Purpose To explore the ultrasound features and early diagnostic clues of fetal myocardial non-compaction.Materials and Methods The clinical data and echocardiographic data of four fetuses who underwent fetal echocardiography in Henan Provincial People's Hospital from January 2015 to February 2023 and were confirmed to have myocardial non-compaction by pathological finding or postnatal examination were collected,and analyzed.Results A total of four fetuses diagnosed as myocardial non-compaction by prenatal ultrasound:two involved the left ventricle with isolated lesions,and apical myocardial non-compaction was confirmed by postnatal echocardiography;two involved the biventricles,and both of which were pathologically confirmed after induction of labor.The prenatal ultrasound of fetal myocardial involvement in four cases showed that:(1)the affected myocardium showed a bilayer structure:the outer layer was compacted myocardium,which showed thin and compacted homogeneous hypoechoic;the inner layer was loose and thickened non-compacted myocardium with enhanced echogenicity;(2)color Doppler flow imaging:the non-compacted myocardium showed sieve mesh blood flow with ventricular communication.Some cases were associated with cardiac enlargement and arrhythmia.Conclusion Prenatal echocardiography can diagnose fetal myocardial non-compaction with a characteristic echographic presentation.Localized myocardial thickening and echogenic enhancement,cardiac enlargement and arrhythmia may be important clues to identify fetal myocardial non-compaction.

Objective:To detect gene mutations in a pedigree with dominant dystrophic epidermolysis bullosa (DDEB) .Methods:A 20-year-old male proband presented with repeated blisters, ulceration, pigmentation, scars on the limbs, and deformation of the nails/toenails after birth. There were 5 patients in the 3-generation family, and they all presented with typical skin lesions. Peripheral blood samples were obtained from 14 members of the pedigree (including the 5 patients) and 100 unrelated healthy controls. Whole-exome sequencing was performed in the proband to identify relevant mutation sites, which were then confirmed in the family by Sanger sequencing.Results:Genetic testing indicated that the proband and the other 4 patients all carried a missense mutation (c.7885G>A) in exon 107 of the COL7A1 gene, resulting in the substitution of glycine by arginine at amino acid position 2629 (p.G2629R). The mutation was identified neither in the 9 healthy relatives nor in the 100 unrelated healthy controls. The mutation co-segregated with DDEB in the family, and was not included in databases such as Pubmed, HGMD or ClinVar, suggesting it was a novel missense mutation. The amino acid encoded by this mutation may alter the structure of type Ⅶ collagen, thereby affecting its function.Conclusion:A novel missense mutation was identified in exon 107 of the COL7A1 gene in the family with DDEB, expanding the spectrum of mutations in the COL7A1 gene.

Objective:To explore the changes of left atrial volume and function in patients with early diabetic nephropathy by four-dimensional auto left atrial quantification (4D Auto LAQ).Methods:Forty patients with early diabetic nephropathy (early diabetic nephropathy group), 40 patients with type 2 diabetes (diabetes group) in Henan Provincial People′s Hospital from March 2020 to April 2021 were selected, and 36 healthy volunteers (control group) were collected during the same period. The parameters of conventional echocardiography were measured, and the four-dimensional volume probe was used to obtain the complete left atrial volume image in 5 cardiac cycles. The 4D Auto LAQ software on the EchoPAC workstation was used for analysis to obtain the left atrial volume and strain indicators: left atrial (LA) maximum volume (LAVmax), left atrial minimum volume (LAVmin), pre-systolic volume (LAVpreA), left atrial volume index (LAVImax), left atrial emptying volume (LAEV), left atrial emptying fraction (LAEF), and long axis and circumferential strains in left atrial reserve phase, pipeline phase and systolic phase (LASr, LASr-c; LAScd, LAScd-c; LASct, LASct-c). The differences of these parameters among 3 groups were analyzed.Results:There were no significant differences in interventricular septum end-diastolic thickness(IVSd), left ventricular posterior wall end-diastolic thickness(LVPWd), left ventricular end-diastolic dimension(LVIDd), left ventricular ejection fraction(LVEF), and E/A (ration of early to late diastolic peak flow velocity of mitral orifice) among 3 groups (all P>0.05), and left atrial diameter(LAD), relative wall thickness(RWT), and E/e′ (ration of early diastolic peak flow velocity of mitral orifice to early diastolic velocity of lateral mitral annulus) among 3 groups were significantly different (all P<0.05). Further pairwise comparison results showed that LAD was only significantly different between the early diabetic nephropathy group and control group ( P=0.001 2), and the differences in RWT and E/e′ were statistically significant among 3 groups (all P<0.05). There were no significant differences in LAEV, LAScd-c, and LASct-c among 3 groups (all P>0.05), and LAVmin, LAVmax, LAVpreA, LAVImax, LAEF, LASr, LAScd, LASct, and LASr-c among the 3 groups were significantly different (all P<0.05). The pairwise comparison showed that, compared with the control group and the diabetes group, LAVmin, AVpreA, and LAVImax in the early diabetic nephropathy group were increased, and LAEF, LAScd, LASct, and LASr-c were decreased (all P<0.05). Compared with the control group, LAVmax, LAVImax and LASct in the diabetes group were increased, and LAEF, LAScd, and LASr-c were decreased (all P<0.05). Conclusions:4D Auto LAQ technology can quantitatively evaluate the changes in left atrium volume and function in patients with early diabetic nephropathy. Patients with early diabetic nephropathy have an increase in left atrium volume and a decrease in strain value.

ObjectiveTo explore the compatibility advantage of Scutellariae Radix-Coptidis Rhizoma in the prevention and treatment of neuroinflammation， and to elucidate the action characteristics and mechanism of the compatibility advantage based on Toll like receptor （TLR4）/myeloid differentiation factor 88 （MyD88）/nuclear factor kappaB （NF-κB） pathway. MethodRepresentative mouse microglia cells （BV2） in vitro were selected and divided into 8 groups： control group， model group， Scutellariae Radix-Coptidis Rhizoma group， Piracetam group， Scutellariae Radix group and Coptidis Rhizoma group. The BV2 cell inflammatory model was established by lipopolysaccharide （LPS）， and the cell activity was detected by cell counting kit-8 （CCK-8）. Cell morphology was observed under bright field. The production and release of pro-inflammatory factors in BV2 cells were determined by enzyme-linked immunosorbent assay （ELISA） and immunofluorescence assay， and the mRNA expressions of TLR4， MyD88 and NF-κB were detected by Real-time fluorescence quantitative polymerase chain reaction （Real-time PCR）. The nuclear translocation of NF-κB p65 was detected by immunofluorescence， and TLR4 signal transduction inhibitor （CLI-095） and NF-κB inhibitor （PDTC） were used to confirm the anti-neuroinflammation targets of Scutellariae Radix-Coptidis Rhizoma. ResultCompared with the conditions in the control group， most cells in LPS-induced model group were activated， and the contents of IL-6， TNF-α and IL-1β in culture medium and cells and the mRNA expressions of TLR4， MyD88， NF-κB p50 and NF-κB p65 were increased （P<0.01）， with obvious nuclear entry of NF-κB p65. Compared with the conditions in the model group， BV2 cell morphology was mostly recovered after pretreatment in Scutellariae Radix-Coptidis Rhizoma and Piracetam groups， and the levels of IL-6， TNF-α and IL-1β and the mRNA expressions of TLR4， MyD88， NF-κB p50 and NF-κB p65 were decreased （P<0.05， P<0.01）， with NF-κB p65 mostly observed in cytoplasm. Compared with the conditions in the model group， cell morphology was slightly recovered in Scutellariae Radix group and Coptidis Rhizoma group， and the levels of pro-inflammatory factors and mRNA expressions of TLR4， MyD88， NF-κB p50 and NF-κB p65 were reduced. In terms of inhibitory effect on pro-inflammatory factors， Scutellariae Radix group and Coptidis Rhizoma group were lower than Scutellariae Radix-Coptidis Rhizoma group （P<0.05）. Compared with the model group， the "Scutellariae Radix-Coptidis Rhizoma+CLI-095" group and "Scutellariae Radix-Coptidis Rhizoma+PDTC" group had lowered mRNA expressions of TLR4， MyD88， NF-κB p50 and NF-κB p65 （P<0.05， P<0.01）， and the transfer of NF-κB p65 into nucleus was obviously inhibited. ConclusionThe anti-neuroinflammation effect of Scutellariae Radix-Coptidis Rhizoma was significantly better than Scutellariae Radix or Coptidis Rhizom alone， and the anti-neuroinflammation advantage was closely related to the inhibition of activation of TLR4/MyD88/NF-κB signaling pathway in microglial cells. It was confirmed that TLR4， MyD88 and NF-κB were potential targets for Scutellariae Radix-Coptidis Rhizoma to exert the compatibility advantage.

