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Hepatology (Baltimore, Md.) May 2024Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on...
Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection and monitoring of hepatobiliary complications in CF were published in 1999. The CF Foundation assembled a committee to evaluate research advances and formulate revised guidelines for CF-associated liver disease. A committee of hepatologists, gastroenterologists, pulmonologists, pharmacists, nurses, dietitians, individuals with CF, and the parents of a child with CF devised "population, intervention, comparison, and outcome" questions regarding hepatobiliary disease in CF. PubMed literature searches were performed for each population, intervention, comparison, and outcome question. Recommendations were voted on with 80% agreement required to approve a recommendation. Public comment on initial recommendations was solicited prior to the formulation of final recommendations. Thirty-one population, intervention, comparison, and outcome questions were assembled, 6401 manuscripts were title screened for relevance, with 1053 manuscripts undergoing detailed full-text review. Seven recommendations were approved for screening, 13 for monitoring of existing disease, and 14 for treatment of CF-associated hepatobiliary involvement or advanced liver disease. One recommendation on liver biopsy did not meet the 80% threshold. One recommendation on screening ultrasound was revised and re-voted on. Through a multidisciplinary committee and public engagement, we have assembled updated recommendations and guidance on screening, monitoring, and treatment of CF-associated hepatobiliary involvement and advanced liver disease. While research gaps remain, we anticipate that these recommendations will lead to improvements in CF outcomes through earlier detection and increased evidence-based approaches to monitoring and treatment.
Topics: Child; Humans; Cystic Fibrosis; Consensus; Mass Screening; Hypertension, Portal; Liver Cirrhosis
PubMed: 37934656
DOI: 10.1097/HEP.0000000000000646 -
Journal of Thoracic Imaging Nov 2023Idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unclear etiology and are distinguished from diffuse parenchymal lung... (Review)
Review
Idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unclear etiology and are distinguished from diffuse parenchymal lung diseases of known cause, such as connective tissue disease-related interstitial lung diseases or hypersensitivity pneumonitis by history, physical exam, imaging, serologic testing, and, when necessary, histopathology. The 2013 American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines are the most widely accepted classification of IIPs and include the following diagnoses: idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, idiopathic lymphocytic interstitial pneumonia, idiopathic pleuro-parenchymal fibroelastosis, respiratory bronchiolitis-interstitial lung disease, and desquamative interstitial pneumonia. The gold standard for diagnosis of IIP involves multidisciplinary discussion among pulmonologists, radiologists, and pathologists. The focus of this review will be to discuss the imaging features of the most common IIPs and the role of multidisciplinary discussion as the gold standard for diagnosis.
Topics: Humans; Diagnosis, Differential; Lung Diseases, Interstitial; Idiopathic Interstitial Pneumonias; Idiopathic Pulmonary Fibrosis; Pneumonia; Lung
PubMed: 37505195
DOI: 10.1097/RTI.0000000000000728 -
Ugeskrift For Laeger Jan 2024Interstitial lung abnormalities (ILA) are incidentally observed specific CT findings in patients without clinical suspicion of interstitial lung disease (ILD). ILA with... (Review)
Review
Interstitial lung abnormalities (ILA) are incidentally observed specific CT findings in patients without clinical suspicion of interstitial lung disease (ILD). ILA with basal and peripheral predominance and features suggestive of fibrosis in more than 5% of any part of the lung should be referred for pulmonologist review. The strategy for monitoring as described in this review is based on clinical and radiological risk factors. ILA are associated with risk of progression to ILD and increased mortality. Early identification and assessment of risk factors for progression are essential to improve outcome.
Topics: Humans; Disease Progression; Lung Diseases, Interstitial; Lung; Risk Factors; Risk Assessment
PubMed: 38235774
DOI: 10.61409/V06230395 -
Pneumologie (Stuttgart, Germany) Aug 2023Chronic cough (i.e., cough lasting >8 weeks) has a global prevalence of approximately 10%. The individual burden can be long-lasting, with some patients experiencing...
Chronic cough (i.e., cough lasting >8 weeks) has a global prevalence of approximately 10%. The individual burden can be long-lasting, with some patients experiencing cough for many years. Although chronic cough is often a symptom of respiratory diseases (e.g., lung cancer, tuberculosis, chronic obstructive pulmonary disease) or associated with triggers (e.g., asthma, gastroesophageal reflux disease, rhinosinusitis) and may resolve after targeted treatment of these conditions, some patients continue to cough despite optimal treatment (refractory chronic cough, RCC) or have no identifiable conditions presumed to be contributing to the cough (unexplained chronic cough, UCC). In patients with chronic cough, it is critical to perform a thorough initial patient assessment with adherence to a diagnostic algorithm (e.g., of the German Respiratory Society Cough Guidelines) to identify the cause of the symptom cough and provide appropriate treatment; or diagnose RCC and UCC. Primary care physicians should provide the initial diagnostic workup of patients with chronic cough (history, physical exam, chest X-ray and spirometry). If no cause of the cough can be identified, referral to specialists (e.g., pulmonologists, gastroenterologists, otolaryngologists) may be appropriate. Increased appreciation of chronic cough as a distinct condition, rather than as only a symptom of other diseases, may help overcome current challenges in diagnosing and managing chronic cough.
