儿童肺泡蛋白沉积症：诊断与治疗结果

Pulmonary Alveolar Proteinosis in Children: Diagnosis and Treatment Outcomes.

作者信息

Hassanzad Maryam, Tashayoie-Nejad Sabereh, Boloursaz Mohammadreza, Mahdaviani Seyed Alireza, Baghaie Nooshin, Ghaffaripour Hosseinali, Aghahosseini Farahnaz, Hossein Ahmadi Zargham, Parsa Tahereh, Farzanegan Behrooz, Fakharian Atefeh, Seyedi Seyed Javad, Velayati Ali Akbar

机构信息

Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Lung Transplantation Research Center, NRITLD, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Tanaffos. 2021 Apr;20(4):363-367.

PMID:36267924

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9577214/

Abstract

BACKGROUND

Pulmonary Alveolar Proteinosis (PAP) is an uncommon pulmonary disease characterized by the accumulation of surfactant composed of proteins and lipids due to disruption of surfactant clearance by alveolar macrophages. The current standard treatment is lung lavage. There are no specific criteria for lavage, but in case of observing these signs it is recommended to perform lavage for the patient: progressive respiratory failure, no labored breathing at rest, and drop in oxygen level during activity (>5%).

MATERIALS AND METHODS

In this study, patients with PAP admitted to Pediatric ward of Masih Daneshvari Hospital were studied. The required data were collected including the patient's demographic data, clinical signs and radiographic data, the number of admissions, the age of diagnosis, detection and treatment methods, number of lavage, current condition of the patient, and in case of death, the cause of death.

RESULTS

In this study, 17 patients with PAP who were admitted during the past 15 years were examined; among which 7 patients were boys (41.2%) and 10 were girls (58.8%). The mean age of population was 11.79±7.21 years. Transbronchial Lung Biopsy (TBLB) (47.1%) and open lung biopsy (52.9%) were used for diagnosis of patients. Lung lavage was used to treat patients, 15 of whom were treated by this method. Five of the patients died because of their serious conditions.

CONCLUSION

Therapy method in the present study was lavage for both lungs, and it was performed for all patients except for two patients due to their anatomical complications. This method is still considered as the gold standard for PAP. Considering the findings from previous studies and the present study, it seems that Whole Lung Lavage (WLL) was fruitful for patients who had the indication for using this therapy and it played a significant role in improving the prognosis of patients. Besides, it is recommended to do follow-up regularly in order to have more therapeutic efficacy and increased patient longevity.

摘要

背景

肺泡蛋白沉积症（PAP）是一种罕见的肺部疾病，其特征是由于肺泡巨噬细胞对表面活性物质清除功能的破坏，导致由蛋白质和脂质组成的表面活性物质积聚。目前的标准治疗方法是肺灌洗。对于灌洗没有具体标准，但如果观察到以下体征，则建议为患者进行灌洗：进行性呼吸衰竭、静息时无呼吸困难、活动期间氧水平下降（>5%）。

材料与方法

在本研究中，对马西·达内什瓦里医院儿科病房收治的PAP患者进行了研究。收集了所需数据，包括患者的人口统计学数据、临床体征和影像学数据、入院次数、诊断年龄、检测和治疗方法、灌洗次数、患者当前状况，以及死亡患者的死亡原因。

结果

在本研究中，对过去15年期间收治的17例PAP患者进行了检查；其中7例为男性（41.2%），10例为女性（58.8%）。人群平均年龄为11.79±7.21岁。经支气管肺活检（TBLB）（47.1%）和开胸肺活检（52.9%）用于患者诊断。采用肺灌洗治疗患者，其中15例接受了该方法治疗。5例患者因病情严重死亡。