Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare inherited metabolic disorder of fatty acid oxidation, with highly heterogeneous clinical manifestations. Late-onset MADD predominantly presenting with myopathic features is easily confused with inflammatory myopathy; early identification and administration of high-dose vitamin B₂ can achieve favorable therapeutic effects. This article reports a MADD patient with main manifestations of exercise intolerance and progressive post-exertion muscle weakness. The case is analyzed by integrating blood and urine metabolic screening results and genetic testing findings, so as to provide a reference for the early diagnosis of this disease.

Objective To explore the effect of management model of clinical pharmacists participating in multidisciplinary collaborative diagnosis and treatment(MDT)for new anti-tumor drugs in the national medical insurance drug negotiation(hereinafter referred to as"national negotiation"),including efficacy,safe-ty,economy and rationality.Methods The medical records of 326 cases using novel anti-tumor drugs by na-tional negotiation and conforming to the including and excluding standards in this hospital from July 2018 to June 2023 were retrospectively analyzed.The patients were divided into the MDT group(n=122)and non-MDT group(n=204).The patients diagnosed as non-small cell lung cancer(NSCLC)in the two groups were extracted and defined as the MDT-NSCLC subgroup(n=41)and non-MDT-NSCLC subgroup(n=77).The progression-free survival(PFS),overall survival(OS),disease control rate(DCR)and the indexes such as survival quality and medical quality control were compared between the groups.Results The median PFS in the two groups was 12.7 months and 8.0 months,the median OS was 75.2 months and 56.3 months,DCR was 96.72％and 81.86％respectively,and the differences were statistically significant(P＜0.05).The COX multivariate regression analysis indicated that the HR value of clinical pharmacists participating in MDT was higher than the other influencing factors.The median PFS time in the MDT-NSCLC subgroup and non-MDT-NSCLC subgroup was 10.5 months and 6.7 months,DCR was 97.30％and 75.64％respectively,and the differences were statistically significant(P＜0.05),the median OS time was 55.1 months and 40.3 months respectively,and the difference was statistically significant(P＞0.05).The COX multivariate regression anal-ysis indicated that the HR value with clinical pharmacists participating in MDT was higher than the other in-fluencing factors;The adverse reaction occurrence rate in the MDT group and non-MDT group was 45.9％and 58.3％respectively,and the difference was statistically significant(P＜0.05).The KPS score after treatment in the MDT group was higher than that in the non-MDT group,and the difference was statistically significant;in the aspect of medical quality control,the average drug proportion in the MDT group and non-MDT group was 63.93％and 64.54％respectively,the rational drug rate of comments on prescription was 98.36％and 88.73％respectively,the patient satisfaction average value was 90.69 points and 87.36 points respectively and the differences were statistically significant(P＜0.05).Conclusion Clinical pharmacists participating in MDT related to novel anti-tumor drugs by national negotiation is beneficial to improve the therapeutic effects,living quality and patient satisfaction,also benefit to management and control of off-label drug use and medical quality control indexes.

Objective To study the influencing factors of intradialytic hypotension(IDH)in diabetic maintenance hemodialysis(MHD)patients,and to provide references for clinical prevention of IDH quality control.Methods A total of 200 diabetic patients from four hemodialysis centers in Beijing from March 2022 to September 2022 were collected as the research objects.According to the definition of IDH[systolic blood pressure during hemodialysis≤90 mmHg(1 mmHg=0.133 kPa)or systolic blood pressure reduction during dialysis≥30 mmHg],the patients were divided into IDH group(frequency of hypotension events during dialysis≥30%during 7 months of follow-up)and non-IDH group.Univariate analysis and multivariate Logistic regression were used to analyze the influencing factors of IDH.receive operating characteristic curve(ROC)curve analysis was used to evaluate the predictive value of each influencing factor for IDH.Results Univariate analysis showed that compared with non-IDH group,IDH group had higher systolic blood pressure,higher blood glucose and lower serum albumin before dialysis(P<0.05).There were more patients with orthostatic hypotension in the IDH group than in the non-IDH group(P<0.05).Multivariate Logistic regression analysis showed that pre-dialysis systolic blood pressure,orthostatic hypotension and serum albumin were the influencing factors of IDH(P<0.05).ROC curve was used to evaluate the diagnostic accuracy of pre-hemodialysis systolic blood pressure for IDH.The area under the ROC curve was 0.787(95%CI:0.720-0.854,P<0.001),the threshold of IDH predicted by the Jorden index was 153 mmHg,the sensitivity was 75.5%,and the specificity was 75.4%.Conclusion Pre-hemodialysis systolic blood pressure,blood albumin and postural hypotension are independent factors of IDH in diabetic patients.In order to predict the occurrence of IDH,the pre-hemodialysis systolic blood pressure threshold was 153 mmHg.

