Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis

AbstractIntroductionThere is a strong association between cystic fibrosis and malnutrition, mainly because of the higher energy needs combined with lower intake. There is also a well-established correlation between good nutritional status and better lung function. To date, however, there are no studies examining nutritional status in childhood and adult lung function. To respond to this need, this innovative study explored the long-term correlations between nutritional status in childhood and lung function in adulthood for the same patient population.MethodsA retrospective patient file study was conducted to identify putative correlations between nutritional status in childhood and lung function in adulthood. The medical archives at Sheba Medical Center were examined for a period of 31  years between 1986 and 2017 for age, gender, mutations, pancreatic sufficiency or insufficiency (PI/PS), sputum cultures, cystic fibrosis related diabetes, body mass index (BMI) at the age of 10, and FEV1 at 20 and 30 in patients who underwent or did not undergo lung transplantation.ResultsThe database was composed of the records of sixty-five patients, thirteen of whom underwent lung transplantation. The correlations (R²) between BMI at age of 10 years and FEV1 at the age of 20 and 30  years were 0.35 and 0.28, respectively,p 
Source: Lung - Category: Respiratory Medicine Source Type: research

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Endocrine Practice,Volume 25, Issue 3, Page 254-262, March 2019.
Source: Endocrine Practice - Category: Endocrinology Authors: Source Type: research
Endocrine Practice,Volume 25, Issue 3, Page 254-262, March 2019.
Source: Endocrine Practice - Category: Endocrinology Authors: Source Type: research
In this study, the median age at LT for CFLD was 15.7 years. Notably, 10 of 13 (77%) CF explants had>5% steatosis and 8 of 13 (61.5%) demonstrated variable fibrosis. The median age, sex, type of transplant (liver vs liver-lung), pancreatic insufficiency status, body mass index (BMI) percentile, genotype, and prevalence of diabetes were comparable in those with and without explant steatosis. More than half of allograft biopsies showed significant steatosis (17/31, 54.8%) and lobular inflammation (16/31, 51.6%). Hepatocyte ballooning was less frequent (5/31, 16.1%). Overall, 6 patients (46.2%) had allograft steatosis that...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
oup 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 f...
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
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
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