Primary Pulmonary Lymphatic Flow Disorder Associated Plastic Bronchitis

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. The clinical symptoms of lymphocytic PB are nonspecific; thus, the diagnosis may be long delayed, especially in the absence of chylothorax. 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. 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. 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.

Clinical Features of Plastic Bronchitis Related to Respiratory Tract Infection in 269 Children

Background. Plastic bronchitis (PB) is a pulmonary disease characterized by the formation of bronchial casts (BCs) that lead to airway blockage. The study aimed to investigate the clinical features of PB related to respiratory tract infection. Methods. A retrospective analysis was performed on data collected over a 5-year period (from January 2015 to December 2019) on children with PB (n=269). The clinical manifestations, laboratory data, imaging findings and management, were investigated. The single fiberoptic bronchoscopy (FOB, n=144) and multiple-treatment groups (n=125) were compared.Results. A total of 269 PB children were included with a mean age of 6.7 ± 2.8 years. The majority of cases (n=241, 89.6%) were diagnosed with Mycoplasma pneumonia (MP) infection. The mean duration of fever and hospitalization was 10.6 ± 3.7 and 9.3 ± 3.2 days, respectively. All patients presented with cough and fever, 62 (23.0%) suffered from hypoxemia, and 144 (53.5%) had extrapulmonary complications. Higher levels of ESR, CRP, PCT, IL-6, LA, LDH, FER and D-dimer were observed. CT findings, including pulmonary consolidation, segmental or lobar atelectasis, pleural effusion and pleural thickening, were observed in 97.4%, 46.5%, 47.9% and 63.2% of cases, respectively. Furthermore, multivariate logistic regression analysis showed that N% >75.5%, LDH >598.5U/L, and D-dimer>1.2mg/L were independent risk factors for multiple therapeutic FOB. Conclusions. MP is a major pathogen responsible for PB in children. Patients with PB are more likely to experience persistent fever and excessive inflammation and have severe radiological findings. FOB is an effective treatment for patients with PB, and children may require multiple FOBs for cast removal. N% >75.5%, LDH >598.5U/L and D-dimer > 1.2mg/L are independent predictors of multiple FOB treatment.

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.

Case Report: Clinical Analysis of Fulminant Mycoplasma pneumoniae Pneumonia in Children

Fulminant Mycoplasma pneumoniae pneumonia (FMPP) accounts for 0.5–2% of all MPP cases, which is considered as MPP combined with severe complications such as hypoxemia, acute respiratory distress syndrome, or acute respiratory failure. It primarily affects young adults with no underlying disease. Although some studies have proved the severity of FMPP, the details about clinical diagnosis and treatment of FMPP in children have been rarely reported. In this case study, we described three cases who suffered from FMPP. These children not only developed acute lung injury and multiple organ involvement within 7 days of treatment, but were also found plastic bronchitis by bronchoscopy. Finally, all the patients were treated successfully with azithromycin, glucocorticoid, and bronchoscopy lavage. We conclude that this case study would contribute to raise awareness with respect to FMPP, which may occur at a younger age with faster disease progression and common extrapulmonary manifestations. It also reinforces the importance of early identification and prompt intervention to save life of children and reduces sequelae. Further studies are needed about mechanism of FMPP.

Plastic bronchitis associated with influenza A virus in children with asthma

Plastic bronchitis (PB) is a rare and potentially fatal disease characterized by acute progressive dyspnea caused by bronchial casts in the bronchial tree. We analyzed two children with asthma and PB who presented with high fever, cough and dyspnea. Both cases showed acute onset and rapid disease progression. Laboratory examination revealed that both children were infected with influenza A virus. Emergency fiberoptic bronchoscopy was performed within 20 hours of admission. Immediately after removing the bronchial casts, their dyspnea symptoms improved significantly, and they recovered after comprehensive treatment with antiviral drugs, antibiotics and glucocorticoids. When children with asthma have acute progressive and difficult-to-relieve dyspnea after infection with influenza A virus, clinicians should be aware of the possibility of PB and perform bronchoscopy as soon as possible to facilitate early diagnosis and treatment and improve patient prognosis.

Analysis of Clinical Characteristics and Risk Factors of Plastic Bronchitis in Children With Mycoplasma pneumoniae Pneumonia

Objective: To analyze the clinical characteristics of plastic bronchitis (PB) in children with Mycoplasma pneumoniae pneumonia (MPP) in order to explore its risk factors.Methods: A retrospective analysis was performed in MPP children receiving bronchoscopy admitted to department of respiratory medicine in Shanghai Children's Hospital from January 2018 to December 2020. According to the bronchoscopic findings, the patients were divided into PB group and non-PB group. The clinical manifestations, laboratory examination, etiology, treatment methods and outcomes of the children were analyzed. Logistic regression was used to analyze the risk factors for PB in children with MPP.Results: A total of 296 children with MPP were enrolled in the study, including 42 (14.2%) children in the PB group and 254 (85.8%) children in the non-PB group. There was no difference in the ratios of gender, age, proportion of fever, cough, wet rales, and wheezing rales between the two groups (P > 0.05). The univariate analysis showed that there were significant differences between the PB group and the non-PB group in LDH, D-dimer, CD3+CD4+(%), CD3+CD4+/CD3+CD8+, CD3 count, CD4 count, CD8 count, complement 3, IL8, IL-1β, IL-2, IL-10 (P < 0.05). The multivariate logistic regression analysis showed that fever duration > 12 d, IL-8 > 2,721.33 pg/ml, LDH > 482 U/L and complement 3 <1.02 g/L were independent risk factors for PB in children with MPP.Conclusions: Children with PB caused by MPP have protracted fever, a strong inflammatory response and immune function disturbance.