Objective:To analyze the clinical characteristics of patients with acute glyphosate herbicide poisoning and the differences in the severity of poisoning.Methods:A retrospective analysis was performed on patients with acute glyphosate herbicide poisoning admitted to the First Affiliated Hospital of Zhengzhou University from January 2014 to December 2020. The general information, exposure time, poisoning dose, poisoning cause, poisoning route, clinical manifestations, laboratory examination results during hospitalization, treatment measures, hospital stays and prognosis of the patients were collected. The patients were graded according to the poisoning severity scoring standard of Chinese Expert Consensus on Diagnosis and Treatment of Acute Poisoning in 2016. The highest severity score during hospitalization was used as the final grade. According to the final grade, asymptomatic and mild patients were included in the mild group, and moderate, severe and death patients were included in the severe group. The independent sample T test or Mann-Whitney U test was used for measurement data, and χ2 test or Fisher's exact test was used for counting data. The differences of general data and clinical data between the two groups were compared. Results:According to the inclusion and exclusion criteria, 83 patients with acute glyphosate herbicide poisoning were selected as the study subjects. All patients survived, mainly mild poisoning (56.6%), with a male to female ratio of 33∶50, and an average age of 39 years. The number of poisoning cases increased yearly (the highest in 2019), and most cases occurred in spring and summer. The main cause of poisoning was suicide (71.1%), direct oral administration (83.1%) was the primary route of poisoning, and the dominating clinical manifestations were digestive symptoms (71.1%). Laboratory tests showed increased white blood cell count (WBC), neutrophil percentage (NEUT %) and D-dimer, and decreased hemoglobin and potassium. Compared with the mild group, patients in the severe group were older [(51±17) years vs. (35±19) years], had a higher proportion of suicide and direct oral administration, a longer hospital stay [8.0 (4.8, 12.0) d vs. 3.0 (2.0, 5.5) d], a higher dose of poisoning [200.0 (50.0, 200.0) mL vs. 30.0 (11.3, 57.5) mL], and higher NEUT % within 24 h of admission [(83.4±10.4) vs. (73.2±12.8)]. The increase of WBC, NEUT %, aspartate aminotransferase, prothrombin time, D-dimer and the decrease of serum potassium were more common in the severe group than the mild group, with statistical significance (all P<0.05). Conclusions:The number of patients with acute glyphosate herbicide poisoning is increasing yearly. Generally, the condition is mild and the prognosis is satisfying. The severity is more serious in the middle-aged and elderly patients andthose with direct oral administration, high toxic dose, and high NEUT % within 24 h of admission. Severe poisoning is more likely to cause changes in laboratory indicators.

Objective:To evaluate the impact of sarcopenia on survival and treatment-related toxicity in postoperative recurrent esophageal squamous cell carcinoma (ESCC) patients treated with chemoradiotherapy.Methods:Clinical data of 147 patients with postoperative locoregional recurrent ESCC receiving chemoradiotherapy in Huai'an First People's Hospital from 2016 to 2017 were retrospectively analyzed. Pectoralis muscle area (PMA) was determined using routine pre-radiotherapy CT simulation scan above the aortic arch level. Sarcopenia was defined as a cut-off value of pectoralis muscle index (PMI) (PMA/height 2) <11.55 cm 2/m 2 for males and <8.69 cm 2/m 2 for females. The incidence of toxicity, 1- and 3-year overall survival (OS) rates were statistically compared between patients with and without sarcopenia. Results:Sarcopenia was detected in 49 of 147 (33.3%) patients. The incidence of grade 3-4 toxicities in sarcopenic patients was significantly higher compared to that in their counterparts without sarcopenia (40.8% vs. 18.4%, P=0.005). In addition, patients with sarcopenia had significantly worse 1-year (61.2% vs. 82.7%) and 3-year OS rates (10.2% vs. 28.6%) than those without sarcopenia (both , P<0.001). Multivariate analysis showed that sarcopenia was an independent prognostic factor for poor OS ( P<0.001). Conclusion:PMI based on CT simulation scan has prognostic value in postoperative locoregional recurrent ESCC patients treated with chemoradiotherapy, which probably serves as a novel diagnostic tool for sarcopenia.

