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BackgroundChina's research on acute intermittent porphyria (AIP) is relatively limited. Due to most medical workers' insufficient understanding, AIP is easily misdiagnosed, and mistreated, leading to delayed appropriate treatment.ObjectiveTo analyze the clinical data of Chinese individuals with AIP with hydroxymethylbilane synthase (HMBS) gene mutation who were reported in published studies, improving medical workers' understanding of AIP.MethodsDatabases of PubMed, CNKI, Wanfang Data and CQVIP were searched for published articles about Chinese AIP cases from inception to June 30, 2021. Literature screening based on the inclusion and exclusion criteria of this study and data extraction were performed by two researchers, separately. Information regarding general data, clinical manifestations, results of laboratory tests, treatments, and gene mutations of AIP patients was analyzed.ResultsA total of 32 articles were included, containing 65 AIP patients (56 females and 9 males) with the age mostly between 20-39 years old 〔83.1% (54/65) 〕. Most of AIP patients' admission were for abdominal pain〔63 cases (96.9%) 〕, often accompanied by nausea, vomiting, and constipation. 56.9% (37/65) of AIP patients suffered from central neuropathy, disturbance of consciousness (25 cases) , epileptiform seizures (22 cases) and respiratory muscle paralysis (6 cases) . 13.8% (9/65) of patients experienced peripheral neuropathy including fatigue (6 cases) and limb numbness (4 cases) . 30.8% (20/65) of patients had autonomic neuropathy such as hypertension and tachycardia. 9.2% (6/65) of patients experienced spirit symptoms, such as depression, anxiety and hallucination. Thirty-five patients had generated multiple acute attacks of AIP. In some patients, the onset of AIP may have been related to menstruation (9 cases) , pregnancy and childbirth (4 cases) , fatigue (3 cases) , alcohol consumption (2 cases) , and dieting (1 case) . 25 patients developed hyponatremia. There were 11 cases of anemia and 11 cases of abnormal liver function. Six patients were in abnormal renal function. 1 case was caught in disorder of cortisol rhythm. Twenty-seven patients received carbohydrate loading therapy, and 25 of them were improved. Two patients were treated with carbohydrate loading combined with heme therapy, and 1 eventually improved. A total of 26 HMBS gene mutations were included in the study, where in the p.R173W, p.R173Q, p.T269R, p.W283X and p.A330P mutations were detected in multiple patients. And different patients with the same gene mutation may also show different clinical phenotypes.ConclusionAIP is a disease with complex and variable clinical manifestations and heterogeneous severity of disease. The acute attack of AIP should be considered if patients, especially young female patients, are clinically found to be with unexplained abdominal pain, epileptiform seizures, disturbance of consciousness, hypertension, tachycardia and hyponatremiain. Currently, carbohydrate loading therapy is mainly applied to control the attack of AIP in China. In terms of factors affecting the clinical phenotype of AIP, further exploration still needs to be carried out.

Taking high speed rotating shaft parts as the research object, the general fault mode of the parts is analyzed. The analysis process of haft parts is expounded by means of finite element analysis method. A variety of fatigue life prediction methods for mechanical equipment parts are reviewed in this paper. And the selection and calculation method of parameters in the life prediction model of shaft parts are summarized. Finally, taking a gear shaft of a reducer as an example, the Morrow linear correction of the elastic stress model, Manson-Halford (M-H) correction model and Smith Watson Topper (SWT) correction model are applied to fatigue life assessments. The prediction results of the SWT correction model are relatively conservative. The prediction results of toe Morrow linear correction of the elastic stress model and the M-H model are similar, and they are larger than the SWT correction model. [ABSTRACT FROM AUTHOR]

Considering that the fault phenomenon of the power head cannot feed under actual working conditions, fuzzy mathematics theory is introduced into fault tree analysis (FTA) according to the structural characteristics of hydraulic system of anchor drilling rigs in this paper. The triangle fuzzy number is used to describe the fault probability of the basic event, the fuzzy probability importance of the basic event is calculated, and the basic events are sorted by comparing the magnitude of the fuzzy probability importance. The results show that the gear wear of auxiliary oil pump, suction phenomena of gear pump, wear and leakage of No. 1 and No. 3 pumps are the key factors leading to power head failure. In order to improve the overall reliability of the hydraulic system, fault diagnosis and maintenance decisions provide a theoretical basis. [ABSTRACT FROM AUTHOR]

Objective To evaluate the training outcomes of refresher physicians in Department of Rheumatic Immunology, Peking Union Medical College Hospital, and to explore the standardized training mode. Methods In September 2021, twenty three intermediate-level refresher physicians from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital participated in a comprehensive multi-scene training program including outpatient tutorials, case-based teaching of difficult and critical cases in wards, specialist lectures and multi-disciplinary lectures. After six months of training, the refresher physicians completed a questionnaire survey covering medical care, scientific research, teaching format, and advanced training duration, with 10 multiple-choice questions and 3 fill-in-the-blank questions. The results were analyzed descriptively. Results After six months of training in a multi-scene comprehensive training program, as shown by the results of the self-assessment by the trainees, the clinical skills and scientific research capacity improved by 55.7% and 50% respectively as compared to the pre-training period. The average improvement of standardized diagnosis and treatment was 60.9%, and the average improvement of diagnosis and treatment skills was 53.5%. The average improvement in the diagnosis and treatment level of difficult cases was 53.0%. The average improvement in the diagnosis and treatment level of rare diseases was 50.4%.There were 65.25% of the refresher physicians believed that the most rewarding learning was in the outpatient clinics, followed by discussion of difficult cases in wards and lectures by the fellowship physicians. 56.5% of the refresher physicians felt that the optimal duration of the advanced training was 12 months.Conclusions A comprehensive training system combining outpatient tutorials, case-based training of difficult cases in the ward, specialized lectures and multidisciplinary lectures for advanced refresher physicians is effective for training rheumatologists.