial AgentsPancreatic ExtractsMutationEpithelial CellsLung TransplantationProportional Hazards ModelsAge FactorsSurvival AnalysisBronchiMucusSurvival RateSeverity of Illness IndexHeterozygote DetectionDisease Models, AnimalMeconiumExpectorantsAlginatesPrognosisUnited StatesBleomycinRisk AssessmentChronic DiseaseIncidencePancrelipaseTracheaBiological MarkersAchromobacter denitrificansMucociliary ClearanceBurkholderia cenocepaciaSweat GlandsCardiovascular DiseasesPhenotypeRespiratory Tract InfectionsGenotypeSex FactorsVital CapacityPredictive Value of TestsMortality, PrematureAminophenolsAspergillosis, Allergic BronchopulmonaryRisk FactorsCell LineLeukocyte ElastaseIon TransportOral Submucous FibrosisAdministration, InhalationMice, Inbred C57BLPerinatal MortalityRegistriesLiver Cirrhosis, ExperimentalAge DistributionGenetic TestingCells, CulturedDisease ProgressionEpitheliumAmiloridePneumoniaColistin
MorbidityPeople with cystic fibrosisDiagnosed with non-cystic fibrosis bronchiectasisCFTRBronchiectasisIdiopathic pulmonarEpidemiologyCFRDPseudomonasInterstitial Lung DiSurvivalManifestationsClinicalPulmonary diseaseAdultsSystematic ReviewLife expectancyDiagnosisBile ductsChronicCOPDDeterminantsSignificantlyNewbornHigher mortality ratesIndependent predictorHigh mortalityDilatationPathogenesisPancreasAutosomalDysfunctionGenetic disorderInfant mortalityRespiratory failurePrevalenceImprovements2019AffectsCFLDDiseaseOutcomeReductionCongenitalDeteriorationAdultPancreaticBurkholderia
Morbidity16
  • The primary cause of morbidity and death in people with cystic fibrosis is progressive lung disease, which eventually leads to respiratory failure. (wikipedia.org)
  • 7 This project will use a coordinated, comprehensive public health approach to improve prevention, early diagnosis, treatment, and management of COPD, by engaging a network of strategic community partners to increase knowledge and adoption of evidence-based strategies that improve COPD morbidity and mortality across the United States. (cdc.gov)
  • Our goal is to improve access to care for people with cystic fibrosis to improve outcomes associated with morbidity and mortality. (kumc.edu)
  • S19.1: Pts with CFRD have a 6-fold increase in morbidity and mortality. (boardhost.com)
  • Recommendations and reports : Morbidity and mortality weekly report. (cdc.gov)
  • While improvements in clinical care have led to decreased mortality of individuals with CF, pulmonary morbidity (often leading to in-patient admissions) had not shown similar decreases prior to ivacaftor. (cdc.gov)
  • Non-resolving inflammation is the main mechanism of morbidity and mortality among patients suffering from cystic fibrosis (CF), the most common life-threatening human genetic disease. (frontiersin.org)
  • Articles met our inclusion criteria if they were (1) randomized clinical trials, (2) observational or epidemiological studies, (3) assessments of the prevalence of IDA in selected disease populations, and (4) studies that correlated IDA with quantitative outcome measures, including morbidity, mortality, survival rate, healthcare utilization, or cost. (ispub.com)
  • Despite remarkable improvements in morbidity and mortality during the last decades, the disease still limits survival and r. (springeropen.com)
  • Mycobacterium abscessus Complex (MABSC) and Mycobacterium avium Complex (MAC) are now recognized as insidious opportunists that can seriously affect morbidity and mortality in CF. Fundamental paradigms of NTM pulmonary disease concerning clinical impact, patient susceptibility, transmission, bacterial modes of growth and implications for lung transplantation are currently being revised. (springer.com)
  • CF is a multisystem disease, principally manifesting in the lungs and up to 95% of morbidity and mortality occurs due to chronic airway infections. (tcd.ie)
  • Respiratory infections are the leading cause of morbidity and mortality in cystic fibrosis. (bmj.com)
  • Although most of its morbidity and mortality stems from pulmonary decline, it was first recognized in 1938 as a cause of severe failure to thrive in infants due to characteristic pancreatic insufficiency. (contemporarypediatrics.com)
  • Cystic fibrosis associated liver disease (CFLD) is emerging as a cause of morbidity and mortality. (pucrs.br)
