Objective:To study the current status of longitudinal extrauterine growth restriction (EUGR) in extremely preterm infants (EPIs) and to develop a prediction model based on clinical data from multiple NICUs.Methods:From January 2017 to December 2018, EPIs admitted to 32 NICUs in North China were retrospectively studied. Their general conditions, nutritional support, complications during hospitalization and weight changes were reviewed. Weight loss between birth and discharge > 1SD was defined as longitudinal EUGR. The EPIs were assigned into longitudinal EUGR group and non-EUGR group and their nutritional support and weight changes were compared. The EPIs were randomly assigned into the training dataset and the validation dataset with a ratio of 7∶3. Univariate Cox regression analysis and multiple regression analysis were used in the training dataset to select the independent predictive factors. The best-fitting Nomogram model predicting longitudinal EUGR was established based on Akaike Information Criterion. The model was evaluated for discrimination efficacy, calibration and clinical decision curve analysis.Results:A total of 436 EPIs were included in this study, with a mean gestational age of (26.9±0.9) weeks and a birth weight of (989±171) g. The incidence of longitudinal EUGR was 82.3%(359/436). Seven variables (birth weight Z-score, weight loss, weight growth velocity, the proportion of breast milk ≥75% within 3 d before discharge, invasive mechanical ventilation ≥7 d, maternal antenatal corticosteroids use and bronchopulmonary dysplasia) were selected to establish the prediction model. The area under the receiver operating characteristic curve of the training dataset and the validation dataset were 0.870 (95% CI 0.820-0.920) and 0.879 (95% CI 0.815-0.942), suggesting good discrimination efficacy. The calibration curve indicated a good fit of the model ( P>0.05). The decision curve analysis showed positive net benefits at all thresholds. Conclusions:Currently, EPIs have a high incidence of longitudinal EUGR. The prediction model is helpful for early identification and intervention for EPIs with higher risks of longitudinal EUGR. It is necessary to expand the sample size and conduct prospective studies to optimize and validate the prediction model in the future.

Objective:To analyze the incidence and risk factors of non-acute symptomatic portal vein thrombosis (PVT) after endoscopic gastric variceal injection (GVI) in the treatment of liver cirrhosis with gastric variceal bleeding (GVB).Methods:66 patients with GVB who were treated with GVI for the first time from July 2017 to October 2019 in Minhang Hospital Affiliated to Fudan University were retrospectively analyzed. The data of gender, age, preoperative Child-Pugh grade, preoperative platelet count, preoperative plasma D-dimer concentration, preoperative splenic length, preoperative portal vein velocity, preoperative splenic vein velocity, preoperative portal vein diameter, preoperative splenic vein diameter, treatment times, total number of injection points, total dose of sclerosing agent and tissue adhesive agent were collected. The patients were divided into PVT group and non-PVT group according to the occurrence of non-acute symptomatic PVT within one year after GVI. Univariate analysis was performed first, and then the factors with P<0.2 were included in the binary logistic regression model to screen the risk factors of PVT after GVI. Results:There were 25 cases (37.88%) in PVT group and 41 cases (62.12%) in non-PVT group. There were significant differences in D-dimer concentration, spleen length, Child-Pugh grade and total dose of sclerosing agent between the two groups ( P<0.05). The D-dimer concentration ( OR=2.319, 95% CI:1.359-3.956), spleen length ( OR=1.044, 95% CI:1.007-1.081) and total dose of sclerosing agent ( OR=1.075, 95% CI:1.004-1.152) were independent risk factors for PVT ( P<0.05). Conclusions:Preoperative D-dimer concentration, spleen length and total dose of sclerosing agent can predict the risk of PVT after GVI. In order to reduce the risk of PVT after GVI, the dose of sclerosing agent should be reduced as much as possible.

