OBJECTIVE: To explore the impact of age on the genetic variant spectrum and prognosis of patients with previously untreated Diffuse large B-cell lymphoma (DLBCL). METHODS: A retrospective analysis was conducted on the clinical data and follow-up information of 254 previously untreated DLBCL patients from 14 hospitals in the Jiangsu Cooperative Lymphoma Group (JCLG) enrolled from July 2018 and July 2023. Following extraction of DNA from tumor tissue samples, next-generation sequencing (NGS) technique was employed to analyze the genetic variant spectrum of the DLBCL patients, with an evaluation of the relationship between age and genetic variants as well as prognosis. This study was approved by the Medical Ethics Committee of the Affiliated Hospital of Nantong University (Ethics No.: 2023-K048-01). RESULTS: The median age of the 254 DLBCL patients was 62 years old, with 55% of patients aged 60 years or above. Clinical evaluation showed that younger (< 60 years) patients had higher complete response (CR) (70% vs. 59%), and objective response rate (ORR) (88% vs. 79%) than older patients, though the difference between the two groups was not statistically. Survival analysis indicated that both the five-year overall survival (OS) (82.7% vs. 71.7%, P = 0.006) and progression-free survival (PFS) (70.6% vs. 50.2%, P < 0.05) rates were significantly higher in younger patients. NGS showed that 99.6% of the patients harbored genetic variants, with PIM1, KMT2D, TP53, MYD88, and CD79B being the most common genes. Age significantly affected the variant frequency of certain genes, with MYC variants serving an adverse prognostic factor for OS in younger patients (P = 0.002), while TP53 (P = 0.024) and BCL2 (P = 0.002) variants significantly impacted OS in older patients. Prognostic analysis identified age ≥ 60 years (HR = 3.439, 95%CI: 1.318~9.874), presence of B symptoms (HR = 2.871, 95%CI = 1.133~7.307), and elevated lactate dehydrogenase (HR = 3.528, 95%CI = 1.231~10.66) as independent adverse prognostic factors. CONCLUSION Age, genetic variants, and clinical factors may significantly affect the prognosis of the DLBCL patients. Younger patients have better survival compared to older patients. Variants of the MYC, BCL2, and TP53 genes are closely associated with poor prognosis.
Humans ; Lymphoma, Large B-Cell, Diffuse/diagnosis* ; Middle Aged ; Female ; Male ; Retrospective Studies ; Aged ; Prognosis ; Adult ; Aged, 80 and over ; High-Throughput Nucleotide Sequencing ; Young Adult ; Adolescent ; Genetic Variation

Objective:To investigate the clinical characteristics, long-term prognosis and changes of pulmonary function in children with idiopathic pulmonary fibrosis (IPF).Methods:The clinical data, long-term prognosis and changes of pulmonary function of children with IPF admitted to the Department of Pediatric Respiratory Center in Children′s Hospital of Chongqing Medical University from January 2008 to December 2018 were retrospectively analyzed.Results:A total of 28 cases were included, with the median age of 3.9 years (range: 0.5 to 15.7 years). Cough (28 cases, 100.0%), tachypnea (25 cases, 89.3%), cyanosis (19 cases, 67.9%), dyspnea (11 cases, 39.3%), Velcho rales (12 cases, 42.9%), inspiratory three concave sign (11 cases, 39.3%), clubbed fingers and toes (6 cases, 21.4%) and diminished breath sounds (5 cases, 17.9%) were main clinical manifestations.Chest high-resolution computed tomography (HRCT) mainly displayed grid shadow, irregular sac-like light-transparent shadow with ho-neycomb changes and dense shadow, partial (7 cases) pulmonary interstitial emphysema/emphysema/pneumomediastinum.Three cases of lung biopsy showed hyperplasia and consolidation of alveolar space and alveolar septal fibrosis, thickening of alveolar wall and coexistence of new and old lesions.In this group, 4 cases did not receive drug therapy due to other reasons (3 cases abandoned therapy, and 1case died of respiratory and circulatory failure during hospitalization). Twenty-four cases were treated with single or combination of oral Prednisone, N-acetylcysteine and Azithromycin.Eleven cases had improved symptoms when discharged, and 13 cases showed no improvement.Twenty-four cases continued to receive oral medication therapy according to the original protocol.Eight cases were followed up for chest HRCT for 3 months to 4 years, the chest HRCT lesions of 7 cases were similar to before, and those of 1 case increased than before.All cases received telephone follow-up for 2 to 7 years; the maximum duration of medication was 4 years.Twelve cases were lost to follow-up, 7 cases had motion limitation, 3 cases died, and 2 cases had no clinical symptom.Three cases were followed up for pulmonary function for 2 to 3 years, among which 2 cases had pulmonary function decreased than before; 1 case had improvements in forced vital capacity as a percentage of the predicted value and peak expiratory flow as a percentage of the predicted value, but decline in forced expiratory volume in the first se-cond as a percentage of the predicted value.Conclusions:The clinical manifestations of children with IPF are lack of specificity.Chest HRCT is of great value in the diagnosis of IPF and preliminary monitoring of the activity of lesion.In the long-term follow-up, some of cases have improvements in symptoms; pulmonary function mostly decreases, but part of indexes can be improved.

