Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Objective To study anatomical structures of the maxillary second molars(MSM)roots and canals and the relationship between root apices and the maxillary sinus.Methods Cone beam computed tomography imaging data of 526 MSM from 263 patients were evaluated retrospectively.The patient age,number of roots and canals,number of fused roots,type of root canal curvature,and anatomical relationship to the sinus floor were all documented and analyzed.Results It was found that the majority of MSM had three roots(59.1%),and the incidence of three root canals with three roots was the highest(47.3%).Fused roots were found in 192(36.5%)of the teeth examined.Palatal root canals of two-root,two-canal,and three-root,three-canal teeth had more curvature in the buccolingual direction than in the mesiodistal direction.The mesial buccal roots of MSM with three roots tended to extend into the max-illary sinus and the palatine roots of the three-rooted MSM tended to separate from the maxillary sinus.The distance between MSM and the sinus floor increased with age(P＜0.05).Conclusion The root and canal shape of MSM varies widely among a sample of individu-als.

Objective:To explore the risk factors of postpartum hemorrhage in parturients undergoing vaginal delivery with a second child and establish a risk prediction model.Methods:Using the convenient sampling method, a total of 2 500 parturients undergoing vaginal delivery with a second child who underwent regular prenatal examinations at Peking University Third Hospital from July 2019 to March 2023 were selected as the research objects. According to the amount of blood loss, parturients with blood loss greater than or equal to 500 ml within 24 hours after delivery were selected as the case group ( n=278), while parturients with blood loss less than 500 ml within 24 hours after delivery in the same period were selected as the control group ( n=2 222). Univariate analysis and binomial Logistic regression were used to analyze the influencing factors of postpartum hemorrhage in parturients with vaginal delivery with a second child. Based on the selected risk factors, a nomogram prediction model was established using R software, and the consistency of the model was tested. Results:The incidence of postpartum hemorrhage in 2 500 parturients undergoing vaginal delivery with a second child was 11.12% (278/2 500). Binomial Logistic regression analysis showed that in in vitro fertilization-embryo transfer, pre-delivery body mass index, lateral perineal incision, neonatal weight, placenta previa, placenta implantation and manual extraction of placenta were the influencing factors for postpartum hemorrhage in parturients undergoing vaginal delivery with a second child ( P<0.05). According to the influencing factors, a nomogram model was established to predict the probability of postpartum hemorrhage in women who gave birth to a second child. The C- index of the prediction model was 0.706, the area under the receiver operating characteristic curve was 0.706, and the χ 2 value of the Hosmer-Lemeshow goodness-of-fit test was 7.720 ( P=0.461) . Conclusions:In vitro fertilization embryo transfer, pre-delivery body mass index, perineal lateral resection, neonatal weight, placenta previa, placental implantation and manual extraction of placenta are risk factors for postpartum hemorrhage in parturients undergoing vaginal delivery for a second child. The prediction model constructed based on risk factors has certain accuracy and clinical value for predicting postpartum hemorrhage in parturients with vaginal delivery of a second child.

Polycystic ovary syndrome (PCOS) is the most common endocrine dysfunction in women of childbearing age. It is considered to be a heterogeneous, multi-faceted disease with polycystic ovarian changes, hyperandrogenism, and endocrine. Clinical features such as dysfunction and metabolic syndrome, due to the presence of ovulation dysfunction, seriously affect the fertility of women with PCOS. With the increase of age, the fertility decline of females is mainly related to factors such as the decrease in the number and quality of follicles, the decrease in the number of available embryos, the decrease in transplant rate and the increase in abortion rate. The existing literature does not agree on the fertility window of elderly PCOS patients. This paper reviews several literatures and discusses the reproductive window problems of elderly PCOS patients.