  • Community-acquired pneumonia (CAP) is one of the most common infectious diseases and an important cause of mortality and morbidity worldwide. (medscape.com)
  • 3. Goal of the WHO strategy against CRDs is for Prevention and Control of to support Member States in their efforts to Chronic Respiratory Diseases reduce the toll of morbidity, disability and (CRDs)1 that was drafted after the expert consultation premature mortality related to chronic held in January 20012. (who.int)
People with cystic fibrosis8
  • Lung problems are responsible for death in 80% of people with cystic fibrosis. (wikipedia.org)
  • Chronic infection of the respiratory tract is nearly universal in people with cystic fibrosis, with Pseudomonas aeruginosa, fungi, and mycobacteria all increasingly common over time. (wikipedia.org)
  • Poor cardiorespiratory fitness is a strong, independent predictor of mortality in people with cystic fibrosis. (kumc.edu)
  • Exercise training has become a valued element of care for improving cardiorespiratory fitness in people with cystic fibrosis. (kumc.edu)
  • The results from this work will inform us on the most effective pulmonary rehabilitation strategies to elicit the greatest improvement in cardiorespiratory fitness and other important patient-centered health outcomes in people with cystic fibrosis. (kumc.edu)
  • The CF Foundation Patient Registry collects information on the health status of people with cystic fibrosis who receive care in CF Foundation-accredited care centers and agree to participate in the Registry. (cff.org)
  • There are 253 million people living with a visual impairment and 70, 000 people with cystic fibrosis. (windowscentral.com)
  • Life expectancy for people with Cystic Fibrosis (PWCF) has improved dramatically over the last 2 decades. (hrb.ie)
Diagnosed with non-cystic fibrosis bronchiectasis1
CFTR10
  • METHODS Polymorphisms in the TGF-β 1 gene defining amino acids of codons 10 and 25 were determined by ARMS-PCR using DNA stored on 171 Caucasian patients who were homozygous for the ΔF508 mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. (bmj.com)
  • Despite advances in the medical management of cystic fibrosis over the last three decades, there are still a number of unexplained differences in its clinical course, especially in the rate of development of respiratory failure of patients who appear to have the same CFTR genotype, degree of bacterial colonisation, and compliance with medication. (bmj.com)
  • It is caused by the presence of mutations in both copies (alleles) of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. (wikipedia.org)
  • The sensitivity and specificity of such testing can now be improved as a result of the recent discovery of the Cystic Fibrosis Transmembrane Conductance Regulatory (CFTR) gene. (cdc.gov)
  • The discovery of the Cystic Fibrosis Transmembrane Conductance Regulatory (CFTR) gene (5) renewed interest in this possibility, as the sensitivity and specificity of testing could be improved through DNA-based testing. (cdc.gov)
  • A prime example of precision medicine is ivacaftor, a small molecule drug originally developed to treat the G551D CFTR gene variant that causes about 3-4% of Cystic fibrosis (CF) cases. (cdc.gov)
  • He participates in research involving gene correction of CFTR in lung stem cells from patients with Cystic fibrosis followed by autologous stem cell transplantation to provide lifelong restoration of physiological activity. (stanford.edu)
  • It's known that CF is associated with a protein known as cystic fibrosis transmembrane conductance regulator (CFTR), the production of which is encoded by the CFTR-gene. (sensiseeds.com)
  • Expression of the cystic fibrosis transmembrane conductance regulator gene ( CFTR ) can be detected in the pancreatic ductules at 18 weeks' gestation. (medscape.com)
  • METHODS: The model projects the impact of ivacaftor vs. standard care in people with CF aged ≥6 years with CFTR gating mutations by combining parametric equations fitted to historical registry survival data, with mortality hazards adjusted for fixed and time-varying person-level characteristics. (bvsalud.org)
Bronchiectasis10
Idiopathic pulmonar6
Epidemiology2
CFRD6
  • Patients with CFRD have worse lung function, poorer nutrition, more frequent hospitalization, and higher mortality rates than those affected by CF but without diabetes. (newswire.ca)
