Diffuse alveolar hemorrhage (DAH) is a rare life‑threatening pulmonary disorder in children, characterized by cough, hemoptysis and dyspnea. The pathogenesis is not fully understood, posing notable challenges for clinical diagnosis and management. Recent advances have gradually revealed potential etiologies and associated immune mechanisms, driving the development and application of novel therapies. The present review aimed to summarize the current understanding of the etiology, diagnostic approaches and therapeutic strategies for pediatric DAH, with a focus on the emerging role of the CD20 monoclonal antibody rituximab, and highlighted clinical evidence supporting its use in immune‑related DAH. The present review aimed to provide a foundation for further research and optimize clinical decision‑making.

BACKGROUND:An imbalance in M1/M2 macrophage polarization has been shown to play a critical role in the pathogenesis of diffuse alveolar hemorrhage (DAH) in mouse models. However, its contribution to immune-associated DAH in humans remains unclear. This study aimed to investigate the role of macrophage M1/M2 polarization imbalance in the pathogenesis of immune-associated DAH in children. METHODS:A total of 24 children with immune-associated DAH (DAH group) and 13 children with acute airway foreign body or mild benign airway stenosis (control group) were enrolled. Bronchoalveolar lavage fluid (BALF) was obtained via bronchoscopy, and supernatant was isolated by centrifugation. Cytokine levels in BALF supernatant were measured using a cytometric bead array. Monocyte-derived macrophages (MDMs) were stimulated in vitro with BALF supernatant. The mRNA expression levels of M1 markers (IL-1β, TNF-α, IL-6, TGM2) and M2 markers (CD163, MRC1) were quantified using real-time PCR. Protein expression of surface markers (CD14, CD80, CD86, CD163, CD206) was analyzed by flow cytometry. RESULTS:(1) Levels of MCP-1, IL-6 and IL-8 in BALF supernatant were significantly higher in the DAH group than those in the control group. (2) MDMs stimulated with BALF supernatant from the DAH group showed significantly elevated mRNA expression of IL-1β and IL-6 and significantly reduced expression of MRC1 compared to those stimulated with control supernatant. (3) Flow cytometry revealed significantly higher CD86 expression and significantly lower CD163 and CD206 expression in MDMs treated with DAH group BALF supernatant. CONCLUSION:This study provides evidence that macrophage M1/M2 polarization imbalance contributes to the pathogenesis of immune-associated DAH in humans, highlighting a potential target for immunomodulatory therapy.

BackgroundPulmonary thromboembolism is rare in children, but can be life-threatening. Timely diagnosis and treatment of pulmonary thromboembolism are crucial for reducing mortality associated with pulmonary thromboembolism in children. While guidelines for pulmonary thromboembolism in adults are available, guidelines for standardized diagnosis and management of pulmonary thromboembolism in children are not. This expert consensus aims to provide recommendations for the management of pulmonary thromboembolism in children based on the current best available evidence.Data sourcesFollowing the World Health Organization Handbook for Guideline Development, the expert panel consisted of 30 members from different clinical areas. The panel identified clinical questions through systematic reviews and expert discussions, systematically reviewed evidence on pulmonary thromboembolism in children, and evaluated the quality of the evidence using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. Using the GRADE Evidence to Decision Framework, the panel made recommendations, considering the effects of interventions, resource use, values and preferences, equity, acceptability, and feasibility.ResultsThe epidemiology, classification, and pathophysiology characteristics are summarized. The expert panel developed 33 recommendations addressing 20 questions related to diagnosis steps, treatment approaches such as anticoagulant therapy, thrombolysis therapy, catheter-based interventional therapy, surgical embolectomy, multidisciplinary team, and treatment of patients with comorbidities, prognosis, education, as well as follow-up. Among these, 18 are weak recommendations based on very low quality evidence, and 15 are good practice statements.ConclusionsThe expert panel provided recommendations for pulmonary thromboembolism in children based on available evidence, which was generally low in quality and volume. The panel urges further research on early identification and diagnosis strategies, preventive and therapeutic regimens, and long-term management for pulmonary thromboembolism.

To characterize the clinical features and outcomes of community-acquired Pseudomonas aeruginosa (PA) pneumonia in immunocompetent children. A retrospective analysis was conducted on seven immunocompetent children with community-acquired PA pneumonia hospitalized between January 2015 and June 2025. Pneumonia was defined by acute respiratory symptoms with new radiographic infiltrates. PA infection was confirmed by culture from sterile sites/lower respiratory tract or metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid. All patients were male (n = 7). Age distribution was as follows: 1–12 months (n = 3), 13–36 months (n = 1), 37–60 months (n = 1), and ≥ 61 months (n = 2). Median age at onset was 18.0 months (IQR: 8.0–123.0). All patients presented acutely with fever and cough; two developed respiratory failure within 72 h. Additional clinical features included dyspnea (n = 4), lung rales (n = 4), hemoptysis (n = 3), chest pain (n = 2), and wheezing (n = 1). Chest imaging showed lobar consolidation (n = 5) or mass-like consolidation (n = 2). A total of seven cases were identified, with PA confirmed by culture in four patients and by mNGS of bronchoalveolar lavage fluid in three patients. All isolates were susceptible to anti-pseudomonal β-lactam antibiotics except aztreonam. Complications included definite or suspected empyema (n = 5), pyopneumothorax (n = 3), and bacteremia (n = 2). Three patients required pediatric intensive care, two received invasive mechanical ventilation, two underwent closed thoracic drainage, and one required decortication. There were no deaths, but 4 patients sustained significant residual lung injury secondary to necrotizing pneumonia. Although rare, community-acquired PA pneumonia in immunocompetent children is associated with severe disease and pulmonary complications. Initial therapy with anti-pseudomonal β-lactam antibiotics appears effective in improving outcomes. Repeated cultures are recommended in the cases who remain symptomatic.

BACKGROUND:Disseminated cryptococcosis is a rare disease in children, especially in children with normal immunity. The understanding of this disease needs to be improved. This study aims to update the global situation of disseminated cryptococcosis in non-HIV infected children for the first time. METHODS:The clinical data of a child with disseminated cryptococcosis was retrospectively analyzed, and disseminated cryptococcosis clinical features of published studies were summarized. Electronic databases were searched in February 2025. Clinical studies that meet the criteria were included in the present study. RESULTS:Totally 116 cases were analyzed in this study, including 1 case in our center and 115 cases from 45 studies. The cohort included 82 males (70.7%) and 34 females (29.3%), with ages ranging from 10 months to 18 years. The main clinical manifestations were fever (79.3%), respiratory symptoms (41.4%), and neurological symptoms (39.7%), followed by hepatosplenomegaly (35.3%), rash (27.6%), lymphadenopathy (18.1%), and gastrointestinal symptoms (16.4%). The most commonly affected organs were the lungs (77.6%), central nervous system (53.4%), and lymph nodes (51.7%). Immunodeficiency was present in 12.9% of children (3.4% domestic cases vs. 9.5% foreign cases). Elevated eosinophils were observed in 43 patients (37.1%), and elevated IgE levels in 35 patients (30.2%). The most common pathogen-positive specimens were cerebrospinal fluid (54 cases, 46.6%), blood cultures (49 cases, 42.2%), lymph node biopsies (26 cases, 22.4%), bone marrow (18 cases, 15.5%), and skin samples (8 cases, 6.9%). Combination therapy was administered to 89 patients (76.7%), while 21 patients (18.1%) received monotherapy. Clinical improvement occurred in 94 patients (81.0%), with 15 fatal cases. CONCLUSIONS:Disseminated cryptococcosis in children often presents with fever, respiratory and neurological symptoms, with the lungs, central nervous system, and lymph nodes being the most frequently involved organs. Most cases do not have immunodeficiency or underlying diseases, and blood tests often reveal eosinophilia and elevated IgE levels. The positive detection rates of pathogens are relatively high in blood cultures, cerebrospinal fluid, bone marrow cultures, and lymph node biopsies. The majority of patients achieved favorable therapeutic outcomes with combination therapy.

Diffuse alveolar hemorrhage (DAH) is characterized by the presence of diffuse ground glass opacities and/or consolidation on chest CT. Emphysema has been reported occasionally. Nevertheless, the pathogenesis, incidence, imaging subtypes, and clinical impact of emphysema in patients with DAH are still unclear. This was a retrospective clinical study. Children with DAH who were admitted to our hospital between January 2013 and December 2019 were included. All chest CT images of the patients were analyzed by two board-certified thoracic radiologists. The clinical features of the patients with diffuse emphysema were further analyzed. A matched case-control study was performed to explore the risk factors for developing diffuse emphysema. Ninety-four patients were included. Chest CT scans revealed emphysema in 28 patients (29.8

Immunoglobulin A vasculitis (IgAV) is the most common cause of systemic vasculitis in childhood. Due to the continued use of the disease name “anaphylactoid purpura” in China, several misunderstandings have arisen in clinical practice and treatment regimens differ widely. In addition, new research and evidence-based data have grown. The Subspecialty Group of Immunology, Society of Pediatrics, Chinese Medical Association and the Chinese Alliance of Pediatric Rheumatic and Immunologic Diseases initiated an update of guidelines for the diagnosis and management of childhood IgAV. The aim therefore was to provide agreed consensus recommendations for diagnosis and treatment for children with IgAV. This study utilized the Delphi technique to develop an evidence-based expert consensus for childhood IgAV. We conducted a systematic literature review to retrieve evidence, which was graded using GRADE (Grading of Recommendations Assessment, Development, and Evaluation) criteria. Two rounds of Delphi voting and a consensus meeting involving 23 experts were conducted. Recommendations were accepted when ≥ 75

Bronchiolitis obliterans syndrome (BOS) after hematopoietic stem cell transplantation (HSCT) is a late-onset complication that significantly impairs patients’ quality of life and is associated with a high mortality rate. Currently, the risk factors for BOS after HSCT remain controversial. We therefore conducted a systematic review and meta-analysis to identify potential risk factors associated with BOS after HSCT. Three primary medical databases (PubMed, Web of Science, Embase) were exhaustively reviewed from their inception through November 2024 to assess contributing factors for BOS occurrence following HSCT. Data synthesis was conducted using RevMan 5.4 for meta-analytic evaluation. Fourteen studies involving 10,317 HSCT recipients were included, 778 of whom developed BOS. Female sex (OR = 1.26; 95

Background Respiratory infection is a common trigger for the episode of alveolar hemorrhage in the pediatric diffuse alveolar hemorrahge (DAH). Whereas, a futher detailed study hasn’t been conducted. The aim of this study was to explore the etiological spectrum, clinical features, treatment strategies and outcomes of the respiratory infection induced episode of alveolar hemorrhage (RIIEAH) in the pediatric DAH. Methods The cases of pediatric DAH who had RIIEAH and a definite etiological diagnosis were included. A retrospective study was conducted. Results 1. A total of 16 cases with 21 RIIEAHs were included. Twelve RIIEAHs occurred at the unstable stage, 6 RIIEAHs occurred at the stable stage and 3 RIIEAHs occurred at the end stage. All the RIIEAHs with respiratory failure (n=5) and requirment of invasive mechanical ventilation (n=3) occurred at the unstable or end stage. 2. In the majority of the RIIEAHs (n=19), etiological diagnosis was identified by detecting the nucleic acid of the pathogens. Of these, bronchoalveolar lavage fluid was the most commonly used specimen in 12 RIIEAHs. The majority of the RIIEAHs (n=19) were caused by a single pathogen including mycoplasma pneumoniae (Mp) in 7 RIIEAHs, coronavirus (CoV) in 3 RIIEAHs, haemophilus influenzae (Hi) in 3 RIIEAHs, chlamydia pneumoniae in 2 RIIEAHs, human metapneumovirus in 2 RIIEAHs, acinetobacter baumannii in 1 RIIEAH and pueumocystis carinii in 1 RIIEAH. The rest 2 RIIEAHs were caused by the mixed pathogens including klebsiella pneumoniae and streptococcus pneumoniae in 1 RIIEAH, rhinovirus and CoV in 1 RIIEAH. 3. The majority of the RIIEAHs (n=19) presented with prodromal symptoms and most of them (n=15) occurred within 3 days from the prodromal symptoms. Either worsening anemia or hemoptysis was found in 13 RIIEAHs and dyspnea was found in 9 RIIEAHs. All the 12 RIIEAHs in which bronchosopy had been performed presented with bleeding on bronchoscopy. 4. An intensive glucocorticoid therapy was administrated in the 19 RIIEAHs and a targeted anti-infection treatment was administrated in the 11 RIIEAHs. The majority of the RIIEAHs (n=19) resolved, whereas there was 1 case death. Conclusions 1. RIIEAH could be caused by a varity of pathogens and could occur at any disease stage of pediatric DAH. 2. It usually occurred at the early stage of respiratory infection and presented with worsening anemia or hemoptysis. 3. Bronchoscopy had a good diagnostic value for RIIEAH and idenifying the etiology. 4. An intensive glucocorticoid therapy seemed to be effective and necessary.

Virus infection is a common cause of lung injury and can result in lung fibrosis in severe cases. Furthermore, it is a significant trigger of disease exacerbation in patients with lung fibrosis. However, nearly all the case reports or case series to date have focused on the adult population rather than the pediatric population. Here, we report three cases of virus infection-induced exacerbations of lung injury and fibrosis in children with diffuse alveolar hemorrhage. To our knowledge, this is one of the first articles to report virus infection-induced lung injury and fibrosis in children.

Background: The factors that cause recurrent wheezing in children are complex, and premature delivery may be one of these factors. Little is known about early wheezing in preterm infants.Methods: Data were sourced from 1616 children born between 2007 and 2013 from 8 hospitals in Guangxi, China. All children were followed up by telephone or questionnaire through the sixth year of life. Children were grouped by gestational age (GA): Group A, GA ≤ 32 weeks; Group B, 32 weeks < GA < 37 weeks; and Group C, 37 weeks ≤ GA < 42 weeks.Results: The incidences and risk factors for early wheezing in preterm infants were analysed. The incidences of early wheezing were as follows: Group A > Group B > Group C. The incidence of persistent early wheezing in Group A or Group B was significantly higher than that in Group C, respectively. SGA (95% CI: 1.097 to 7.519) was a risk factor for early wheezing in group A. Male sex (95% CI: 1.595 to 4.501) and family history of allergies (95% CI: 1.207 to 3.352) were risk factors for early wheezing in group B.Conclusions: 1. New-borns with younger GAs had a higher risk of early wheezing. 2. The incidence of persistent early wheezing for preterm infants (GA ≤ 32 weeks and 32 weeks < GA < 37 weeks) was higher than that for full-term infants (37 weeks ≤ GA < 42 weeks). 3. SGA was a risk factor for early wheezing in preterm infants with a GA ≤ 32 weeks. 4. Male sex, personal history of allergies and family history of allergies were all possible factors affecting early wheezing in preterm infants with a GA > 32 weeks but < 37 weeks and full-term infants. Among them, male sex and family history of allergies were risk factors for early wheezing. 5. Mode of delivery, passive smoking, breastfeeding and invasive mechanical ventilation were not possible risk factors for early wheezing in infants of different GAs.

Background This study investigates the clinical characteristics and outcomes of pediatric patients with rheumatic diseases infected with COVID-19 in China. Methods We conducted a retrospective analysis of pediatric patients with rheumatic diseases who contracted COVID-19. Data were collected via a comprehensive questionnaire with a 14-day follow-up. Multivariable logistic regression was used to assess severe outcomes, and network analyses evaluated symptom correlations. Results A total of 1070 cases were collected. Fever (88.05%) and cough (62.75%) were the most common symptoms. Cough, nasal congestion, and runny nose exhibited a stronger correlation with each other. A higher incidence of fever reduced the incidence of two single symptoms (nasal congestion [r = -0.833], runny nose [r = -0.762]). Vaccinated children showed a shorter time to negative COVID-19 conversion (7.21 days vs. 7.63 days, p < 0.05) and lower hospitalization rates (p = 0.025). Prolonged symptom duration was associated with older age (OR: 1.07 [1.04-1.11]; p < 0.001) and systemic lupus erythematosus (OR: 1.47 [1.01-2.12]; p = 0.046). Conclusions Pediatric patients with rheumatic diseases exhibited a wide range of clinical symptoms after COVID-19 infection. The infection generally did not lead to severe outcomes in this study. COVID-19 vaccination was associated with reduced hospitalization risk and expediting the time to negativity for virus. Impacts This manuscript demonstrates a comprehensive analysis of the clinical characteristics and outcomes of COVID-19 infection in pediatric patients with rheumatic diseases in China. It provides critical insights into the specific challenges faced by this vulnerable population and offers practical recommendations for improving patient management during periods of increased infectious risk.

BACKGROUND:Respiratory syncytial virus (RSV) is a leading cause of severe illness in infants, with no effective treatment. Results of a phase 2 trial suggested that ziresovir may have efficacy in the treatment of infants hospitalized with RSV infection. METHODS:In a phase 3, multicenter, double-blind, randomized, placebo-controlled trial conducted in China, we enrolled participants 1 to 24 months of age who were hospitalized with RSV infection. Participants were randomly assigned, in a 2:1 ratio, to receive ziresovir (at a dose of 10 to 40 mg, according to body weight) or placebo, administered twice daily, for 5 days. The primary end point was the change from baseline to day 3 (defined as 48 hours after the first administration) in the Wang bronchiolitis clinical score (total scores range from 0 to 12, with higher scores indicating greater severity of signs and symptoms). The intention-to-treat population included all the participants with RSV-confirmed infection who received at least one dose of ziresovir or placebo; the safety population included all the participants who received at least one dose of ziresovir or placebo. RESULTS:The intention-to-treat population included 244 participants, and the safety population included 302. The reduction from baseline in the Wang bronchiolitis clinical score at day 3 was significantly greater with ziresovir than with placebo (-3.4 points [95% confidence interval {CI}, -3.7 to -3.1] vs. -2.7 points [95% CI, -3.1 to -2.2]; difference, -0.8 points [95% CI, -1.3 to -0.3]; P = 0.002). The reduction in the RSV viral load at day 5 was greater in the ziresovir group than in the placebo group (-2.5 vs. -1.9 log10 copies per milliliter; difference, -0.6 log10 copies per milliliter [95% CI, -1.1 to -0.2]). Improvements were observed in prespecified subgroups, including in participants with a baseline bronchiolitis score of at least 8 and in those 6 months of age or younger. The incidence of adverse events related to the drug or placebo was 16% with ziresovir and 13% with placebo. The most common adverse events that were assessed by the investigator as being related to the drug or placebo were diarrhea (in 4% and 2% of the participants, respectively), an elevated liver-enzyme level (in 3% and 3%, respectively), and rash (in 2% and 1%). Resistance-associated mutations were identified in 15 participants (9%) in the ziresovir group. CONCLUSIONS:Ziresovir treatment reduced signs and symptoms of bronchiolitis in infants and young children hospitalized with RSV infection. No safety concerns were identified. (Funded by Shanghai Ark Biopharmaceutical; AIRFLO ClinicalTrials.gov number, NCT04231968.).

Lymphangiomatosis is a rare disease,which ischaracterized by abnormal proliferation of lymphaticendothelial cells. The proliferative lesions are usually diffuseand multicentric in lymphangiomatosis. It is referred to asdiffuse pulmonary lymphangiomatosis when the thoracicviscera including lung,mediastinum and pleura are involved.Usually, diffuse pulmonary lymphangiomatosis has poorprognosis. Recently,the understanding of the mechanisms ofdiffuse pulmonary lymphangiomatosis has been impoved bythe breakthough of genetics as well as cellular signalingpathway research. What’s more,the clinical application oftargeted drug and their significant effects have brought a newhope to the treatment of diffuse pulmonary lymphangiomatosis.This article mainly introduces the pathogenesis, clinicalmanifestations,diagnostic methods and treatment progress ofdiffuse pulmonary lymphangiomatosis in children.