Objective To study the influencing factors of intradialytic hypotension(IDH)in diabetic maintenance hemodialysis(MHD)patients,and to provide references for clinical prevention of IDH quality control.Methods A total of 200 diabetic patients from four hemodialysis centers in Beijing from March 2022 to September 2022 were collected as the research objects.According to the definition of IDH[systolic blood pressure during hemodialysis≤90 mmHg(1 mmHg=0.133 kPa)or systolic blood pressure reduction during dialysis≥30 mmHg],the patients were divided into IDH group(frequency of hypotension events during dialysis≥30%during 7 months of follow-up)and non-IDH group.Univariate analysis and multivariate Logistic regression were used to analyze the influencing factors of IDH.receive operating characteristic curve(ROC)curve analysis was used to evaluate the predictive value of each influencing factor for IDH.Results Univariate analysis showed that compared with non-IDH group,IDH group had higher systolic blood pressure,higher blood glucose and lower serum albumin before dialysis(P<0.05).There were more patients with orthostatic hypotension in the IDH group than in the non-IDH group(P<0.05).Multivariate Logistic regression analysis showed that pre-dialysis systolic blood pressure,orthostatic hypotension and serum albumin were the influencing factors of IDH(P<0.05).ROC curve was used to evaluate the diagnostic accuracy of pre-hemodialysis systolic blood pressure for IDH.The area under the ROC curve was 0.787(95%CI:0.720-0.854,P<0.001),the threshold of IDH predicted by the Jorden index was 153 mmHg,the sensitivity was 75.5%,and the specificity was 75.4%.Conclusion Pre-hemodialysis systolic blood pressure,blood albumin and postural hypotension are independent factors of IDH in diabetic patients.In order to predict the occurrence of IDH,the pre-hemodialysis systolic blood pressure threshold was 153 mmHg.

Smart healthcare plays an important role in easing the strain on medical resources and improving the continuity of chronic disease management. This study analysed the iatrogenic risks from the intrinsic attributes and the external environment of smart healthcare, including doctor-patient conflict risk, technical operation risk, information leakage risk, humanistic absence risk, legal risk, regulatory risk and ethical risk. Based on the " structure process result" model, suggestions were proposed to optimize the construction of a smart healthcare platform for chronic diseases, improve the legal system and industry standards, strengthen talent cultivation and capacity building, establish an integrated regulatory system, and regularly evaluate the effectiveness of chronic disease management. These suggestions provided references for creating a healthy, orderly, and safe smart healthcare environment for chronic disease patients.

Objective·To explore the diagnostic and treatment methods for patients with cystic fibrosis(CF)complicated by allergic bronchopulmonary aspergillosis(ABPA),and to enhance clinicians'understanding of these two diseases.Methods·A retrospectively analysis was conducted on the clinical data of 5 patients with CF complicated by ABPA admitted to the Department of Respiratory Medicine,Shanghai Children's Hospital,Shanghai Jiao Tong University School of Medicine,from July 2023 to August 2024.A literature search was performed in PubMed,Web of Science,Cochrane Library,and CNKI for studies published in the past 10 years regarding the co-existence of these diseases.Clinical manifestations,treatment courses,and current epidemiological research were summarized and analyzed.Results·Common symptoms of patients with CF complicated by ABPA included aggravated cough and expectoration,wheezing,fever,and dyspnea.Whole-exome aequencing indicated mutations in the cystic fibrosis transmembrane conductance regulator(CFTR)gene,and an increase in the concentration of chloride ions in sweat.The levels of total serum immunoglobulin E(IgE)and Aspergillus fumigatus-specific IgE increased,and chest computed tomography(CT)showed bronchiectasis and mucus plugging.CF complicated by ABPA is often missed or misdiagnosed for asthma.In China,ABPA is often diagnosed before CF,whereas in Caucasian populations CF is typically diagnosed first.Initial treatment usually involves long-term oral administration of antifungal drugs such as voriconazole combined with glucocorticoids such as prednisone.For patients with frequent relapses or severe side effects,alternative antifungal agents or omalizumab therapy may be considered.Co-infection with Pseudomonas aeruginosa is common,often requiring intravenous antibiotics such as cefoperazone-sulbactam.Current epidemiological research focuses mainly on clinical characteristics,treatment regimens,and novel diagnostic methods.Conclusion·ABPA and CF have overlapping symptoms.Accurate diagnosis of CF complicated by ABPA requires genetic testing,sweat chloride measurement,chest CT,and serological tests.The coexistence of these diseases often leads to missed,delayed,or incorrect diagnosis,increasing patient burden.Present epidemiological studies mainly address clinical characteristics with a lack of targeted clinical drug trials for this patient population.

