Proteinose alveolar pulmonar: série de quatro casos

Thompson, João Carlos; Kishima, Marina Okuyama; Gomes, Mariana Ulbricht; Menezes, Mariano de Almeida; Neto, José Perandré; Pereira, Paula Tapia Gomes

doi:10.1590/s1806-37132006000300013

Cited by 4 publications

(2 citation statements)

References 15 publications

(55 reference statements)

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“…Few patients require more than six WLL cycles, and less than 10% of patients do not respond to WLL. 25 Asymptomatic or oligosymptomatic patients with normal or near-normal lung function and without hypoxemia require periodic evaluation rather than immediate treatment. Symptomatic patients with moderate to severe lung function impairment, hypoxemia, or both can undergo WLL; although there is no consensus regarding which PAP patients should undergo WLL, those with moderate or severe dyspnea at rest or dyspnea on exertion and a PaO 2 of < 60 mmHg at rest or an SpO 2 of < 90% during exercise testing are candidates for the procedure.…”

Section: Discussionmentioning

confidence: 99%

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

et al. 2018

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Objective:Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods:This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results:We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions:To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

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Section: Discussionmentioning

confidence: 99%

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

et al. 2018

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“…There are only few case reports describing PAP during pregnancy. [59606162] Its presence may lead to perinatal or postnatal complications. [59606162] Whether physiologic changes in pregnancy or alteration in levels of various growth factors predispose to PAP is not known.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

et al. 2016

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Background:Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by deposition of lipoproteinaceous material within alveoli, with a variable clinical course. Here, we report an experience of management of PAP at our center. A systematic review of previously reported cases from India is also included in the article.Materials and Methods:This study included patients with primary PAP managed at our center from 2009 to 2015. Diagnosis of primary PAP was based on histopathologic diagnosis on bronchoalveolar lavage or transbronchial lung biopsy and absence of causes of secondary PAP. For systematic review of Indian publications, the literature search was performed using PubMed and EMBASE databases using the terms “pulmonary alveolar proteinosis'” or “alveolar proteinosis” and “India” or “Indian.”Results:During the above-specified period, five patients with diagnosis of PAP were admitted at our center. Median age of patients was 32 years (interquartile range [IQR] 30.5–59); 80% were female. Mean duration (± standard deviation) of symptoms was 6.2 (±1.79) months. Anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies were elevated in 4 out of 5 patients (80%). For management, whole lung lavage (WLL) was done for four patients with median volume of 32.5 (IQR 18–74) L per patient. All the patients showed significant symptomatic as well as improvement in physiological parameters. Subcutaneous GM-CSF and ambroxol were given to 3 patients and 1 patient, respectively. The median follow-up of all patients was 18 (IQR 5–44) months. A systematic review of all Indian studies of PAP revealed thirty publications.Conclusions:WLL is the most common, effective, and safe therapy in patients with PAP. GM-CSF administration is an efficacious treatment for patients with incomplete response after WLL.

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Pulmonary alveolar proteinosis: a case report and world literature review

Huaringa

Francis

2016

Respirology Case Reports

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Proteinose alveolar pulmonar: série de quatro casos

Abstract: In the cases evaluated, whole-lung lavage proved an efficient treatment for pulmonary alveolar proteinosis. Although some patients presented a certain resistance to the procedure, it might lead to complete remission of the disease in others.

Cited by 4 publications

References 15 publications

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

Pulmonary alveolar proteinosis: a case report and world literature review

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