Objective:To explore the clinical characteristics and the therapeutic approach in children with pulmonary embolism(PE).Methods:The clinical data of 8 patients with PE who hospitalized at the Children′s Hospital of Chongqing Medical University from March 2001 to October 2018 were retrospectively analyzed.Results:Among the 8 cases with PE, 3 cases were male and 5 cases were female, the age of subjects ranged from 0.6 to 11.7 years old, the median age was 7.96 years old.All of them had underlying diseases, among them, congenital heart disease with infective endocarditis accounted for 37.5%(3 case). Among the 8 cases, 4 cases presented with symptoms of respiratory tract infection, 7 cases had shortness of breath, 5 cases had cough, 3 cases had chest pain, 2 cases had hemoptysis, and 1 case had typical triad of PE with chest pain, dyspnea and hemoptysis.Among the 8 cases, 7 cases did the arterial blood gas analysis and showed hypoxemia; 6 cases did the D-dimer and the value>500 μg/L; 5 cases did the electroca-rdiogram and 4 cases(80.0%) showed sinus tachycardia, and 2 cases(40.0%) had ST-T changes, all of them did echocardiography and 3 cases(37.5%) of which indicated pulmonary artery excrescence, 7 cases of which did spiral CT pulmonary angiography and 5 cases(71.4%) of them prompted pulmonary vascular filling defects, 6 cases(75.0%) of which were embolized in the lower lobe of the lung.Three cases received anticoagulant therapy, and 1 of them was treated with combined thrombectorny, 1 case of them died.Two cases received thrombectomy, 1 case of them died.Three cases were not treated with thrombectomy and anticoagulation therapy, 2 cases of them died.Conclusions:The mortality of PE in children is high.The clinical manifestations of PE in children are not typical and difficult to distinguish from respiratory infections.For children with high risk factors of PE, once clinical symptoms related to PE occur, D-dimer, echocardiography, and spiral CT pulmonary angiography should be done soon for early diagnosis and treatment.

Objective:To explore clinical features, etiology and mechanism of pulmonary bullae in children.Methods:The clinical data of children with diagnosis or suspected diagnosis of pulmonary bullae, including the general situation, etiology, pathogen composition, number and location, prognosis and so on, in the Inpatient Department of Children′s Hospital of Chongqing Medical University from March 1993 to August 2019 were retrospectively analyzed.Results:Among the 163 patients, there were 130 cases of respiratory tract infection, and 11 cases of pneumothorax alone.Totally, 22 cases were found pulmonary bullae in the chest CT examination without typical symptoms.Etiology: viruses accounted for 19.02%(31/163 cases), with mainly respiratory syncytial virus (9.20%, 15/163 cases); bacterial infection took up 28.83%(47/163 cases), mostly Haemophilus influenzae (13/163 cases, 7.98%) and Staphylococcus aureus (10/163 cases, 6.13%). Pulmonary bullae was more common in the right lung (82 cases). It took 7 days to 9 months for bullae to reduce, shrink or disappear.In some cases, there was no significant change in bullae even after 19 months. Conclusion:Pulmonary bullae can be seen in infection, tumor, auto-immune diseases and so on, most of which are bacterial infections.Bullae may exist for a long time.The mechanism of pulmonary bullae may include the narrowing of lumen, followed by the thickening of bronchial wall, ischemic necrosis and alveolar expansion due to the clogging of distal small vessels or capillaries, the degradation of connective tissue and dissociation of elastic tissue, the destruction of the bronchiolar wall, disturbing ion channels and mitochondria metabolism and destroying the connection of epithelial cells.

Asthma is the most common chronic airway disease in childhood.Recent studies have shown that pulmonary rehabilitation can alleviate dyspnea, improve lung function and quality of life.However, there are few studies on pulmonary rehabilitation for children with asthma.This article will expound the application of pulmonary rehabilitation in the management of pediatric asthma.