Objective To summarize the etiology and clinical features of Mounier-Kuhn syndrome (MKS) so as to enhance the acknowledgement of MKS. Methods Three cases of MKS were reported and the keywords with " Tracheobronchomegaly”, " Mounier-Kuhn syndrome” were analyzed through the mode of literature retrieval in CNKI, VIP and Pubmed databases. Results There were 214 cases around the world including 14 cases of MKS in China. With unknown etiology and non-specific clinical feature, MKS is characteristic with congenital absence of tracheal or bronchial elastic tissue. MKS is diagnosed with remarkably dilated trachea and bronchus through chest CT or bronchofibroscope, accompanied with genetic defects. The ratio of men to women is 8.5 to 1. There is no radical cure. Symptomatic treatment and surgical treatment when necessary could be available. Conclusions MKS is a rare progressive hereditary disease, irrelevant to smoking and sharing a similar cause with generalized elastolysis, which need pay much attention to the physical examination of skin or elastic tissue. The diagnosis of MKS should be based on not merely airway diameter but also the overall clinical, pathologic, and radiologic profile.
Objective To evaluate the control status and knowledge level about disease in asthmatic patients in hospitals of different grades in Gansu province, and provide a basis for proposing a work plan forprevention and treatment of asthma in Gansu province.Methods We performed a questionnaire investigation in a face-to-face manner in asthmatic patients from hospitals of three different grades, which included a grade 3A hospital, a grade 3B hospital, and a grade 2A hospital.Results A total of 542 asthmatic patients were investigated. The percentage of complete controlled, well controlled and uncontrolled were 3. 5% , 16. 9% and 79. 5% respectively. The rate of complete controlled was low in three hospitals without significant difference ( 3. 6% , 3. 4% , and 3. 3% , respectively) . The rate of well controlled in the grade 3A and 3B hospitals was higher than that in the grade 2A hospital ( 20. 4% and 18. 3% vs. 8. 2% ,P lt;0. 05) . 41. 4% of the respondents had been hospitalized for exacerbation in the past one year, and the rate was higher in the grade 2A hospital than that in the grade 3A and 3B hospitals ( 58. 2% vs. 31. 8% and 42. 9%, P lt;0. 01) . 46. 9% of the patients had visited the emergency department, and the rate was higher in the grade 2A hospital than that in the grade 3A and 3B hospitals ( 63. 9% vs. 41. 6% and 42. 3% , P lt;0. 01) . 23. 6% of the respondents had underwent lung function test follow-up during the past one year, andthe rate in the grade 3A hospital was higher than that in the grade 3B and 2A hospitals( 79. 2% vs. 44% and 40. 2%, P lt;0. 01) . Only 2. 8% of the patients in the grade 3A hospital had used peak flowmeter regularly.There were 19. 7% of the patients received a long-term treatment plan by specialists, and the rate in the grade 3A and 3B hospitals was higher than that in the grade 2A hospital ( 22. 4% and 21. 7% vs. 11. 5% ,P lt;0. 05) . 30. 6% of the respondents insisted on using inhaled corticosteroid ( ICS) or combination of ICS and long acting β2 -agonist ( ICS/LABA) , and the rate was higher in the grade 3A and 3B hospitals than that in the grade 2A hospital ( 33. 9% and 32. 6% vs. 21. 3% , P lt;0. 05) . 27. 7% of the patients had used the theophylline drugs regularly, and there were no significant differences in three hospitals. 30. 3% of the patients did not used the drugs regularly, and most of these patients were from the grade 2A and 3B hospitals ( 54. 1% and 32. 9% respectively) . 11. 4% of the patients has used antibiotics, herbal medicine or other drugs. And most of these patients were from the grade 2A and 3B hospitals ( 50% and 30. 6% respectively) . 26. 2% of the patients believed that asthma is an inflammatory disease. 51. 3% of the patients selected ICS ( or ICS/LABA) as the first line medicine, and the rate in the grade 3A hospital was higher than that in the grade 3B and 2A hospitals ( P lt;0. 05) . Only13. 1% -14. 7% of the patients attended the lecture about asthma in hospitals in the past one year, and the rate in the grade 3A hospital was thehighest, but the difference was not significant. Conclusions The lack of the awareness, the poor control, and the non-standardized treatment and management of the disease are the main obstacles in the control of asthma in Gansu province, especially in township hospitals.
