[Objective] To explore the clinical features of IgG4-related urinary diseases so as to provide reference for the diagnosis and treatment of such diseases. [Methods] The clinical data of 4 cases of IgG4-related urinary system diseases diagnosed and treated in Xijing Hospital of Air Force Medical University during Aug.2019 and Dec.2023 were retrospectively collected.Here, we report on the diagnosis and treatment of these patients, analysing their symptoms, serology, imaging and pathology as well as their treatment and outcomes. [Results] The patients included 2 male and 2 female.The lesions were involved with the retroperitoneum and urinary system.Three patients had symptoms of lumbar pain.The imaging manifestations were complex, including retroperitoneal mass involving urinary system organs in 2 cases, tabdense shadow of the right kidney in 1 case, and simple cystic mass of kidney in 1 case.Serum IgG4 value was not detected before surgery.All patients underwent radical surgical treatment.Postoperative pathology showed fibrous tissue hyperplasia with a large number of plasma cells, lymphocytes, a few neutrophil infiltrates, and lymphoid follicles and obliterated vasculitis in some specimens.The number of IgG4⁺ plasma cells was more than 10 in all tissues under high power microscope.After surgery, 3 patients had symptoms improved, and serum IgG4 value was within the normal range; 1 patient (patem 3) had elevated IgG4 value during follow-up, received subsequent hormone therapy, and the serum IgG 4 level remained stable. [Conclusion] The symptoms of IgG4-related diseases involving the urinary system are non-specific, and the imaging findings are various, easily confused with other diseases.Early detection of serum IgG4 and biopsy pathology can help clinicians make correct diagnosis in the early stage.

Objective To investigate the impact of two different surgical methods, orthotopic kidney transplantation and abdominal heterotopic kidney transplantation, on the surgical outcomes of pig-to-pig kidney transplantation and the short-term survival of recipient pigs after surgery. Methods Twenty-four Bama miniature pigs were divided into two groups, with 12 pigs in each group, and underwent orthotopic kidney transplantation and abdominal heterotopic kidney transplantation, respectively. The perioperative indicators of the recipient pigs, renal blood perfusion, the overall incidence rate of complications and survival rate were compared between the two surgical methods. Results The total surgical time, renal artery anastomosis time, renal vein anastomosis time, cold ischemia time and total ischemia time were all shorter in the abdominal heterotopic kidney transplantation group than in the orthotopic kidney transplantation group, with statistically significant differences (all P<0.05). The number of satisfactory renal perfusion cases was higher in the abdominal heterotopic kidney transplantation group than in the orthotopic kidney transplantation group (83% vs. 75%), but the difference was not statistically significant (P>0.05). The total incidence of postoperative complications was 33% in the heterotopic kidney transplantation group, with a survival rate of 92%, and the cause of death was rupture of the vascular anastomosis. The total incidence of postoperative complications was 50% in the orthotopic kidney transplantation group, with a survival rate of 83%, and the causes of death were renal vein thrombosis and renal artery thrombosis. There were no statistically significant differences in the total incidence of postoperative complications and survival rates between the two groups (all P>0.05). Conclusions Compared with orthotopic kidney transplantation, abdominal heterotopic kidney transplantation showes better surgical outcomes in pig-to-pig kidney transplantation and is more beneficial for the short-term survival of recipient pigs after surgery. This provides experience for improving the stability of pig-to-non-human primate kidney xenotransplantation models in the future.

Organ shortage has become one of the major challenges hindering the development of organ transplantation. Xenotransplantation is one of the most valuable methods to resolve global organ shortage. In recent years, the development of genetic engineering technique and research and development of new immunosuppressant have provided novel theoretical basis for xenotransplantation. International scholars have successively carried out researches on xenotransplantation in genetically modified pigs to non-human primates or brain death recipients, making certain substantial progresses. However, most of the researches are still in the preclinical stage, far from clinical application. Therefore, according to the latest preclinical experimental research progress at home and abroad, the history of xenotransplantation, the development of gene modification technology, xenotransplantation rejection and immunosuppression regimens were reviewed, aiming to provide reference for subsequent research of xenotransplantation, promote clinical application of xenotransplantation and bring benefits to more patients with end-stage diseases.

