scholarly journals Young Cystic Fibrosis Lung Transplant Recipients: Predisposing Factors for End-stage Disease

2021 ◽  
Author(s):  
George Mallory ◽  
Ernestina Melicoff ◽  
Fadel Ruiz ◽  
Kathleen Hosek
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Stage Disease ◽  
High Prevalence ◽  
The Usa ◽  
History Of ◽  
Lung Transplant Recipients ◽  
End Stage ◽  
Age Range

Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those who in the pubertal or post pubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the USA. Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years. Results: Notable findings were a high prevalence of methicillin-resistant Staphylococcus aureus, a high prevalence of diabetes mellitus and an impressive prevalence of consolidated lobar or whole lung disease. Post-transplant outcomes, however, were comparable to those older than 10 years of age in our center. Conclusions: In an era of increasingly effective medications modifying the natural history of CF, identification of risk factors for early severe lung disease in CF remains relevant and potentially more important for effective intervention in order to extend life.

Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?

CHEST Journal ◽  
2021 ◽  
Author(s):  
Oded Breuer ◽  
David Shoseyov ◽  
Shifra Koretz ◽  
Nadia Alyan ◽  
Joel Reiter ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Ethical Dilemma ◽  
End Stage

scholarly journals NOVOSTI V OBRAVNAVI PLJUČNE BOLEZNI PRI OTROCIH IN MLADOSTNIKIH S CISTIČNO FIBROZO

2015 ◽  
Vol 84 (2) ◽  
Author(s):  
Marina Praprotnik ◽  
Ana Kotnik Pirš ◽  
Barbara Salobir ◽  
Majda Oštir ◽  
Matjaž Turel ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Respiratory Insufficiency ◽  
Clinical Skills ◽  
Holistic Approach ◽  
Patient Registries ◽  
Recent Developments ◽  
Chronic Respiratory Insufficiency ◽  
Infection And Inflammation

Cystic fibrosis (CF) is a multi-organ disease,  affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.The purpose of this review is to survey recent developments in the treatment of lung disease  in children and adolescents with CF.We describe newborn screening for CF.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies  and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in  clinical trials.  The most important development  in the recent years is  modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

412: Antibiotic Desensitisation in Cystic Fibrosis Patients with a History of Hypersensitivity Undergoing Lung Transplantation Is Not Mandatory

2010 ◽  
Vol 29 (2) ◽  
pp. S136-S136
Author(s):  
T. Tse ◽  
P.M.A. Hopkins ◽  
M.W. France ◽  
P.J. Masel ◽  
S.C. Bell ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
History Of

scholarly journals Impact of Nocturnal Noninvasive Ventilation on Pulmonary Rehabilitation in Patients with End-Stage Lung Disease Awaiting Lung Transplantation

Respiration ◽  
2017 ◽  
Vol 95 (3) ◽  
pp. 161-168 ◽  
Author(s):  
Nikolaus Kneidinger ◽  
Rainer Gloeckl ◽  
Ursula Schönheit-Kenn ◽  
Katrin Milger ◽  
Wolfgang Hitzl ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Lung Disease ◽  
Noninvasive Ventilation ◽  
Pulmonary Rehabilitation ◽  
End Stage

scholarly journals Chronic Pulmonary Silicone Embolism from Breast Augmentation Is Not a Common Finding in Explanted Lungs

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Jarmanjeet Singh ◽  
Hanine Inaty ◽  
Sanjay Mukhopadhyay ◽  
Atul C. Mehta
Keyword(s):  
Lung Transplantation ◽  
Pulmonary Disease ◽  
Breast Augmentation ◽  
Lung Transplant ◽  
Common Finding ◽  
Transplant Recipients ◽  
Chronic Lung Diseases ◽  
Pathologic Features ◽  
History Of ◽  
Lung Transplant Recipients

Objective. Acute pulmonary silicone embolism (APSE) related to subcutaneous silicone injections is a well-known entity. Recently, a few cases of pathologically confirmed chronic pulmonary silicone embolism (CPSE) from breast implants have been reported. The prevalence of CPSE in women with breast augmentation is unknown. This study was done to determine the prevalence of CPSE in female lung transplant recipients with a history of breast augmentation and to determine whether breast augmentation plays a role in chronic lung diseases requiring lung transplantation. Methods. A retrospective chart review was performed to identify female lung transplant recipients with a history of breast augmentation prior to or at the time of lung transplantation. Ten patients meeting these criteria were identified. The pathologic features of the explanted lungs of these patients were reexamined for CPSE by a board-certified pathologist with expertise in lung transplantation and pulmonary embolism. Results. Of 1518 lung transplant recipients at Cleveland Clinic, 578 were females. Of 578 females, 10 (1.73%) had history of breast augmentation. A total of 84 H&E-stained slides from the explanted lungs from 10 cases were examined. No pathologic evidence of chronic silicone embolism was seen in any of the 10 cases. Conclusions. CPSE is not associated with pulmonary disease leading to lung transplantation. Breast augmentation is not a significant contributor to pulmonary disease requiring lung transplantation. Further studies are required to ascertain the prevalence of CPSE in the general breast augmentation populace and to define the relationship between breast augmentation and pulmonary disease.

Selection of Candidates for Lung Transplantation and Controversial Issues

2018 ◽  
Vol 39 (02) ◽  
pp. 117-125 ◽  
Author(s):  
Christian Merlo ◽  
Jonathan Orens
Keyword(s):  
Lung Transplantation ◽  
Lung Disease ◽  
Significant Loss ◽  
Candidate Selection ◽  
Therapeutic Interventions ◽  
Controversial Issues ◽  
Successful Transplantation ◽  
Transplant Candidates ◽  
End Stage ◽  
Selection Of

AbstractLung transplantation is a widely accepted treatment to manage the advanced stages of many lung diseases that have failed to respond to all other therapeutic interventions. There have been ever-expanding indications for lung transplantation as a treatment for lung disease. The International Society for Heart and Lung Transplantation (ISHLT) updated guidelines for candidate selection in 2014. This document was published to serve as a guide in selecting appropriate candidates for lung transplantation. Ideal candidates for lung transplantation are those with near-end stage disease, limited life expectancy due to their lung disease, and who experience significant loss in quality of life. At the same time, candidates should be free of significant comorbidities that might negatively impact successful transplantation. Despite well thought out published guidelines for candidate selection, many controversial issues surrounding selection of lung transplant candidates remain. This review will focus on some of the more common controversial issues and explain how most centers approach these issues when choosing candidates for lung transplantation.

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