Objective:To investigate the predictive value of peritoneal protein clearance (Pcl) for cardiovascular events and cardiovascular mortality in peritoneal dialysis (PD) patients.Methods:Eligible PD patients were prospectively enrolled from January 2014 to April 2015 in the PD Center of Renji Hospital, School of Medicine, Shanghai Jiao Tong University. All patients were followed up until death, withdrawing from PD, transferring to other centers, or the end of study period (October 1, 2018). The patients were divided into high Pcl group and low Pcl group by the median Pcl, and the differences of related indicators between the two groups were compared. A multiple linear regression model was used to analyze the influencing factors of Pcl. The Kaplan-Meier method and Log-rank test were used to compare the cumulative survival rates of patients between the two groups. A multivariate Cox regression model was used to estimate the risk of cardiovascular events and cardiovascular mortality in relation to Pcl in PD patients.Results:A total of 271 patients were enrolled, with 135 males (49.8%), age of (56.92±0.84) years old and a median PD duration of 38.77(19.00, 63.10) months. There were 70 patients (25.8%) comorbiding with diabetes and 81 patients (29.9%) with cardiovascular diseases (CVD). The median Pcl of this cohort was 67.93(52.31, 88.36) ml/d. Compared with the low Pcl group (Pcl<67.93 ml/d), the high Pcl group (Pcl≥67.93 ml/d) had older age, and greater proportion of CVD, body mass index (BMI), pulse pressure, brain natriuretic peptide, mass transfer area coefficient of creatinine (MTACcr), and lower serum albumin (all P<0.05). There was no significant difference in gender, dialysis duration, proportion of diabetes, proportion of angiotensin converting enzyme inhibitor and angiotensin receptor blocker, proportion of continuous ambulatory PD, high sensitivity C reactive protein, fluid removal including 24 h urine volume and 24 h ultrafiltration, and residual renal function between the two groups (all P>0.05). Multiple linear regression analysis showed that serum albumin ( β=-0.388, P<0.001), BMI ( β=0.189, P<0.001), and MTACcr ( β=0.247, P<0.001) were independently related to lg(Pcl). During the study period, 55 patients experienced one or more cardiovascular events and 39 patients had cardiovascular mortality. According to Kaplan-Meier analysis, cardiovascular mortality in the high Pcl group was higher than that of low Pcl group (Log-rank χ2=6.902, P=0.009). Multivariate Cox regression analysis showed that, high lg(Pcl) was an independent influencing factor of cardiovascular events in PD patients ( HR=7.654, 95% CI 1.676-34.945, P=0.009). Conclusions:Serum albumin, BMI and MTACcr are independently associated with Pcl, and Pcl is an independent predictor of cardiovascular events in PD patients.

BACKGROUND: Perioperative treatment has become an increasingly important aspect of the management of patients with non-small cell lung cancer (NSCLC). Small-scale clinical studies performed in recent years have shown improvements in the major pathological remission rate after neoadjuvant therapy, suggesting that it will soon become an important part of NSCLC treatment. Nevertheless, neoadjuvant immunotherapy may be accompanied by serious adverse reactions that lead to delay or cancelation of surgery, additional illness, and even death, and have therefore attracted much attention. The purpose of the clinical recommendations is to form a diagnosis and treatment plan suitable for the current domestic medical situation for the immune-related adverse event (irAE). METHODS: This recommendation is composed of experts in thoracic surgery, oncologists, thoracic medicine and irAE related departments (gastroenterology, respirology, cardiology, infectious medicine, hematology, endocrinology, rheumatology, neurology, dermatology, emergency section) to jointly complete the formulation. Experts make full reference to the irAE guidelines, large-scale clinical research data published by thoracic surgery, and the clinical experience of domestic doctors and publicly published cases, and repeated discussions in multiple disciplines to form this recommendation for perioperative irAE. RESULTS: This clinical recommendation covers the whole process of prevention, evaluation, examination, treatment and monitoring related to irAE, so as to guide the clinical work comprehensively and effectively. CONCLUSIONS Perioperative irAE management is an important part of immune perioperative treatment of lung cancer. With the continuous development of immune perioperative treatment, more research is needed in the future to optimize the diagnosis and treatment of perioperative irAE.

