Objective To investigate the feasibility and safety of bedside transesophageal echocardiography(TEE)-guided atrial septostomy in patients undergoing extracorporeal membrane oxygenation(ECMO)support.Methods Eight patients(2 females and 6 males)who developed pulmonary edema during VA-ECMO treatment between January 2020 and October 2024 were enrolled.All patients underwent bedside TEE-guided atrial septal puncture,followed by gradual balloon dilation using an Inoue balloon(diameter:18-24 mm).The endpoint for dilation was defined as mean left atrial pressure＜15 mmHg.Post-procedure evaluation included assessment of the septal defect size,mean left atrial pressure,and improvement in bilateral pulmonary edema.Results The mean age of all 8 patients was(43.50±16.84)years old.Atrial septostomy was successfully performed in all patients.The mean balloon dilation diameter was(22.75±2.68)mm,resulting in a mean septal defect size of(6.31±0.37)mm.The mean left atrial pressure significantly decreased from(26.63±1.77)mmHg pre-procedure to(12.75±1.28)mmHg post-procedure(P＜0.001).All patients demonstrated marked improvement in bilateral pulmonary edema,and six patients also showed a significant reduction in the severity of mitral regurgitation following the procedure.No procedure-related complications were observed.Conclusions Bedside TEE-guided atrial septostomy is safe and effective for reducing left heart loading during ECMO support and improving pulmonary edema.

Objective To investigate the feasibility and safety of bedside transesophageal echocardiography(TEE)-guided atrial septostomy in patients undergoing extracorporeal membrane oxygenation(ECMO)support.Methods Eight patients(2 females and 6 males)who developed pulmonary edema during VA-ECMO treatment between January 2020 and October 2024 were enrolled.All patients underwent bedside TEE-guided atrial septal puncture,followed by gradual balloon dilation using an Inoue balloon(diameter:18-24 mm).The endpoint for dilation was defined as mean left atrial pressure＜15 mmHg.Post-procedure evaluation included assessment of the septal defect size,mean left atrial pressure,and improvement in bilateral pulmonary edema.Results The mean age of all 8 patients was(43.50±16.84)years old.Atrial septostomy was successfully performed in all patients.The mean balloon dilation diameter was(22.75±2.68)mm,resulting in a mean septal defect size of(6.31±0.37)mm.The mean left atrial pressure significantly decreased from(26.63±1.77)mmHg pre-procedure to(12.75±1.28)mmHg post-procedure(P＜0.001).All patients demonstrated marked improvement in bilateral pulmonary edema,and six patients also showed a significant reduction in the severity of mitral regurgitation following the procedure.No procedure-related complications were observed.Conclusions Bedside TEE-guided atrial septostomy is safe and effective for reducing left heart loading during ECMO support and improving pulmonary edema.

Objective To investigate the distribution and antimicrobial resistance profiles of clinically isolated Haemophilus influenzae and Moraxella catarrhalis in hospitals across China from 2015 to 2021,and provide evidence for rational use of antimicrobial agents.Methods Data of H.influenzae and M.catarrhalis strains isolated from 2015 to 2021 in CHINET program were collected for analysis,and antimicrobial susceptibility testing was performed by disc diffusion method or automated systems according to the uniform protocol of CHINET.The results were interpreted according to the CLSI breakpoints in 2022.Beta-lactamases was detected by using nitrocefin disk.Results From 2015 to 2021,a total of 43 642 strains of Haemophilus species were isolated,accounting for 2.91％of the total clinical isolates and 4.07％of Gram-negative bacteria in CHINET program.Among the 40 437 strains of H.influenzae,66.89％were isolated from children and 33.11％were isolated from adults.More than 90％of the H.influenzae strains were isolated from respiratory tract specimens.The prevalence of β-lactamase was 53.79％in H.influenzae strains.The H.influenzae strains isolated from children showed higher resistance rate than the strains isolated from adults.Overall,779 strains of H.influenzae did not produce β-lactamase but were resistant to ampicillin(BLNAR).Beta-lactamase-producing strains showed significantly higher resistance rates to these antimicrobial agents than the β-lactamase-nonproducing strains.Of the 16 191 M.catarrhalis strains,80.06％were isolated from children and 19.94％isolated from adults.M.catarrhalis strains were mostly susceptible to both amoxicillin-clavulanic acid and cefuroxime,evidenced by resistance rate lower than 2.0％.Conclusions The emergence of antibiotic-resistant H.influenzae due to β-lactamase production poses a challenge for clinical anti-infective treatment.Therefore,it is very important to implement antibiotic resistance surveillance for H.influenzae and guide rational antibiotic use.All local clinical microbiology laboratories should actively improve antibiotic susceptibility testing and strengthen antibiotic resistance surveillance for H.influenzae.

