OBJECTIVE: To summarize the clinical and genetic characteristics of children with Silver-Russell syndrome (SRS) and improve the recognition of this disease. METHODS: A retrospective analysis was conducted on the clinical manifestations and genetic testing results of 29 children with SRS diagnosed at the Children's Hospital Affiliated to Zhengzhou University between March 2016 and June 2025. RESULTS: The 29 children had included 18 boys and 11 girls, with the age ranging from 2 months to 16 years. Their primary clinical manifestations included postnatal growth retardation (100%), small for gestational age (SGA) (100%), characteristic facial features (90%), limb asymmetry (83%), feeding difficulties (76%), ulnar deviation of the fifth finger (69%), body mass index (BMI) of < -2 SD (62%), and abnormal bone age (55%), including 15 cases with delayed bone age for an average of 1.5 years and 1 case with advanced bone age for 2.5 years. Additional manifestations included abnormal sexual development in 11 cases (38%), dental malocclusion in 11 cases (38%), allergic diseases in 10 cases (34%), cardiac diseases in 9 cases (31%), skeletal abnormalities in 7 cases (24%), renal hypoplasia in 5 cases (17%), and abnormal cranial MRI findings in 5 cases (17%). Twenty children were treated with recombinant human growth hormone (rhGH) at a dose of 0.1 ~ 0.15 U/(kg.d). Among them, 7 cases achieved annual height increase of ≥ 10 cm, 11 cases achieved annual height increase of ≥ 5 ~ 9 cm, and 2 cases achieved annual height increase < 5 cm. Twenty three children exhibited hypomethylation of imprinted genes in the chromosome region of 11p15, 4 presented maternal uniparental disomy of chromosome 7 [UPD(7)mat], and 2 had harbored nonsense variants of the HMGA2 gene. CONCLUSION SRS patients may present with diverse clinical manifestations including postnatal growth retardation, SGA, characteristic facial features, limb asymmetry, feeding difficulties, and ulnar deviation of the fifth finger. Most patients may exhibit abnormal methylation in the 11p15 region. rhGH therapy can improve the height of these patients.
Humans ; Silver-Russell Syndrome/diagnosis* ; Male ; Female ; Child ; Child, Preschool ; Infant ; Adolescent ; Retrospective Studies

The high-quality development of new quality productivity and internal audit constitutes a significant theoretical and practical innovation under the background of the new era,jointly forming the key impetus for the sustainable development of public hospitals.Based on the connotation and characteristics of the new quality productivity and the high-quality development of internal audit,it expounds the internal logic of the two-way driving,analyzes the realistic challenges faced by the high-quality develop-ment of internal audit.Moreover,specific implementation paths are proposed from the aspects of policy guidance,service supply,resource allocation,talent cultivation,and data intelligence linkage.

Objective:To evaluate the value of D-dimer(D-D) levels in the diagnosis of thromboembolism in children with severe Mycoplasma pneumoniae pneumonia (SMPP).Methods:A case control study was conducted on 51 SMPP patients admitted to Qingdao Women and Children′s Hospital Affiliated to Qingdao University and Jining First People′s Hospital from January 1, 2021 to August 1, 2024.They were divided into a thrombus group (19 cases) and a non-thrombus group (32 cases) according to whether they had thromboembolism.The characteristics of the cases were analyzed.Logistic regression was used to analyze the relationship between increased D-D levels and the risk of thrombosis.The value of D-D levels in predicting thromboembolism in SMPP patients was evaluated by the receiver operating characteristic curve.Results:Of the 19 patients with thrombosis, 11 had with pulmonary thrombosis, 6 had cerebrovascular thrombosis, 3 had heart thrombosis, 1 had spleen artery thrombosis, and 3 had multiple site thrombosis.Compared with the non-thrombus group, the thrombus group had higher D-D levels[(18.5±4.9) mg/L vs.(3.1±0.8) mg/L, t=3.118, P=0.006], higher C-reactive protein levels[25.5(15.5, 92.7) mg/L vs.7.9(3.9, 21.4) mg/L, Z=3.292, P=0.001], higher lactate dehydrogenase levels[484(351, 743) U/L vs.347(285, 396) U/L, Z=2.770, P=0.006] and a higher proportion of pleural effusion[63.1%(12/19) vs.25.0%(8/32), χ2=7.282, P=0.009].Increased D-D levels were an independent risk factor for thromboembolism in SMPP patients ( P=0.005, OR=1.254, 95% CI: 1.069-1.472).When the D-D level was used for predicting thromboembolism in SMPP patients, its cut-off value was 4.46 mg/L, its Youden index was 0.707, its area under the curve was 0.893(95% CI: 0.807-0.979), its sensitivity was 89.5%, its specificity was 81.2%, and its negative predictive value was 92.9%. Conclusions:D-D levels have high value in predicting thromboembolism in SMPP patients, and it can help timely identify patients at high risk.