Objective:To explore the association of abdominal aortic calcification score (AACS) with cardiovascular disease (CVD) outcomes in peritoneal dialysis (PD) patients.Methods:The patients who underwent regular PD at Renji Hospital between July 2011 and July 2014 were recruited and prospectively followed up until the end of the study (August 31, 2018), death, or dropout PD. Abdomen lateral X-ray was used to determine AACS for each patient at enrollment. Patients were divided into three groups based on the tertiles of AACS: non-calcified group, AACS group (AACS=0), mild-moderate calcification group AACS group (0

Objective To investigate the role of BMP-2/Smad signaling pathway in the osteogenic differentiation of human aortic smooth muscle cells (HASMCs) caused by hyperphosphatemia -induced calcium phosphate (CaP) crystals.Methods High-phosphate medium was incubated at 37℃ for 3 days.CaP crystals and supernatant were isolated by ultracentrifugation.Scanning electron microscope and energy dispersive X-ray spectroscopy were performed for analysis of physicochemical characteristics of CaP crystals.HASMCs were cultured in vitro,and divided into high-phosphate,control,crystals and supernatant groups.Calcification was visualized by Alizarin red staining.Calcium loads in cells were quantified by o-cresolphthalein complexone method.Protein expression of bone morphogenetic protein-2 (BMP-2),Runt-related transcription factor 2 (RUNX2),osteopontin (OPN),phospho-Smad1/5/9 (p-Smad1/5/9) were quantified by Western blotting.After knockdowns of BMP-2 and Smad1 with small hairpin RNA (shRNA) interfering respectively in HASMCs,protein expressions were measured by Western blotting.Results High-phosphate medium induced the formation of CaP crystals.Compared with the cells in control group,CaP crystals significantly induced HASMCs calcification,increased calcium loads and up-regulated the levels of BMP-2,RUNX2 and OPN proteins (all P ＜ 0.05).After the addition of CaP crystals into HASMCs,the level of p-Smad 1/5/9 protein peaked at 30 min (P ＜ 0.05).After BMP-2 was knocked down in HASMCs,the expression of p-Smad1 caused by CaP crystals was blocked completely,and the expressions of RUNX2 and OPN caused by CaP crystals were reduced significantly (all P ＜ 0.05).After Smad1 was knocked down in HASMCs,the expressions of RUNX2 and OPN caused by CaP crystals were decreased significantly (all P ＜ 0.05).Conclusions Hyperphosphatemia-induced CaP crystals promoted osteogenic differentiation of HASMCs through the BMP-2/Smad signaling pathway.

Adult ; Aged ; Coma ; physiopathology ; Consciousness ; physiology ; Consciousness Disorders ; physiopathology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Persistent Vegetative State ; physiopathology ; Photic Stimulation ; methods ; Recovery of Function ; physiology ; Treatment Outcome

With the global warming, the incidence of heat stroke was significantly higher than before. Severe heat stroke has a high mortality, high morbidity and consolidated central nervous system injury characteristics. The main features of severe heat stroke cerebral injury include cognitive impairment, delirium, convulsions and coma. Its mechanism is related with heat shock induced cerebral tissue ischemia and hypoxia, vascular dysfunction, secondary cascade inflammation and so on. Currently, the main treatment of heat stroke cerebral injury is the hypothermia therapy, dehydration for the reduction of intracranial pressure, naloxone and other cerebral protection and nutrition treatments. Hyperbaric oxygen therapy (HBOT) is effective in treating brain injury. HBOT can alleviate tissue ischemia and hypoxia, improve circulation, reduce cerebral edema, and anti-inflammatory, anti-oxidative damage, anti-apoptosis and other molecular biological effects. HBOT also play a wake up-promoting effect of nerve repair in the cerebral injury. The treatment of cerebral injury has been the difficulty and weakness of heat stroke research. Therefore, this article reviewed the epidemiology, pathogenesis, the therapeutic effect and mechanism of hyperbaric oxygen on cerebral injury in severe heat stroke to clarify the advantages of HBOT and to provide experimental basis for further research.