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.
摘要:目的: 报道同卵双生子间肾移植效果,探讨免疫抑制剂及激素的使用、鉴定同卵双生子的方法以及术后随访。 方法 :个案报道结合文献综述。 结果 :手术获得成功。术后随访8月,患者恢复良好。 结论 :同卵双生间的肾移植安全有效,术后不需要使用免疫抑制剂也能维持移植肾功能正常。Abstract: Objective: Reported the effects of renal transplantation between identical twins,explored the use of immunosuppressive drugs and glucocorticoid, identification method of the identical twins and postoperative followup . Methods :Combining case report and literature review. Results :The operation is success.Followup in 8 months,the patient recover well. Conclusion : The renal transplantation between identical twins is safe and effective,the immunosuppressant is not need for the postoperative patients to maintain the graft`s function.
We reported one case of MTX-induced aplastic anemia and reviewed related literature to investigate the mechanism of action of MTX, and summarize the clinical feature, diagnostic criteria, risk factor, and interventions. These were hoped to arouse the attention of clinicians and clinical pharmacists, in order to effectively prevent, diagnose, and treat MTX-induced aplastic anemia.
Objective To discuss the clinical characteristics, radiological characteristics, diagnosis, and treatment of hepatocellular carcinoma with bile duct tumor thrombus (HCCBDTT), and to improve the level of diagnosis and treatment for it. Methods Clinical data of 2 cases of HCCBDTT admitted in March 2016 and July 2016 in our hospital were analyzed retrospectively, and the related literatures were reviewed. Results Two cases of HCCBDTT were misdiag- nosed as hilar cholangiocarcinoma before operation, and then proved to be HCCBDTT after operation. The 2 cases were both alive during the follow-up period (20 months and 13 months respectively). Conclusions HCCBDTT patients should be comprehensively analyzed basing on the clinical data for diagnosis, and avoiding misdiagnosis. Active surgical treatment can effectively improve the quality of life in HCCBDTT patients, and prolong the survival time.
Objective To investigate the clinical characteristics and diagnosis and treatment of fungal pulmonary embolism, and to improve the understanding of this disease. Methods The diagnosis and treatment of two patients with fungal main pulmonary embolism in the First Affiliated Hospital of Guangzhou Medical University were summarized and analyzed. Literatures were retrieved from Wanfang database, China national knowledge internet database and Pubmed database with search terms of “pulmonary embolism AND mucor”, “pulmonary embolism AND aspergillus”, “pulmonary embolism AND fungi”, “pulmonary embolism AND Candida”, “pulmonary embolism AND cryptococcus”. Results Case 1, a 53-year-old female was referred, with cough, high fever, breathlessness for 2 years, chest pain for 1 year. The patient had rheumatoid arthritis and systemic lupus erythematosus history with long term prednisone treatment. Finally, the patient was diagnosed main pulmonary artery embolism (aspergillus) and disseminated aspergillosis. Although treatment with voriconazole, amphotericin B, and caspofungin were given for more than 1 year, the patient died with uncontrolled aspergillus infection. Case 2, a 67-year-old female was referred with cough, chest distress, chest pain for 8 months, breathlessness for 6 months. The patient had a history of chronic viral hepatitis C. Finally the patient was diagnosed as main pulmonary artery embolism and pulmonary valve endocarditis (aspergillus, mucor). The patient underwent pulmonary artery lesion resection and tricuspid valvuloplasty (DeVega method). After surgery, the patient was delivered with amphotericin B and posaconazole for 3 months. During the follow-up period of 1 year, the patient recovered almost totally without relapse signs. A total of 42 cases of fungal pulmonary embolism from 1980 to 2021 were retrieved (including 2 cases in this article), and 6 of these cases were main pulmonary artery embolism. Of all the cases, the median age was 49 years and 22 (54.3%) were males. 20 cases were immunocompromised. The infection pathogens included: Aspergillus (21, 50%), Candida (11, 26.2%), Mucor (7, 16.7%), and Aspergillus combined with Mucor (1, 2.5%), Coccidioides spp (1, 2.5%), and Cryptococcus (1, 2.5%). Fifteen cases were complicated with infection other than cardiopulmonary. Twenty-two cases were treated with surgery combined with antifungal medicine, and 9 cases with antifungal medicine alone. Twenty-two cases were dead and the overall mortality rate was 52.4%. There were statistically significant differences in the effects of fungal species, dissemination of other organs other than the heart and lung, and surgical treatment on the survival rate. The survival rate of different fungal species was significantly different. Dissemination to organs other than the heart and lungs reduces survival, whereas surgical treatment improves survival. Conclusions Fungal pulmonary embolism, a disease with high mortality, rarely involves the main pulmonary artery. The possibility of fungal pulmonary embolism should be considered when the cause of pulmonary thrombosis is unknown and the anticoagulant effect is poor. Although there is no unified treatment at present, early surgical combined with standard antifungal treatment may improve the prognosis of patients.
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.