The high-quality development of new quality productivity and internal audit constitutes a significant theoretical and practical innovation under the background of the new era,jointly forming the key impetus for the sustainable development of public hospitals.Based on the connotation and characteristics of the new quality productivity and the high-quality development of internal audit,it expounds the internal logic of the two-way driving,analyzes the realistic challenges faced by the high-quality develop-ment of internal audit.Moreover,specific implementation paths are proposed from the aspects of policy guidance,service supply,resource allocation,talent cultivation,and data intelligence linkage.

Objective:To evaluate the value of D-dimer(D-D) levels in the diagnosis of thromboembolism in children with severe Mycoplasma pneumoniae pneumonia (SMPP).Methods:A case control study was conducted on 51 SMPP patients admitted to Qingdao Women and Children′s Hospital Affiliated to Qingdao University and Jining First People′s Hospital from January 1, 2021 to August 1, 2024.They were divided into a thrombus group (19 cases) and a non-thrombus group (32 cases) according to whether they had thromboembolism.The characteristics of the cases were analyzed.Logistic regression was used to analyze the relationship between increased D-D levels and the risk of thrombosis.The value of D-D levels in predicting thromboembolism in SMPP patients was evaluated by the receiver operating characteristic curve.Results:Of the 19 patients with thrombosis, 11 had with pulmonary thrombosis, 6 had cerebrovascular thrombosis, 3 had heart thrombosis, 1 had spleen artery thrombosis, and 3 had multiple site thrombosis.Compared with the non-thrombus group, the thrombus group had higher D-D levels[(18.5±4.9) mg/L vs.(3.1±0.8) mg/L, t=3.118, P=0.006], higher C-reactive protein levels[25.5(15.5, 92.7) mg/L vs.7.9(3.9, 21.4) mg/L, Z=3.292, P=0.001], higher lactate dehydrogenase levels[484(351, 743) U/L vs.347(285, 396) U/L, Z=2.770, P=0.006] and a higher proportion of pleural effusion[63.1%(12/19) vs.25.0%(8/32), χ2=7.282, P=0.009].Increased D-D levels were an independent risk factor for thromboembolism in SMPP patients ( P=0.005, OR=1.254, 95% CI: 1.069-1.472).When the D-D level was used for predicting thromboembolism in SMPP patients, its cut-off value was 4.46 mg/L, its Youden index was 0.707, its area under the curve was 0.893(95% CI: 0.807-0.979), its sensitivity was 89.5%, its specificity was 81.2%, and its negative predictive value was 92.9%. Conclusions:D-D levels have high value in predicting thromboembolism in SMPP patients, and it can help timely identify patients at high risk.

OBJECTIVE To identify and analyze adverse drug event(ADE)signals associated with tirzepatide based on the FDA Adverse Event Reporting System(FAERS)database,providing a reference for clinical medication safety.METHODS ADE reports from January 1,2022,to June 30,2024,with tirzepatide as the primary suspected drug,were extracted from the FAERS database.Medical Dictionary for Regulatory Activities was used to systematically categorize the selected system organ class(SOC)and preferred term of ADE.Signal mining and analysis were performed using the reporting odds ratio method and the proportional reporting ratio method.RESULTS A total of 39 229 ADE reports related to tirzepatide were obtained,including 3 934 severe ADE reports(10.03%).The majority of severe ADE reports were related to hospitalization or prolonged hospitalization(3.82%),involving 131 positive ADE signals.Among the reports with documented patient gender and age,26 195 were female(66.77%),7 869 were male(20.06%),and the majority of patients were aged 18-64 years(54.26%).The top three most frequently reported ADE were injection site pain,nausea,and injection site hemorrhage.Strong ADE signals not mentioned in the tirzepatide instruction included injection site coldness,starvation ketoacidosis,injection site hemorrhage,hunger,elevated adrenaline,injection site skin cracking,binge eating,skin laxity,intestinal sepsis,lack of satiety,and dysesthesia.Subgroup analysis for patient's gender and age showed differences in the proportion of ADE reports across different SOC.Male patients or those aged≥65 years had a higher risk of gastrointestinal system disorders compared to female patients or those aged＜65 years.CONCLUSIONS In clinical use of tirzepatide,in addition to monitoring ADE listed in the instruction,attention should also be paid to ADE not mentioned in the instruction,such as injection site coldness,starvation ketoacidosis,injection site hemorrhage,elevated adrenaline,and intestinal sepsis,to ensure patient safety.