Objective:To assess the value of ultrasonography in the diagnosis of fetal isomerism syndrome in the first trimester.Methods:Sonographic features of 15 fetuses with isomerism syndrome diagnosed in the Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region from January 2015 to March 2020 were reviewed. Ultrasonic characteristics in the first trimester were analyzed, and the ultrasonic characteristics of early mid-trimester and pathological anatomical results were combined for comparison.Results:There were 6 cases of left isomerism syndrome (LIS) and 9 cases of right isomerism syndrome (RIS) in the 15 fetues.Increased nuchal translucency(NT) (≥3.0 mm, 6 cases), reversed A wave in ductus venosus (10 cases), and atrio-ventricular valve regurgitation (14 cases) were found during the first trimester. There were 14 cases with abnormal visceral laterality. Of the 15 fetues, 14 cases with cardiac malformations, including 6 cases of functional single ventricle, 8 cases of complete atrioventricular septal defect (CAVSD), and 12 cases with great artery abnormalities. All of the 6 LIS cases had bradycardia, 3 cases had interruption of inferior vena cava (IVC). Six cases of RIS had juxtaposition of descending aorta and IVC, and 1 case of RIS had total anomalous pulmonary vein drainage. The major structural malformations were consistent with the early mid-trimester ultrasound examination or autopsy. Karyotype and chromosomal microarray were available in 12 cases and all were normal.Conclusions:Isomerism syndrome has high positive rate of fetal aneuploidies ultrasonographic marker, especially with the atrio-ventricular valve regurgitation, but the risk of chromosome abnormality is low. Ultrasound screening for fetal cardiac structural abnormalities is beneficial to the early diagnosis of isomerism syndrom in the first trimester.

Objective:To explore the feasibility and prognosis of prenatal ultrasonic diagnosis of fetal isolated vascular ring at 11-13 + 6 weeks. Methods:A total of 36 996 fetuses were selected to compare the results of ultrasound screening at 11-13 + 6 weeks and 20-24 weeks of pregnancy in Guangxi Maternity & Child Healthcare Hospital from January 2016 to January 2020, the reasons for missed diagnosis and misdiagnosis of fetal isolated vascular ring by ultrasonography in the first trimester were summarized. Results:Thirty-five cases were diagnosed as isolated vascular ring in the first trimester, including 19 cases right aortic arch combined with aberrant left subclavian artery with "U-shaped" vascular ring, 7 cases double aortic arch with "O-shaped" vascular ring, and 9 cases aberrant right subclavian artery with "C-shaped" vascular ring. While 155 cases were diagnosed as isolated vascular ring at 20-24 weeks of pregnancy, consisting of 18 cases of combined with aberrant left subclavian artery, 9 cases of double aortic arch, 126 cases of aberrant right subclavian artery and 2 cases of pulmonary artery sling, the diagnosis coincidence rates were 94.74%, 77.78%, 7.14%, 0(Kappa value were 0.97, 0.88, 0.13, 0). For the diagnosis of right aortic arch combined with aberrant left subclavian artery, double aortic arch and aberrant right subclavian artery in the first trimester, the specificities were 99.99%, 100%, 100%, the sensitivities were 100%, 77.78%, 7.14%, the false-positive rates were 0.01%, 0, 0, the false-negative rates were 0, 22.22%, 92.86%, and the Youden′s indices were 0.99, 0.78, 0.07.Conclusions:Right aortic arch combined with aberrant left subclavian artery and double aortic arch have high coincidence rate by ultrasonography in the first trimester, while the diagnostic coincidence rate of isolated aberrant right subclavian artery was low. Ultrasound screening for isolated vascular ring in the first trimester still needs ultrasound examination in the second trimester to exclude the minor cardiac malformations that are difficult to diagnose in the first trimester.

Objective:To explore the values of Z-scores of fetal heart circumference (HC) and heart area (HA) in prediction of homozygous α-thalassemia.Methods:From February 2014 to March 2019, 233 fetuses of 15 to 23 gestation weeks with risk of homozygous α-thalassemia were examined by prenatal ultrasound in the Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region. HC, HA and cardiothoracic ratio (CTR) were measured. HC and HA were converted into Z-scores, respectively. The ROC curves were established and analyzed based on HC Z-score, HA Z-score or CTR respectively to compare their predicting efficacies for fetal homozygous α-thalassemia. Finally, the sensitivity, specificity, positive predictive value and negative predictive value were obtained by the best cutoff values.Results:①Sixty-five fetuses were diagnosed as homozygous α-thalassemia and classified as α-thalassemia group. One hundred and sixty-eight fetuses were mild and normal and were classified as control group. ②HC Z-score, HA Z-score and CTR in the α-thalassemia group were significantly higher than those in the control group, and the differences between the two groups were statistically significant ( P<0.001). ③The area under ROC curve of HA Z-score was the largest compared with HC Z-score and CTR, and the prediction efficacy was the highest ( Z test=2.144 and 2.517 respectively, P<0.05). ④The best cutoff values were HC Z-score>1.67, HA Z-score>2.06 and CTR>0.53. Sensitivities of predicting homozygous α-thalassemia in 15 to 23 gestation weeks were 92.31%, 100% and 89.23%, respectively. Specificities were 94.05%, 95.83% and 93.45%, respectively. Positive predictive values were 84.43%, 89.00% and 84.05%, respectively. Negative predictive values were 96.91%, 100% and 95.57%, respectively. Conclusions:Fetal heart HC Z-score and HA Z-score are safe and effective novel ultrasonic indexes for predicting homozygous α-thalassemia. Especially compared with traditional CTR, HA Z-score has a significantly higher predicting efficacy, which can improve the detection rate of homozygous α-thalassemia and reduce the need for invasive examination.

