Objective:To summarize the clinical manifestations of anti-synthetase syndrome (ASS) with interstitial lung disease (ILD) patients with different antibody subtypes and the skeletal muscle pathology of ASS.Methods:A total of 106 ASS-ILD patients admitted to the First Medical Center of Chinese People′s Liberation Army General Hospital from May 11, 2015 to June 25, 2023 were included. Their intramuscular and extramuscular clinical manifestations were collected. The correlation between different antibody subtypes in patients with ASS and the various subtypes of ILD was investigated. The skeletal muscle pathological characteristics of 13 ASS patients were also summarized.Results:Among the 106 ASS-ILD patients, 56 (52.8%) were anti-JO-1 antibody positive, 19 (17.9%) were anti-PL-7 antibody positive, 11 (10.4%) were anti-PL-12 antibody positive, 14 (13.2%) were anti-EJ antibody positive, and 6 (5.7%) were anti-OJ antibody positive. All the patients had ILD [including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), mixed pneumonia]. In all the patients, 46.2% (49/106) had cardiac damage, 37.7% (40/106) had arthritis, 29.2% (31/106) had myasthenia gravis, 24.5% (26/106) had myalgia, and 19.8% (21/106) had Raynaud′s phenomenon. The incidence of NSIP was 75.0% (42/56) in the anti-JO-1 antibody-positive group, significantly higher than other groups (anti-PL-7 antibody-positive group, 8/19;anti-PL-12 antibody-positive group, 3/11;anti-EJ antibody-positive group, 5/14;anti-OJ antibody-positive group, 2/6; P=0.001). UIP was most common in the anti-PL-7 antibody-positive group (8/19). OP was most frequent in the anti-PL-12 antibody-positive group (5/11). The incidence of arthritis was highest in the anti-JO-1 antibody-positive group (51.8%, 29/56). The anti-Ro-52 antibody-positive rate was significantly higher in the anti-EJ antibody-positive group (12/14) than in the other 4 groups [anti-JO-1 antibody-positive group, 33.9% (19/56); anti-PL-7 antibody-positive group, 10/19; anti-PL-12 antibody-positive group, 6/11; anti-OJ antibody-positive group, 0/6; P=0.001]. ASS skeletal muscle pathology was manifested as necrotizing myopathy pattern (6/13), inflammatory myopathy pattern (4/13), and nonspecific myopathy pattern (3/13). All the 106 patients received methylprednisolone as the basic treatment. Among them, 69 patients (65.1%) received methylprednisolone alone, while 37 patients (34.9%) received combination therapy involving immunosuppressants, whose symptoms improved after treatment. Conclusions:A discernible correlation exists between the clinical manifestations of ASS with ILD and specific antibody subtypes. ASS patients generally respond well to immunotherapy. ASS can manifest as 3 distinct skeletal muscle pathological patterns: necrotizing myopathy pattern, inflammatory myopathy pattern, and nonspecific myopathy pattern.

Objective:To investigate the incidence, clinical characteristics, and impact of spontaneous portosystemic shunt (SPSS) in patients with liver cirrhosis combined with hepatic encephalopathy (HE).Methods:The basic clinical and follow-up data were retrospectively analyzed for patients diagnosed with cirrhosis combined with HE at Mengchao Hepatobiliary Hospital of Fujian Medical University from January 2017 to December 2022. The patients were divided into large and small SPSS groups and a control group based on the results of abdominal enhanced CT or MRI.The clinical characteristics and outcome differences were compared among the three groups. Kaplan-Meier survival curves were used to compare HE-free survival time and overall survival time among the three groups. The log-rank test was used to compare the differences between groups. Cox regression analysis was used to identify the relevant risk factors affecting HE-free survival time and overall survival time.Results:A total of 223 cases with liver cirrhosis combined with HE were enrolled, including 150 in the SPSS and 73 in the control groups. The incidence rate of SPSS was 67.3% (150/223). The group was divided into small SPSS (79/150, 52.7%) and large SPSS group (71/150, 47.3%) according to the cross-sectional area of the diversion channel. The HE-free survival was shorter in the small and large SPSS groups compared with the control group (35.5 months in the small SPSS group and 21.3 months in the large SPSS group; P<0.001). The HE-free survival time was shorter in the large SPSS than with small SPSS group ( P=0.003). The overall survival time in the small SPSS group and the large SPSS group was shorter compared with the control group (small SPSS group: 39.4 months, large SPSS group: 52.9 months; P<0.001). There was no statistically significant difference in overall survival time between the small SPSS and large SPSS groups ( P=0.700). Cox regression analysis showed that SPSS was an independent risk factor affecting patients' HE-free survival time and overall survival time ( P<0.05). Conclusion:SPSS is more common in patients with liver cirrhosis combined with HE. Patients who combined with SPSS showed significant reductions in both HE-free survival time and overall survival time, especially evident in those with combined large SPSS.