BACKGROUND:Establishing a stable and reliable animal model of acute pancreatitis is of great significance for understanding its pathogenesis,pathophysiological characteristics,and clinical medication.Domestic and foreign studies have shown that cerulein,L-arginine,and sodium taurocholate can induce acute pancreatitis,but their pathophysiological characteristics and model characteristics are still unclear. OBJECTIVE:To establish an acute pancreatitis rat model using cerulein,L-arginine,and sodium taurocholate and to observe the changing patterns of model features at different time points. METHODS:Ninety-six healthy male Sprague-Dawley rats were randomly divided into normal group,cerulein group,L-arginine group,and sodium taurocholate group,with 24 rats in each group.Within each group,there were three subgroups(n=8 per group):12-,24-,and 48-hour subgroups.Cerulein was administered via intraperitoneal injection six times with a 1-hour interval.L-arginine was administered through two intraperitoneal injections with a 1-hour interval.Sodium taurocholate was injected for inducing acute pancreatitis models through retrograde injection into the bile-pancreatic duct.By examining the rat survival rate,gross morphology of the pancreas,calculating the pancreatic organ index,and measuring levels of amylase,lipase,alanine transaminase,aspartate transaminase,blood urea nitrogen,and creatinine,as well as observing pancreatic tissue pathological features through hematoxylin-eosin staining and conducting a pancreatic injury scoring,we evaluated the changing patterns of model features at different time points. RESULTS AND CONCLUSION:Compared with the normal group,the overall survival rate of rats was 100%in the cerulein group,88%in the L-arginine group,and 96%in the sodium taurocholate group.The pancreatic organ index was increased in all groups.Gross observation indicated that,In the cerulein group,pancreatic edema,blurred lobes,and looseness were visible.In the L-arginine group,the pancreatic glands were enlarged and thickened with patchy bleeding.In the sodium taurocholate group,pancreatic tissue showed varying degrees of congestion and edema accompanied by scattered flakes of hemorrhage and necrosis.The levels of serum alanine transaminase,aspartate transaminase,blood urea nitrogen,creatinine,amylase,and lipase in rats exhibited consistent changes.In the cerulein group,these parameters possibly peaked at 12 hours(P<0.05)and then showed a declining trend.In the L-arginine group,they reached the highest levels at 24 hours(P<0.05)and significantly decreased at 48 hours.In the sodium taurocholate group,serum amylase and lipase remained at higher levels at 12 hours with a slow decline trend(P<0.05).Compared with the normal group,microscopic examination revealed mild acinar edema and widened interlobular spaces in the cerulein group,with a higher presence of inflammatory cells.In the L-arginine group,there was widening of interlobular spaces,extensive infiltration of inflammatory cells,and patchy necrotic areas.In the sodium taurocholate group,significant pancreatic edema,structural disarray,extensive necrotic foci,and inflammatory cell infiltration were observed.Compared with the normal group,the pathological scores of induced acute pancreatitis in all three models were significantly different at each time point(P<0.05).Moreover,the pathological scores in each group increased over time,indicating a gradual worsening of pancreatic tissue damage.When comparing different models at the same time,there were differences in pathological scores,with the sodium taurocholate group having the highest scores,followed by the L-arginine group,and the cerulein group having the lowest scores.Analyzing the three models at the same time point,the most severe condition was in the sodium taurocholate group,which was characterized by pancreatic hemorrhage and necrosis,followed by the L-arginine group,which was characterized by necrosis,and the least severe condition was in the cerulein group,mainly characterized by edema.The serum biochemical index levels of the cerulein and L-arginine groups decreased at 48 hours,indicating that these two models may have a tendency to self-heal and belong to a self-limiting disease course.The serum biochemical index levels of the sodium taurocholate group decreased slowly after 12 hours.Therefore,pancreatic injury in the sodium taurocholate group might not be relieved after 48 hours or longer.

