Dataset Information

Efficacy of whole lung lavage in pulmonary alveolar proteinosis: a 20-year experience at a reference center in Thailand.

ABSTRACT:

Background

Pulmonary alveolar proteinosis (PAP) is a rare lung syndrome. The current standard treatment is whole lung lavage (WLL). We reviewed PAP cases treated with WLL during a 243-month period. The primary objective was to describe the efficacy of WLL. We compared chest imaging resolution and pulmonary function tests (PaO₂ and DLCO) before the first and after the last WLL. The secondary objectives were to compare mMRC dyspnea scores, other lung function parameters, and complications of WLL.

Methods

We retrospectively reviewed PAP patients from 1 January 2000 to 31 March 2020. Demographic data, pulmonary function tests, and the efficacy of WLL were collected from the electronic medical database and analyzed by descriptive analysis. Differences in data used the student t-test to compare parameters pre- and post-WLL.

Results

A total of 19 PAP patients and 50 WLL procedures were included. Eleven patients (57.9%) were females and the mean age was 51.5±11.7 years. Dyspnea (100%) and cough (94.7%) were the two leading symptoms. The most common indication for WLL was progressive dyspnea. There were significant improvements in SpO₂ from 86% to 94% (P<0.001), PaO₂ from 49.3 to 66.1 (P<0.001), DLCO from 31.8% to 52.5% predicted (P=0.013), and the mMRC dyspnea score from 3 to 2 (P<0.001) without major complications.

Conclusions

WLL is an effective standard treatment for PAP cases. It is safe and can be used as a primary treatment in case of inhaled anti GM-CSF is not available.

SUBMITTER: Kaenmuang P

PROVIDER: S-EPMC8264701 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Efficacy of whole lung lavage in pulmonary alveolar proteinosis: a 20-year experience at a reference center in Thailand.

Kaenmuang Punchalee P Navasakulpong Asma A

Journal of thoracic disease 20210601 6

<h4>Background</h4>Pulmonary alveolar proteinosis (PAP) is a rare lung syndrome. The current standard treatment is whole lung lavage (WLL). We reviewed PAP cases treated with WLL during a 243-month period. The primary objective was to describe the efficacy of WLL. We compared chest imaging resolution and pulmonary function tests (PaO<sub>2</sub> and DLCO) before the first and after the last WLL. The secondary objectives were to compare mMRC dyspnea scores, other lung function parameters, and com ...[more]

PMID: 34277049

Similar Datasets

Project description:BackgroundTruncus arteriosus (TA) is a complex congenital heart disease that carries morbidities in the first year of life. Previous authors have reported an operative mortality of 50%. In this report, we aim to report on the survival of patients with TA in our medical center in the recent era.MethodsA retrospective review of all patients diagnosed with TA in Siriraj Hospital, Thailand from August 1995 to March 2018 was performed. Patients with single ventricle, hemiTA were excluded. The characteristics and outcomes of repaired and unrepaired TA patients with a known recent functional status in 2018 were reviewed. Operative mortality risks were analyzed using a multivariate model.ResultsA total of 74 patients (median age at referral: 70 days) were included in the cohort. One-third of the patients had associated anomalies including DiGeorge syndrome (13.5%). Anatomical repair was not performed in 22 patients (29.7%). The median age at time of repair for the 52 patients was 133 days (range: 22 days to 16.7 years). Complex TA was 10%. Early mortality occurred in 16 patients (30.8%). Five patients (9.6%) had late deaths at 0.3-1.2 years. Significant mortality risk was weight at time of operation <4 kg (HR 3.05, 95% CI [1.05-8.74], p-value 0.041). Of the 31 operation survivors, 17 required re-intervention within 0.4-11.4 years. Eight patients had reoperation at 8.7 years (range: 2.7-14.6 years) post-repair. Freedom from reoperation was 93%, 70.4%, and 31%, at 5, 10, and 15 years, respectively. All late survivors were in functional class I-II. Of the 22 unrepaired TA patients, 11 patients (50%) died (median age: 13.6 years; range: 14 days-32.8 years). Survival of unrepaired TA patients was 68.2%, 68.2%, and 56.8, at 5, 10, and 15 years of age, respectively. At the end of study, 11 survivors of TA with palliative treatment had a recent mean oxygen saturation value of 84.1 ± 4.8% and a mean weight for height of 81.4 ± 12.7%, which were significantly lower than those of 31 late-survivors who had undergone anatomical repair.ConclusionContemporary survival rates of patients with TA following operation in the center has been gradually improved over time. Most of the operative mortality occurs in the early postoperative period. Compared to patients with TA who had palliative treatment, operative survivors have a better functional status even though they carry a risk for re-intervention.