  • Patients with cystic fibrosis experience decreased nutritional and pulmonary health several years before they're diagnosed with cystic fibrosis-related diabetes (CFRD). (diabeticlive.com)
  • According to Amanda Leonard, a senior pediatric clinical dietician at the Johns Hopkins Cystic Fibrosis Center, early diagnosis and treatment can significantly improve life expectancy in patients who develop CFRD. (diabeticlive.com)
  • Patients with CFRD typically experience decline in lung function, protein catabolism, weight loss, and an increased rate of mortality. (diabeticlive.com)
  • In cystic fibrosis patients at the age of 40, the rate of CFRD is over 50 percent. (diabeticlive.com)
  • Nutritional recommendations for patients with CFRD are based on those for patients with cystic fibrosis. (diabeticlive.com)
Pseudomonas6
  • Isles A , Maclusky I , Corey M , Gold R , Prober C , Fleming P , Pseudomonas cepacia infection in cystic fibrosis: an emerging problem. (cdc.gov)
  • Govan JR , Brown PH , Maddison J , Doherty CJ , Nelson JW , Dodd M , Evidence for transmission of Pseudomonas cepacia by social contact in cystic fibrosis. (cdc.gov)
  • Person-to-person transmission of Pseudomonas cepacia between patients with cystic fibrosis. (cdc.gov)
  • Pegues DA , Carson LA , Tablan OC , FitzSimmons SC , Roman SB , Miller JM , Acquisition of Pseudomonas cepacia at summer camps for patients with cystic fibrosis. (cdc.gov)
  • Pseudomonas aeruginosa infections are the leading cause of lung dysfunction and mortality in Cystic Fibrosis (CF) patients. (auburn.edu)
  • Chronic infection with Pseudomonas aeruginosa is associated with an adverse prognosis in adults with cystic fibrosis (CF). 1 P aeruginosa induces a prolonged inflammatory response which leads to progressive pulmonary tissue destruction. (bmj.com)
Interstitial Lung Di2
  • 2) Eisenmenger syndrome, (3) congenital heart disease without Eisenmenger syndrome, (4) idiopathic pulmonary arterial hypertension (IPAH), (5) cystic fibrosis, (6) chronic obstructive pulmonary disease (COPD) and/or emphysema), and (7) diffuse interstitial lung disease. (unican.es)
  • Fibrosis and diffuse interstitial lung disease. (who.int)
Survival8
  • Aerobic fitness, as measured by peak oxygen uptake (VÌ O2 peak), correlates with survival in children and adults with cystic fibrosis (CF). We sought to evaluate the effects of VÌ O2 peak on mortality rates in subjects with CF. (bvsalud.org)
  • People like me are living longer - median survival for someone with cystic fibrosis is now 56 thanks to research and care. (cff.org)
  • Clinical outcomes of interest included mortality and survival rates. (ispub.com)
  • A multivariate Cox proportional hazards model showed higher RI-SUV and higher extent of fibrosis score as independent predictors of shorter progression-free survival. (snmjournals.org)
  • Several physiologic and radiologic parameters that predict poor survival in patients with IPF have been identified, including lower forced vital capacity (FVC), diffusion capacity for carbon monoxide (DL CO ), and extent of fibrosis shown by high-resolution CT (HRCT) ( 8 - 10 ). (snmjournals.org)
  • The aim of this study is to determine the association between severity of liver disease and survival in patients with cystic fibrosis. (pucrs.br)
  • A directed acyclic graph was used to assess possible predictors of survival in cystic fibrosis patients. (pucrs.br)
  • We used real-world evidence of 5-year mortality impacts of ivacaftor in a US registry population to validate a CF disease-progression model that estimates the impact of ivacaftor on survival. (bvsalud.org)
Manifestations3
Clinical6
  • Acute severe clinical deterioration of patients with cystic fibrosis (CF) may mandate endotracheal intubation. (nih.gov)
  • ic" or the " Corporation") today reported that it had been approved to commence the clinical trial of its orally active anti-fibrotic lead drug candidate, PBI-4050, in patients suffering from cystic fibrosis ("CF"), following the CTA clearance by Health Canada. (newswire.ca)
  • It is the earliest clinical manifestation of cystic fibrosis (CF) and occurs in either a simple or a complicated form in approximately 16-20% of patients who have CF, though it also occurs in patients who do not have CF. (medscape.com)
  • The study will compare the time to a composite endpoint of relative decline in lung function [10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplantation, or all-cause mortality] The secondary objectives will be to examine the effect of NAC on the components of the primary composite endpoint, the rates of clinical events, change in physiology, change in health status, and change in respiratory symptoms. (stanford.edu)
  • Patients at the Queensland Children's Hospital Cystic Fibrosis Service (Brisbane, Australia) were evaluated in a 27-year clinical cohort study. (pucrs.br)
  • Because of the variable clinical presentations, congenital hepatic fibrosis is believed to represent a broad spectrum of hepatic and renal lesions rather than a single clinical entity. (medscape.com)
Pulmonary disease1
  • Important paradigms of pulmonary disease with nontuberculous mycobacteria (NTM) are currently shifting based on an increasing attention within the field of cystic fibrosis (CF). These shifts are likely to benefit the management of all patients with pulmonary NTM, regardless of underlying pathology. (springer.com)
Adults2
  • Aggressive antibiotic eradication therapy of new isolation P aeruginosa has similar efficacy in adults and children with cystic fibrosis. (bmj.com)
  • 2006). Determinants of mortality for adults with cystic fibrosis admitted in intensive care unit: A multicenter study [Eletronic version]. (bvsalud.org)
Systematic Review3
  • Peak Oxygen Uptake and Mortality in Cystic Fibrosis: Systematic Review and Meta-Analysis. (bvsalud.org)
  • In this issue, Chen and colleagues report the results of a systematic review and meta-analysis to determine if inhaled therapy containing ICSs reduces all-cause mortality. (chestnet.org)
  • 2020. Impact of colorectal cancer screening on cancer-specific mortality in Europe: A systematic review. (who.int)
Life expectancy4
Diagnosis1
Bile ducts2
  • Pathologically, it is defined by its variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. (medscape.com)
  • Congenital hepatic fibrosis is a ductal plate malformation of the small interlobular bile ducts, whereas Caroli disease involves the large intrahepatic bile ducts. (medscape.com)
Chronic6
  • Cystic fibrosis (CF) is a disease of exocrine gland function that involves multiple organ systems but chiefly results in chronic respiratory infections, pancreatic enzyme insufficiency, and associated complications in untreated patients. (medscape.com)
  • Her research focuses on characterising the diverse community of microorganisms in the airways of people with chronic lung diseases, such as cystic fibrosis (CF), and understanding how these microbial communities or the 'microbiome' impacts disease progression. (tcd.ie)
  • Cystic Fibrosis is a chronic condition where excess, sticky mucus builds up in the organs of an affected person's body. (windowscentral.com)
  • Acute kidney injury (AKI) is a life-threatening disease with high mortality characterized by an abrupt decrease of the kidney glomerular filtration rate, extra-kidney consequences (cardiovascular diseases, lung injury, neurological impairment) and high risk of secondary chronic kidney disease (CKD). (hrb.ie)
  • More patients with cystic fibrosis are being transferred from paediatric to adult CF services without a history of chronic P aeruginosa infection. (bmj.com)
  • Data suggest a possible increase in mortality and in the incidence of heart failure with long-term use of vitamin E (400 IU or more) in patients with chronic diseases. (medscape.com)
COPD5
  • Other indications for HLT have virtually disappeared, mainly lung diseases (IPAH, COPD, cystic fibrosis). (unican.es)
  • and longer in IPAH, COPD and/or emphysema, and cystic fibrosis. (unican.es)
  • My lab is focused on understanding the causes of and working towards specific molecular and cell-based treatments for lung diseases like cancer, pulmonary fibrosis, COPD. (stanford.edu)
  • It is unclear if inhaled corticosteroid (ICS) therapy can reduce all-cause mortality in patients or subgroups of patients with COPD. (chestnet.org)
  • They found that ICSs, particularly when included as triple therapy, were associated with a reduction in all-cause mortality among patients with COPD (OR 0.73). (chestnet.org)
Determinants1