Objective:To investigate the protective effect of hyperbaric oxygen therapy(HBOT)on myocardial fibrosis and oxidative stress induced by D-galactose(D-gal)in senescent model mice and its possible mechanism.Methods:Three-month-old male Kunming mice(n=27)were randomized into control, D-gal, and D-gal + HBOT groups.The control group received subcutaneous sterilized saline(5 ml · kg -1· d -1)for 8 weeks; the remaining 2 groups received subcutaneous D-gal(200 mg · kg -1· d -1)for 8 weeks. The D-gal + HBOT group underwent HBOT intervention at week 7~8.At the end of the experiment, the histopathological changes were analyzed by hematoxylin-eosin(HE)staining, and the fibrosis changes were analyzed by Masson staining and Sirius red staining.Oxidative stress kit was used to detect catalase(CAT), total superoxide dismutase(T-SOD)activity and malon-di-aldehyde(MDA)content in serum of mice.Western blotting was used to detect the expression levels of the aging-related proteins p53 and p16 in mouse heart tissue, the heart-function-related proteins atrial natriuretic peptide(ANP)and brain natriuretic peptide(BNP), and the oxidative stress-related protein superoxide dismutase 1(SOD1), superoxide dismutase 2(SOD2)and catalase(CAT). Results:Cardiac morphologic staining indicated that as compared with the control group, mice of D-gal group exhibited features of senescence and the increased fibrosis area, and senescence and fibrosis were obviously improved after HBOT intervention as compared with the D-gal group.The findings of the oxidative stress kit measurement indicated that as compared with the control group, the D-gal group had markedly decreased activities of CAT and T-SOD, significantly increased MDA content in the serum.After HBOT treatment, as compared with d-gal group, serum CAT and T-SOD activities were increased, while MDA content was decreased( F=126.85, 32.89, 157.50, all P<0.05).Furthermore, as compared with the control group, the D-gal group had obviously increased contents of p53, p16, ANP and BNP, while the content of CAT, SOD1 and SOD2 were obviously decreased.After HBOT intervention, as compared with the D-gal group, the contents of p53, p16, ANP、BNP were reduced, while the content of CAT, SOD1 and SOD2 were increased( F=36.37, 14.81, 23.28, 58.41, 12.79, 80.08, 6.63, all P<0.05). Conclusions:HBOT intervention could protects against cardiac injury in aging mice, which may be related to attenuating myocardial fibrosis, inducing the expression of antioxidant enzymes, and reducing oxidative stress.

ObjectiveTo compare the differences in resistance and structure of skin between acupoints and non-acupoints， and to study the difference in skin permeability characteristics of Corydalis Rhizoma total alkaloid patches （CTTP） after administration at Shenque acupoint and non-acupoint， so as to provide experimental support for its clinical acupoint application to prevent and treat chronic pain. MethodTaking corydaline （CD）， tetrahydropalmatine （THP） and corydalis L （CDL） as evaluation indexes， and the quantitative analysis was carried out by high performance liquid chromatography （HPLC）. The mobile phase was methanol-0.04 mol·L^-1 phosphoric acid aqueous solution （70∶30， pH 6.0 adjusted with triethylamine）， the detection wavelength was 281 nm. In vitro transdermal test in Franz diffusion cell and in vivo transdermal test were used to study the skin permeability characteristics of CTTP through Shenque acupoint and non-acupoint administration. At the same time， the skin resistance between Shenque acupoint and non-acupoint was measured before and after the administration， and the distribution of the drug in each layer of the skin was compared by freezing sectioning， and visual verification was performed with fluorescence inverted microscope. ResultAfter 24 h of administration， the results of in vivo and in vitro experiments showed that the cumulative permeation and retention of CD， THP and CDL at Shenque acupoint skin were higher than those at non-acupoint skin （P<0.05， P<0.01）， the skin resistance of Shenque acupoint was lower than that of non-acupoint at all time points. The fluorescence microscopic observation results showed that the drug content of each layer of the skin was all Shenque acupoint>non-acupoint， indicating that the skin of Shenque acupoint had better effect on drug penetration and storage than non-acupoint. ConclusionThe 24 h cumulative permeation and retention of CTTP in Shenque acupoint skin are higher than those in non-acupoint skin， and the mechanism may be related to the thin skin， low electrical resistance and large number of hair follicle bodies at Shenque acupoint.

Objective:To systematically evaluate the influencing factors on unplanned shutdown of continuous blood purification, and to provide reference basis for the prevention of unplanned shutdown.Methods:The literatures related to the influencing factors of unplanned shutdown of continuous blood purification in CNKI, Wanfang Database, Chinese Biomedical Literature Database, Chinese Science and Technology Periodical Full-text Database, PubMed and Web of Science were searched.The retrieval time of Chinese database was from the establishment of the database to March 2021.English databases were searched from March 2016 to March 2021.Literature selection, quality evaluation and data extraction were independently conducted by two researchers, and Meta-analysis was performed by Stata 14.0 software.Results:A total of 11 studies were included, including 3 031 cases of continuous blood purification treatment and 1 412 cases of unplanned discontinuation.The combined OR value and 95% CI of all influencing factors were as follows: treatment mode 2.22 (1.06-4.62), blood flow velocity 0.91 (0.776-1.09), agitation 4.54 (2.33-8.86), ventilator 2.67 (1.63-4.38), transfusing blood products and fat milk 1.07 (0.34-3.36), one-time catheter success 0.26 (0.05-1.42), catheterization site (femoral vein vs.jugular vein) 2.24 (0.83-6.02). Conclusion:Unplanned deplaning is influenced by many factors.Treatment mode, agitation and ventilator use are the risk factors for unplanned deplaning.There is no correlation between blood flow velocity, transfusing blood products and fat milk, one-time catheterization success, catheterization site and unplanned deplaning.