ObjectiveTo enhance the understanding of common variable immune deficiency (CVID) combined with celiac disease in order to diagnose and treat the disease as early as possible and delay its complications through the treatment of the disease and study on related literatures. MethodThe experience of diagnosing and treating one case of CVID combined with celiac disease in November 2013 was reported in the present study, and the related literatures were reviewed. ResultsAfter strict gluten-free diet and infusion of intravenous immune globulin, the patient's diarrhea relieved and serum immunoglobulin elevated. ConclusionsCVID is the most frequent symptomatic primary immune deficiency which is related to celiac disease closely, a gluten-sensitive condition characterized by a variable degree of villous atrophy. Once diagnosed, gluten-free diet can alleviate the symptoms.
Objective To enhance the understanding of the primary salivary glandtype lung cancer (PSGLC) and improve the diagnostic rate of the disease. Methods The clinical data of 41 patients with PSGLC pathologically confirmed in West China Hospital between October 2009 and October 2015 were analyzed in terms of clinical features, therapy and prognosis. Results All the 41 patients (21 males and 20 females) accounted for 0.22% (41/18 738) of the primary malignant lung tumor diagnosed in the same period. The patients aged from 16 to 72 with the median age of 43.6. The disease course was 1 month to 6 years, and 12 had smoking history. There were 23 cases of adenoid cystic carcinoma, 15 of mucoepidermoid carcinoma and 3 of acinic cell carcinoma. The symptoms, chest-computed tomography and fiberbronchoscopy examination had no specificity. There were 34 patients who had undergone surgery, in whom 3 had fiberbronchoscopy, 4 had oral traditional Chinese medicine treatment, 5 had chemotherapy and radiotherapy at the same time, 9 received chemotherapy only, and 4 recived radiotherapy only. Follow-up period lasted for 2-65 months, with the median time of 38 months. Two patients had recrudescence, 6 patients had distant metastasis and 1 patient died. Conclusions PSGLC have no specific clinical features; its diagnosis basically rely on pathological examination. After comprehensive treatment mainly on surgery, the prognosis of the disease is better than other malignant lung cancers.
Objective To investigate the levels and clinical significance of cold-inducible RNA-binding protein (CIRBP) and mucin 5AC (MUC5AC) in serum and bronchoalveolar lavage fluid (BALF) of patients with interstitial lung disease (ILD). Methods A total of 63 ILD patients who were hospitalized in the Department of Pulmonary and Critical Care Medicine, The First Hospital of Lanzhou University from January 2022 to February 2024 were collected, including 22 patients with idiopathic pulmonary fibrosis (IPF), 20 patients with connective tissue disease-related interstitial lung disease (CTD-ILD), and 21 patients with other types of ILD. Thirty healthy individuals with matching demographic characteristics during the same time period were selected as the healthy group. Enzyme linked immunosorbent assay was used to detect the levels of CIRBP and MUC5AC in serum of healthy group, the levels of CIRBP and MUC5AC in serum and BLAF of ILD patients. The levels of CIRBP and MUC5AC in serum were compared between the healthy group and the ILD group, and the levels of CIRBP and MUC5AC in serum and BALF were compared among the patients with different types of ILD. The correlations were analyzed between the levels of CIRBP and MUC5AC and the lung function, blood gas analysis, and 6-minute walk distance (6MWD) in the patients with different types of ILD. The receiver operating characteristic curve was drawn according to the levels of CIRBP and MUC5AC in serum. The optimal cut-off value, sensitivity and specificity of serum CIRBP and MUC5AC for diagnosis of ILD were determined. Results The serum CIRBP level in the three groups of ILD patients was higher than that of the