Objective:To construct Z-score ranges for normal fetal middle cerebral artery peak systolic velocity(MCA-PSV).Methods:From May 2017 to October 2019, 865 normal singleton fetuses of 10th to 40th gestational weeks underwent prenatal ultrasound in the Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region. Using fetal biometric parameters as independent variables, and measurement of MCA-PSV on standard section as dependent variables, the regression analyses of the mean(M) and the standard deviation(SD) for each parameter were calculated separately and then the best fitting equation was selected. A group of diseases which might cause the abnormal MCA-PSV were assessed using these standards.Results:①Strong correlations were found between MCA-PSV and fetal biometric parameters ( r=0.935-0.939, P<0.001). ②Quadratic or cubic regression equations were fitted to the models of the means of the MCA-PSV, whereas linear equations were fitted to the SDs. ③In these case groups, intrauterine growth restriction, severe preeclampsia, intrauterine infection and homozygous α-thalassemia-1 demonstrated Z-score>2 reflective of increased MCA-PSV with varying degrees, especially with the homozygous α-thalassemia-1 fetus being the most significant (17/20, 85%). Conclusions:The calculation of Z-score for MCA-PSV as a function of fetal biometric parameters is intuitive and simple, it can be used as an important indicator especially for homozygous α-thalassemia-1.

Objective: To investigate the echocardiographic characteristics, pathological anatomy and genetic abnormality of congenital absent semilunar valves in first trimester. Methods: Eleven cases of congenital absent semilunar valve fetus diagnosed at 11-13^{+ 6} weeks of gestation in Guangxi Magernity & Child Healthcare Hospital from December 2014 to December 2018 were analyzed. The characteristics of echocardiography and the abnormal microanatomy of cardiac tissue after labor induction was compared. Results: The crown-lump length of the 11 fetus was 46-74(62.0±9.2)mm, and nuchal translucency thickness(NT) was 2.4-10.4 (6.4±2.6)mm. The NT of 10 cases were greater than 3.0 mm. Color Doppler flow imaging revealed that biphasic bidirectional flow in the aortic arch and/or pulmonary artery at the 3VT view( "to-and-fro" ) in those 11 cases, and pansystolic turbulence and pandiastolic reflux spectrum were showed on spectral Doppler. Among them, there were 10 cases of " stealing type" , including 2 cases of isolate absent aortic valves, 3 cases of absent pulmonary valves and 5 cases of absent both aortic and pulmonary valves; and all the 10 cases had secondary ultrasonic manifestations of fetal heart failure. Chromosome analysis and detection of genes showed that 4 cases with Trisomy 13 syndrome, 3 cases with Trisomy 18 syndrome, 1 case with 22q11.2 deletion, 1 case with 12q24.32q24.33 deletion and 1 case was normal. Pathological anatomy revealed enlarged heart in 8 cases, isolate absent aortic valves in 2 cases (1 case complicated with pulmonary atresia, absence of ductus arteriosus and thymus), absent pulmonary valves in 3 cases, absent both aortic and pulmonary valves in 3 cases, relics of semilunar valves in 3 cases. And 2 cases of absent pulmonary valves and 3 cases of absent both aortic and pulmonary valves with short and thick ductus arteriosus. Only 1 case was congenital absent semilunar valve in " non-stealing type" without secondary ultrasonic manifestations of fetal heart failure. Chromosome analysis and detection of genes showed 22q11.2 deletion, and there were relics of semilunar valves in the pathological anatomy. It also combined with tetralogy of Fallot and absence of ductus arteriosus. Conclusions In first trimester, congenital absent semilunar valves are more common as " stealing type" . The echocardiographic features of congenital absent semilunar valves are the " in-out sign" of aorta arch and/or pulmonary artery and biphasic spectrum in spectral Doppler. Trisomy 13 syndrome and trisomy 18 syndrome significantly increased the risk of congenital absent semilunar valves in " stealing type" in first trimester.

