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Cystic lung

Deepa Patel, Sarah Popple, Alison Claydon, Deborah E Modha, Erol A Gaillard
There is emerging evidence for the role of posaconazole in the management of Aspergillus-related cystic fibrosis (CF) lung disease. The tolerability and efficacy of posaconazole in paediatric CF is not well established. We report a prospective study over a fifty-three month period evaluating the safety, tolerability, and efficacy of posaconazole in pediatric CF. Fourteen children (seven males, median age 13 years, range 3-17 years) received a total of twenty-three courses of posaconazole (13 oral suspension and 10 tablet formulation)...
March 16, 2019: Medical Mycology: Official Publication of the International Society for Human and Animal Mycology
Laura I Marquez Loza, Eric C Yuen, Paul B McCray
Despite the continued development of cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs for the treatment of cystic fibrosis (CF), the need for mutation agnostic treatments remains. In a sub-group of CF individuals with mutations that may not respond to modulators, such as those with nonsense mutations, CFTR gene transfer to airway epithelia offers the potential for an effective treatment. Lentiviral vectors are well-suited for this purpose because they transduce nondividing cells, and provide long-term transgene expression...
March 14, 2019: Genes
Michelle Klingel, Sanja Stanojevic, Elizabeth Tullis, Felix Ratjen, Valerie Waters
RATIONALE: Intravenous (IV) tobramycin is frequently used to treat pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF), but there is concern that azithromycin may interact with tobramycin making it less effective against Pseudomonas aeruginosa. OBJECTIVE: The objective of this study was to determine whether oral azithromycin use was associated with worse lung function response to intravenous tobramycin treatment for PExs in a cohort of pediatric CF patients with chronic P...
March 15, 2019: Annals of the American Thoracic Society
Yao-Lung Chang, An-Shine Chao, Shuenn-Dyh Chang, Wen-Fang Li, Po-Jen Cheng
BACKGROUND: Transient donor hydrops (TDH) is defined as donor hydrops developed within days after laser therapy for twin-twin transfusion syndrome (TTTS) followed by resolution later. The purpose of this study was to evaluate the incidence, neonatal outcomes and predisposing factors of post laser therapy TDH in severe TTTS. METHODS: A total of 142 patients with severe TTTS who received laser therapy were included into this study. The pre-operative characteristics and neonatal outcomes were compared between TTTS with and without post laser therapy TDH...
March 11, 2019: BMC Pregnancy and Childbirth
Ashraf Imam, Tawfik Khoury, Dani Weis, Harbi Khalayleh, Muhammad Adeleh, Abed Khalaileh
Cystic echinococcosis (CE) is a widely endemic helminthic disease caused by infection with the Echinococcus granulosus tapeworm. Following ingestion of eggs, hydatid cysts develop, most frequently in the liver and lungs, but occasionally in other organs. Infection of the pancreas by hydatid cysts is very rare, even in endemic areas. Most cases of pancreatic hydatid cysts reported in the literature were treated surgically using traditional open laparotomy. There are only few case reports describing laparoscopic treatment for this disease...
February 2019: Annals of Hepato-Biliary-Pancreatic Surgery
Robert Henker, Annett Oltmanns, Alexandra Wald, Jan Tuennemann, Sabine Opitz, Albrecht Hoffmeister, Hubert Wirtz, Joachim Mössner, Boris Jansen-Winkeln, Thomas Karlas
The relevance of gastrointestinal manifestations of cystic fibrosis (CF) is increasing due to an improved life expectancy. We report on 2 adult patients with prior lung transplantation who presented with a severe inflammatory disorder of the ileocecal region. One patient underwent ileocecal resection; the second patient died after emergency surgery for intestinal perforation. Both cases did not show typical signs of CF-related distal intestinal obstruction syndrome or extensive fibrosing colonopathy. However, the clinical and histopathological findings revealed CF-induced inflammatory alterations of the intestinal mucosa...
March 2019: Zeitschrift Für Gastroenterologie
Patricia J Mergo, Carlos A Rojas
No abstract text is available yet for this article.
March 12, 2019: Radiology
Yang Tan, Jie Gao, Chongchong Wu, Shaohong Zhao, Jing Yu, Ruiping Zhu, Qing Zhang, Guangyao Wu, Xinying Xue, Jianlin Wu
There are increasing reports of a type of lung cancer that manifests as solitary cystic airspaces. The purpose of this case series was to identify the CT features and possible mechanisms of solitary cystic lung cancer, on the basis of CT observations and pathologic characteristics. The clinical, imaging, and pathologic data of 106 patients with solitary cystic lung cancer were collected and analyzed between January 2011 and December 2017. CT images were reviewed independently by three radiologists who were blinded to pathologic findings...