healthy group (P<0.01), but there was no significant difference in the serum CIRBP level among the three groups of ILD patients (P>0.05). There was no significant difference in serum MUC5AC levels between the three groups of ILD patients and the healthy group, as well as among the three groups of ILD patients (P>0.05). There was no significant difference in the levels of CIRBP and MUC5AC in BALF among the three groups of ILD patients (P>0.05). CIRBP in BALF of the IPF patients was negatively correlated with 6MWD, while CIRBP in serum of the CTD-ILD patients was negatively correlated with partial pressure of oxygen and arterial oxygen saturation. CIRBP in serum of other ILD patients was negatively correlated with total lung capacity as a percentage of predicted value (TLC%pred), forced vital capacity as a percentage of predicted value (FVC%pred), diffusion capacity of carbon monoxide of lung as a percentage of predicted value (DLCO%pred), and 6MWD, while CIRBP in BALF was negatively correlated with DLCO%pred. The MUC5AC in BALF of IPF patients was negatively correlated with DLCO%pred, while the MUC5AC in BALF of CTD-ILD patients was negatively correlated with TLC%pred, DLCO%pred, and 6MWD. The serum MUC5AC in other ILD patients was negatively correlated with FVC%pred, DLCO% pred, and 6MWD. The sensitivity was 77.6%, and the specificity was 96.7% for diagnosing ILD with serum CIRBP. Sensitivity was 55.1%, and specificity was 76.7% for diagnosing ILD with serum MUC5AC. The combination of the two indicators resulted in a sensitivity of 87.8% and a specificity of 86.7% for the diagnosis of ILD. Conclusion The high expression levels of CIRBP and MUC5AC have certain diagnostic efficacy and disease assessment effects on ILD, and may serve as potential biomarkers for ILD.
Objective To establish the finite element model of varus-type ankle arthritis and to implement the finite element mechanical analysis of different correction models for tibial anterior surface angle (TAS) in supramalleolar osteotomy. Methods A female patient with left varus-type ankle arthritis (Takakura stage Ⅱ, TAS 78°) was taken as the study object. Based on the CT data, the three-dimensional model of varus-type ankle arthritis (TAS 78°) and different TAS correction models [normal (TAS 89°), 5° valgus (TAS 94°), and 10° valgus (TAS 99°)] were created by software Mimics 21.0, Geomagic Wrap 2021, Solidworks 2017, and Workbench 17.0. The 290 N vertical downward force was applied to the upper surface of the tibia and 60 N vertical downward force to the upper surface of the fibula. Von Mises stress distribution and stress peak were calculated. Results The finite element model of normal TAS was basically consistent with biomechanics of the foot. According to biomechanical analysis, the maximum stress of the varus model appeared in the medial tibiotalar joint surface and the medial part of the top tibiotalar joint surface. The stress distribution of talofibular joint surface and the lateral part of the top tibiotalar joint surface were uniform. In the normal model, the stress distributions of the talofibular joint surface and the tibiotalar joint surface were uniform, and no obvious stress concentration was observed. The maximum stress in the 5° valgus model appeared at the posterior part of the talofibular joint surface and the lateral part of the top tibiotalar joint surface. The stress distribution of medial tibiotalar joint surface was uniform. The maximum stress of the 10° valgus model appeared at the posterior part of the talofibular joint surface and the lateral part of the top tibiotalar joint surface. The stress on the medial tibiotalar joint surface increased. Conclusion With the increase of valgus, the stress of ankle joint gradually shift outwards, and the stress concentration tends to appear. There was no obvious obstruction of fibula with 10° TAS correction. However, when TAS correction exceeds 10° and continues to increase, the obstruction effect of fibula becomes increasingly significant.