1.Pelvic epithelioid inflammatory myofibroblastic sarcoma complicated with peritoneal metastasis: a case report
Wenpeng HUANG ; Lele SONG ; Xiaoyan XIAO ; Liming LI ; Yongkang QIU ; Jianbo GAO ; Lei KANG
Journal of Chinese Physician 2025;27(3):357-360
Objective:To analyze the clinical features, imaging features and diagnosis and treatment process of a female pelvic epithelioid inflammatory myofibroblastic sarcoma (EIMS) with peritoneal metastasis, so as to improve the clinical understanding and diagnostic ability of the disease and avoid misdiagnosis and missed diagnosis.Methods:The clinical data of a female patient with pelvic EIMS combined with peritoneal metastasis in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.Results:The triad examination of the patient involved an irregular solid mass in the pelvic cavity about 8.0 cm×9.0 cm in size. Laboratory examination revealed human epididymal protein 4(HE4)154.00 pmol/L. The ultrasonography showed multiple low-echo masses in deep pelvic cavity with unclear boundary and uneven internal echo, and color doppler flow imaging (CDFI) showed abundant internal blood flow signals. Enhanced CT showed uneven and obvious enhancement of the lesion. Pathological examination showed the infiltration of inflammatory cells in the mucous interstitial background. The tumor cells were round and epithelioid, with large nuclei, deep staining and obvious nucleolus. Immunohistochemistry showed anaplastic lymphoma kinase (ALK) positive, and molecular pathology fluorescence in situ hybridization showed ALK gene amplification (positive). Combined with pathological, immunohistochemical and genetic tests, EIMS was diagnosed. Conclusions:EIMS should be considered when there are single or multiple solid or cystic nodules or masses in the pelvic cavity with obvious enhancement, invasive growth and peritoneal implantation metastasis, and the correct diagnosis can be made according to the pathological findings, immunohistochemistry and genetic test results.
2.Analysis on the Characteristics of Personnel Disputes in Domestic Public Hospitals Based on Grounded Theory
Lufei XU ; Tiefu KUI ; Zhi SONG ; Kai ZHENG ; Jianbo LEI
Chinese Hospital Management 2025;45(2):79-81
Objective To systematically understand the current situation of personnel disputes and put forward tar-geted opinions and suggestions,providing theoretical basis and data support for future research on personnel dis-putes in public hospitals.Methods Based on grounded theory and using NVivo 12 software,it makes a qualitative study on 374 second-instance judgments of personnel disputes in public hospitals retrieved from China Judgment Document Network.Results Through three-level coding of the judgments,found that personnel disputes in public hospitals had characteristic rules in three core nodes:the focus of the dispute(437 reference points),the applica-ble laws and regulations(1 005 reference points),and the winning party(374 reference points),among which the focus of the dispute was mostly the performance and termination of the employment contract.The applicable laws and regulations were mostly general laws and regulations,while the exclusive laws and regulations on personnel were lacking,and the winning parties were mostly hospitals.Conclusion Public hospitals can prevent and reduce the occurrence of personnel disputes from four aspects:strengthening the management of employment contracts,ful-ly fulfilling the obligation of informing establishing an in-hospital mediation mechanism and actively responding to dis-putes.
