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肺泡蛋白沉积症患者出现意外的弥漫性肺部病变:一例报告。

Unexpected diffuse lung lesions in a patient with pulmonary alveolar proteinosis: A case report.

作者信息

Jian Li, Zhao Qi-Quan

机构信息

Department of Endocrinology, Dazu Hospital of Chongqing Medical University, The People's Hospital of Dazu, Chongqing 402360, China.

Department of Respiratory and Critical Care Medicine, Dazu Hospital of Chongqing Medical University, The People's Hospital of Dazu, Chongqing 402360, China.

出版信息

World J Clin Cases. 2023 Jul 16;11(20):4932-4936. doi: 10.12998/wjcc.v11.i20.4932.

Abstract

BACKGROUND

Pulmonary alveolar proteinosis (PAP) often presents nonspecifically and can be easily confused with: (1) Idiopathic interstitial lung fibrosis; (2) alveolar carcinoma; (3) pulmonary tuberculosis; and (4) other lung diseases such as viral pneumonia, mycoplasma pneumonia, and chlamydial pneumonia.

CASE SUMMARY

Diagnosis: In this case, a patient was diagnosed with PAP through transbronchial cryobiopsy (TBCB) and quantitative metagenomic next-generation sequencing, which confirmed the impairment of surfactant turnover as the underlying cause of PAP. Interventions: High-volume total lung lavage was performed for this patient. Outcomes: The patient's clinical condition had improved significantly by the 6-month follow-up, with a 92% finger oxygen saturation. A repeat chest computed tomography scan revealed scattered patchy ground-glass shadows in both lungs, which was consistent with alveolar protein deposition but with a lower density than in the radiograph from October 23, 2022.

CONCLUSION

TBCB has unique advantages in diagnosing atypical alveolar protein deposition, particularly for enabling the early detection of PAP. This information can help patients take preventive measures to prevent or halt PAP development by avoiding dusty environments and seeking treatment with total lung lavage and inhaled granulocyte macrophage colony-stimulating factor.

摘要

背景

肺泡蛋白沉积症(PAP)通常表现不具有特异性,容易与以下疾病混淆:(1)特发性间质性肺纤维化;(2)肺泡癌;(3)肺结核;以及(4)其他肺部疾病,如病毒性肺炎、支原体肺炎和衣原体肺炎。

病例摘要

诊断:在本病例中,一名患者通过经支气管冷冻活检(TBCB)和定量宏基因组下一代测序被诊断为PAP,这证实了表面活性剂更新受损是PAP的根本原因。干预措施:对该患者进行了大容量全肺灌洗。结果:到6个月随访时,患者的临床状况有显著改善,手指血氧饱和度为92%。重复胸部计算机断层扫描显示双肺散在斑片状磨玻璃影,这与肺泡蛋白沉积一致,但密度低于2022年10月23日的X光片。

结论

TBCB在诊断非典型肺泡蛋白沉积方面具有独特优势,特别是能够早期发现PAP。这些信息可以帮助患者采取预防措施,通过避免多尘环境以及寻求全肺灌洗和吸入粒细胞巨噬细胞集落刺激因子治疗来预防或阻止PAP的发展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb2/10424030/6b2758770509/WJCC-11-4932-g001.jpg

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