Cystic fibrosis complicated by cor pulmonale: The first case report in Taiwan

I read with great interest the article entitled Cystic Fibrosis Complicated by Cor Pulmonale: The First Case Report in Taiwan by Chen et  al.1
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Letter to the Editor Source Type: research

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Lung transplantation is an established therapeutic option for patients with irreversible end-stage pulmonary disease limiting life expectancy and quality of life. Common indications for lung transplantation include chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, pulmonary arterial hypertension, and alpha-1 antitrypsin deficiency. Complications of lung transplantation can be broadly divided etiologically into surgical, infectious, immunologic, or neoplastic. Moreover, specific complications often occur within a certain time interval following surgery, which can be broadly classified as...
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Review Articles Source Type: research
Conclusions: Liver US patterns in children with CF correlate with platelet count, spleen size and indices of liver fibrosis. Multivariable models of these biomarkers have excellent discriminating ability for NL versus NOD, and good ability to distinguish other US patterns, suggesting that US patterns correlate with clinically relevant liver disease.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Guidelines recommend breastfeeding in cystic fibrosis (CF) but breastfeeding rates decline rapidly in CF. We initiated a quality improvement project to improve breastfeeding in CF by incorporating International Board-Certified Lactation Consultants (IBCLC) into the initial CF-diagnosis visit to support mothers who were breastfeeding at diagnosis. In the preintervention group, only 8/14 (57%) continued to provide breast milk after the first visit, whereas postintervention, 16/17 (94%) mothers continued to do so (P = 0.03). The duration of any (or partial) breastfeeding increased to an average of 7.7 months from an avera...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Short Communication: Pancreatology Source Type: research
Cystic fibrosis (CF) is a genetic disorder that doctors usually diagnose in early childhood. Find out about the diagnostic procedure and the treatments that may help reduce CF symptoms.
Source: Health News from Medical News Today - Category: Consumer Health News Tags: Cystic Fibrosis Source Type: news
Shortcomings of inhaled antibiotic treatments for Pseudomonas aeruginosa infection in patients with cystic fibrosis (CF) include poor drug penetration, inactivation by sputum, poor efficiency due to protective biofilm, and short residence in the lung.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research
BackgroundThere is a paucity of data evaluating antibiotic use in anterior skull ‐base surgery (ASBS). The goal of this study was to determine antibiotic prescribing patterns and factors that influence antibiotic use in ASBS.MethodsAn online ‐based survey was distributed to the membership of the North American Skull Base Society in 2018. Outcomes included practitioner preference regarding intraoperative and postoperative antibiotic use, practice location and environment, surgeon experience, and patient factors influencing antibiotic u se.ResultsThere were 208 respondents (25.6% response rate) of which 182 (87.5%) perfo...
Source: International Forum of Allergy and Rhinology - Category: Allergy & Immunology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Cystic fibrosis (CF) is an autosomal recessive disorder, caused by genetic mutations in CF transmembrane conductance regulator (CFTR) protein. Several reports have indicated the presence of specific fatty acid (FA) alterations in CF patients, most notably decreased levels of plasmatic and tissue docosahexaenoic acid (DHA), the precursor of Specialized Pro-resolving Mediators (SPMs). We hypothesized that an imbalance between arachidonic acid (AA)- and DHA-derived products could contribute to the chronic pulmonary inflammation observed in CF subjects. Sputum samples from CF and Chronic Obstructive Pulmonary Disease (COPD) s...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
Publication date: Available online 21 August 2019Source: Progress in Lipid ResearchAuthor(s): Dušan Garić, Juan B. De Sanctis, Juhi Shah, Daciana Catalina Dumut, Danuta RadziochAbstractCeramides, the principal building blocks of all sphingolipids, have attracted the attention of many scientists around the world interested in developing treatments for cystic fibrosis, the most common genetic disease of Caucasians. Many years of fruitful research in this field have produced some fundamentally important, yet controversial results.Here, we aimed to summarize the current knowledge on the role of long- and very-long- cha...
Source: Progress in Lipid Research - Category: Lipidology Source Type: research
There is a great deal of interest in the airway microbiota, its diversity and the role of specific microbial taxa in the pathophysiology of lung disease [1]. Non-cystic fibrosis bronchiectasis is a significant public health problem in many countries, including New Zealand, where prevalence is high and morbidity and mortality are substantial [2–4]. A role for bacteria in the pathophysiology of bronchiectasis is widely accepted but poorly characterised due to inherent difficulties with lower airway sampling, especially in young children. Culture-based studies demonstrate associations with Haemophilus influenzae, Moraxe...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Research letters Source Type: research
This report highlights the usefulness of bronchoscopy in case of recurrent pneumonia with the same localization even in CF patients where the presence of bronchiectasis as promoting factor of infections could delay the diagnosis. AbstractThis report highlights the usefulness of bronchoscopy in case of recurrent pneumonia with the same localization even in CF patients where the presence of bronchiectasis as promoting factor of infections could delay the diagnosis.
Source: Clinical Case Reports - Category: General Medicine Authors: Tags: CASE REPORT Source Type: research
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