3.Analysis of clinical characteristics and genetic variations in a case of self-improving collodion ichthyosis in the adult stage
Siming HU ; Mengyao ZHANG ; Weixia WANG ; Jinghui SONG ; Jianguo LI ; Jianbo WANG
Chinese Journal of Dermatology 2025;58(5):469-472
Objective:To investigate clinical characteristics and genetic variations in a case of self-improving collodion ichthyosis in the adult stage.Methods:An adult patient with clinically suspected self-improving collodion ichthyosis was collected from the Department of Dermatology, Henan Provincial People′s Hospital in April 2023. Clinical data were collected from the patient and her parents. Peripheral blood samples were obtained from them, and whole blood DNA was extracted. Whole-exome sequencing was performed to screen genetic variation sites, which were then verified by Sanger sequencing. The deleteriousness of the identified variants was assessed using pathogenicity analysis software.Results:The 54-year-old female patient presented with facial and neck flushing, mild dry skin on the trunk and limbs, sheepskin-like skin of the dorsal hand, and short fingers. Genetic testing identified two in-frame deletion mutations c.406_408del (p.E136del) and c.769_801del (p.H257_Q267del) in the non-repetitive region of the ALOX12B gene in the patient, which were inherited from her father and mother respectively. Bioinformatics analysis revealed that both genetic variations were deleterious pathogenic mutations.Conclusions:Two in-frame deletion mutations c.406_408del (p.E136del) and c.769_801del (p.H257_Q267del) were identified in the non-repetitive region of the ALOX12B gene in the patient with self-improving collodion ichthyosis, which may contribute to the clinical phenotype of the patient. The mutation c.769_801del had not been reported in literature.
4.Hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis: case report and literature review of genotype-phenotype correlations
Weixia WANG ; Chen WANG ; Jinfa DOU ; Lu BIAN ; Jinghui SONG ; Zhenlu LI ; Jianguo LI ; Jianbo WANG
Chinese Journal of Dermatology 2025;58(4):356-360
Objective:To report a case of hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis (POIKTMP), and analyze the genotype-phenotype correlation through a literature review.Methods:The clinical manifestations and genetic testing results of a Chinese Han child with POIKTMP were reported. Relevant literature was searched in databases using ′FAM111B gene′, ′hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis′ or ′POIKTMP′ as keywords, and the clinical manifestations, mutation sites of the FAM111B gene, and the correlation between them were statistically analyzed.Results:A 6.5-year-old girl developed POIKTMP at 6 months of age. Dermatological examination showed irregular brown patches and dotted hypopigmentation on the face and neck, mainly on the forehead and around the mouth, telangiectasia on the cheeks and nose, pigmentation and hypopigmentation on the limbs and trunk, as well as sparse, pale eyebrows. A total of 39 cases of POIKTMP were retrieved, including this case, all of which had clinical data and were definitively diagnosed. Fourteen variants of the FAM111B gene had been reported, including 1 in-frame deletion variant and 13 missense variants. Among the 39 cases, the incidence of poikiloderma/photosensitivity/facial erythema/telangiectasia was 100% (39/39), alopecia was 87.2% (34/39), and that of hypohidrosis/heat intolerance was 82.1% (32/39). The incidence of extracutaneous manifestations was as follows: tendon contractures/digital sclerosis, 69.2% (27/39) ; elevated liver transaminases, 46.2% (18/39) ; muscle pain/weakness/amyotrophy, 43.6% (17/39). The incidence of eczema-like lesions, bullous lesions, and elevated liver transaminases was significantly higher in the young versus the adult group ( P < 0.05) . Conclusions:This case of POIKTMP was characterized by brown patches, hypopigmentation, and sparse eyebrows. POIKTMP is a progressive multisystem disorder with age-related clinical manifestations. Early genetic testing is crucial for evaluating potential complications and providing genetic counseling.
5.A case of pheochromocytoma crisis misdiagnosed as severe viral myocarditis
Yafen JIANG ; Zhong ZHONG ; Yan XU ; Fengxian HUANG ; Lijuan XU ; Chengqiang MO ; Qiao HE ; Song YANG ; Jianbo LI
Chinese Journal of Nephrology 2025;41(9):687-690
Pheochromocytoma is a neuroendocrine tumor that produces catecholamines, leading to elevated blood pressure and metabolic changes in patients. It can result in serious complications affecting the heart, brain, kidneys, and blood vessels, potentially becoming a primary cause of death. Most pheochromocytoma patients present with atypical symptoms, making misdiagnosis or missed diagnosis common. This article reports a case of pheochromocytoma crisis misdiagnosed as severe viral myocarditis and includes a review of the relevant literature.
