Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis.

Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis. Am J Respir Crit Care Med. 2018 Oct 15;: Authors: Hebestreit H, Hulzebos EH, Schneiderman JE, Karila C, Boas SR, Kriemler S, Dwyer T, Sahlberg M, Urquhart DS, Lands LC, Ratjen F, Takken T, Varanistkaya L, Rücker V, Hebestreit A, Usemann J, Radtke T, Prognostic value of CPET in CF study group Abstract RATIONALE: The prognostic value of cardiopulmonary exercise testing (CPET) for survival in cystic fibrosis (CF) in the context of current clinical management, when controlling for other known prognostic factors is unclear. OBJECTIVES: To determine the prognostic value of CPET-derived measures beyond peak oxygen uptake (VO2peak) following rigorous adjustment for other predictors. MEASUREMENTS AND MAIN RESULTS: Data from 10 CF-centers in Australia, Europe and North America were collected retrospectively. 510 patients completed a cycle CPET between January 2000 and December 2007, of which 433 fulfilled the criteria for a maximal effort. Time to death/lung transplantation (LTx) was analyzed using Cox proportional hazards regression. In addition, phenotyping using hirarchical Ward's clustering was performed to characterize high risk subgroups. Cox regression showed - even after adjustment for sex, forced expiratory volume in 1s (%predicted), body mass index (z-score), age at CPET, Pseudomonas aeruginosa status, and CF-related diabetes as covariates in th...
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research

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Abstract Cystic fibrosis (CF) is the most common life-limiting genetic disease in Caucasian patients. Continued advances have led to improved survival, and adults with CF now outnumber children. As our understanding of the disease improves, new therapies have emerged that improve the basic defect, enabling patient-specific treatment and improved outcomes. However, recurrent exacerbations continue to lead to morbidity and mortality, and new pathogens have been identified that may lead to worse outcomes. In addition, new complications, such as CF-related diabetes and increased risk of gastrointestinal cancers, are c...
Source: Annual Review of Medicine - Category: General Medicine Authors: Tags: Annu Rev Med Source Type: research
Doctors in Scotland believe a woman’s large tattoo on her leg may have been the cause of her unexplained inflammation and severe leg pain. The authors of a report on the woman’s case, published in BMJ Case Reports, say it is a reminder to physicians that they should be mindful of their patients’ tattoos if they encounter unusual and unexplained symptoms. The 31-year-old woman was suffering a condition called inflammatory myopathy, which affected her left leg and caused pain severe enough to disrupt her sleep. The woman had a double lung transplant in 2009, and was taking drugs to suppress her immune syst...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized healthytime onetime tattoos Source Type: news
A woman with cystic fibrosis and lung transplants suffered chronic pain for three years after she had a tattoo on her thighGetting a tattoo if you have a weakened immune system could put you at risk of complications, doctors have warned. The caution comes after a woman with cystic fibrosis and lung transplants developed thigh and knee pain after having body art inked on her leg.Doctors say those taking immunosuppressant drugs should take precautions if considering body art. These medicines are often given after an organ transplant or to treat autoimmune conditions such as Crohn ’s disease, lupus or rheumatoid arthrit...
Source: Guardian Unlimited Science - Category: Science Authors: Tags: Tattoos Medical research Immunology Biochemistry and molecular biology Health Cystic fibrosis Diabetes Source Type: news
CONCLUSIONS: The lack of currently available evidence makes it impossible to draw conclusions about the comparative efficacy and safety of the various immunosuppressive drugs among people with cystic fibrosis after lung transplantation. A 2013 Cochrane Review comparing tacrolimus with cyclosporine in all lung transplant recipients (not restricted to those with cystic fibrosis) reported no significant difference in mortality and risk of acute rejection. However, tacrolimus use was associated with lower risk of broncholitis obliterans syndrome and arterial hypertension and higher risk of diabetes mellitus. It should be noted...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Poster Sessions Source Type: research
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Workshops Source Type: research
This article is protected by copyright. All rights reserved. PMID: 29700855 [PubMed - as supplied by publisher]
Source: Clinical Lung Cancer - Category: Cancer & Oncology Authors: Tags: Clin Transplant Source Type: research
Conclusion: IT following lung and liver transplantation, with injection of islets into a transplanted organ, is feasible. It improves C-peptide secretion, decreases insulin needs, and lowers HbA1c.Horm Res Paediatr
Source: Hormone Research in Paediatrics - Category: Endocrinology Source Type: research
Conclusions:VB is an uncommon complication of CF cirrhosis and can herald the diagnosis, but does not affect ACM. Adverse liver outcomes and ACM are frequent by 10 years after cirrhosis report. Objectives: Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis. Methods: We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and exa...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Article: Pancreatology Source Type: research
Discussion Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are l...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
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