1.von Hippel-Lindau disease manifesting as a sellar and spinal cord solid-cystic mass: a case report
Yuzhen WEI ; Gehong DONG ; Decai TIAN ; Jia LI ; Shiwei LI ; Huabing WANG
Chinese Journal of Neurology 2025;58(9):981-985
von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary tumor predisposition syndrome. Central nervous system hemangioblastoma is one of its major clinical manifestations, commonly occurring in the infratentorial region and spinal cord, while supratentorial involvement is rare. A 23-year-old female patient with sellar and spinal hemangioblastomas, along with polycystic pancreas, who presented with back pain and decreased vision in the left eye, was reported. The diagnosis of VHL disease was confirmed based on family history, pathological findings, and genetic testing. This case was reported to enhance clinicians′ awareness of this disease, facilitating early diagnosis and intervention for patients and their families to improve clinical outcomes.
2.Construction and application evaluation of a core competency framework-based training program for cardiovascular nurse specialists
Haiyan YU ; Yuting LAI ; Bi LIN ; Hong CAI ; Sailan LI ; Junqin ZHANG ; Huabing LEI ; Yuan CHEN
Chinese Journal of Medical Education Research 2025;24(10):1408-1414
Objective:To construct and evaluate a training program for cardiovascular nurse specialists based on a core competency framework.Methods:Among 61 trainees participating in the first training class for cardiovascular nurse specialists organized by a provincial nursing society, a training program focusing on the nine core competencies for cardiovascular nurse specialists was implemented, which consisted of 4-week theoretical and 4-week practical training. The effectiveness of the training program was evaluated using the Kirkpatrick model. Data analyses were performed by using SPSS 26.0. Categorical data were presented as the number of cases; continuous variables in normal distribution were expressed as mean±standard deviation; and continuous variables in non-normal distribution were presented as median (interquartile range) and compared using non-parametric tests.Results:At the reaction level, the satisfaction rates of trainees with the theoretical and practical sections of the training program were 98.36% (60/61) and 95.08% (58/61), respectively. At the learning level, the comprehensive assessment score of the trainees was (83.01±3.39) points, and all of them successfully obtained their completion certificates, with a pass rate of 100.00% (61/61). At the behavior level, the core competencies for cardiovascular nurse specialists were significantly improved after training [the total score increased from 291.00 (254.00, 334.25) to 410.50 (354.50, 433.00), P<0.001]. At the results level, at six months after training, there were significant increases in the number of participants engaging in cardiovascular care practices, clinical nursing education and guidance, leadership roles, and research projects within their units as well as the number of individuals achieving career advancement (all P<0.05). Conclusions:The trainees are highly satisfied with the core competency-focused cardiovascular nurse training program, which can improve core competencies and cardiovascular nursing capabilities, expand the scope of cardiovascular nursing services, and foster the sustained advancement of the participants.