Objective Summarize the endocrine characteristics of 5 children with Wiedemann-Steiner syndrome(WDSTS)to enhance the early recognition capability of clinicians for this condition.Methods A retrospective analysis of the medical history,clinical manifestations,genetic testing,and treatment of 5 children with Wiedemann-Steiner syndrome treated in the endocrinology department of capital center for children's health,capital medical university from October 2020 to December 2024,summarizing the clinical characteristics of the disease.Results Among the 5 cases of WDSTS,there were 2 males and 3 females,with ages ranging from 1.9 to 10 years at the time of diagnosis.Cases 1 and 3 were born prematurely,and cases 1,2 and 5 were diagnosed as small for gestational age(SGA)infants.All 5 children exhibited distinctive facial features,hirsutism,sacral dimple,developmental delay,and adenoid hypertrophy,premature tooth replacement,feeding difficulties,and a high incidence of urinary system abnormalities.Four children complained of slow growth.Cases 1 and 4 were diagnosed with short stature.Cases 3 and 5 had normal height but were slightly shorter with advanced bone age.Case 5 also experienced early puberty.Case 2 was diagnosed with premature adrenarche and idiopathic central precocious puberty due to the presence of pubic hair and breast enlargement.Cases 1 and 3 were treated with recombinant human growth hormone(rhGH),which resulted in significant height growth(approximately 10 cm/year).However,treatment was discontinued in Case 1 due to a significant advancement in bone age.Conclusions WDSTS should be considered in patients presenting with short stature,distinctive facial features,hirsutism,developmental delay,and multiple system deformities.While rhGH treatment can help improve height in these patients,the potential for accelerated bone age maturation during treatment requires careful monitoring.

