ObjectiveTo explore the clinical features, diagnosis, differential diagnosis, pathological characteristics, management and prognosis of airway hemangioma, and improve the level of the diagnosis and therapy.MethodsThe clinical data of a patient with airway hemangioma admitted to West China Hospital of Sichuan University were retrospectively analyzed. The related literatures were reviewed. Databases including PubMed, Ovid Medline, Embase, VIP, Wanfang and Chinese National Knowledge infrastructure were searched using the keywords as " Trachea” OR " Bronchus” AND " Hemangioma” from January 1976 to October 2016.ResultsThe patient was a 32-year-old male presented with hemoptysis and backache more than 10 days. His enhanced chest CT scan revealed thickening of the esophagus wall and narrowing of the lumen of esophagus. A wide range of vascular tumor like changes in the trachea and the two sides of bronchus were found by the fiberoptic bronchoscopy and gastroscope examination suggested esophageal varices. Although the patient had a wide range of airway lesions, the symptom of hemoptysis was relieved after the conservative treatment. The patient’s condition was stable until now. From January 1976 to October 2016, a total of 34 related articles were retrieved and 36 cases of airway hemangioma were reported. The etiology of the disease remains unknown. Recurrent hemoptysis was the common symptoms of airway hemangioma, and imaging changes lacking characteristics. Histopathological examination revealed dilated, thin-walled and different size of vascular or hyperplastic capillary and immunohistochemical staining was positive for vascular endothelial cell specific CD34 and Ⅷ factor.ConclusionsHemangioma is a common benign tumor of head and neck in infants and children. Hemangioma is very rarely occurred in trachea in adults. Clinical and imaging manifestations are not specific, and diagnosis relies on histopathological examination. Fiberoptic bronchoscopy and three dimensional reconstruction of thoracic vessels with CT play important roles in the whole process of diagnosis and treatment of airway hemangioma. It should be differentiated from lymphangioma. The prognosis of airway hemangioma is good.
Coronavirus disease 2019 epidemic is a serious and infectious public health event that causes physical damage and may also have an impact on mental health. West China Hospital of Sichuan University combines the internet hospital platform with the traditional telephone model, establishes a new coronavirus disease 2019 intervention integration platform, and builds a new “four-in-one” medical service system, which contains epidemic consultation, psychological consultation, self-evaluation and home prevention and control. It has established a way for the public to communicate with professional, reduced the spread of coronavirus disease 2019 in space, and effectively carried out psychological decompression and comfort work, disseminated the knowledge of fighting coronavirus disease 2019, and provided medical advice. This article mainly introduces the out-of-hospital psychological intervention model of West China Hospital of Sichuan University during the coronavirus disease 2019 epidemic, and aims to provide a reference for exploring the psychological intervention methods for major public health emergencies.
Unhealthy diet, habits and drug abuse cause a variety of liver diseases, including steatohepatitis, liver fibrosis, liver cirrhosis and liver cancer, which seriously affect human health. The fabrication of highly simulated cell models in vitro is important in the treatment of liver diseases and drug development. This article summarized the common strategies for the construction of liver pathology models in vitro. It introduced four typical cell models in vitro related to liver disease and provided a reference for the study of liver disease models.
Transcatheter aortic valve replacement (TAVR) is the best treatment for severe aortic stenosis with high surgical risk, and low body weight significantly increased the risk of surgery and postoperative all-cause mortality. A case of elderly female diagnosed with severe aortic valve stenosis and extremely low body weight was presented in this article. Additionally, horizocardia and low located coronary orifice were also found in this patient, which markedly increased the risk and complexity of the TAVR procedure. During the operation, circulatory collapse occurred, and prosthetic valve was quickly released under emergency cardiopulmonary resuscitation. The operation was successful and the patient’s vital signs recovered soon. The follow-up showed that the patient was in good condition.
