Cyclists to raise funds for UCLA Blood and Platelet Center; young patient kicks off race

WHAT:Some 1,000 cyclists will raise funds for the UCLA Blood and Platelet Center while enjoying a fresh-air tour of more than 65 scenic, cultural and historic landmarks in Los Angeles during the 16th annual Volkswagen City of Angels Fun Ride. Riders, who can choose from a 51-mile advanced race or a 31-mile recreational route, will be escorted by Los Angeles Police Department motorcycle officers, with rolling closures of city streets for traffic-free cycling.     Proceeds support UCLA's mobile blood-donation program, which collects 22,000 units of blood a year, supplying half the transfusions for patients at UCLA Health System hospitals. Last year's event raised $73,500 for the center.  A finish-line festival, featuring a free lunch catered by Sharky's Woodfired Mexican Grill and the raffle of a VW Beach Cruiser bicycle, a carbon-fiber Super Bike and many more prizes, will conclude the event, which is sponsored by Volkswagen, KHS Bicycles, Pace Sportswear and the Los Angeles Police Revolver and Athletic Club.  WHO:A young UCLA patient who benefited from blood donations will kick off the race by driving the pace car with her fiancé and parents:   Jennifer Golden Golden, 21, of Las Vegas, is a college student with cystic fibrosis who underwent a rare double-organ transplant to replace her infected lungs and deteriorating liver at Ronald Reagan UCLA Medical Center in 2011. Her life was sustained during her 13-hour surgery by lifesaving donatio...
Source: UCLA Newsroom: Health Sciences - Category: Universities & Medical Training Source Type: news

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Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant.
Source: Clinics in Liver Disease - Category: Gastroenterology 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
Authors: Regard L, Martin C, Chassagnon G, Burgel PR Abstract Introduction Cystic fibrosis (CF) is a genetic disease that primarily affects the respiratory system and often leads to respiratory failure and premature death. Although pulmonary complications contribute to 85% of deaths, non-pulmonary complications are responsible for significant morbidity and mortality in adults with CF. Areas covered This review summarizes acute and chronic non-pulmonary complications in CF patients, with emphasis on emerging complications and in the context of the current growth and ageing of the CF adult population. It also address...
Source: Expert Review of Respiratory Medicine - Category: Respiratory Medicine Tags: Expert Rev Respir Med Source Type: research
Authors: West NE, Flume PA Abstract INTRODUCTION: Cystic fibrosis (CF) outcomes and survival have improved over the last century primarily due to advancements in antibiotics, nutritional and pulmonary therapies. Reviewed here are the significant unmet needs that exist for individuals with CF. Areas Covered: With the recent development of medications that address the underlying defect in the CF protein, there is hope that there will be continued improvement in CF outcomes. However, there remains a need to prevent or stop progression of CF related complications, as the CF protein is important to several body systems....
Source: Expert Review of Respiratory Medicine - Category: Respiratory Medicine Tags: Expert Rev Respir Med 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
In this study we investigated regulatory t cells after combined lung and liver transplantation, or after isolated lung transplantation, in cystic fibrosis patients and correlated the findings with long term outcomes
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: 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
Abstract ObjectivesTo assess the characteristics that correlate with better outcomes after lung transplantation for patients with cystic fibrosis (CF). MethodsWe retrospectively reviewed the charts of all patients with CF who underwent lung transplantation between 1996‐2014 at Rabin Medical Center, Israel. ResultsFifty patients with CF underwent 55 lung transplantations. Eighteen patients (36%) died during the study period. Actuarial survival was 83%, 68%, 62% and 39% at 1, 3, 5 and 10 years, respectively. Better survival correlated with: BMI at 6‐months and 1‐year after transplantation (p=0.002 and p=0.003, respecti...
Source: Clinical Transplantation - Category: Transplant Surgery Authors: Tags: Original Article Source Type: research
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