Objective To investigate the mechanism of electroacupuncture-mediated PTEN-induced kinase 1(PINK 1)/Parkin signal pathway activation in treating functional dyspepsia model rats.Methods Thirty-six SD rats were randomly divided into the blank control(n=8)and the modeling groups(n=28)using the random number table method.The functional dyspepsia model was established using the compound etiology modeling method.After successful modeling,24 rats were divided into the model,electroacupuncture,and mosapride groups using the random number table method,with eight rats per group.The blank group was not intervened.The electroacupuncture,mosapride,and model groups were bound for 30 min,and only the electroacupuncture group was administered"Neiguan"(PC 6)and"Gongsun"(SP 4)while bound.The electroacupuncture and model groups were administered the same amount of normal saline,and the mosapride group was given mosapride citrate solution(1.35 mg/kg).The above intervention method were performed once a day for 7 consecutive days to observe the general condition of rats.Gastric emptying and small intestinal propulsion experiments were used to observe gastric emptying rate and small intestine propulsion rate in each group of rats.The interstitial cells of the Cajal(ICC)ultrastructure of gastric antrum were observed under a transmission electron microscope.The protein and mRNA expressions of C-kit proto-oncogene protein(C-kit),microtubule-associated protein 1 light chain 3(LC3)Ⅱ/Ⅰ,ubiquitin-binding protein p62(p62),Bcl-2 interacting protein 1(Beclin1),PINK1,and Parkin were detected using Western blotting and real-time fluorescence PCR.Results Compared with the blank group,the rats in the model group exhibited lethargy,dull and dry fur,sluggish responses,and substantial decreases in food intake and body weight.Their feces alternated between dry and loose consistency.Furthermore,the gastric emptying and small intestinal propulsion rates were reduced(P<0.01).The ultrastructural morphology of ICC and their mitochondria in the gastric antrum tissue showed apparent damage.The protein and mRNA expressions of C-kit and p62 were decreased(P<0.01),whereas those of PINK1,Parkin,Beclin1,and LC3Ⅱ/Ⅰ were increased(P<0.01).Compared with the model group,the rats in the electroacupuncture and mosapride groups showed notable improvements in their mental state,with neat fur,enhanced activity and responsiveness,gradual increases in food intake and body weight,and well-formed feces.Additionally,the gastric emptying and intestinal propulsion rates increased(P<0.01).The ultrastructural morphology of ICC and their mitochondria in the gastric antrum tissue were notably improved.The protein and mRNA expressions of C-kit and p62 were increased(P<0.01),whereas those of PINK1,Parkin,Beclin1,and LC3Ⅱ/Ⅰwere decreased(P<0.01).Conclusion Electroacupuncture improves the gastrointestinal motility of functional dyspepsia rats,and its mechanism may be related to upregulating C-kit and p62 expression,downregulating PINK1,Parkin,Beclin1,and LC3Ⅱ/Ⅰ expression,inhibiting abnormal PINK1/Parkin signal pathway activation,and blocking the excessive mitophagy of ICC.

Arsenic is a semi-metal,and lipid-soluble arsenic compounds are one of the widespread forms in the environment and food chain,but there is a lack of standards for lipid-soluble arsenic compounds,which is one of the bottlenecks in the current analytical detection and toxicological studies of organic arsenic.In this study,four saturated arsenic-containing hydrocarbons,AsHC 318,AsHC 332,AsHC 346,and AsHC 374(The number is relative molecular mass),were successfully synthesized in three steps by using dimethylarsinic acid,potassium iodide,sodium hydroxide,and four brominated alkanes(1-Bromotetradecane,1-bromopentadecane,1-bromohexadecane,and 1-bromooctadecane)as raw materials.The structures of these four saturated arsenic-containing hydrocarbons were characterized by proton nuclear magnetic resonance(1H NMR)spectroscopy,13C nuclear magnetic resonance(13C NMR)spectroscopy,and high-resolution mass spectrometry(HR-MS).The yields of the method were 8%-10%,and the synthesized compounds could be used in subsequent toxicity evaluation experiments to assess the toxic effects and mechanisms of action of arsenic-containing hydrocarbons.This study provided an effective method for synthesis of arsenic-containing hydrocarbons,enriching the synthesis methods of arsenic-containing hydrocarbons,and provided raw materials for the subsequent toxicological studies of arsenic-containing hydrocarbons.

Childhood primary renal tubular diseases are chronic kidney diseases characterized by impaired renal tubular reabsorption. Primary renal tubular disease has diverse clinical manifestations and lacks of specificity. Laboratory tests are limited，making it prone to missed diagnosis and misdiagnosis. Based on the current knowledge of renal tubular diseases，authors propose early warning signals of renal tubular diseases such as family history of primary tubular diseases，unexplained polyhydramnios during pregnancy，polydipsia，polyuria，delayed growth and development or rickets，decreased muscle strength and tone，unexplained electrolyte disturbance，hyperuricemia，acid-base disturbance，positive urine sugar test，renal tubular proteinuria，urinary imaging examination suggesting kidney stones，calcium deposition，renal cysts and early onset of eye，ear，joint and neuron injury.Meanwhile，some universal management strategies for primary renal tubular disease are proposed，emphasizing the importance of multidisciplinary collaboration，genetic testing and individualized intervention to improve the long-term prognosis of childhood primary renal tubular diseases.

