Pennsylvania girl who got lung transplants recovering from more surgery
(Reuters) - A 10-year-old Pennsylvania girl with cystic fibrosis who sparked a national debate about child access to organ donations that led to two lung transplant operations was in recovery on Tuesday after surgery to repair her diaphragm, her family said.
Purpose: Exercise improves pulmonary function and physical conditioning in cystic fibrosis. Exercise capacity was assessed in the inpatient setting to determine whether pulmonary exacerbation treatment with intravenous antibiotics, airway clearance, and exercise would improve exercise capacity independently of lung function. Methods: Exercise capacity was measured at hospital admission and hospital discharge by numbers of completed shuttles using the Modified Shuttle Walk Test (MSWT). Resting heart rate, oxyhemoglobin saturation, and assessments of breathlessness and perceived exertion were measured before and after e...
A clinical trial led by a pharmaceutical company with additional support from an NIDDK-supported Cystic Fibrosis Center has shown that a combination of two medications provides significant clinical benefit in a subgroup of patients with cystic fibrosis (CF).
Proteins involved in ensuring that other proteins are in their proper shapes may have a role to play in mitigating the effects of cystic fibrosis.
AbstractThe pharmacokinetics (PK) of β-lactam antibiotics in cystic fibrosis (CF) patients has been compared with that in healthy volunteers for over four decades; however, no quantitative models exist that explain the PK differences between CF patients and healthy volunteers in older and newer studies. Our aims were to critically eva luate these studies and explain the PK differences between CF patients and healthy volunteers. We reviewed all 16 studies that compared the PK of β-lactams between CF patients and healthy volunteers within the same study. Analysis of covariance (ANCOVA) models were developed. I...
Authors: Gea J, Sancho-Muñoz A, Chalela R Abstract Nutritional abnormalities are frequent in different chronic respiratory diseases such as chronic obstructive pulmonary disease (COPD), bronchiectasis, cystic fibrosis (CF), interstitial fibrosis and lung cancer, having important clinical consequences. However, nutritional abnormalities often remained underdiagnosed due to the relative lack of awareness of health professionals. Therefore, systematic anthropometry or even better, assessment of body composition, should be performed in all patients with chronic respiratory conditions, especially following exacer...
EXCLUSIVE: Benat Broderick, from Dublin, argued his life has been transformed since he was given Orkambi. But UK watchdogs have denied patients access to the £100,000 medication.
Previous studies have demonstrated that CF epithelial cells exhibit increased cholesterol content at the plasma membrane compared to wild type controls as measured by electrochemical methods. Microtubule dysregulation that impacts intracellular transport has also been identified in CF cells and is reversible with histone deacetylase 6 (HDAC6) inhibition, a regulator of tubulin acetylation. The hypothesis of this study is that increased membrane cholesterol content in CF cells is dependent on HDAC6 regulation.
There is no effective way to predict cystic fibrosis (CF) pulmonary exacerbations (CFPE) before they become symptomatic or to assess satisfactory treatment responses.
A 29-year-old female patient with significant past medical history of cystic fibrosis (CF) complicated by Pseudomonas colonization in the lungs, pancreatic insufficiency, and chronic sinusitis presented to the Emergency Department (ED) after an episode of confusion, disorientation, and headache at home. The patient also reported “cough and cold” symptoms, which were above her baseline chronic symptoms related to CF; and her boyfriend had had an upper respiratory infection over the past several weeks.
To identify prenatal and perinatal predictors of surgery and describe surgical findings/outcomes of neonates with Meconium Ileus (MI) secondary to Cystic Fibrosis (CF).