Lung Transplantation for Interstitial Lung DiseasesLung Transplantation for Interstitial Lung Diseases

Select patients with interstitial lung diseases and pulmonary hypertension may benefit from lung transplantation, even though the number of transplants performed is limited by the supply of donor organs. Which patients should be on the waiting list? Seminars in Respiratory and Critical Care Medicine
Source: Medscape Today Headlines - Category: Consumer Health News Tags: Pulmonary Medicine Journal Article Source Type: news

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CONCLUSIONS: PH-NF1 is characterized by a female predominance, a low DLCO and severe functional and hemodynamic impairment. Despite a potential benefit of PAH treatment, prognosis remains poor, and double-lung transplantation is an option for eligible patients. PMID: 32437637 [PubMed - as supplied by publisher]
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
The age of patients with pulmonary arterial hypertension (PAH) has increased, with registries now reporting mean ages of 50-65 years old. Limited data exist on age-related differences in hemodynamic and functional assessments in PAH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Introduction Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients. Methods ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary hypertension Source Type: research
Conclusion PAH due to TBX4 mutations may occur with or without skeletal abnormalities across a broad age range from birth to late adulthood. PAH is usually severe and associated with bronchial and parenchymal abnormalities.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Genetics, Pulmonary vascular disease Original Articles: Pulmonary hypertension and genetics Source Type: research
CONCLUSION: Poor sleep quality was found to be common among patients on the lung transplantation waitlist in Japan. The two most significant factors responsible for impaired sleep quality were anxiety and respiratory symptoms. Additional care should be taken to ensuring a better quality of sleep for such patients. PMID: 32399697 [PubMed - as supplied by publisher]
Source: Sleep and Breathing - Category: Sleep Medicine Authors: Tags: Sleep Breath Source Type: research
ConclusionPoor sleep quality was found to be common among patients on the lung transplantation waitlist in Japan. The two most significant factors responsible for impaired sleep quality were anxiety and respiratory symptoms. Additional care should be taken to ensuring a better quality of sleep for such patients.
Source: Sleep and Breathing - Category: Respiratory Medicine Source Type: research
One in five children with end-stage lung failure (ESLF) die while awaiting lung transplant. No suitable animal model of ESLF exists for the development of artificial lung devices for bridging to transplant. Small lambs weighing 15.7 ± 3.1 kg (n = 5) underwent ligation of the left anterior pulmonary artery (PA) branch, and gradual occlusion of the right main PA over 48 hours. All animals remained hemodynamically stable. Over seven days of disease model conditions, they developed pulmonary hypertension (mean PA pressure 20 ± 5 vs. 33 ± 4 mm Hg), decreased perfusion (SvO2 66 ± 3 vs. 55 ± 8%)...
Source: ASAIO Journal - Category: Medical Devices Tags: Pulmonary Source Type: research
An 11-year-old girl presented with pulmonary hypertension (PH), World Health Organization (WHO) functional class (FC) III associated with sickle cell disease (SCD) and history of a congenital diaphragmatic hernia (CDH) repair after birth. Her past medical history was significant for multiple episodes of pneumonia and pain crises. At age 7 years, her mean pulmonary artery pressure (mPAP) was 32 mmHg and pulmonary vascular resistance index (PVRi) was 3.6 Wood units x m2 by right heart cardiac catheterization (RHC) at another institute.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
Background: Screening and early diagnosis of pulmonary hypertension (PH) is critical for managing progression and preventing associated mortality, there are no tools for this purpose. We developed and validated an artificial intelligence (AI) algorithm for predicting PH using electrocardiography (ECG).Methods: This historical cohort study included data from consecutive patients from two hospitals. The patients in one hospital were divided into derivation (56,670 ECGs from 24,202 patients) and internal validation (3,174 ECGs from 3,174 patients) dataset, while the patients in the other hospital were included in only an exte...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Semin Respir Crit Care Med 2020; 41: 280-287 DOI: 10.1055/s-0040-1702211Pulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive disorder that is caused by mutations in SCL34A2 that encodes for the type IIb sodium-dependent phosphate cotransporter (Npt2b). The loss of Npt2b transporter function from alveolar epithelial cells results in failure to export inorganic phosphate from the alveolar lining fluid, which then accumulates, binds to calcium, and forms hydroxyapatite microliths. Radiographs and computed tomography of the chest demonstrate hyperdense infiltrates that are often quite dramatic and distinctive...
Source: Seminars in Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Review Article Source Type: research
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