6.Tirzepatide in the treatment of type 2 diabetes mellitus:a rapid health technology assessment
Yunxuan ZHANG ; Haifeng ZHOU ; Ningzhou GAO ; Jianbo WU ; Cheng QIAN ; Min ZHANG ; Han GUO ; Zhongjuan SONG ; Xiaoyan LIU
Chinese Journal of Pharmacoepidemiology 2025;34(9):1057-1065
Objective To conduct a rapid health technology assessment(rHTA)of the efficacy,safety,and cost-effectiveness of tirzepatide for the treatment of type 2 diabetes mellitus(T2DM),and to provide evidence for clinical medication.Methods PubMed,Web of Science,Embase,Cochrane Library,WanFang Data,CNKI databases,and health technology assessment(HTA)websites were searched to collect systematic reviews/Meta-analysis,pharmacoecomic literature and HTA reports of tirzepatide in the treatment of T2DM from inception to February 28,2025.Two researchers independently conducted literature screening,data extraction,and quality assessment,and then summarized and analyzed the results.Results A total of 13 articles were included,comprising 2 HTA reports,8 systematic reviews/Meta-analyses,and 3 pharmacoeconomic studies.In terms of efficacy,tirzepatide outperformed placebo or other antidiabetic drugs in reducing glycated hemoglobin(HbA1c),increasing the rate of HbA1c<7%,lowering blood glucose,and reducing weight.The antihyperglycemic and weight-loss effects of tirzepatide were dose-dependent,and it also had certain advantages in reducing the risk of cardiovascular events and renal composite endpoint events.Regarding safety,the incidence of gastrointestinal adverse events in the tirzepatide group was higher than that in the placebo group and the insulin group,mainly manifested as diarrhea,nausea,and vomiting.However,it did not increase the risk of serious gastrointestinal adverse events,nor did it increase the risk of hypoglycemia and pancreatitis.In terms of cost-effectiveness,tirzepatide had cost-utility and cost-effectiveness advantages compared with semaglutide and other antidiabetic drugs.Conclusion Compared with other antidiabetic drugs,tirzepatide has better efficacy,safety,and cost-effectiveness in the treatment of T2DM.
7.Analysis of Influencing factors on the therapeutic effect of endoscopic retrograde cholangiopancreatography stent implantation for Malignant obstructive jaundice
Hongxu LI ; Jinzhi SONG ; Yang CHEN ; Jianbo XIAO ; Shoujun WANG
Journal of Clinical Surgery 2025;33(8):856-859
Objective To investigate the influencing factors of ERCP stent implantation for patients with malignant obstructive jaundice.Methods 130 patients with malignant obstructive jaundice who received ERCP stent implantation in our hospital from January 2017 to January 2024 were retrospectively included,and grouped according to the jaundice control effect 4 weeks after surgery.Univariate and multivariate analysis of the efficacy of ERCP stent placement in malignant obstructive jaundice after 4 weeks.Construction of a predictive model for the efficacy of ERCP stent placement in malignant obstructive jaundice after 4 weeks and clinical efficacy analysis.Results There were 64 cases for jaundice resolved in 4 weeks after surgery among all 130 patients with the regression rate for 49.23%.The results of univariate analysis showed that the type of biliary obstruction,stent type,and preoperative Child Pugh grading may all be related to the efficacy of ERCP stent placement in malignant obstructive jaundice after 4 weeks(P<0.05).The results of Logistic multivariate analysis showed that the type of biliary obstruction,stent type,and preoperative Child Pugh grade were all independent influencing factors on the efficacy of ERCP stent placement for malignant obstructive jaundice at 4 weeks(P<0.05).Using the independent influencing factors and P-value prediction probability of Logistic regression model to predict the prognosis of patients,the ROC curve was used,with areas under the curve of 0.713,0.823,0.907,and 0.971,respectively.Conclusion The clinical effects of ERCP stent implantation in malignant obstructive jaundice was closely related to the type of biliary obstruction,stent type and preoperative Child-Pugh grade.The data model constructed using the above three factors has shown good performance in predicting the prognosis of patients.
