A 30-year-old gentleman with a medical history of HIV, on HAART therapy and pulmonary amyloidosis proven by lung biopsy presented with progressive, worsening shortness of breath and chronic productive cough over the last year. Pulmonary function tests showed a severely obstructive process and CT scan of the thorax manifested diffusely thin-walled, air-filled cysts. After reviewing the patient's prior histories, PFTs and CTof the thorax, the most likely cause of his symptoms was amyloid-related cystic lung disease. After being placed on a LABA/ICS, and LAMA inhaler, the man's condition remained stable. Active oncologic and pulmonary surveillance were continued every three to six months as the patient's respiratory status, PFTs and imaging remained stable. Thoughts of chemotherapy and lung transplant have been suggested, but not initiated at this time. Amyloid-related cystic lung disease is a rare presentation of amyloidosis in the thorax. It has been associated with collagen vascular diseases and mucosa-associated lymphoid tissue lymphoma. After an extensive literature search, it does not appear that this condition has been referenced in an HIV-positive patient; thus making this report unique. More case reports will need to be published and reviewed to see whether an association with HIV and amyloid-associated cystic lung disease exists.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.5603/ARM.a2021.0007 | DOI Listing |
Background: Due to its increasing prevalence and suboptimal treatment, non-tuberculous mycobacterial (NTM) infection is an emerging problem in patients with cystic fibrosis (CF). Detailed description of regional NTM prevalence and distribution, and identification of predictors of NTM acquisition in CF are essential to optimise treatment and surveillance guidelines.
Methods: A retrospective, multi-center analysis was conducted between the years 2020 and 2022 on data from 232 adult patients registered in the Hungarian CF Registry in 2022.
BMJ Open
December 2024
Department of Rehabilitation, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China
Introduction: Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, primarily affecting the respiratory and digestive systems. Respiratory rehabilitation techniques play a crucial role in managing pulmonary symptoms and maintaining lung function in CF patients. Although various techniques have been developed and applied, there is currently no globally recognised optimal respiratory rehabilitation regimen.
View Article and Find Full Text PDFArch Dis Child Educ Pract Ed
January 2025
National Heart and Lung Institute, Imperial College London, London, UK.
Transformative changes in the health of children with cystic fibrosis (CF) mean that more sensitive outcome measures are needed to monitor paediatric CF lung disease. Multiple breath washout (MBW) and its primary readout lung clearance index are gaining increasing traction as an endpoint for clinical trials in the CF space and show promise as a clinical investigation. In this article, we use four clinically based questions to explore what MBW can and cannot (yet) do and highlight some of its strengths and weaknesses as an investigation.
View Article and Find Full Text PDFLancet Respir Med
January 2025
Division of Respiratory Medicine and Gastroenterology, University of Dundee, Ninewells Hospital and Medical School, Dundee, UK. Electronic address:
Background: Bronchiectasis is a disease with a global impact, but most published data come from high-income countries. We aimed to describe the clinical characteristics of patients with bronchiectasis in China.
Methods: The Chinese Bronchiectasis Registry (BE-China) is a prospective, observational cohort enrolling patients from 111 hospitals in China.
Introduction: The Lung Clearance Index (LCI) is an established research test, but its role in clinical decision-making is not well defined. This study estimated the proportion of treatment decisions that are changed or supported by the added information provided by LCI.
Methods: A mixed methods prospective observational study was conducted in North America.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!