Objective:To explore the value of pressure-strain loop (PSL) for non-invasive quantitative assessment of left ventricular myocardial work index (GWI), global work efficiency (GWE), global constructive work (GCW), global wasted work (GWW) in the evaluation of left ventricular myocardial work in patients with hypertrophic cardiomyopathy (HCM).Methods:Thirty-one HCM patients (HCM group) and 36 healthy volunteers (control group) from December 2018 to September 2019 in Henan Provincial People′s Hospital were selected. Relevant clinical data were collected, left ventricular ejection fraction (LVEF), left ventricular end-diastolic diameter (LVEDd), left ventricular end systolic diameter (LVEDs), left atrial diameter (LAD), the maximum wall thickness (MWT), left ventricular mass index (LVMI), the global longitudinal strain (GLS), the peak strain dispersion(PSD) and GWI, GWE, GCW and GWW between the two groups were compared.Results:Compared with the control group, MWT, LAD, E/e′, LVMI in HCM group were significantly increased (all P<0.05); left ventricular myocardial functional parameters of GLS, GWI, GWE, GCW were significantly decreased, and GWW and PSD were significantly increased (all P<0.05). Left ventricular GLS, PSD and GWI, GCW, GWW, GWE have better repeatability within the observer and between observers. ICC within the observer were 0.852, 0.707, 0.917, 0.955, 0.675, 0.618, respectively. And their ICC between observers were 0.837, 0.631, 0.927, 0.944, 0.555, 0.670, respectively. Correlation analysis showed that GLS was positively correlated with GWI, GWE, and GCW ( r=0.765, 0.737, 0.815; all P<0.001), and negatively correlated with GWW and PSD ( r=-0.517, -0.606; all P<0.001). Conclusions:The left ventricular GWI, GWE and GCW decreased in HCM patients, while the GWW increased. PSL can evaluate the damage of left ventricular myocardial work in HCM patients.

Objective To investigate the value of ultrasonic two‐dimensional speckle tracking imaging ( 2D‐ST I) layer‐specific strain and transmural gradient in evaluating the changes of hypertensive patients′left ventricular three layers myocardial function . Methods Thirty‐five hypertensive patients without renal insufficiency were selected as group A ,and 25 hypertensive patients with renal insufficiency as group B .For comparison ,40 healthy volunteers were gathered as control group . T hen ,the systolic peak longitudinal strain of the left ventricle( LPS) ,endocardium( LPS Endo ) ,mid‐cardium ( LPSMid ) and epicardium ( LPSEpi ) were collected .After that ,features of transmural gradient ( ΔLS ) and its percentage ( ΔLS% ) were analyzed . Results For each group ,gradient features exist in each layer of left ventricular myocardium :LPS Endo ＞LPSMid＞LPSEpi . For each group ,the differences between LPSEndo and LPSMid ,and that between LPSEndo and LPSEpi were both statistically significant( P ＜0 .05) . When it came to the differences between LPS Mid and LPSEpi ,those of group A and control group were both statistically significant ( P ＜0 .05 ) . Compared with control group ,the LPSEndo of group A ,and LPSEndo ,LPSMid and LPSEpi of group B declined ,all the differences were statistically significant ( P ＜0 .05 ) . Compared with group A ,LPS Endo ,LPSMid and LPSEpi of group B declined ,the differences were statistically significant ( all P ＜0 .05) . For ΔLS′s differences among all three groups ,there was no statistical significance( P ＞0 .05) . Compared with control group ,the ΔLS% Endo‐Mid of group A rised , and the ΔLS% Endo‐Mid , ΔLS% Mid‐Epi of group B rised , the differences were statistically siginificant ( all P ＜ 0 .05 ) . Compared with group A ,the ΔLS% Endo‐Mid ,ΔLS% Mid‐Epi of group B rised ,the differences were statistically siginificant( all P ＜0 .05) . Conclusions T he layer‐specific strain technique can quantitatively evaluate the changes of systolic function of the left ventricular myocardium in hypertensive patients . ΔLS% may have better sensitivity than ΔLS in dection of systolic function damage of the left ventricular myocardium and can provide more reference for the evaluation of left ventricular systolic function .