Objective:To analyze the expression of MXD1 and Ki-67 in breast invasive ductal carcinoma (IDC) and their relationship with clinicopathologic features.Methods:Clinical data of 68 patients with breast invasive ductal carcinoma admitted to Shanxi Maternal and Child Health Hospital and Shanxi Cancer Hospital from Mar. 2022 to Mar. 2023 were retrospectively analyzed,and the expressions of MXD1 and Ki67 in cancer tissues and adjacent normal tissues of patients with breast invasive ductal carcinoma were measured. The expressions of MXD1 and Ki67 in cancer tissues of patients with different clinicopathological characteristics were analyzed. The relationship between the expression of MXD1 and Ki67 and clinicopathological features; Follow-up until March 2024 was conducted to analyze the survival of different expressions of MXD1 and Ki67.Results:The high expression of MXD1 and positive Ki67 in cancer tissues of 68 patients were 63.24% and 69.12%, respectively, which were significantly higher than 8.82% and 11.76% in normal tissues ( χ2=43.67, 46.43, P<0.05). The proportion of high expression of MXD1 and Ki67 positive in histological grade Ⅲ, low differentiation, TNM stage Ⅲ or Ⅳ was significantly higher than that in histological grade Ⅰ or Ⅱ, middle and high differentiation, TNM stage Ⅰ or Ⅱ ( χ2=4.05, 5.87, 6.00, 4.00, 5.78, 5.39, P<0.05). The expressions of MXD1 and Ki67 were positively correlated with histological grade and TNM stage, and inversely correlated with differentiation degree ( r=0.34, 0.39, 0.39, 0.38, -0.39, -0.34, P<0.05). The survival curves of different expressions of MXD1 and Ki67 were statistically significant (Log-Rank χ2=10.13,10.72, P<0.05). At the end of follow-up,the median survival time of low and high expression of MXD1 was 17 months (95% CI: 15.059-18.941) and 14 months (95% CI: 12.452-15.548), respectively, and the median survival time of negative and positive expression of Ki67 was 19 months (95% CI: 13.335-24.665), 14 months (95% CI: 12.432-15.568) . Conclusion:MXD1 is highly expressed and Ki67 positiverate is higher in patients with breast invasive ductal carcinoma, which is related to histological grade, TNM stage, differentiation degree and survival time.

Objective:To retrospectively analyze the treatment status of tyrosine kinase inhibitors (TKI) in newly diagnosed patients with chronic myeloid leukemia (CML) in China.Methods:Data of chronic phase (CP) and accelerated phase (AP) CML patients diagnosed from January 2006 to December 2022 from 77 centers, ≥18 years old, and receiving initial imatinib, nilotinib, dasatinib or flumatinib-therapy within 6 months after diagnosis in China with complete data were retrospectively interrogated. The choice of initial TKI, current TKI medications, treatment switch and reasons, treatment responses and outcomes as well as the variables associated with them were analyzed.Results:6 893 patients in CP ( n=6 453, 93.6%) or AP ( n=440, 6.4%) receiving initial imatinib ( n=4 906, 71.2%), nilotinib ( n=1 157, 16.8%), dasatinib ( n=298, 4.3%) or flumatinib ( n=532, 7.2%) -therapy. With the median follow-up of 43 ( IQR 22-75) months, 1 581 (22.9%) patients switched TKI due to resistance ( n=1 055, 15.3%), intolerance ( n=248, 3.6%), pursuit of better efficacy ( n=168, 2.4%), economic or other reasons ( n=110, 1.6%). The frequency of switching TKI in AP patients was significantly-higher than that in CP patients (44.1% vs 21.5%, P<0.001), and more AP patients switched TKI due to resistance than CP patients (75.3% vs 66.1%, P=0.011). Multi-variable analyses showed that male, lower HGB concentration and ELTS intermediate/high-risk cohort were associated with lower cytogenetic and molecular responses rate and poor outcomes in CP patients; higher WBC count and initial the second-generation TKI treatment, the higher response rates; Ph + ACA at diagnosis, poor PFS. However, Sokal intermediate/high-risk cohort was only significantly-associated with lower CCyR and MMR rates and the poor PFS. Lower HGB concentration and larger spleen size were significantly-associated with the lower cytogenetic and molecular response rates in AP patients; initial the second-generation TKI treatment, the higher treatment response rates; lower PLT count, higher blasts and Ph + ACA, poorer TFS; Ph + ACA, poorer OS. Conclusion:At present, the vast majority of newly-diagnosed CML-CP or AP patients could benefit from TKI treatment in the long term with the good treatment responses and survival outcomes.