The paper reports a 32-year-old female acute myeloid leukemia patient who developed graft-versus-host disease after paternal hematopoietic stem cell transplantation, which subsequently led to renal thrombotic microangiopathy. She subsequently required a kidney transplant from the same donor 5 years later due to renal failure. Considering that both the bone marrow and kidney were from the same donor and the recovery of renal function was favorable, immunosuppressive therapy was discontinued after a short course of anti-rejection treatment, with maintained stable kidney function. This case suggests that under the condition of high chimerism, allogeneic hematopoietic stem cell transplantation and kidney transplantation from the same donor can achieve immune tolerance, potentially improving solid organ transplantation success rate. The findings provide a novel therapeutic approach for solid organ transplantation following allogeneic hematopoietic stem cell transplantation.

Objective:To analyze the clinical and genetic characteristics of children diagnosed with Noonan-syndrome associated with loose anagen hair (NS-LAH).Methods:A retrospective analysis was conducted on the clinical data of 5 children diagnosed with NS-LAH by the Endocrinology Department of the Capital Institute of Pediatrics from January 2018 to June 2024. This analysis encompassed the patients′ demographic information, clinical manifestations, distinguishing features, treatment regimens, and prognostic outcomes to elucidate their clinical characteristics. Additionally, whole-exome sequencing and Sanger sequencing were utilized to investigate the genetic etiology within the families, and the identified variations were interpreted according to the guidelines of the American College of Medical Genetics and Genomics.Results:Among the 5 NS-LAH patients, there were 3 boys and 2 girls, with ages at diagnosis ranging from 2.3 to 7.7 years old. All patients presented with short stature as a primary complaint. Birth histories were generally unremarkable, though case 2 and 5 of macrosomia were noted. In addition to the characteristic facial features of Noonan syndrome, short stature, and varying degrees of intellectual and motor developmental delay, all 5 patients exhibited sparse hair that was easily shed, as well as enlarged head circumferences. Four patients showed structural cardiac abnormalities, which included a case of hypertrophic cardiomyopathy, 2 cases of atrial septal defect, and 1 case of patent foramen ovale. Genetic analysis revealed heterozygous missense variantion in SHOC2 gene in 4 patients, comprising 3 cases with c.4A>G (p.S2G) and one case with c.519G>C (p.M173I). Additionally, one patient was found to have a heterozygous missense variantion c.146C>G (p.P49R) in PPP1CB gene. Three children were diagnosed with growth hormone deficiency and treated with growth hormone for 1.7, 2.7 and 0.5 years. This resulted in significant improvements in height, with annual increases of 11.8, 8.4 and 13.0 cm, respectively. Among the 4 patients with SHOC2 variantions, 2 developed systemic lupus erythematosus and 1 exhibited symptoms of arthritis.Conclusions:Growth failure is the primary complaint in patients with NS-LAH. Key characteristic findings include enlarged head circumference and sparse, loose hair. Growth hormone deficiency is commonly associated with NS-LAH, and growth hormone therapy is generally effective. Furthermore, patients carrying the classic variantion in SHOC2 (c.4A>G) may have an increased risk of developing autoimmune diseases.

Turning to critical illness is a common stage of various diseases and injuries before death. Patients usually have complex health conditions, while the treatment process involves a wide range of content, along with high requirements for doctor′s professionalism and multi-specialty teamwork, as well as a great demand for time-sensitive treatments. However, this is not matched with critical care professionals and the current state of medical care in China. Telemedicine, which shortens the distance of medical professionals and the gap of disease diagnosis and treatments in various regions through electronic information, can effectively solve the current problem. Therefore, there is an urgent need to develop a standardized, high-quality visualization telemedicine round system .Therefore, experts have been organized to search domestic and foreign literature on telemedicine round for critically ill patients and to form this consensus based on clinical experiences so as to further improve the level of critical care treatments in regions.