Objective To explore the effects of recombinant human growth hormone(rh-GH) on immune function in children with tetralogy of Fallot (TOF) after radical operation. Methods Thirty children with TOF were divided into two groups: conventional (n=20) and rh-GH (n=10, 0.2U/kg rhGH, sc., three times per week for 4 weeks). The immunoglobulin, complement fraction, lymphocyte subsets, and interleukins were determined. Results After treatment, the abnormal elevation of IgG, IgM, C_3, C_4, CD8~+ and CD19~+ in rhGH group decreased significantly at 1 week to 3 weeks; moreover, the abnormal descend of IgA, CD3~+, CD4~+, CD4~+/CD8~+, CD3~+/HLA-DR~+, and CD3~+/CD~ (16+56) in rh-GH group increased significantly at 1 week to 2 weeks compared to those of conventional group; and all recovered to the levels of control at 4 weeks. The interleukin-6 and tumor necrosis factor-? levels in the plasma and supernatant of peripheral blood mononuclearcytes decreased gradually at 1 week to 2 weeks after treatment compared to those of conventional group, and also recovered to the levels of control at 4 weeks. Conclusion rh-GH therapy could significantly improve immune function of children with TOF after radical operation.

Objective To summarize the clinical phenotype profiles and mitochondrial DNA mutation in maternally inherited diabetes and deafness ( MIDD ) , and to improve the diagnosis and treatment of this disease in clinical practice. Methods Sixteen patients with MIDD in six families from Peking Union Medical College from 2007 to Dec 2014 were confirmed as carrying the mitochondrial ( mt) DNA 3243 A to G mutation. Sanger sequencing was used to detect the mt DNA 3243 A to G mutation. The peak height G/A ratio was used to determine mutation heteroplasmy levels. Results The patients with early onset of diabetes (35. 0 ± 14. 6 years), deafness, normal or lower body mass index ( BMI) , and maternal hereditary tendency suggested the diagnosis of MIDD. The peak height G/A ratio was significantly different according to the onset age of MIDD [≤25 years (61. 6 ± 20. 17)%;25-45 years (16.59±8.64)%;>45 years(6.37±0.59)%;P<0.01]. The peak height G/A ratio was negatively correlated with the onset age of MIDD(r=-0. 785,P=0. 001). Conclusion Early onset of diabetes with deafness, normal/lower BMI, and maternal hereditary tendency strongly suggests the diagnosis of MIDD. The peak height G/A ratio might provide a simple prediction regarding the onset age and severity of MIDD.

Chronic kidney disease （CKD） is a global health problem， and its incidence increases year by year. Studies have revealed that the progression of CKD into end-stage renal disease （ESRD） is related to its inability to effectively eliminate toxins due to decreased renal function. Additionally， intestinal microflora produces a large amount of gut-derived uremic toxins （GDUTs） during protein fermentation. The theory of gut-kidney axis holds that gut and kidney interact with each other， and CKD reduces the ability to remove uremic toxins （UTs）， resulting in the accumulation of UTs in the blood. The accumulation of UTs also accelerates the deterioration of renal function， leading to a vicious circle. This paper focused on the sources of indoxyl sulfate and p-cresol sulfate in GDUTs and their mechanisms against CKD （such as inducing renal tubular cell death， oxidative stress and endothelial injury， promoting renal fibrosis and down-regulating renal protective protein） as well as the sources of trimethylamine oxide and its mechanisms against CKD （such as promoting renal fibrosis and inflammation）. Moreover， starting from gut-kidney axis， this paper summarized the ways of diet and nutrition regulation， toxin adsorption， enhanced dialysis to increase the clearance， inhibiting the sources of gut-derived toxins and traditional Chinese medicine （TCM） therapy （TCM preparations and TCM active ingredients） to regulate intestinal microecology and reduce the generation of GDUTs， aiming to provide new therapeutic ideas for delaying the progression of CKD.

Tumor-associated tertiary lymphoid structures(TLSs)are ectopic lymphoid formations within tumor tissue,with mainly B and T cell populations forming the organic aggregates.The presence of TLSs in tumors has been strongly associated with patient responsiveness to immunotherapy regimens and improving tumor prognosis.Researchers have been motivated to actively explore TLSs due to their bright clinical application prospects.Various studies have attempted to decipher TLSs regarding their formation mechanism,structural composition,induction generation,predictive markers,and clinical utilization.Meanwhile,the scientific approaches to qualitative and quantitative descriptions are crucial for TLS studies.In terms of detection,hematoxylin and eosin(H&E),multiplex immunohistochemistry(mIHC),multiplex immunofluorescence(mIF),and 12-chemokine gene signature have been the top approved methods.However,no standard methods exist for the quantitative analysis of TLSs,such as absolute TLS count,analysis of TLS constituent cells,structural features,TLS spatial location,density,and maturity.This study reviews the latest research progress on TLS detection and quantification,proposes new directions for TLS assessment,and addresses issues for the quantitative application of TLSs in the clinic.

Objective:To investigate the incidence and prothrombotic risk factors of postoperative venous thromboembolism(VTE) in Cushing′s disease and to further develop an assessment model to identify those at high risk of postoperative VTE events.Methods:A retrospective study was performed in 82 patients who were admitted to Huashan Hospital, Fudan University during January 2019 and January 2020 and diagnosed with Cushing′s disease. These patients underwent the evaluation about their clinical, hormonal, and coagulation parameters, as well as ultrasonography and pulmonary angio-CT when necessary. The least absolute shrinkage and selection operator(LASSO) regression analysis was used to screen independent risk factors, and a nomogram model for postsurgical VTE risk assessment in Cushing′s disease was initially established, and Bootstrap method was used for internal verification. Finally, the predictive model was evaluated for calibration and clinical applicability in the study cohort.Results:Nineteen patients(23.17%) developed VTE events, with 14 cases occurring after endoscopic transsphenoidal surgery. Compared to patients without VTE, those in the VTE group were older( P<0.001), had longer postoperative bed rest, higher rates of current infection, higher HbA 1C levels, and more severe glucose tolerance impairment(all P<0.05). Through LASSO regression analysis, two independent risk factors for postoperative VTE were identified: Age and current infection. Then a VTE risk assessment nomogram model was established to predict the patients at high risk of VTE. In the nomogram model for VTE risk assessment, the area under the receiver operating characteristic curve was 0.868(95% CI 0.787-0.949), with the calibration curve closely aligning with the ideal diagonal line and the clinical decision curve exceeding the two extreme curves. Conclusions:Advanced perioperative assessment needs to be taken to screen those with high VTE risks in patients diagnosed with Cushing′s disease. Additionally, during the perioperative period, patients with Cushing′s disease should undergo mandatory physical activity or prophylactic anticoagulant therapy.

Apoptosis ; Autophagy ; Ferroptosis ; Iron/metabolism* ; Sequestosome-1 Protein/metabolism* ; Ubiquitin-Specific Proteases/metabolism*