March 12, 2019: Radiology
Xiao Cai, Huixia Cai, Qing Gan, Wenxu Chang, Fang Yuan, Wei Luo, Jie Sun, Jing An
Cystic echinococcosis (CE) is a common, chronic, and endemic zoonotic disease usually localized in a single organ; multivisceral cases are rare, especially outside the liver or lung. Here, we describe an unusual case of a 43-year-old Tibetan man with echinococcosis of the infratemporal fossa, heart, liver, pancreas, abdomen, and pelvic cavity. He only presented with diminished vision of the left eye, especially when chewing. Computed tomography and magnetic resonance imaging revealed multivisceral CE. The patient underwent surgery for the excision of a cyst in the infratemporal fossa, as well as chemotherapy, and the diagnosis was confirmed by histopathological examination...
March 11, 2019: American Journal of Tropical Medicine and Hygiene
Nikolai Wajda, Nishant Gupta
Purpose of review: Spontaneous pneumothorax (SP) is a common manifestation of patients with diffuse cystic lung diseases (DCLDs) such as lymphangieoleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH) and Birt-Hogg-Dubé syndrome (BHD). Air travel may pose an additional risk for the development of SP. Here, we summarize the literature pertaining to air travel related SP in DCLDs in order to assist patients and clinicians in appropriate decision-making with regards to air travel...
June 2018: Current Pulmonology Reports
Alya A Heirali, Nicole Acosta, Douglas G Storey, Matthew L Workentine, Ranjani Somayaji, Isabelle Laforest-Lapointe, Winnie Leung, Bradley S Quon, Yves Berthiaume, Harvey R Rabin, Barbara J Waddell, Laura Rossi, Michael G Surette, Michael D Parkins
BACKGROUND: To improve clinical outcomes, cystic fibrosis (CF) patients with chronic Pseudomonas aeruginosa infections are prescribed inhaled anti-pseudomonal antibiotics. Although, a diverse microbial community exists within CF airways, little is known about how the CF microbiota influences patient outcomes. We hypothesized that organisms within the CF microbiota are affected by inhaled-antibiotics and baseline microbiome may be used to predict therapeutic response. METHODS: Adults with chronic P...
March 8, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Sabine Mainbourg, François Philit, Sandrine Touzet, Raphaele Nove-Josserand, Stéphane Durupt, Agathe Sénéchal, Pauline Occelli, Stéphanie Poupon-Bourdy, Jean-Michel Maury, François Tronc, Jean-François Mornex, Isabelle Durieu, Quitterie Reynaud
OBJECTIVE: To describe the prevalence of cystic fibrosis-related diabetes (CFRD) before and after lung transplantation (LT); to analyse the survival and renal function after LT according to the CFRD status before LT. METHODS: Sixty cystic fibrosis (CF) patients transplanted at the Lyon University Hospital between 2004 and 2014 were included. Genotype, pancreatic status, age at LT, survival were recorded. Glucose tolerance status, daily insulin dose requirement, glomerular filtration rate (GFR), and daily glucocorticoid (GC) dose were recorded before LT and until December 2016...
March 10, 2019: Pediatric Pulmonology
Yuichi Okata, Yuko Bitoh, Makiko Yoshida, Yoshinobu Akasaka
No abstract text is available yet for this article.
March 10, 2019: Pediatrics International: Official Journal of the Japan Pediatric Society
Massimo Boffini, Erika Simonato, Davide Ricci, Fabrizio Scalini, Matteo Marro, Stefano Pidello, Matteo Attisani, Paolo Solidoro, Paolo Olivo Lausi, Vito Fanelli, Cristina Barbero, Luca Brazzi, Mauro Rinaldi
Background: Lung transplantation is the treatment of choice for end-stage pulmonary disease in selected patients. However, severe primary graft dysfunction is a significant complication of transplant and requires the implantation of an extracorporeal support. The aim of the study is to evaluate the impact of extracorporeal membrane oxygenation (ECMO) after transplant in our center. Methods: From January 2008 till June 2018, 195 consecutive unselected patients receiving a lung transplant were considered...