Periarticular fracture of the shoulder is a common type of fractures in the elderly. Postoperative adverse events such as internal fixation failure, humeral head ischemic necrosis and upper limb dysfunction occur frequently, which seriously endangers the exercise and health of the elderly. Compared with the fracture with normal bone mass, the osteoporotic periarticular fracture of the shoulder is complicated with slow healing and poor rehabilitation, so the clinical management becomes more difficult. At present, there is no targeted guideline or consensus for this type of fracture in China. In such context, experts from Youth Osteoporosis Group of Chinese Orthopedic Association, Orthopedic Expert Committee of Geriatrics Branch of Chinese Association of Gerontology and Geriatrics, Osteoporosis Group of Youth Committee of Chinese Association of Orthopedic Surgeons and Osteoporosis Committee of Shanghai Association of Chinese Integrative Medicine developed the Chinese expert consensus on the diagnosis and treatment of osteoporotic periarticular fracture of the shoulder in the elderly ( version 2023). Nine recommendations were put forward from the aspects of diagnosis, treatment strategies and rehabilitation of osteoporotic periarticular fracture of the shoulder, hoping to promote the standardized, systematic and personalized diagnosis and treatment concept and improve functional outcomes and quality of life in elderly patients with osteoporotic periarticular fracture of the shoulder.

Chronic refractory wound (CRW) is one of the most challengeable issues in clinic due to complex pathogenesis, long course of disease and poor prognosis. Experts need to conduct systematic summary for the diagnosis and treatment of CRW due to complex pathogenesis and poor prognosis, and standard guidelines for the diagnosis and treatment of CRW should be created. The Guideline forthe diagnosis and treatment of chronic refractory wounds in orthopedic trauma patients ( version 2023) was created by the expert group organized by the Chinese Association of Orthopedic Surgeons, Chinese Orthopedic Association, Chinese Society of Traumatology, and Trauma Orthopedics and Multiple Traumatology Group of Emergency Resuscitation Committee of Chinese Medical Doctor Association after the clinical problems were chosen based on demand-driven principles and principles of evidence-based medicine. The guideline systematically elaborated CRW from aspects of the epidemiology, diagnosis, treatment, postoperative management, complication prevention and comorbidity management, and rehabilitation and health education, and 9 recommendations were finally proposed to provide a reliable clinical reference for the diagnosis and treatment of CRW.