Hepatic encephalopathy (HE) is a kind of neuropsychiatric syndrome caused by acute or chronic liver failure or portosystemic venous shunt (hereinafter referred to as portosystemic shunt), which can lead to the occurrence of functional impairment, personality and behavioral abnormalities, coma, and even death. Most patients with cirrhosis combined with HE have spontaneous portosystemic shunt (SPSS), especially those with recurrent or persistent HE. Internal medicine's current standard of treatment for HE associated with SPSS in cirrhotic patients is unsatisfactory, and even after treatment, recurrent HE episodes may still occur. Although interventional therapy has shown significant results and has been applied in clinical practice for many years for SPSS-associated HE, the number of treatment-related cases is relatively small, and there is a lack of large samples and well-designed research. Currently, interventional therapy for SPSS-associated HE in patients with cirrhosis is still under continuous exploration.

Objective:To analyze the influencing factors for choice of secondary disciplines in eight-year medical students majoring in clinical medicine based on the self-determination theory.Methods:Fourteen eight-year students majoring in clinical medicine in 2018 and 2019 from an affiliated medical school of Peking University Health Science Center were selected to conduct semi-structured interviews using purposive sampling and descriptive phenomenological study methods. The thematic framework method was used to analyze the interview data and refine the theme.Results:Through the analysis of interview data, a total of 3 dimensions were obtained, including 6 first-level topics and 19 second-level topics: ①autonomy dimension (interest factor and value factor); ②dimension of competence (personal trait factor and subject requirement factor); and ③belonging dimension (department factor and mentor factor).Conclusions:The sense of autonomy based on interests and values, the sense of competence matched with personal ability and the characteristics of the discipline, and the sense of belonging brought by the atmosphere of the department and the mentor are important factors influencing the choice of secondary disciplines in eight-year medical students. Teachers and teaching managers should pay attention to the cultivation of students' interests and value guidance, early contact with secondary disciplines, provision of competency training, and promotion of good interaction between students and department tutors, so as to enhance students' sense of belonging and optimize the training model of eight-year excellent doctors.

Objective:To explore the application value of cinematic rendering reconstruction technology in laparoscopic resection of giant adrenal tumors.Methods:As many as 21 patients with large adrenal tumors who underwent laparoscopic resection in Yongchuan Hospital Affiliated to Chongqing Medical University from January 2021 to January 2024 were retrospectively analyzed, with a median age of 54.0 (40.5, 58.0) years and a median tumor diameter of 7.3 (6.8, 8.8) cm. All patients underwent preoperative cinematic rendering 3D reconstruction imaging. All patients underwent enhanced CT scans. Their CT images were three-dimensionally reconstructed on the post-processing workstation platform. By changing image perspectives, adjusting the visualization of organs or blood vessels, and using physical volume rendering, real-shadow-rendered images were obtained. With these images, surgeons can intuitively understand important preoperative information, like the relationship between the tumor and surrounding organs and the path of tumor-feeding blood vessels, for preoperative planning. Following thorough preoperative preparation, laparoscopic transabdominal resection was performed. During surgery, 20 tumors were found to be located in the adrenal gland, and 1 in the retroperitoneum, with 13 on the left side and 8 on the right side. Preoperative cinematic rendering 3D imaging was consistent with intraoperative findings.Results:All 21 patients underwent successful surgeries, with an average operation time of (199.0±95.3) minutes, a median blood loss of 220 (150, 500) ml, and a median blood transfusion volume of 200 (150, 400) ml. No significant damage of vital organs or major blood vessels occurred, and there were no case of conversion to open surgery.Conclusions:For retroperitoneal giant adrenal tumors, utilizing cinematic rendering 3D reconstruction imaging enables a comprehensive understanding of the relationship between the tumor and surrounding organs and vessels preoperatively. This approach can reduce intraoperative bleeding and collateral injuries, improve the success rate of laparoscopic resection, and enhance overall surgical safety.