3.Clinical characteristics of malignant insulinomas and benign insulinomas
Yan LIU ; Jie YU ; Yiwen LIU ; Fan PING ; Huabing ZHANG ; Lingling XU ; Yuxiu LI
Basic & Clinical Medicine 2025;45(10):1356-1361
Objective To analyze the differences in clinical indicators between malignant insulinoma and benign in-sulinoma,in order to provide diagnostic and therapeutic insights for the early detection and diagnosis of malignant insulinoma.Methods A retrospective analysis was conducted in patients diagnosed and treated for insulinoma at Peking Union Medical College Hospital from January 2018 to June 2022.Among them,10 cases were diagnosed as malignant insulinoma.Twenty cases of benign insulinoma patients matched for age,sex,and body mass index(BMI),were randomly selected.Statistical analysis was performed to compare the differences between malignant and benign insulinomas.Results 1)Compared to benign insulinoma,malignant insulinoma showed significantly ele-vated C-peptide(CP)and C-peptide to glucose ratio(CPGlu)during hypoglycemia(blood glucose<3.0 mmol/L)[6.04(3.40,6.76)vs 1.68(1.39,2.47)ng/mL,P<0.05),2.25(1.12,3.58)vs 0.74(0.54,1.54),P<0.05].The tumor diameter(DIA)was larger(1.9±0.6 vs 1.4±0.3 cm,P<0.05),and the insulin level at 300 minutes(INS300)during the 5-hour oral glucose tolerance test(5 h OGTT)was significantly elevated(30.47±5.67 vs 9.67±3.32)μIU/mL,P<0.01).Levels of blood tumor markers AFP,CEA,and CA724 were also increased(P<0.05).2)Correlation analysis indicated that CP,CPGlu,DIA,INS300,AFP,CEA,and CA724 were positively correlated with malignant insulinoma during hypoglycemia.3)The ROC curve analysis suggested that the optimal cut-off points for distinguishing malignant from benign insulinomas were CP 2.49 ng/mL,CPGlu 1.31,DIA 1.85 cm,and INS300 20.22 μIU/mL,respectively.Conclusions In clinical practice,if an insulinoma patient has a CP level higher than 2.49 ng/mL and a tumor diameter larger than 1.9 cm during hypoglycemia,the possibility of malignant insulinoma should be considered,warranting further examinations and enhanced follow-up.Persistent elevation of AFP,CEA,and CA724 may indicate malignant insulinoma.
4.A Case Report of Mitochondrial Diabetes Mellitus Caused by Large Fragment Deletion of Mitochondrial Gene and Literature Review
Ran LI ; Jinhao LIAO ; Hanhui FU ; Hui PAN ; Yuxiu LI ; Jiangfeng MAO ; Hongbo YANG ; Huabing ZHANG
Medical Journal of Peking Union Medical College Hospital 2025;16(3):634-640
Mitochondrial diabetes mellitus(MDM)is a genetically heterogeneous disorder caused by mitochondrial DNA(mtDNA)or nuclear DNA mutations,characterized by multi-system involvement and diverse clinical phenotypes.We report a pediatric case presenting with growth retardation followed by subsequent development of diabetes mellitus.Systematic evaluation revealed concurrent bilateral sensorineural hearing loss,bilateral basal ganglia calcification,and electroencephalographic abnormalities.A post-exercise lactate test demonstrated significant elevation of serum lactate levels immediately after physical exertion.Genetic analysis identified a large-scale mitochondrial DNA deletion spanning from m.8649 to m.16084.This case re-port is complemented by a literature review focusing on the pathogenesis,genetic characteristics,and therapeu-tic approaches of mitochondrial diabetes,with particular emphasis on mitochondrial disorders exhibiting large-scale mtDNA deletions alongside diabetic manifestations.Our comprehensive analysis aims to enhance clinical understanding and inform diagnostic strategies for this complex disease entity.
5.Application of a rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings in children undergoing radical operation on Hirschsprung's disease
Huabing LI ; Xianghua JI ; Xianjie GENG ; Liang ZHOU ; Chunxia ZHANG ; Guangjun HOU
Chinese Journal of Modern Nursing 2025;31(29):4020-4024
Objective:To investigate the effect of a rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings in the postoperative care of children undergoing radical operation on Hirschsprung's disease.Methods:A total of 91 children with Hirschsprung's disease and their parents who underwent radical surgery at the Children's Hospital Affiliated to Zhengzhou University (Henan Children's Hospital, Zhengzhou Children's Hospital) between January 2022 and January 2024 were selected by convenience sampling. Using a computer-generated random number table, they were randomly divided into an intervention group ( n=46) and a control group ( n=45). The control group received routine surgical nursing, while the intervention group received a rapid rehabilitation nursing model based on Rogers' theoretical framework in addition to routine care. Postoperative pain stress, anorectal function, gastrointestinal functional recovery, and parental satisfaction were compared between the two groups. Results:On the third postoperative day, the intervention group had lower scores on the FLACC scale, better anorectal function, and shorter times to first bowel sound, first anal exhaust, and first defecation than the control group. The overall parental satisfaction rate in the intervention group was 86.96% (40/46), higher than 51.11% (23/45) in the control group, and the differences were statistically significant (all P<0.05) . Conclusions:The rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings showed significant effectiveness in children undergoing radical operation on Hirschsprung's disease. It reduced postoperative pain, promoted recovery of anorectal and gastrointestinal function, and improved parental satisfaction.