Objective:To investigate the application effects of nitrous oxide and oxygen mixed inhalation technology on analgesia and sedation during debridement and dressing change in children with moderate or severe burns.Methods:A retrospective non-randomized contemporary controlled study was conducted. From December 2019 to November 2021, 140 burn children with moderate or severe burns, aged 1 to 3 years, who met the inclusion criteria were admitted to Central Hospital Affiliated to Shandong First Medical University. During debridement and dressing change 3 to 14 days after injury, 42 children, including 23 males and 19 females, who received nurse-centered pain management mode and analgesia and sedation with nitrous oxide and oxygen mixed inhalation technology were included in nitrous oxide group (the dressing change process using the above-mentioned technology for the first time was selected for the follow-up study). Another 42 children, including 24 males and 18 females, were included in non-nitrous oxide group from 98 children who did not apply analgesia or sedation treatment during dressing change with stratified random sampling (one dressing change process was randomly selected for the follow-up study). The face, legs, activity, cry, and consolability scale and Ramsay sedation scale were used to evaluate the pain intensity and degree of sedation, respectively, at 30 minutes before dressing change (hereinafter referred to as before dressing change), immediately after debridement, and at 30 minutes after finishing dressing change (hereinafter referred to as after dressing change). After dressing change, the self-made satisfaction scale was used to evaluate the satisfaction degree of dressing change surgeons and guardians of children for analgesic effects during dressing change. The duration of dressing change and the healing time of deep partial-thickness burn wounds were recorded. The heart rate and percutaneous arterial oxygen saturation (SpO 2) before, during, and after dressing change and the occurrence of adverse events such as nausea and vomiting during dressing change were recorded. Data were statistically analyzed with Mann-Whitney U test, chi-square test, analysis of variance for repeated measurement, independent sample t test, and Bonferroni correction. Results:There were no significant differences in the score of pain intensity and score of sedation degree between children in two groups before and after dressing change ( P>0.05). Immediately after debridement, the score of pain intensity of children in nitrous oxide group was 2.5±0.7, which was significantly lower than 7.6±1.0 in non-nitrous oxide group ( t=-26.69, P<0.05); the score of sedation degree of children in nitrous oxide group was 1.83±0.38, which was significantly higher than 1.21±0.42 in non-nitrous oxide group ( t=7.15, P<0.05). After dressing change, the satisfaction degree scores of dressing change surgeons and guardians of children for analgesic effects during dressing change of children in nitrous oxide group were significantly higher than those in non-nitrous oxide group (with t values of 10.53 and 2.24, respectively, P<0.05). The dressing change duration of children in nitrous oxide group was significantly shorter than that in non-nitrous oxide group ( t=-5.33, P<0.05). The healing time of deep partial-thickness burn wounds in children between the two groups had no significant difference ( P>0.05). The heart rate of children in nitrous oxide group was significantly lower than that in non-nitrous oxide group during dressing change ( t=-12.40, P<0.05), while the SpO 2 was significantly higher than that in non-nitrous oxide group ( t=5.98, P<0.05). During dressing change, 2 children had nausea and 1 child had euphoria in nitrous oxide group, while heart rate of all children in non-nitrous oxide group continued to be higher than the normal range. Conclusions:In the process of debridement and dressing change in children with moderate or severe burns, the use of nurse-centered pain management mode and the standardized use of nitrous oxide and oxygen mixed inhalation technology can safely and effectively control pain and sedation.

Adventitia ; Atherosclerosis/genetics* ; Cell Communication ; Humans ; Hyperhomocysteinemia/genetics* ; Sequence Analysis, RNA

Objective:To retrospectively analyze the clinical and genetic features of PCDH19 gene mutation related epilepsy in 11 families. Methods:The clinical manifestations and genetic mutation characteristics of 13 children (from 11 families) diagnosed with PCDH19 gene mutation related epilepsy at the Department of Pediatric Neurology, the Third Affiliated Hospital of Zhengzhou University from March 2013 to July 2019 were analyzed. Results:(1) The results of PCDH19 gene mutations: among 11 probands, 10 children had point mutations of PCDH19 gene and one child was with Exon 5 deletion.One male patient was detected with mosaic PCDH19 mutation, which was c. 840C > A, and the proportion of variation was 34.27%.Five hereditary and 6 de novo mutations were identified in 11 probands.Three patients inherited mutations from their clinically asymptomatic fathers with hemizygous mutation.Two patients inherited from their mothers, 1 case was diagnosed with epilepsy and the other was asymptomatic carrier.(2) Clinical features: there were 12 females and 1 male in the enrolled 13 children, with the age of onset of less than 2 years old.The clinical phenotypes: epilepsy with mental retardation in 9 patients, which including 3 patients with Dravet syndrome, and the remaining 4 patients were epilepsy without mental retardation.The phenotypic heterogeneity was observed in females with identical mutations from the same family, and a few girls can be asymptomatic.In all patients, seizures in clusters were observed in all 13 cases, fever sensitivity in 12 cases, and status epilepticus was only found in 3 cases.Of all the patients, only 2 cases had no seizures for more than 2 years, 3 cases with Dravet syndrome were given 6 to 8 kinds of antiepileptic drugs successively, but there were still frequent seizures. Conclusions:Most patients with PCDH19 mutations-related epilepsy are females, while rare mosaic males can be affected, phenotypic heterogeneity is obvious.Seizures in clusters and fever sensitivity are the major clinical features, and most patients are companied with different levels of intellectual impairment.Mutations in PCDH19 can be inherited or de novo, most of which are point mutations.