Significantly3
  • 9 Previous animal and human studies have shown that high TGF-β 1 producers develop significantly more lung fibrosis in response to a number of inflammatory triggers such as radiation, 10 chemotherapy, 11 and lung transplantation. (bmj.com)
  • In addition, subjects with a lower VÌ O2 peak had a significantly higher mortality risk ( relative risk 4.896, 95% CI = 1.086 to 22.072, P = .039) in an 8-y follow-up period. (bvsalud.org)
  • however, the risk of mortality while waiting for that procedure is significantly greater for patients with IPF than for those with cystic fibrosis or emphysema ( 6 , 7 ). (snmjournals.org)
Newborn3
  • Cystic fibrosis (CF) is a genetic disease that can be detected in newborn infants (i.e., those aged less than or equal to 1 month) by immunotrypsinogen testing. (cdc.gov)
  • Since the development of the immunoreactive trypsinogen test (IRT) for cystic fibrosis (CF), experts in the field of CF have considered adding this test to the newborn screening panel. (cdc.gov)
  • Title : Improving newborn screening for cystic fibrosis using next-generation sequencing technology: a technical feasibility study Personal Author(s) : Baker, Mei W.;Atkins, Anne E.;Cordovado, Suzanne K.;Hendrix, Miyono;Earley, Marie C.;Farrell, Philip M. (cdc.gov)
Higher mortality rates1
  • This is especially true for women with the disease, who tend to have higher mortality rates and shorter lifespans. (monaghanmed.com)
Independent predictor1
  • In the elderly, anemia is an independent predictor of fragility, function, and mortality(4;5). (ispub.com)
High mortality1
Dilatation1
  • The family of fibropolycystic diseases are characterized by varying degrees of persistent bile duct structures, fibrosis, and duct dilatation. (medscape.com)
Pathogenesis1
  • However, the exact pathogenesis of association between congenital hepatic fibrosis and ADPKD still requires further research and study. (medscape.com)
Pancreas5
  • Cystic fibrosis (CF) is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. (wikipedia.org)
  • The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas. (wikipedia.org)
  • We have already demonstrated a significant improvement in the blood glucose levels in patients with metabolic syndrome with Type 2 diabetes treated with PBI-4050, and a significant reduction of fibrosis in the lungs, pancreas and liver in multiple animal models. (newswire.ca)
  • We believe that the unique mode of action of PBI-4050 could indeed provide much relief for the CF patients by reducing the level of fibrosis in their lungs and pancreas and by improving the production of insulin" concluded Dr. Moran. (newswire.ca)
  • Cystic fibrosis (CF), also known as mucoviscidosis, is a life-threatening, genetic disease that affects especially the lungs but also the pancreas, liver, kidneys, and gut. (newswire.ca)
Autosomal5
  • Cystic fibrosis is inherited in an autosomal recessive manner. (wikipedia.org)
  • Cystic fibrosis (CF) is an autosomal recessively inherited disease, caused by mutations in the CF gene located at chromosome 7. (hindawi.com)
  • Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the United States, occurring in 2000 to 4000 newborns. (contemporarypediatrics.com)
  • Congenital hepatic fibrosis (CHF) is an autosomal recessive disease that primarily affects the hepatobiliary and renal systems. (medscape.com)
  • Congenital hepatic fibrosis is one of the fibropolycystic diseases, which also include Caroli disease , autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). (medscape.com)
Dysfunction3
  • This study was undertaken to examine the association between TGF-β 1 gene polymorphisms and the development of pulmonary dysfunction in patients with cystic fibrosis. (bmj.com)
  • DISCUSSION TGF-β 1 genotypes may have a role in mediating pulmonary dysfunction in patients with cystic fibrosis. (bmj.com)
  • 12 In this study we have investigated the correlation between high TGF-β 1 production and pulmonary dysfunction in cystic fibrosis by determination of polymorphisms in the TGF-β 1 gene. (bmj.com)
Genetic disorder2
Infant mortality1
Respiratory failure1
  • In this issue, Zakrajsek and colleagues report the findings of a retrospective, observational cohort study designed to determine if treatment with ECMO is associated with lower mortality in refractory asthma exacerbations with respiratory failure. (chestnet.org)
