[Research progress of cell therapy in hereditary pulmonary alveolar proteinosis]

Zhonghua Jie He He Hu Xi Za Zhi. 2023 Jul 12;46(7):730-734. doi: 10.3760/cma.j.cn112147-20230107-00008.
[Article in Chinese]

Abstract

Hereditary pulmonary alveolar proteinosis (hPAP) is a rare interstitial lung disease caused by mutation in CSF2RA/CSF2RB, characterized by the deposition of pulmonary surfactant due to the alveolar macrophage dysfunction. The whole lung lavage can effectively alleviate the symptoms but is associated with potential complications. Cell therapy is a novel approach with advances that provide a new therapeutic strategy for the treatment of hPAP.

遗传性肺泡蛋白沉积症(hPAP)是一种罕见的间质性肺疾病,由CSF2RA或CSF2RB突变引起,其特征为肺泡巨噬细胞功能失衡引起表面活性物质沉积。全肺灌洗治疗可有效缓解症状,但具有一定风险。细胞治疗是一种新型治疗方法,已在hPAP治疗中取得进展,为hPAP的治疗提供了新的治疗策略。.

Publication types

  • English Abstract

MeSH terms

  • Bronchoalveolar Lavage
  • Cell- and Tissue-Based Therapy
  • Humans
  • Macrophages, Alveolar
  • Mutation
  • Pulmonary Alveolar Proteinosis* / genetics
  • Pulmonary Alveolar Proteinosis* / therapy
  • Pulmonary Surfactants*

Substances

  • Pulmonary Surfactants