8.Analysis of clinical characteristics and genetic variations in a case of self-improving collodion ichthyosis in the adult stage
Siming HU ; Mengyao ZHANG ; Weixia WANG ; Jinghui SONG ; Jianguo LI ; Jianbo WANG
Chinese Journal of Dermatology 2025;58(5):469-472
Objective:To investigate clinical characteristics and genetic variations in a case of self-improving collodion ichthyosis in the adult stage.Methods:An adult patient with clinically suspected self-improving collodion ichthyosis was collected from the Department of Dermatology, Henan Provincial People′s Hospital in April 2023. Clinical data were collected from the patient and her parents. Peripheral blood samples were obtained from them, and whole blood DNA was extracted. Whole-exome sequencing was performed to screen genetic variation sites, which were then verified by Sanger sequencing. The deleteriousness of the identified variants was assessed using pathogenicity analysis software.Results:The 54-year-old female patient presented with facial and neck flushing, mild dry skin on the trunk and limbs, sheepskin-like skin of the dorsal hand, and short fingers. Genetic testing identified two in-frame deletion mutations c.406_408del (p.E136del) and c.769_801del (p.H257_Q267del) in the non-repetitive region of the ALOX12B gene in the patient, which were inherited from her father and mother respectively. Bioinformatics analysis revealed that both genetic variations were deleterious pathogenic mutations.Conclusions:Two in-frame deletion mutations c.406_408del (p.E136del) and c.769_801del (p.H257_Q267del) were identified in the non-repetitive region of the ALOX12B gene in the patient with self-improving collodion ichthyosis, which may contribute to the clinical phenotype of the patient. The mutation c.769_801del had not been reported in literature.
9.Clinical significance of skeletonization dissection for No.12 lymph nodes after neoadjuvant therapy in advanced gastric cancer
Bohao ZHOUYE ; Kaiyu SUN ; Zhewei WEI ; Jianbo XU ; Xinhua ZHANG ; Shirong CAI ; Wu SONG
Chinese Journal of Gastrointestinal Surgery 2025;28(9):1052-1058
Objective:To explore the clinical significance of skeletonized lymph node dissection of No.12 lymph nodes after neoadjuvant therapy in patients with advanced gastric cancer.Methods:For this retrospective case-cohort study we collected data from patients with advanced gastric cancer who underwent neoadjuvant chemotherapy and D2 or more extensive curative resection including No.12 lymph node dissection at the First Affiliated Hospital of Sun Yat-sen University from January, 2011 to December, 2022. Patients were divided into two groups based on whether they received skeletonized dissection of No.12 lymph nodes: 177 cases were in the skeletonized group, and 55 cases were in the nonskeletonized group. The differences of prognosis between the two groups were compared, and logistic regression models were used to analyze the factors affecting No.12 lymph node metastasis in the overall cohort and No.12b or No.12p lymph node metastasis in the skeletonized group.Results:A total of 232 patients were included, with 84 females (36.2%) and 148 males (63.8%), with an average age of 56.4±11.6 years. The proportion of female and ycT4 patients was significantly higher in the skeletonized group than in the nonskeletonized group (both P<0.05). Among all 232 patients, No. 12a metastasis occurred in 14 cases (6.0%). In the skeletonized group of 177 patients, No. 12b and No. 12p metastases were observed in 6 patients each (3.4%), and 4 patients had concurrent metastases in both No. 12b and No. 12a. The 5-year overall survival (OS) rates were 45.5% in the skeletonized group and 42.8% in the nonskeletonized group, with no statistical difference (HR=0.755, 95%CI: 0.488-1.168, P=0.580). The 5-year disease-free survival (DFS) rates were 39.8% and 41.0%, respectively, also with no statistical difference (HR=0.775, 95%CI: 0.513-1.172, P=0.584). 