BACKGROUND:Early-onset pharyngeal airway collapse (PAC) in infants, which presents with onset within 6-months old is relatively rare. This disease has not been given enough attention in clinic. The aim of this study was to explore the clinical features, endoscopic findings and outcomes of early-onset PAC in infants.METHODS:The children of PAC with onset within 6-months old were included. A retrospective study was conducted.RESULTS:(1) Total 26 cases were included. The age of onset was neonatal period in 20 cases, 1 to 3-months old in 5 cases, and 4 to 6-months old in 1 case. (2) The main clinical manifestations were noisy breathing (26/26), suprasternal retraction (18/26), snoring (14/26) and hypoxic episode (13/26). (3) Based on the endoscopic findings, collapse at the retropalatal level was most common (24/26). (4) Twelve cases underwent pharyngolaryngeal CT examination, which revealed abnormal findings in 7 cases. (5) Fifteen cases were accompanied with the other airway malformations. (6) In the group with comorbidities of cerebral impairment or craniofacial abnormalities, 1 case was lost to follow up, 4 cases died, and 10 cases survived, in which 9 cases had neurodevelopmental disorders. In the group without comorbidities, 2 cases were lost to follow up, 9 cases survived, in which 1 case had neurodevelopmental disorders. The incidence of poor prognosis including death and neurodevelopmental disorders was significantly higher in the group with comorbidities than that without comorbidities (P<0.01). (7) An symptomatic improvement of PAC was found in the majority of the survived cases (18/19) with age.CONCLUSIONS:Early-onset PAC in infants usually exhibits varying degrees of relief with age, whereas the cases with comorbidities had a poor prognosis.

Objective: To evaluate the awareness, diagnosis and treatment of chest tightness variant asthma (CTVA) among pediatricians in China. Methods: The survey was conducted by convenient sampling method. Pediatricians with professional title of attending physician and above from different grades hospitals in 30 provinces were invited to conduct online questionnaire surveys through WeChat, pediatricians scan QR codes to complete electronic questionnaires in the mini program from January 16th to February 4th, 2021. The contents of questionnaire included the awareness, diagnosis and treatment of CTVA, and comparing the differences between pediatricians in secondary hospitals and tertiary hospitals. Results: A total of 1 529 pediatricians participated in the survey, and 1 484 (97.06%) pediatricians completed the questionnaire and included in the analysis, including 420 males (28.30%). The awareness rate of CTVA among pediatricians was 77.83 % (1 155/1 484). Pediatricians in tertiary hospitals had higher rates of awareness of CTVA than pediatricians in secondary hospitals [81.86% (898/1 097) vs 66.41% (257/387), P<0.001] and had better execution of the guidelines [89.15% (978/1 097) vs 79.59% (308/387), P<0.001]. A total of 93.06 % (1 381/1 484) of pediatricians' first-line treatment included inhaled corticosteroids (ICS) for CTVA. Among them, a higher proportion of pediatricians in tertiary hospitals used ICS included regimens for first-line treatment of CTVA compared with pediatricians in secondary hospitals [94.90% (1 041/1 097) vs 87.86% (340/387), P<0.001]. The reported well control rate of CTVA was 32.08% (476/1 484), which was significantly lower in secondary hospitals than that in tertiary hospitals [17.31% (67/387) vs 37.28% (409/1 097), P<0.001]. Conclusion: Most pediatricians are well aware of CTVA, among which there is a certain gap in clinical practice between pediatricians in secondary hospitals and tertiary hospitals in terms of understanding, diagnosis, and treatment of CTVA.

