Abstract

Background:

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by intra-alveolar accumulation of
surfactant material, resulting in impaired gas exchange and functional limitations. Whole-lung lavage (WLL) remains the
standard therapy; however, objective assessment of functional status is important during longitudinal follow-up, particularly
for patients who require monitoring outside specialized centers.
Objectives: This study evaluated the association between home-based six-minute walk test (6MWT) parameters and the need
for repeat WLL in patients with PAP.

Methods: 

In this exploratory observational study, 21 patients with PAP who had undergone at least one WLL were followed
using a telemedicine-based monitoring approach. Patients completed home-based 6MWT assessments, including walking
distance, oxygen saturation, and heart rate response. Dyspnea severity was assessed using the modified Medical Research
Council (mMRC) scale; oxygen dependence and symptom-related parameters were also recorded. Exploratory binary logistic
regression analysis was performed to evaluate factors associated with repeat WLL.

Results: 

Patients requiring repeat WLL had significantly shorter 6MWT distances (297.6 ± 109.5 m vs. 429.1 ± 97.4 m, P = 0.009)
and higher mMRC dyspnea scores (1.91 ± 0.94 vs. 0.72 ± 0.89, P = 0.002) than those not requiring repeat WLL. In an exploratory
multivariable logistic regression analysis, a lower 6MWT distance was associated with the requirement for repeat WLL; however,
after adjustment, none of the evaluated predictors showed a statistically significant independent association. Other
parameters, including oxygen desaturation and demographic characteristics, were not independently associated with repeat
WLL.

Conclusions: 

The home-based 6MWT provides clinically relevant information on functional impairment and may serve as an
adjunctive marker for longitudinal monitoring of patients with PAP. Telemedicine-based assessment is a feasible approach for
remote collection of functional data; however, its effectiveness in improving clinical outcomes or guiding WLL decisions
requires confirmation in larger prospective studies using validated assessment protocols.

Keywords: Pulmonary Alveolar Proteinosis, Whole Lung Lavage, Six-minute Walk Test, Telemedicine, Functional Capacity

 

لینک مقاله: 

https://brieflands.com/journals/jcma/articles/173477