5-year OS for patients without No.12 lymph node metastasis was 48.8%, which was higher than the 15.9% for those with metastasis (HR=0.349, 95% CI: 0.209-0.584, P=0.003). Additionally, the 5-year DFS for those without metastasis was 44.3%, significantly higher than the 5.7% for those with metastasis (HR=0.444, 95%CI: 0.276-0.716, P<0.001). For patients without No. 12b or No. 12p lymph node metastasis, the 5-year OS was 47.6%, and the 5-year DFS was 42.3%, both of which were significantly higher than the 16.7% and 8.3% for those with No.12b or No. 12p lymph node metastasis, respectively (HR=0.353, 95%CI: 0.183-0.681, P=0.005; HR=0.457, 95%CI: 0.244-0.855, P=0.006). Multivariate analysis showed that more advanced ypN stage (OR=3.908, 95%CI:1.638-9.323, P=0.002) and tumor location in the lower stomach or whole stomach (OR=3.533, 95%CI: 1.312-9.511, P=0.012) were independent risk factors for No.12 lymph node metastasis and also for No.12b and No.12p lymph node metastasis (OR=2.426, 95%CI: 1.212-4.856, P=0.012 and OR=4.908, 95%CI:1.182-20.373, P=0.028, respectively). Conclusion:Patients with advanced gastric cancer who have more advanced ypN stage and tumor location in the lower stomach or whole stomach have a higher risk of No.12b and No.12p metastasis and thus require further skeletonized lymph node dissection of No.12.
10.Hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis: case report and literature review of genotype-phenotype correlations
Weixia WANG ; Chen WANG ; Jinfa DOU ; Lu BIAN ; Jinghui SONG ; Zhenlu LI ; Jianguo LI ; Jianbo WANG
Chinese Journal of Dermatology 2025;58(4):356-360
Objective:To report a case of hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis (POIKTMP), and analyze the genotype-phenotype correlation through a literature review.Methods:The clinical manifestations and genetic testing results of a Chinese Han child with POIKTMP were reported. Relevant literature was searched in databases using ′FAM111B gene′, ′hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis′ or ′POIKTMP′ as keywords, and the clinical manifestations, mutation sites of the FAM111B gene, and the correlation between them were statistically analyzed.Results:A 6.5-year-old girl developed POIKTMP at 6 months of age. Dermatological examination showed irregular brown patches and dotted hypopigmentation on the face and neck, mainly on the forehead and around the mouth, telangiectasia on the cheeks and nose, pigmentation and hypopigmentation on the limbs and trunk, as well as sparse, pale eyebrows. A total of 39 cases of POIKTMP were retrieved, including this case, all of which had clinical data and were definitively diagnosed. Fourteen variants of the FAM111B gene had been reported, including 1 in-frame deletion variant and 13 missense variants. Among the 39 cases, the incidence of poikiloderma/photosensitivity/facial erythema/telangiectasia was 100% (39/39), alopecia was 87.2% (34/39), and that of hypohidrosis/heat intolerance was 82.1% (32/39). The incidence of extracutaneous manifestations was as follows: tendon contractures/digital sclerosis, 69.2% (27/39) ; elevated liver transaminases, 46.2% (18/39) ; muscle pain/weakness/amyotrophy, 43.6% (17/39). The incidence of eczema-like lesions, bullous lesions, and elevated liver transaminases was significantly higher in the young versus the adult group ( P < 0.05) . Conclusions:This case of POIKTMP was characterized by brown patches, hypopigmentation, and sparse eyebrows. POIKTMP is a progressive multisystem disorder with age-related clinical manifestations. Early genetic testing is crucial for evaluating potential complications and providing genetic counseling.

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