Objective To explore the role of hydrogen peroxide (H2O2) in inducing chronic oxidative stress in microglia aging. Methods BV2 microglia purchased from ATCC in less than 10 generations were treated with 0, 50, 100, 200 μmol/L H2O2 at different concentrations. According to the concentration of H2O2 used, the BV2 microglia were divided into a control group and H2O2 -50 μmol/L Group, H2O2 -100 μmol/L Group, H2O2 -200 μmol/L Group. Cell proliferation was measured by CCK8 cell proliferation assay. Age-related β-galactosidase (SA-β-gal) staining assay, and expression of age-related cyclin molecules p16, p21, p53 and senescence sssociated secretory phenotype interleukin 1 beta (IL-1β), transforming growth factor-β (TGF-β) and matrix metalloprotein 9 (MMP9) detected by quantitative real-time polymerase chain reaction were used to measure celluar senescence. Results During the induction process, H2O2-200 μmol/L caused significant damage to BV2 microglia, therefore no subsequent testing was conducted. Finally, the control group, H2O2-50 μmol/L group and H2O2-100 μmol/L group cells were collected. The differences in cell survival rate (F=46.176, P<0.001) and positive rate of SA-β-gal staining (F=553.1, P<0.001) among the three groups were statistically significant. The cell survival rate of H2O2-50 μmol/L group had no significant change (P>0.05), while the cell survival rate of H2O2-100 μmol/L group decreased significantly (P<0.001). The positive rate of SA-β-gal staining in H2O2-50 μmol/L group and H2O2-100 μmol/L group was increased (P<0.001), and the positive rate of SA-β-gal staining in H2O2-100 μmol/L group was higher than that in H2O2-50 μmol/L group (P<0.001). The mRNA levels of senescence related cyclin molecules p16, p21 and p53 were up-regulated under the induction of 50, 100 μmol/L H2O2 (P<0.05), and the expressions of IL-1β, TGF-β and MMP9 of senescence associated secretory phenotype were increased (P<0.05). The increase of H2O2-50 μmol/L group was more obvious (P<0.05). Conclusion The aging model of BV2 microglia can be successfully established by inducing 8 d with 100 μmol/L H2O2, and the mechanism may be related to promoting the secretion of p16, p21, p53, IL-1β, TGF-β and MMP9.
Objective To evaluate the incidence and characteristics of spontaneous pneumothorax in patients with Marfan syndrome (MFS), and to analyze its related factors. Methods The medical record of patients with MFS who were hospitalized in the First Affiliated Hospital of University of Science and Technology of China from March 1, 2017 to December 31, 2022 were retrospectively collected, to analyze the incidence of pneumothorax and related risk factors. Results A total of 53 patients (including 29 males, 55%) were included, with a median age of 32 years (ranging from 13 to 65 years). All patients denied family history of pneumothorax. Four cases (8%) had one episode of spontaneous pneumothorax, with pulmonary bullae involved the ipsilateral apex. Among them, 3 cases (75%) had single pulmonary bulla and thoracic deformity. Of the 49 patients without previous pneumothorax history, 9 (18%) had pulmonary bullae, of which 6 (67%) were located at the apex of the lung, and the remaining 3 (33%) were in the middle and lower lobes of the lung; In addition, 6 cases (12%) were coexisted with thoracic deformity. The incidence of pneumothorax in patients with pulmonary bullae in MFS was significantly higher than those without pulmonary bullae (31% vs. 0%, P=0.002), and the incidence of pneumothorax in patients with thoracic deformities was significantly higher than those with normal thorax (33% vs. 2%, P=0.013). Of the 4 patients with pneumothorax, 2 underwent bullectomy, 1 underwent thoracic drainage, and the other underwent thoracic puncture and aspiration procedures, respectively. No recurrence of pneumothorax was observed during the follow-up period. Sixteen cases of MFS with spontaneous pneumothorax (11 males, 69%) were reported in Chinese literature, with a median age of 17 years (ranging from 13 to 28 years). Among them, 3 cases (19%) had a family history of pneumothorax, and 14 cases (88%) had thoracic deformities. Bullae was found in all 5 patients with pneumothorax, 4 of them with chest CT images involved the ipsilateral upper lobe. Forty-four percents of 16 patients had pneumothorax recurrence. Conclusions The incidence of MFS complicated with spontaneous pneumothorax is significantly higher. However, most of them have no family history of pneumothorax. The incidence of pneumothorax with MFS is associated with pulmonary bullae (especially the apex of lung) and thoracic deformity. Timely surgical intervention should be carried out when pneumothorax happened.