Topics: Humans; Cough; Carcinoma, Renal Cell; Pulmonary Disease, Chronic Obstructive; Asthma; Kidney Neoplasms
PubMed: 37619576
DOI: 10.1055/a-1849-4345 -
Ugeskrift For Laeger Jan 2024Extrapulmonary tuberculosis (TB) in the gastrointestinal tract is a rare, but yet an essential differential diagnosis to patients with complex fistula disease, since the...
Extrapulmonary tuberculosis (TB) in the gastrointestinal tract is a rare, but yet an essential differential diagnosis to patients with complex fistula disease, since the initiation of immunosuppressive therapy for presumed Crohn's disease can lead to a fulminant course of TB. This case report presents a young Danish woman with a progressive complex fistula disease, where cause and treatment were uncovered by interprofessional collaboration between pulmonologists, gastroenterologists and surgeons including a screening test for TB as well as multiple biopsies from the anal fistula tissue.
Topics: Female; Humans; Crohn Disease; Rectal Fistula; Tuberculosis, Extrapulmonary; Tuberculosis, Gastrointestinal; Biopsy
PubMed: 38305264
DOI: 10.61409/V09230551 -
Journal of Bronchology & Interventional... Oct 2023
Topics: Humans; Pulmonologists; Bronchoscopy
PubMed: 37784236
DOI: 10.1097/LBR.0000000000000939 -
The New England Journal of Medicine Jun 2024Many persons with chronic obstructive pulmonary disease (COPD) or asthma have not received a diagnosis, so their respiratory symptoms remain largely untreated. (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Many persons with chronic obstructive pulmonary disease (COPD) or asthma have not received a diagnosis, so their respiratory symptoms remain largely untreated.
METHODS
We used a case-finding method to identify adults in the community with respiratory symptoms without diagnosed lung disease. Participants who were found to have undiagnosed COPD or asthma on spirometry were enrolled in a multicenter, randomized, controlled trial to determine whether early diagnosis and treatment reduces health care utilization for respiratory illness and improves health outcomes. Participants were assigned to receive the intervention (evaluation by a pulmonologist and an asthma-COPD educator who were instructed to initiate guideline-based care) or usual care by their primary care practitioner. The primary outcome was the annualized rate of participant-initiated health care utilization for respiratory illness. Secondary outcomes included changes from baseline to 1 year in disease-specific quality of life, as assessed with the St. George Respiratory Questionnaire (SGRQ; scores range from 0 to 100, with lower scores indicating better health status); symptom burden, as assessed with the COPD Assessment Test (CAT; scores range from 0 to 40, with lower scores indicating better health status); and forced expiratory volume in 1 second (FEV).
RESULTS
Of 38,353 persons interviewed, 595 were found to have undiagnosed COPD or asthma and 508 underwent randomization: 253 were assigned to the intervention group and 255 to the usual-care group. The annualized rate of a primary-outcome event was lower in the intervention group than in the usual-care group (0.53 vs. 1.12 events per person-year; incidence rate ratio, 0.48; 95% confidence interval [CI], 0.36 to 0.63; P<0.001). At 12 months, the SGRQ score was lower than the baseline score by 10.2 points in the intervention group and by 6.8 points in the usual-care group (difference, -3.5 points; 95% CI, -6.0 to -0.9), and the CAT score was lower than the baseline score by 3.8 points and 2.6 points, respectively (difference, -1.3 points; 95% CI, -2.4 to -0.1). The FEV increased by 119 ml in the intervention group and by 22 ml in the usual-care group (difference, 94 ml; 95% CI, 50 to 138). The incidence of adverse events was similar in the trial groups.
CONCLUSIONS
In this trial in which a strategy was used to identify adults in the community with undiagnosed asthma or COPD, those who received pulmonologist-directed treatment had less subsequent health care utilization for respiratory illness than those who received usual care. (Funded by Canadian Institutes of Health Research; UCAP ClinicalTrials.gov number, NCT03148210.).
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Asthma; Early Diagnosis; Forced Expiratory Volume; Pulmonary Disease, Chronic Obstructive; Quality of Life; Spirometry; Canada; Facilities and Services Utilization; Patient Acceptance of Health Care
PubMed: 38767248
DOI: 10.1056/NEJMoa2401389 -
Future Microbiology Sep 2023Diagnosing tuberculous pleural effusion (TPE) is challenging for pulmonologists and laboratory scientists. The gold standards for TPE diagnosis are pleural fluid... (Review)
Review
Diagnosing tuberculous pleural effusion (TPE) is challenging for pulmonologists and laboratory scientists. The gold standards for TPE diagnosis are pleural fluid Ziehl-Neelsen staining, () culture and pleural biopsy. These tools have limitations, including low sensitivity, long turnaround time and invasiveness. The nucleic acid amplification test (NAAT) is a rapid and minimally invasive tool for diagnosing TPE. This review summarizes the diagnostic accuracy of available NAATs for TPE, with a focus on the evidence from systematic reviews and meta-analyses. The NAATs summarized in this review include in-house NAATs, GeneXpert-MTB/RIF, GeneXpert-MTB/RIF Ultra, simultaneous amplification and testing-tuberculosis, FluoroType MTB and loop-mediated isothermal amplification.
Topics: Humans; Sensitivity and Specificity; Tuberculosis; Mycobacterium tuberculosis; Pleural Effusion; Nucleic Acid Amplification Techniques
PubMed: 37702019
DOI: 10.2217/fmb-2023-0025