6.Application of a rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings in children undergoing radical operation on Hirschsprung's disease
Huabing LI ; Xianghua JI ; Xianjie GENG ; Liang ZHOU ; Chunxia ZHANG ; Guangjun HOU
Chinese Journal of Modern Nursing 2025;31(29):4020-4024
Objective:To investigate the effect of a rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings in the postoperative care of children undergoing radical operation on Hirschsprung's disease.Methods:A total of 91 children with Hirschsprung's disease and their parents who underwent radical surgery at the Children's Hospital Affiliated to Zhengzhou University (Henan Children's Hospital, Zhengzhou Children's Hospital) between January 2022 and January 2024 were selected by convenience sampling. Using a computer-generated random number table, they were randomly divided into an intervention group ( n=46) and a control group ( n=45). The control group received routine surgical nursing, while the intervention group received a rapid rehabilitation nursing model based on Rogers' theoretical framework in addition to routine care. Postoperative pain stress, anorectal function, gastrointestinal functional recovery, and parental satisfaction were compared between the two groups. Results:On the third postoperative day, the intervention group had lower scores on the FLACC scale, better anorectal function, and shorter times to first bowel sound, first anal exhaust, and first defecation than the control group. The overall parental satisfaction rate in the intervention group was 86.96% (40/46), higher than 51.11% (23/45) in the control group, and the differences were statistically significant (all P<0.05) . Conclusions:The rapid rehabilitation nursing model under the theoretical framework of Rogers' Science of Unitary Human Beings showed significant effectiveness in children undergoing radical operation on Hirschsprung's disease. It reduced postoperative pain, promoted recovery of anorectal and gastrointestinal function, and improved parental satisfaction.
7.Construction and application evaluation of a core competency framework-based training program for cardiovascular nurse specialists
Haiyan YU ; Yuting LAI ; Bi LIN ; Hong CAI ; Sailan LI ; Junqin ZHANG ; Huabing LEI ; Yuan CHEN
Chinese Journal of Medical Education Research 2025;24(10):1408-1414
Objective:To construct and evaluate a training program for cardiovascular nurse specialists based on a core competency framework.Methods:Among 61 trainees participating in the first training class for cardiovascular nurse specialists organized by a provincial nursing society, a training program focusing on the nine core competencies for cardiovascular nurse specialists was implemented, which consisted of 4-week theoretical and 4-week practical training. The effectiveness of the training program was evaluated using the Kirkpatrick model. Data analyses were performed by using SPSS 26.0. Categorical data were presented as the number of cases; continuous variables in normal distribution were expressed as mean±standard deviation; and continuous variables in non-normal distribution were presented as median (interquartile range) and compared using non-parametric tests.Results:At the reaction level, the satisfaction rates of trainees with the theoretical and practical sections of the training program were 98.36% (60/61) and 95.08% (58/61), respectively. At the learning level, the comprehensive assessment score of the trainees was (83.01±3.39) points, and all of them successfully obtained their completion certificates, with a pass rate of 100.00% (61/61). At the behavior level, the core competencies for cardiovascular nurse specialists were significantly improved after training [the total score increased from 291.00 (254.00, 334.25) to 410.50 (354.50, 433.00), P<0.001]. At the results level, at six months after training, there were significant increases in the number of participants engaging in cardiovascular care practices, clinical nursing education and guidance, leadership roles, and research projects within their units as well as the number of individuals achieving career advancement (all P<0.05). Conclusions:The trainees are highly satisfied with the core competency-focused cardiovascular nurse training program, which can improve core competencies and cardiovascular nursing capabilities, expand the scope of cardiovascular nursing services, and foster the sustained advancement of the participants.