OBJECTIVE: To investigate the clinical characteristics and prognosis of patients with IgD multiple myeloma (MM). METHODS: The clinical data of 8 patients with IgD MM admitted to Gansu Provincial Hospital from September 2013 to February 2023 were collected, and their clinical characteristics and prognosis were retrospectively analyzed and summarized. RESULTS: Among the 8 enrolled patients, there were 4 males and 4 females, with a median age of 60 (44-74) years. All patients had symptoms of renal insufficiency and anemia. There were 3 cases of bone invasion, 3 cases of splenomegaly, 7 cases of IgD-λ type, and 1 case of IgD-κ type. FISH examination was performed in 7 cases, and 6 of them were positive for 1q21 . There were 6 cases in DS stage III and 2 cases in DS stage II; According to ISS staging, there were 6 cases in stage III, 1 case in stage II, and 1 case in stage I; According to R-ISS staging, there were 5 cases in stage III and 3 cases in stage II. All patients received bortezomib-based combination chemotherapy, with 1 case undergoing autologous stem cell transplantation (ASCT) and 2 cases receiving daratumumab in combination. The median treatment period was 6 (1-15) cycles. The short-term efficacy was evaluated after 4-6 courses of treatment. Among the 6 patients with assessable efficacy, 1 case experienced disease progression (PD), and 5 cases achieved complete remission (CR). The median follow-up time was 26 (11-33) months, and the median progression-free survival (PFS) and median overall survival (OS) of the patients were 11.25 (3-26) months and 18.5 (4-33) months, respectively. Among the 8 patients, 4 cases died. Among the deceased patients, 3 cases were in R-ISS stage III and 3 cases were 1q21 positive. 2 of the 5 patients with early CR died due to disease progression. CONCLUSION The incidence of IgD MM is low, the symptoms of early renal damage, blood system damage and bone erosion in IgD MM patients are obvious, and the median survival time is short. ASCT and / or daratumumab may bring lasting relief for IgD MM patients, but large-scale clinical studies are still needed.
Humans ; Multiple Myeloma/therapy* ; Middle Aged ; Male ; Female ; Aged ; Prognosis ; Immunoglobulin D ; Adult ; Retrospective Studies

OBJECTIVE: To explore the significance of the plasma cell percentage and immature plasma cells in the prognosis of patients with multiple myeloma (MM). METHODS: The clinical data of 126 newly diagnosed MM patients in Gansu Provincial Hospital from June 2017 to November 2022 were retrospectively analyzed. The enrolled patients were divided into a higher plasma cell percentage group (group A) and a lower plasma cell percentage group (group B) according to the median plasma cell percentage (33.5%). The clinicopathological data of the two groups were compared, and the effect of plasma cell percentage on the prognosis of MM patients was analyzed using survival curves. On this basis, group A and group B were divided into subgroups with immature plasma cells (A1 group, B1 group) and subgroups without immature plasma cells (A2 group, B2 group), respectively, then the survival curves were used to analyze the effect of immature plasma cells on the prognosis of MM patients. RESULTS: Among the 126 patients with MM, the proportions of patients with ISS stage III, elevated β₂-microglobulin(β₂-MG) level, and immature plasma cells in Group A were significantly higher compared those in Group B ( P =0.015, P =0.028, P =0.010). The median overall survival(OS) and progression-free survival(PFS) of group A were 32 months and 10 months, respectively. The median OS of group B was not reached, and the median PFS was 32 months. The 3-year OS rates of patients in group A and group B were 46.7% and 62.2%, respectively ( P =0.021), and the 3-year PFS were 29.2% and 42.5%, respectively ( P =0.033). There were no significant differences in OS and PFS between group A1 and group A2, or between group B1 and group B2 ( P >0.05). Multivariate COX survival analysis showed that the plasma cell percentage ≥33.5%（HR=1.253, 95%CI : 0.580-2.889, P =0.018）, age ≥65 years (HR=2.206, 95%CI : 1.170-3.510, P =0.012), lactate dehydrogenase(LDH) ≥250 U/L (HR=1.180, 95%CI : 0.621-2.398, P =0.048) and β₂-MG ≥3.5 mg/L (HR=1.507, 95%CI : 0.823-3.657, P =0.036) were independent risk factors affecting OS in MM patients. CONCLUSION MM patients with a higher plasma cell percentage (≥33.5%) at the initial diagnosis have a later disease stage, poorer OS and PFS, compared to the patients with a lower percentage(<33.5%) of plasma cells. The presence or absence of immature plasma cells has no significant impact on the survival of MM patients.
Humans ; Multiple Myeloma/pathology* ; Prognosis ; Plasma Cells/cytology* ; Retrospective Studies ; Male ; Female ; Middle Aged ; Aged ; Bone Marrow