Objective To quantitatively evaluate left ventricular ( LV ) systolic function in bicuspid aortic valve (BAV) using layer-specific strain ( LSS) . Methods Thirty BAV patients were divided into normal function (NF) group (10 cases) and non-normal function (N-NF) group (20 cases) based on aortic valvular lesion types ,and 20 healthy volunteers were taken as control group . Longitudinal strain( LS) and circumferential strain (CS) of three-layer myocardium and full thickness myocardium were assessed using layer-specific speckletracking imaging ,available by GE Vivid E9 and EchoPac workstation . Results There was no significant difference in left ventricular ejection fraction( LVEF) among the N-NF group ,NF group and control group ( P > 0 .05) ,all of them within the normal range[(63 .3 ± 7 .1)％ ,(64 .6 ± 6 .2)％ , ( 65 .3 ± 3 .9)％ ] . It showed a gradient decrease from the endocardium to the epicardium in both control and BAV group . LS of endocardium ( LSendo) and LS of epicardium ( LSepi) in N-NF group and NF group were significantly reduced compared with those in control group ( P <0 .05) [ LSendo :( -21 .19 ± 3 .12)％vs ( -23 .06 ± 2 .07 )％ vs ( -25 .53 ± 2 .51 )％ ;LSepi:( -16 .08 ± 2 .68 )％ vs ( -18 .85 ± 2 .12 )％ vs ( -20 .72 ± 2 .28)％ ] . Compared with control group ,there was no significant difference in NF group in CS of the three-layer myocardial and full-thickness myocardium as well as the LS of the whole medial myocardial layers and full-thickness ( P > 0 .05 ) . Compared with NF group [ CS :( -19 .57 ± 2 .9 )％ vs ( -13 .43 ± 2 .19)％ vs ( -20 .03 ± 3 .04)％ ;LS :( -21 .38 ± 2 .05)％ vs ( -18 .85 ± 2 .12)％ vs ( -21 .09 ± 2 .03)％ ] and control group[CS :( -21 .63 ± 3 .01)％ vs ( -14 .34 ± 2 .55)％ vs ( -21 .48 ± 2 .16)％ ;LS :( -22 .18 ± 2 .30 )％ vs ( -20 .72 ± 2 .28 )％ vs ( -22 .89 ± 2 .30 )％ ] , CS [ ( -16 .78 ± 3 .65 )％ vs ( -11 .40 ± 3 .78 )％ vs ( -15 .83 ± 2 .61 )％ ] and LS [ ( -18 .34 ± 2 .85 )％ vs ( -16 .08 ± 2 .68 )％ vs ( -18 .51 ± 2 .86)％ ] of middle myocardium ,epicardial myocardium and full-thickness myocardium in N-NF group were decreased significantly ( P < 0 .05) . Conclusions It is essential to maintain normal valvular function to prevent the progress of myocardial deterioration . LSendo and LSepi can be used to sensitively identify early left ventricular systolic dysfunction in BAV patients with normal LVEF .

Objective To explore the efficacy of alprostadil combined with Bailing capsule in the treatment of early chronic kidney disease. Methods A total of 94 early stage chronic kidney disease patients were selected and divided into treatment group(n=46) and control group(n=48).The patients in control group were treated with Bailing capsule,5 capsules, tid,po.The patients in treatment group were treated with Bailing capsule combined with 2 mL alprostadil in 20 mL 0.9% sodium chloride injection,intravenous injection,qd.The patients were treated for 4 weeks as a course of treatment in both groups.After 2 courses of treatment,the improvement of renal function,the changes in cytokine levels including NK cells and T cell subsets CD+3, CD+4,CD+8,adverse reactions of two groups were observed. Results The effective rates of the control group and the treatment group were 60.42%,91.30%,respectively(P<0.05).The renal function index 24 h urine protein were(1.15± 0.35) g,serum creatinine were(78.52±10.63) μmol·L-1,urea nitrogen were(8.23±1.65) mmol·L-1,all of which were decreased significantly (P<0.05).The levels of NK cells were(21.89±2.73)%,T cell subsets CD+3were(71.02±5.61)%,CD+4were(38.84±3.52)%, CD+4/CD+8were(1.28 ± 0.14),which were increased significantly,while the level of CD+8were(30.21± 3.03)% was decreased significantly(P<0.05).There was no significant difference between two groups in the adverse reactions(P>0.05). Conclusion The combination of alprostadil and Bailing capsule is effective to early stage chronic kidney disease by improving the renal function and regulating the level of cytokines.