Publications by authors named "Alexandre F Amaral"
Lymphangioleiomyomatosis (LAM) is a rare disease, characterized as a low-grade neoplasm with metastatic potential that mainly affects women of reproductive age, in which there is proliferation of atypical smooth muscle cells (LAM cells) and formation of diffuse pulmonary cysts. It can occur in a sporadic form or in combination with tuberous sclerosis complex. In recent decades, a number of advances have been made in the understanding of the pathophysiology and management of LAM, leading to improvements in its prognosis: identification of the main genetic aspects and the role of the mechanistic target of rapamycin (mTOR) pathway; relationship with hormonal factors, mainly estrogen; characterization of pulmonary and extrapulmonary manifestations in imaging studies; identification and importance in the diagnosis of VEGF-D; a systematic diagnostic approach, often without the need for lung biopsy; use of and indications for the use of mTOR inhibitors, mainly sirolimus, for pulmonary and extrapulmonary manifestations; pulmonary rehabilitation and the management of complications such as pneumothorax and chylothorax; and the role of and indications for lung transplantation.
View Article and Find Full Text PDFClinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.
Lung
December 2024
Article Synopsis
- Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting women, leading to decreased health-related quality of life (HRQoL), with limited understanding of the factors influencing this impairment.
- A study assessed 45 women with LAM, finding the lowest HRQoL scores in general health and vitality, while anxiety (35%) and depression (17%) were prevalent among the participants.
- Results indicated that depression symptoms and exercise capacity significantly impacted HRQoL, while lung function showed weak or no correlation with quality of life.
Article Synopsis
- Drug-induced lung disease (DILD) happens when certain medicines harm the lungs, affecting between 3% and 5% of people with lung diseases.
- Symptoms can appear quickly after starting treatment and may range from not feeling sick at all to having serious breathing problems.
- To treat DILD, doctors usually stop the harmful medication and may give steroids, but the chances of recovery can be very different for each person and some cases can be very serious.
Clinical, tomographic and functional comparison of sporadic and tuberous sclerosis complex-associated forms of lymphangioleiomyomatosis: a retrospective cohort study.
ERJ Open Res
March 2024
Article Synopsis
- Lymphangioleiomyomatosis (LAM) can present in two forms—sporadic (S-LAM) and associated with tuberous sclerosis complex (TSC-LAM)—and the study aims to compare their clinical and functional differences.
- A total of 107 patients were analyzed between 1994 and 2019, revealing that while TSC-LAM patients had more associated health issues, both groups had similar pulmonary function tests.
- The study found that S-LAM patients experience a greater decline in lung function and more lung cysts, while TSC-LAM impacts quality of life more significantly, especially regarding vitality and emotional health.
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article.
View Article and Find Full Text PDFDesaturation-Distance Ratio During Submaximal and Maximal Exercise Tests and Its Association With Lung Function Parameters in Patients With Lymphangioleiomyomatosis.
Front Med (Lausanne)
July 2021
The desaturation-distance ratio (DDR), the ratio of the desaturation area to the distance walked, is a promising, reliable, and simple physiologic tool for functional evaluation in subjects with interstitial lung diseases. Lymphangioleiomyomatosis (LAM) is a rare neoplastic condition frequently associated with exercise impairment. However, DDR has rarely been evaluated in patients with LAM.
View Article and Find Full Text PDFConcentration of Serum Vascular Endothelial Growth Factor (VEGF-D) and Its Correlation with Functional and Clinical Parameters in Patients with Lymphangioleiomyomatosis from a Brazilian Reference Center.
Lung
April 2019
Introduction: Serum vascular endothelial growth factor-D (VEGF-D) is a lymphangiogenic growth factor that is considered a valuable tool in the diagnosis of lymphangioleiomyomatosis (LAM). Previous studies have reported a wide variability in VEGF-D serum levels in LAM patients and it seems to be associated with pulmonary impairment and lymphatic involvement.
Methods: We conducted a cross-sectional study from 2009 to 2017 that evaluated VEGF-D serum levels in a cohort of LAM patients who were never treated with mTOR inhibitors and compared them to healthy age-matched volunteers.
Background: Heart failure and diabetes often occur simultaneously in patients, but the prognostic value of glycemia in chronic heart failure is debatable. We evaluated the role of glycemia on prognosis of heart failure.
Methods: Outpatients with chronic heart failure from the Long-term Prospective Randomized Controlled Study Using Repetitive Education at Six-Month Intervals and Monitoring for Adherence in Heart Failure Outpatients (REMADHE) trial were grouped according to the presence of diabetes and level of glycemia.
Background: Peculiar aspects of Chagas cardiomyopathy raise concerns about efficacy and safety of sympathetic blockade. We studied the influence of beta-blockers in patients with Chagas cardiomyopathy.
Methods And Results: We examined REMADHE trial and grouped patients according to etiology (Chagas versus non-Chagas) and beta-blocker therapy.