January 2019: Annals of Cardiothoracic Surgery
Kathleen Kate Zink, Barbara Chini, Joyce Cowens, Lois Kremer, Li Lin
Background: Cystic Fibrosis (CF) is an autosomal recessive disorder of exocrine glands characterized by abnormal production of thick mucus, primarily in bronchi of the lungs. Individuals experience recurrent respiratory infections, increased work of breathing, cough and musculoskeletal changes with pain. Previous research found that massage therapy (MT) decreased pain, muscle tightness, and anxiety in individuals with CF, but did not use valid/reliable measurements of quality of life (QOL)...
March 2019: International Journal of Therapeutic Massage & Bodywork
Jennifer Yan, Ajay Kevat, Elena Martinez, Nicky Teese, Kareena Johnson, Sarath Ranganathan, Jo Harrison, John Massie, Andrew Daley
BACKGROUND: Mycobacterium abscessus is an emerging pathogen in cystic fibrosis (CF) lung disease. Hospital transmission of M. abscessus has been described. This paper details the investigation into possible cross-transmission of M. abscessus locally at our paediatric hospital CF centre, and the subsequent infection control response. METHODS: Whole genome sequencing (WGS) of M. abscessus respiratory isolates with epidemiological linkage analysis using hospital electronic medical records...
March 7, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Jinxue Ruan, Hiroyuki Hirai, Dongshan Yang, Linyuan Ma, Xia Hou, Hong Jiang, Hongguang Wei, Carthic Rajagopalan, Hongmei Mou, Guoshun Wang, Jifeng Zhang, Kui Li, Yuqing E Chen, Fei Sun, Jie Xu
Cystic fibrosis (CF) is a lethal autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Nuclease-mediated precise gene editing (PGE) represents a promising therapy for CF, for which an efficient strategy that is free of viral vector, drug selection, and reporter enrichment (VDR free) is desirable. Here we compared different transfection methods (lipofectamine versus electroporation) and formats (plasmid DNA versus ribonucleoprotein) in delivering the CRISPR/Cas9 elements along with single-stranded oligodeoxynucleotides (ssODNs) to clinically relevant cells targeting major CFTR mutation loci...
February 16, 2019: Molecular Therapy. Nucleic Acids
Maxim Kostylev, Daniel Y Kim, Nicole E Smalley, Indraneel Salukhe, E Peter Greenberg, Ajai A Dandekar
The bacterial pathogen Pseudomonas aeruginosa activates expression of many virulence genes in a cell density-dependent manner by using an intricate quorum-sensing (QS) network. QS in P. aeruginosa involves two acyl-homoserine-lactone circuits, LasI-LasR and RhlI-RhlR. LasI-LasR is required to activate many genes including those coding for RhlI-RhlR. P. aeruginosa causes chronic infections in the lungs of people with cystic fibrosis (CF). In these infections, LasR mutants are common, but rhlR-rhlI expression has escaped LasR regulation in many CF isolates...
March 8, 2019: Proceedings of the National Academy of Sciences of the United States of America
Heino Stass, Johannes Nagelschmitz, Dominik Kappeler, Knut Sommerer, Astrid Patzlaff, Boris Weimann
BACKGROUND: As non-cystic fibrosis bronchiectasis (NCFB) progresses, patients suffer irreversible lung damage and deterioration in lung function. This study explored whether inhalational parameters (peak inspiratory flow [PIF, primary endpoint], inspiratory volume and time [secondary endpoints]) represent barriers to complete dosing in patients with poor lung function who are using Ciprofloxacin dry powder for inhalation (DPI) (a drug-device combination of the T-326 inhaler device and a Ciprofloxacin dry powder formulation)...
March 8, 2019: Journal of Aerosol Medicine and Pulmonary Drug Delivery
Gilmar de Souza Osmundo Junior, Rodrigo Abensur Athanazio, Samia Zahi Rached, Rossana Pulcineli Vieira Francisco
OBJECTIVES:  To assess the perinatal and maternal outcomes of pregnant women with cystic fibrosis (CF) and severe lung impairment. METHODS:  This was a series of cases aiming to review the maternal and fetal outcomes in cases of singleton pregnant women with a diagnosis of CF. We have included all of the cases of singleton pregnancy in patients with CF who were followed-up at the obstetrics department of the Medical School of the Universidade de São Paulo, between 2003 and 2016...
March 7, 2019: Revista Brasileira de Ginecologia e Obstetrícia
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