Objective To observe and analyze the clinical characteristics of children with autosomal dominant hereditary microcephaly with or without chorioretinopathy, lymphedema, or intellectual disability syndrome (MCLMR). MethodsA retrospective clinical study. In September 2023, the first patient and three family members (parents, brother) of MCLMR who were diagnosed through ophthalmic examination and genetic testing at Department of Ophthalmology of Henan Children's Hospital were included in the study. Clinical data were collected, inquired about medical history and family history in detail, and performed best corrected visual acuity (BCVA), optical coherence tomography (OCT), fluorescein angiography (FFA), flash visual evoked potential (F-VEP), full field electroretinogram (ERG), cranial magnetic resonance imaging (MRI), and systemic examination. 3 ml of peripheral venous blood were collected from the proband, her parents and younger brother, and extracted whole genome DNA. Second generation sequencing technology was used for gene sequencing. For suspected pathogenic sites, Sanger sequencing was used for validation, and bioinformatics analysis was performed to determine the pathogenicity of the genetic variant sites. The relevant literature of PubMed of the National Library of Medicine and Wan Fang Med Online by computer were searched. The genetic characteristics and conducted literature review were summarized. ResultsThe proband (Ⅱ-1) was an 8-year-old and 5-month-old female. Her head was relatively small, the lower jaw was small, the ears protrude, the nose was wide, the eyelid was tilted upwards, philtrum was long. Mild intellectual disability, no history of lymphedema. The BCVA values for the right and left eyes were 0.08 and 0.1, respectively. Bilateral nystagmus. Atrophic lesioned in the macular area and below choroid retina of both eyes. FFA examination showed mottled fluorescent staining in the macular area and the below retina, with no obvious fluorescein leakage in the late stage. OCT examination revealed shallow macular fovea morphology, absence of ellipsoidal bands, unclear layers, thinning of the entire retina, and significant atrophy of the choroid and retina beneath the macula. F-VEP examination, no waveform was detected in both eyes. Full field ERG examination showed severe reduction in amplitude of a wave and b wave in both eyes. Head magnetic resonance imaging showed widening of the subarachnoid space in the left temporal region, with no significant abnormal signals observed in the brain parenchyma. Her father (Ⅰ-1) had mild nystagmus and strabismus. The phenotypes of the eyes of the mother (Ⅰ-2) and brother (Ⅱ-2) were not significantly abnormal. The genetic testing results showed that the proband (Ⅱ-1) had a heterozygous missense mutation c.895A>G (p.Ile299Val) in exon 8 of the KIF11 gene, which was a known mutation. Her parents (Ⅰ-1, Ⅰ-2) and younger brother (Ⅱ-2) were both wild-type. The bioinformatics analysis results indicated that this mutation is a potentially pathogenic variant. A total of 109 cases were retrieved from 20 relevant literatures. Among them, 55 were male, 54 were females. There were 61 cases with family history and 48 cases without family history, respectively. Among the 109 cases, 98 cases (89.9%, 98/109) had microcephaly, 2 cases had premature closure of cranial sutures, and 11 patients underwent cranial MRI, which showed 11 cases of small head with simplified development of the cerebral gyrus. 50 cases (45.9%, 50/109) of lymphedema. 83 cases (76.1%, 83/109) of intellectual developmental disorders. 92 cases (84.4%, 92/109) had ocular abnormalities, 69 cases (63.3%, 69/109) had chorioretinopathy, 20 cases (18.3%, 20/109) had retinal folds, 10 cases (9.2%, 10/109) had nystagmus, and 17 cases (15.6%, 17/109) had retinal detachment. ConclusionsThe main clinical manifestations of MCLMR are microcephaly, chorioretinopathy, with or without lymphedema, and intellectual disability. The main manifestations of eye diseases are low vision, nystagmus, and chorioretinopathy. The heterozygous missense mutation c.895A>G (p.Ile299Val) in exon 8 of KIF11 gene is the pathogenic variant of this family.