8.von Hippel-Lindau disease manifesting as a sellar and spinal cord solid-cystic mass: a case report
Yuzhen WEI ; Gehong DONG ; Decai TIAN ; Jia LI ; Shiwei LI ; Huabing WANG
Chinese Journal of Neurology 2025;58(9):981-985
von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary tumor predisposition syndrome. Central nervous system hemangioblastoma is one of its major clinical manifestations, commonly occurring in the infratentorial region and spinal cord, while supratentorial involvement is rare. A 23-year-old female patient with sellar and spinal hemangioblastomas, along with polycystic pancreas, who presented with back pain and decreased vision in the left eye, was reported. The diagnosis of VHL disease was confirmed based on family history, pathological findings, and genetic testing. This case was reported to enhance clinicians′ awareness of this disease, facilitating early diagnosis and intervention for patients and their families to improve clinical outcomes.
9.A Case Report of Mitochondrial Diabetes Mellitus Caused by Large FragmentDeletion of Mitochondrial Gene and Literature Review
Ran LI ; Jinhao LIAO ; Hanhui FU ; Hui PAN ; Yuxiu LI ; Jiangfeng MAO ; Hongbo YANG ; Huabing ZHANG
Medical Journal of Peking Union Medical College Hospital 2024;16(3):634-640
Mitochondrial diabetes mellitus (MDM) is a genetically heterogeneous disorder caused by mitochondrial DNA (mtDNA) or nuclear DNA mutations, characterized by multi-system involvement and diverse clinical phenotypes. We report a pediatric case presenting with growth retardation followed by subsequent development of diabetes mellitus. Systematic evaluation revealed concurrent bilateral sensorineural hearing loss, bilateral basal ganglia calcification, and electroencephalographic abnormalities. A post-exercise lactate test demonstrated significant elevation of serum lactate levels immediately after physical exertion. Genetic analysis identified a large-scale mitochondrial DNA deletion spanning from m.8649 to m.16084. This case report is complemented by a literature review focusing on the pathogenesis, genetic characteristics, and therapeutic approaches of mitochondrial diabetes, with particular emphasis on mitochondrial disorders exhibiting large-scale mtDNA deletions alongside diabetic manifestations. Our comprehensive analysis aims to enhance clinical understanding and inform diagnostic strategies for this complex disease entity.
10.Geometric Shape Validation of Knee Statistical Shape Model
Huabing DUAN ; Xuelian GU ; Xiaohu LI ; Jincheng ZHOU ; Yihao CHANG ; Jie WANG ; Xiaochen GUO
Journal of Medical Biomechanics 2024;39(2):243-249
Objective To obtain the range of anatomical parameters of healthy knee joints in Chinese males and validate a statistical shape model(SSM)based on the geometric shape of a healthy knee to provide references for the design of knee SSM-based prostheses.Methods Computed tomography(CT)images of knee joints from 112 healthy males were acquired to build three-dimensional(3D)knee joint models.Each model was the target model separately,and the remaining models were used as the training set for principal component analysis(PCA).The obtained knee SSM was fitted to the target model to predict the SSM.The exact anatomical measurement points were marked on the sample and SSM prediction models,and 17 linear and 3 angular parameters were derived.The values of the anatomical parameters were statistically tested using an independent samples t-test and Mann-Whitney U-test,and the validity of the SSM in terms of geometric shape was demonstrated if the resulting P-values were all greater than 0.05.Results Qualitative and quantitative comparative analyses of anatomical parameters showed that the mean deviation of linear parameters was less than 6 mm,and that of angular parameters was less than 2.5°.The results of statistical tests showed P>0.05 for all anatomical parameters,proving that the knee SSM prediction model was not statistically different from the true healthy model in terms of geometric shape.Conclusions This study derived a reference range of anatomical parameters for a healthy knee and demonstrated that the knee SSM model was consistent with the real healthy model in terms of shape.The results provide a reference for the design of knee SSM-based prostheses.

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