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Vol. 156. Issue 11.
Pages 555-557 (June 2021)
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Vol. 156. Issue 11.
Pages 555-557 (June 2021)
Brief report
Pulmonary alveolar proteinosis: A single center retrospective analysis of 14 cases
Proteinosis alveolar: estudio unicéntrico de 14 casos
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Ning Zhanga,b, Zhilong Jiangb, Changzhou Shaob,
Corresponding author
shaoczzsh@126.com

Corresponding author.
a Department of Pulmonary Medicine, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen 361000, China
b Shanghai Institute of Respiratory Diseases, Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai 200032, China
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Table 1. Baseline characteristics of the fourteen cases.
Table 2. Pretreatment and follow-up parameters of the fourteen cases.
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Abstract
Introduction

Pulmonary alveolar proteinosis (PAP) is a rare lung disease, characterized by abnormal alveolar accumulation of enlarged foamy macrophages and periodic acid-Schiff (PAS)-positive materials. Knowledge of the disease characteristics is still lacking.

Objective

To help clinicians gain a better understanding of this rare disease.

Methods

We undertook a retrospective analysis of 14 adult patients with PAP, treated in Zhongshan Hospital, Fudan University.

Results

Serum lactate dehydrogenase (LDH) was correlated with the arterial partial pressure of oxygen (PaO2) and diffusion capacity for carbon monoxide (DLCO). Transbronchial lung biopsy (TBLB) established a definitive diagnosis for a positive rate of 100%. The patients underwent whole lung lavage (WLL) and exhibited varying degrees of remission. The patients with mild symptoms received only supportive care and observation, and remained stable during follow-up.

Conclusion

LDH may correlate with disease severity. Bronchoscopy is sufficiently sensitive for a definite diagnosis. Conventional bilateral whole lung lavage proved a reliable treatment for indicated patients, but selective unilateral lung lavage or observation may be a rational choice in certain patients.

Keywords:
Pulmonary alveolar proteinosis
Interstitial lung disease
Rare lung disease
Resumen
Introducción

La proteinosis alveolar pulmonar (PAP) es una enfermedad pulmonar rara, caracterizada por una acumulación alveolar anormal de macrófagos espumosos agrandados y materiales periódicos de ácido-Schiff (PAS). Todavía se carece de la cognición de las características de la enfermedad.

Objetivo

Para ayudar a los médicos a comprender mejor esta enfermedad rara.

Métodos

Realizamos un análisis retrospectivo de 14 pacientes adultos con PAP, tratados en el Hospital Zhongshan, Universidad de Fudan.

Resultados

La lactato deshidrogenasa (LDH) sérica se correlacionó con la presión parcial de oxígeno arterial (PaO2) y la capacidad de difusión del monóxido de carbono (DLCO). La biopsia pulmonar transbronquial (TBLB) que establece un diagnóstico definitivo para una tasa positiva del 100%. Los pacientes recibieron lavado pulmonar completo (WLL) y exhibieron diferentes grados de remisión. Los pacientes con síntomas leves aceptaron solo atención de apoyo y observaciones, se mantuvieron estables durante el seguimiento.

Conclusión

La LDH puede correlacionarse con la gravedad de la enfermedad. La broncoscopia es suficientemente sensible para un diagnóstico definitivo. El lavado pulmonar bilateral convencional demostró ser un tratamiento confiable para los pacientes indicados, pero el lavado selectivo unilateral u observación pulmonar puede ser una opción racional en ciertos pacientes.

Palabras clave:
Proteinosis alveolar pulmonar
Enfermedad pulmonar intersticial
Enfermedad pulmonar rara

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