Project description:RationaleWhole lung lavage (WLL) is a widely accepted palliative treatment for autoimmune pulmonary alveolar proteinosis (aPAP) but does not correct myeloid cell dysfunction or reverse the pathological accumulation of surfactant. In contrast, inhaled recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF) is a promising pharmacological approach that restores alveolar macrophage functions including surfactant clearance. Here, we evaluate WLL followed by inhaled rGM-CSF (sargramostim) as therapy of aPAP.Methods18 patients with moderate-to-severe aPAP were enrolled, received baseline WLL, were randomised into either the rGM-CSF group (receiving inhaled sargramostim) or control group (no scheduled therapy) and followed for 30 months after the baseline WLL. Outcome measures included additional unscheduled "rescue" WLL for disease progression, assessment of arterial blood gases, pulmonary function, computed tomography, health status, biomarkers and adverse events. Patients requiring rescue WLL were considered to have failed their assigned intervention group.ResultsThe primary end-point of time to first rescue WLL was longer in rGM-CSF-treated patients than controls (30 versus 18 months, n=9 per group, p=0.0078). Seven control patients (78%) and only one rGM-CSF-treated patient (11%) required rescue WLL, demonstrating a 7-fold increase in relative risk (p=0.015). Compared to controls, rGM-CSF-treated patients also had greater improvement in peripheral arterial oxygen tension, alveolar-arterial oxygen tension difference, diffusing capacity of the lungs for carbon monoxide and aPAP biomarkers. One patient from each group withdrew for personal reasons. No serious adverse events were reported.ConclusionsThis long-term, prospective, randomised trial demonstrated inhaled sargramostim following WLL reduced the requirement for WLL, improved lung function and was safe in aPAP patients. WLL plus inhaled sargramostim may be useful as combined therapy for aPAP.

Project description:BACKGROUND:Trocar-site hernia (TSH) is a serious complication after laparoscopic procedures. Although it is a rare entity, it can have life-threatening consequences. This study aimed to retrospectively analyze the potential associated factors for TSH following gynecologic laparoscopy and summarize prevention strategies based on our experience. METHODS:We searched for gynecological laparoscopic surgeries in the medical records system of Peking Union Medical College Hospital (PUMCH) from August 1998 to July 2018 and further sifted through the results for cases involving TSH. All included patients were divided into different groups according to patient characteristics, and the rate of TSH was compared among groups. Moreover, the detailed information of all patients with TSH was recorded and analyzed. Statistical analyses were performed with GraphPad Prism 6. RESULTS:The approximate total rate of post-operative TSH among gynecologic laparoscopy procedures performed at PUMCH in the last 20 years was 0.016% (9/55,244). The rate of TSH was significant higher in elder patients (?60 years old; 3/2686, 0.112%) than in younger patients (<60 years old, 6/52,558; 0.011%, P?=?0.008). Moreover, the approximate rate of TSH was significantly higher after single-incision laparoscopic surgery (SILS, 2/534, 0.357%) than conventional laparoscopic surgery (7/54,710, 0.013%, P?=?0.003). The average age of patients with TSH was 53.4 years (range, 35.0-79.0 years). Two of the nine patients had late-onset TSH following SILS; the other seven had early-onset TSH following conventional laparoscopy. Five TSH cases occurred at right lateral port sites, and the remaining three occurred at the umbilical port site. All patients underwent repair surgery, and one required small bowel resection. CONCLUSION:Older age and SILS are potential associated factors for TSH development, while reducing excessive manipulation and improving suturing skills, especially at the umbilical trocar site following SILS and the right lateral trocar site, can avoid herniation.

Dataset Information

Efficacy of whole lung lavage in pulmonary alveolar proteinosis: a 20-year experience at a reference center in Thailand.

Background

Methods

Results

Conclusions

Publications

Efficacy of whole lung lavage in pulmonary alveolar proteinosis: a 20-year experience at a reference center in Thailand.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets