Objectives: Primary ciliary dyskinesia (PCD) is a rare genetic disease mainly involved in lung dysfunction. PCD patient outcomes after azithromycin (AZM) treatment have rarely been reported. This study was aimed to assess AZM treatment effects on disease progression of pediatric PCD patients. Study design: This retrospective follow-up study involved PCD patients diagnosed from December 2009 to December 2020. Changes of clinical outcomes, pulmonary function, and chest computed tomography findings were compared between untreated and AZM-treated patients. Results: Of 63 enrolled patients (median follow-up duration of 3.1 years), 30 received AZM (AZM-treated group) and 33 received no AZM (AZM-untreated group). At diagnosis, no significant intergroup differences in age, sex, height, weight, respiratory infection frequency, and FEV1% and FVC% predicted values were found, although FEF25-75% predicted was lower in AZM group. Between treatment initiation and follow-up, patients in AZM-treated group had less respiratory infection frequency than that of controls (1.4 ± 0.8 VS 3.0 ± 2.1 times/year, respectively, P = 0.001) and fewer AZM-treated group patients exhibited exercise intolerance. Increases above baseline of AZM-treated group FEV1% and FVC% predicted values exceeded corresponding control increases, but intergroup differences were insignificant (FEV1% predicted: 5.3 (-13.4, 9.4) VS 1.8 (-12.1, 9.5), P = 0.477; FVC% predicted: 6.7 (-7.6, 18.8) VS 1.6 (-5.6, 7.6), P = 0·328). Conclusions: Long-term AZM treatment can reduce respiratory infection frequency and may maintain pulmonary diseases stable in pediatric PCD patients.

Background: Lymphatic plastic bronchitis (PB) most commonly occurs in children with congenital heart disease as a result of secondary pulmonary lymphatic flow disorder (PLFD). However, PB caused by primary PLFD is rare. We made a retrospective analysis of two children diagnosed with PB due to primary PLFD, in order to contribute to further understanding of these disorders. Results: Patient 1, an eight-year-old boy, presented with chronic productive cough and expectorated milky-white mucous plugs accompanied by intermitted wheezing for one year. Patient 2, a nine-month-old girl, presented with episodes of acute respiratory distress with expectoration of milky-white bronchial casts for four months. There was no obvious evidence of infection in either child. Bronchoscopy showed massive milky-white casts blocking the airway in patient 2; no casts were observed in patient 1. Bilateral thickening of bronchovascular bundles and interlobular septal, as well as multiple patchy ground-glass opacities were seen on chest computed tomography (CT) in both patients. Lymphangioscintigraphy demonstrated pulmonary lymph reflux in both patients and slowed lymphatic drainage of the lower limbs in patient 1. Primary PLFD was considered for both patients, and a diagnosis of yellow nail syndrome was made in patient 1. Both patients received lymphatic interventional treatment, but all experienced recurrence following the procedure. Conclusions: Primary PLFD is a rare but significant cause of PB in children. Chest CT findings have highly suggestive significance for the diagnosis. The lymphatic interventional procedure may be effective for short-term resolution of symptoms, but prone to recurrence.

there are several advantages to the use of transbronchial lung cryobiopsy (TBLC), including less trauma, fewer complications, large and high-quality specimens, and lower cost. This procedure is mainly used for the etiological diagnosis of diffuse parenchymal lung diseases (DPLD) [2] and its most common complications, including bleeding and pneumothorax. Virtual bronchoscopy navigation (VBN) can assist bronchoscopists in pinpointing the target area in the lung and designing an appropriate navigation pathway to accurately reach the target lesion [3]. Thus, VBN improves the rate of diagnosis and reduces the risk of bleeding and pneumothorax. In this report, which we believe to be the first of its kind, we performed TBLC guided by VBN.

Objectives: We aimed to determine the bronchoscopic features of children with severe Mycoplasma pneumoniae pneumonia (SMPP), and correlation with obliterative bronchitis (OB), so as to help early clinical evaluation and treatment of pediatric SMPP. Methods: 213 pediatric SMPP cases admitted to Beijing Children’s Hospital were included. Medical records and bronchoscopic manifestations at different SMPP stages were retrospectively analyzed . Results: Of 213 acute-stage pediatric SMPP patients, bronchoscopy revealed 22 cases (10.3%) with subacute-stage or recovery-stage OB, for an OB incidence rate in cases with mucous embolus of 48.8% (22/47), a significantly higher rate than the rate without mucous embolus (0/166, 0%) (P < 0.001). Notably, the OB incidence rate for children undergoing bronchoscopy within 10 days (9/142, 6.3%) of SMPP onset was significantly lower than in children undergoing bronchoscopy 10 days post-disease onset (13/71, 76.5%) (P < 0.007). Conclusions: In pediatric SMPP patients, airway mucus and debris from epithelial necrosis and exfoliation can block airway subbranches, readily leading to OB.