Prevalence3
  • The prevalence of nasal polyps is increased in children with cystic fibrosis and persons with known aspirin hypersensitivity. (medscape.com)
  • The prevalence is increased in patients with cystic fibrosis and aspirin-hypersensitivity triad. (medscape.com)
  • Obesity associated sleep related breathing disorders (which are associated with hypertension and excess cardio and cerebrovascular mortality) is also increasingly being recognised in South Asia and also probably has a higher prevalence amongst Afro Caribbeans. (who.int)
Improvements1
  • Significant improvements in cystic fibrosis (CF) care have focused primarily on the pulmonary system, but addressing the gastrointestinal complications of CF presents a major opportunity for improvement in disease management. (contemporarypediatrics.com)
20191
  • Nov. 8, 2021: NIMHD-supported study analyzes U.S. state-level mortality due to police violence from 1980 to 2019 that disaggregates by race and ethnicity. (nih.gov)
Affects1
  • Congenital hepatic fibrosis is characterized by the intrahepatic form of portal hypertension, which is caused by the intrahepatic obstruction that affects the blood supply to the liver and subsequently leads to the development of cavernous transformations of the portal vein with a rise in portal venous pressure. (medscape.com)
CFLD2
  • In the Irish Longitudinal Study of CFLD (ILSCFLD), we found that those with CFLD had a mortality rate difference of 6.6 versus those with no liver. (hrb.ie)
  • The study shows evidence that severe CFLD may be associated with an increased risk of death in patients with cystic fibrosis when adjusted for sex, diabetes and ursodeoxycholic acid therapy. (pucrs.br)
Disease6
  • Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. (hindawi.com)
  • Announced as the most important emerging threat to cystic fibrosis (CF) patients at the 2013 European Cystic Fibrosis Conference, the perception of nontuberculous mycobacteria (NTM) disease has once again undergone a notable shift. (springer.com)
  • In addition to Type 1 and Type 2 diabetes, there is another variant of the disease: cystic fibrosis-related diabetes. (diabeticlive.com)
  • OBJECTIVES: Cystic fibrosis (CF) is a rare genetic disease characterized by life-shortening lung function decline. (bvsalud.org)
  • It is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. (medscape.com)
  • Congenital hepatic fibrosis is associated with an impairment of renal functions, usually caused by an ARPKD, which is a severe form of polycystic kidney disease . (medscape.com)
Outcome1
  • The main outcome evaluated was the mortality of subjects with CF. Whenever possible, and if appropriate, a random effect meta-analysis was performed. (bvsalud.org)
Reduction1
  • Treatment duration of >6 months (OR 0.9), the use of medium-dose ICSs (0.71), the use of low-dose ICSs (0.88), and the use of budesonide (0.75) were all associated with a reduction in all-cause mortality. (chestnet.org)
Congenital4
  • Congenital hepatic fibrosis results from a malformation of the ductal plate (the embryological precursor of the biliary system), secondary biliary strictures, and periportal fibrosis. (medscape.com)
  • The classic renal lesion associated with congenital hepatic fibrosis is ARPKD, which results in an impairment of renal functions. (medscape.com)
  • The relationship of ARPKD to congenital hepatic fibrosis remains a controversial issue. (medscape.com)
  • [ 9 ] Most cases of ARPKD and congenital hepatic fibrosis are genetically homogeneous. (medscape.com)
Deterioration2
  • RESULTS Patients with cystic fibrosis of a TGF-β 1 high producer genotype for codon 10 had more rapid deterioration in lung function than those with a TGF-β 1 low producer genotype. (bmj.com)
  • Our results demonstrate that positive RI-SUV is strongly predictive of earlier deterioration of pulmonary function and higher mortality in patients with IPF. (snmjournals.org)
Adult1
  • 2 National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland. (jci.org)
Pancreatic1
  • In patients with CF, abnormal pancreatic secretions obstruct the duct system, leading to autodigestion of the acinar cells, fatty replacement, and ultimately, fibrosis. (medscape.com)
Burkholderia3