Objective·To explore the diagnostic and treatment methods for patients with cystic fibrosis(CF)complicated by allergic bronchopulmonary aspergillosis(ABPA),and to enhance clinicians'understanding of these two diseases.Methods·A retrospectively analysis was conducted on the clinical data of 5 patients with CF complicated by ABPA admitted to the Department of Respiratory Medicine,Shanghai Children's Hospital,Shanghai Jiao Tong University School of Medicine,from July 2023 to August 2024.A literature search was performed in PubMed,Web of Science,Cochrane Library,and CNKI for studies published in the past 10 years regarding the co-existence of these diseases.Clinical manifestations,treatment courses,and current epidemiological research were summarized and analyzed.Results·Common symptoms of patients with CF complicated by ABPA included aggravated cough and expectoration,wheezing,fever,and dyspnea.Whole-exome aequencing indicated mutations in the cystic fibrosis transmembrane conductance regulator(CFTR)gene,and an increase in the concentration of chloride ions in sweat.The levels of total serum immunoglobulin E(IgE)and Aspergillus fumigatus-specific IgE increased,and chest computed tomography(CT)showed bronchiectasis and mucus plugging.CF complicated by ABPA is often missed or misdiagnosed for asthma.In China,ABPA is often diagnosed before CF,whereas in Caucasian populations CF is typically diagnosed first.Initial treatment usually involves long-term oral administration of antifungal drugs such as voriconazole combined with glucocorticoids such as prednisone.For patients with frequent relapses or severe side effects,alternative antifungal agents or omalizumab therapy may be considered.Co-infection with Pseudomonas aeruginosa is common,often requiring intravenous antibiotics such as cefoperazone-sulbactam.Current epidemiological research focuses mainly on clinical characteristics,treatment regimens,and novel diagnostic methods.Conclusion·ABPA and CF have overlapping symptoms.Accurate diagnosis of CF complicated by ABPA requires genetic testing,sweat chloride measurement,chest CT,and serological tests.The coexistence of these diseases often leads to missed,delayed,or incorrect diagnosis,increasing patient burden.Present epidemiological studies mainly address clinical characteristics with a lack of targeted clinical drug trials for this patient population.

Smart healthcare plays an important role in easing the strain on medical resources and improving the continuity of chronic disease management. This study analysed the iatrogenic risks from the intrinsic attributes and the external environment of smart healthcare, including doctor-patient conflict risk, technical operation risk, information leakage risk, humanistic absence risk, legal risk, regulatory risk and ethical risk. Based on the " structure process result" model, suggestions were proposed to optimize the construction of a smart healthcare platform for chronic diseases, improve the legal system and industry standards, strengthen talent cultivation and capacity building, establish an integrated regulatory system, and regularly evaluate the effectiveness of chronic disease management. These suggestions provided references for creating a healthy, orderly, and safe smart healthcare environment for chronic disease patients.

This article focuses on a case study of sitosterolemia in a child who initially presented with hemolytic anemia and thrombocytopenia. Sitosterolemia is a rare autosomal recessive lipid metabolism disorder, difficult to diagnose due to its non-typical clinical manifestations. The 8-year-old patient was initially misdiagnosed with pyruvate kinase deficiency. Comprehensive biochemical and molecular biology analyses, including gene sequencing, eventually led to the correct diagnosis of sitosterolemia. This case highlights the complexity and diagnostic challenges of sitosterolemia, emphasizing the need for increased awareness and accurate diagnosis in patients presenting with similar symptoms.

【Objective】 To retrospectively analyze the indexes of autologous blood transfusion during heart valve replacement, in order to provide reference for allogeneic blood transfusion during heart valve replacement surgery under direct vision. 【Methods】 The data of 180 patients who underwent heart valve replacement in our hospital from January 2020 to December 2021 were analyzed retrospectively. The patients were divided into allogeneic and non-allogeneic blood transfusion group based on whether allogeneic blood was transfused during the operation, and the general data and 24 hours pre- and post-operative clinical examination indexes were compared. 【Results】 Multivariate logistic regression analysis showed that age (OR=1.110, 95% CI: 1.058-1.165, P<0.05) and intraoperative cardiopulmonary bypass time (OR=1.062, 95% CI: 1.038-1.086, P<0.05) were risk factors for allogeneic blood transfusion, and preoperative Hb content (OR=0.910, 95%CI: 0.868-0.953, P<0.05) was a protective factor. The RBC count(4.16±0.73 vs 4.52±0.71)×10¹²/L and Hb(120.94±17.97 vs 136.57±19.33) g/L at 24 hours preoperative in the allogeneic transfusion group were lower than those in the non-allogeneic transfusion group, and the RBC(3.51±0.53 vs 4.13±0.78)×10¹²/L, Hb(114.15±11.68 vs 124.79±14.96)g/L and platelet count(124.28±32.11 vs 148.29±26.62)×10⁹/L at 24 hours postoperative were significantly lower than those in the non-allogeneic transfusion group (P<0.05). 【Conclusion】 Age and intraoperative cardiopulmonary bypass time are the risk factors for autologous and allogeneic blood transfusion during heart valve replacement under direct vision, and the preoperative Hb content is a protective factor. It is necessary to evaluate the symptomatic treatment of patients before operation and reduce allogeneic blood transfusion.