Objective To evaluate the position of the fetal conus medullaris during pregnancy and its value in detecting tethered cord syndrome(TCS). Methods Nine hundred and seventy-four normal fetuses and 46 fetuses with TCS between 15 and 41 weeks gestation were involved in the study.Parameters D 1 (the distance between the end of the conus medullaris and the caudal edge of last vertebral body ossification center) and D2 (the distance from the end of the conus medullaris to the caudal skin namely the intersection point of the extending line of D1 and the skin) were measured in the caudal midsagittal plane of the spine. Sixty normal fetuses were chosed randomly for interobserver variability.Correlation analysis between these two parameters and gestational age(GA) were conducted and the normal reference value of these parameters were calculated in normal group. The ratios of growth parameters ( Biparietal diameter, Head circumference,Abdominal circumference,Femur length) to D1 and D2 were calculated separately to observe the difference of the ratios between two groups. All the parameters and ratios of normal fetuses were compared with that of TCS cases.Results There was no significant difference in D1 and D2 between two observers.A significant linear correlation between the parameters and GA was found in normal group,linear regression equations were D1=0.251 GA -2.265 cm (R2=0.926,P <0.01) and D2=0.267 GA -1.812 cm(R2=0.928,P <0.01),respectively.D1 and D2 were much lower in normal group than in abnormal group (all P <0.01). The ratios of the growth parameters to D1 and D2 were relatively stable and had statistically differences between two groups in different gestational age. Conclusions The methods that determination of D1 and D2 are simple and feasible,and could help to the prenatal diagnosis of TCS.

Objective To evaluate the efficacy of the ratio of the fetal cardiac diameter to biparietal diameter( CBR) as a predictor of homozygous α-thalassemia-1 . Methods Single mid-pregnancies ( 15-22weeks) at risk of homozygous α-thalassemia-1 were enrolled . A total of 251 singleton pregnancies were recruited ,in which 63 cases were homozygous α-thalassemia-1 fetuses and the rest were unaffected . The CBR and cardiothoracic ratio(CTR) were measured by two-dimensional ultrasound . Then the accuracy of these variables were analyzed and compared with each other by ROC curves . Results ①The CBR and CTR in affected fetuses were significantly higher than those in the unaffected( P ＜0 .01) . ②With CBR＞0 .43 and CTR ＞ 0 .52 as the best cut off values ,the sensitivity and specificity of predicting homozygous α-thalassemia-1 fetuses in 15-22 gestational weeks were 95 .74% , 92 .06% and 94 .15% , 85 .71% , respectively ;the area under ROC curve were compared with Z test and there was no significant difference between them ( Z ＝ 1 .500 , P ＝ 0 .1335) . ③ When CBR and CTR were combined ,the sensitivity and specificity of the prediction were significantly increased ( the sensitivity of series experiment : 99 .75% ,the specificity of parallel experiment : 98 .87% ) . Conclusions CBR is a novel , effective and noninvasive predictor of homozygous α-thalassemia-1 in mid-pregnancy whose prediction efficiency is the same as traditional CTR . The measurement of CBR is easier to standardize and is not affected by thoracic lesions such as pleural cavity ,pericardial effusion and skeletal dysplasia . If combined with CTR ,it may play an important role in improving the prenatal detection rate of homozygous α-thalassemia-1 fetuses .

Objective To evaluate the efficacy of fetal heart diameter(HD)Z-score as predictors of homozygous α-thalassemia-1.Methods Two hundred and fourteen cases of Single mid-pregnancies(1 5-22 W)at risk of homozygous α-thalassemia-1 were enrolled.Fetal HD were first measured.Next,the Z-scores of HD were calculated separately based on previously constructed Z-score models.Finally,the accuracy of this variable was analyzed and compared with that from the cardiothoracic ratio(CTR)by ROC curves analysis.Results ①A total of 214 singleton pregnancies were recruited in which 57 cases were homozygous α-thalassemia-1 fetuses and the other 157 cases were unaffected.②The affected fetal HD and Z-score were significantly higher than those in the unaffected fetuses(P <0.01).③With the HD Z-score >2.76 as the best cutoff value,the sensitivity and specificity of predicting homozygous α-thalassemia-1 fetuses in 1 5-22 gestational week were 92.98% and 100%;If a best cut-off value of CTR >0.52 was used for prediction,the sensitivity was 87.72% and the specificity was 91.72%.Compared with CTR,the discriminative power of HD Z-score was better(Z value=2.286,P <0.01).Conclusions HD Z-score is a novel,effective and noninvasive predictor of homozygous α-thalassemia-1 in mid-pregnancy.Its prediction efficiency is higher than that of traditional CTR.It can improve the prenatal detection rate of homozygous α-thalassemia-1 fetus,reduce unnecessary invasive operation and save expenses.