目的:分析婴儿喉软化症(ILM)的临床特点及预后转归.方法:收集2013年12月至2021年1月在广西医科大学第一附属医院儿科经支气管镜确诊ILM的住院患儿76例.根据ILM患儿的病情严重程度分为轻中度组36例和重度组40例;根据合并症分为有同步气道病变组(SALs组)40例和无SALs组36例、有神经肌肉发育异常组(NMD组)35例和无NMD组41例、有先天性心脏病组(CHD组)40例和无CHD组36例、有先天综合征组(CS组)23例和无CS组53例.回顾性分析各组临床特点,随访出院后症状改善情况以及重度ILM术后转归情况.结果:与重度组比较,Ⅱ型ILM在轻中度组的所占比例较高(P＜0.05).重度组出院后半年生长发育不良(FTT)改善率低于轻中度组(13.8%vs 53.3%,P＜0.05),而出院后1年差异无统计学意义(P＞0.05).NMD组出院后1年FTT改善率低于无合并NMD组(32.0%vs 73.7%,P＜0.05).在重度ILM患儿中,共有12例进行了支气管镜介入手术,其中声门上成形术6例,喉咽部/会厌肿物/囊肿切除术6例,两种术式术后患儿的临床症状均得到缓解.结论:合并NMD对ILM患儿生长发育影响较大;重度ILM早期及时干预可较早改善临床症状;选择手术方式应个体化.

1例主诉为咳嗽、活动耐力下降2个月余的3岁3月龄患儿（女）2020年12月就诊于广西医科大学第一附属医院儿科。经过肺部CT平扫、支气管镜下肺泡灌洗液检查、全外显子基因检测、血清粒细胞和巨噬细胞集落刺激因子水平及其信号传导的检测，诊断为遗传性肺泡蛋白沉积症。该疾病罕见且病情严重，在给予支气管镜联合支气管封堵器行全肺灌洗术并辅以口服吡格列酮治疗后，患儿氧合状态好转，能脱离呼吸机和吸氧，恢复基本活动，肺部CT复查也较前改善。

目的 回顾性分析广西医科大学第一附属医院近15年来不同科室收治的气管支气管异物病例的临床特点,为气管支气管异物的诊治提供临床经验与参考.方法 收集并整理2005年1月-2019年12月就诊于耳鼻咽喉头颈外科、儿科、呼吸内科及其他科室诊断为气管支气管异物的病例资料.本研究每5年作为1个阶段,将所收集病例分为2005-2009年、2010-2014年、2015-2019年3个阶段,总结比较不同阶段、不同科室气管支气管异物病例的诊断治疗特点.结果 ①本研究共收集了 699例气管支气管异物病例,其中耳鼻咽喉头颈外科549例(78.5％),儿科70例(10％),呼吸内科65例(9.3％),其他科15例(2.1％).在第1个5年阶段中,患者以耳鼻咽喉头颈外科收治为主(90.1％);至2019年,儿科和呼吸内科收治患者比例增至56.1％,纤维支气管镜的应用比例由2012年的6.3％增至2019年的61％;②影像检查方式包括胸部X线253例(36.2％),胸部CT 404例(57.8％);胸部X线检出率为71.1％,胸部CT检出率为91.3％;③各科室15年总的平均住院日比较,其中耳鼻咽喉头颈外科3.68 d、儿科6.89 d、呼吸内科及其他科11.00 d;④手术相关并发症发生率为3.4％(24/699),其中耳鼻咽喉头颈外科为1.8％(10/549)、儿科8.6％(6/70)、呼吸内科10.8％(7/65)、其他科为6.7％(1/15);硬质支气管镜为2.0％(12/591)、纤维支气管镜为11.1％(12/108).结论 ①气管异物影像检查以胸部X片、CT为主,检出阳性率较高;②气管支气管异物主要收治科室为耳鼻咽喉头颈外科,儿科、呼吸内科近年来收治病例数增加.气管异物取出方式逐渐由硬性支气管镜转向纤维支气管软镜;③随着诊治经验增加,病种平均住院日有缩短趋势;④并发症发生率或与不同科室及手术操作经验有关.

1例主诉为“反复咳嗽伴痰鸣2年余”的2岁10月龄患儿就诊。患儿有反复呼吸道感染、活动不耐受，查体见杵状指，鼻窦CT示鼻窦炎。患儿婴儿期有可疑脂肪泻，血脂示甘油三酯、低密度脂蛋白胆固醇、载脂蛋白B明显降低。结合全基因组外显子测序检测结果，最终诊断为原发性纤毛运动障碍合并无β脂蛋白血症。