OBJECTIVE: To analyze the clinical characteristics and prognosis of patients with T-cell large granular lymphocytic leukemia (T-LGLL). METHODS: The clinical data of 7 patients with T-LGLL in Gansu Provincial Hospital from March 2016 to June 2023 were analyzed retrospectively. RESULTS: Among the 7 patients, 5 were male and 2 were female, with a median age of 51(28-83) years old. At the onset of illness, 6 cases showed symptoms of fatigue and anemia, 4 cases had enlarged lymph nodes, and 5 cases had splenomegaly. Examination showed that 4 cases were antinuclear antibody(ANA) positive, 5 cases were anemia. The median hemoglobin (Hb) level was 83(61-151) g/L, the median white blood cell count (WBC) was 5.6(2.0-8.7)×10⁹ /L, and the median percentage of lymphocytes in peripheral blood was 66.2(13.9-89.1)%. There were 3 cases with extremely active bone marrow hyperplasia, 2 cases with active hyperplasia, and 2 cases with decreased hyperplasia. There were 5 cases with mild myelofibrosis (MF-1), and 1 case with moderate myelofibrosis (MF-2). The median percentage of T cells was 64.3 (31.5-80.6)%. 5 cases showed the classic immunophenotype (CD3 ⁺ CD4^- CD8 ⁺), 6 cases were CD57 ⁺, 3 cases were TCRα/β ⁺, and 3 cases were TCRγ/δ ⁺. TCRG rearrangement was detected in 5 cases.The median follow-up time was 55(4-87) months, one patient died of heart disease, and the other 6 patients are surviving. CONCLUSION The incidence of T-LGLL is low. The initial symptoms of T-LGLL include anemia, fatigue, lymph node enlargement, splenomegaly, and higher percentage of lymphocytes in peripheral blood, the percentage of abnormal T cells in bone marrow was significantly increased. Analysis of flow cytometric immunophenotyping, TCR gene rearrangement, and hot spot genes such as STAT3 and STAT5b, can improve the diagnostic accuracy.
Humans ; Leukemia, Large Granular Lymphocytic/diagnosis* ; Male ; Middle Aged ; Female ; Aged ; Prognosis ; Adult ; Aged, 80 and over ; Retrospective Studies

BACKGROUND: The clinical impact of post-percutaneous coronary intervention (PCI) quantitative flow ratio (QFR) in patients treated with PCI for chronic total occlusion (CTO) was still undetermined. METHODS: All CTO vessels treated with successful anatomical PCI in patients from PANDA III trial were retrospectively measured for post-PCI QFR. The primary outcome was 2-year vessel-oriented composite endpoints (VOCEs, composite of target vessel-related cardiac death, target vessel-related myocardial infarction, and ischemia-driven target vessel revascularization). Receiver operator characteristic curve analysis was conducted to identify optimal cutoff value of post-PCI QFR for predicting the 2-year VOCEs, and all vessels were stratified by this optimal cutoff value. Cox proportional hazards models were employed to calculate the hazard ratio (HR) with 95% CI. RESULTS: Among 428 CTO vessels treated with PCI, 353 vessels (82.5%) were analyzable for post-PCI QFR. 31 VOCEs (8.7%) occurred at 2 years. Mean value of post-PCI QFR was 0.92 ± 0.13. Receiver operator characteristic curve analysis shown the optimal cutoff value of post-PCI QFR for predicting 2-year VOCEs was 0.91. The incidence of 2-year VOCEs in the vessel with post-PCI QFR < 0.91 (n = 91) was significantly higher compared with the vessels with post-PCI QFR ≥ 0.91 (n = 262) (22.0% vs. 4.2%, HR = 4.98, 95% CI: 2.32-10.70). CONCLUSIONS Higher post-PCI QFR values were associated with improved prognosis in the PCI practice for coronary CTO. Achieving functionally optimal PCI results (post-PCI QFR value ≥ 0.91) tends to get better prognosis for patients with CTO lesions.