Objective:To evaluate right atrial (RA) volume and function in patients with hepatitis B cirrhosis by four-dimensional automatic quantitation analysis technique, and to explore its correlation with liver function grading.Methods:Ninety patients with hepatitis B cirrhosis who were clinically confirmed in Henan Provincial People′s Hospital from December 2020 to July 2021 were randomly enrolled as the research subjects. According to Child-Pugh liver function score criteria, the patients were divided into three groups: Child-Pugh A group ( n=31), Child-Pugh B group ( n=31), Child-Pugh C group ( n=28). Another 30 healthy volunteers with gender and age matched at the same period were selected as the control group. The RA maximum volume (RAVmax), RA minimum volume (RAVmin), RA presystolic volume (RAVpreA), RA maximum volume index (RAVImax), RA emptying volume (RAEV), RA ejection fraction (RAEF), RA reservoir longitudinal and circumferential strains (RASr, RASr-c), RA conduit longitudinal and circumferential strains (RAScd, RAScd-c), RA contraction longitudinal and circumferential strains (RASct, RASct-c) were obtained by four-dimensional automatic quantitation analysis technique. Spearman correlation analysis was used to explore the correlation between the volume and strain parameters of right atrium and liver function grading, and the independent correlation factors of RASr-c were analyzed by univariate and multivariate linear regression analyses. Results:Compared with the control group, Child-Pugh A and Child-Pugh B groups, RAVmax, RAVmin, RAVpreA, RAVImax, and RAEV were all increased in Child-Pugh C group (all P<0.05). Compared with control group, the absolute values of RASr, RASct, RASr-c and RASct-c were increased, and the absolute values of RAScd and RAScd-c were decreased in Child-Pugh A group (all P<0.05). Compared with control group, Child-Pugh A and Child-Pugh B groups, the absolute values of RASr, RAScd, RASct, RASr-c, RAScd-c and RASct-c were decreased in the Child-Pugh C group (all P<0.05). Correlation analysis showed that the volume and strain parameters of the right atrium were correlated with liver function grading. RASr-c was independently correlated with E/e, ALB and BNP(β=-0.543, 0.521, and -0.562 respectively, all P<0.05). Conclusions:During the compensatory stage in patients with hepatitis B cirrhosis, the RA functions of reservoir and contractile were increased, but the function of conduit was decreased. With the aggravation of liver cirrhosis, the RA functions of reservoir, conduit and contractile were all decreased. The four-dimensional volume and strain parameters of the right atrium were correlated with Child-Pugh liver function grading, and RASr-c was independently correlated with E/e, ALB and BNP.

Objective:To investigate the change of left atrial volume and function in patients with hypertensive disorders of pregnancy (HDPs) by four-dimensional automatic left atrial quantitative analysis (4D LAQ) and analyze the predictive value of risk stratification.Methods:A total of 60 patients diagnosed with hypertensive disorder of pregnancy in Henan Provincial People′s Hospital from March to December 2021 were randomly enrolled, which were divided into gestational hypertension group (low risk group, n=30) and preeclampsia group (medium and high risk group, n=30) according to the disease development and risk stratification method reported in the literature; another 30 healthy pregnant women matched for age, gestational weeks and body mass index were selected as the control group. Left atrial anteroposterior diameter (LAd), interventricular septum thickness (IVSd), left ventricular end diastolic diameter (LVd), left ventricular ejection fraction (LVEF), left ventricular myocardial mass index (LVMI), peak early diastolic (E) and late diastolic (A) velocities of mitral inflow, and e′ values on the septal and lateral mitral annulus sides were routinely measured to calculate E/A and E/e′ values. Left atrial volume and strain parameters were obtained using 4D LAQ technique, including left atrial minimum volume (LAVmin), left atrial maximum volume (LAVmax), left atrial presystolic volume (LAVpreA), left atrial maximum volume index (LAVImax), left atrial inflation index (LAEI), left atrial stroke volume (LAEV), left atrial fraction (LAEF), longitudinal strain of left atrial reserve, conduit and systolic period (LASr, LAScd, LASct), circumferential strain of left atrial reserve, conduit and systolic period(LASr-c, LAScd-c, LASct-c). The differences among the three groups were compared. Multiple Logistics regression analysis was used to obtain the relevant indicators of risk stratification of HDPs and ROC curves were used for assessment. Results:Compared with the control group, E/e′, LAVmin, LAVpreA, LAScd, and LAScd-c increased, and LAEI, LAEF, LApEF, LASr, and LASr-c decreased in the gestational hypertension group (all P<0.05). Compared with the control group and gestational hypertension group, LAd, IVSd, LVd, LVMI, E/e′ LAVmin, LAVmax, LAVpreA, LAVImax, LAEV, LAScd, and LAScd-c increased, and LVEF, LAEF, LAEI, LApEF, LASr, and LASr-c decreased in the preeclamptic group, and the differences were statistically significant (all P<0.05). The results of multiple Logistics regression showed that LAVmax, LAScd-c and LASr were the indicators relevant to risk stratification of HDPs(β=0.344, 0.216 and -0.249, respectively, all P<0.05). ROC analysis showed when the cut-off value of left atrial strain parameter LASr was 30.5%, the AUC, sensitivity, and specificity were 0.725, 0.58, 0.90, respectively; when the cut-off value of LAVmax was 44.5 ml, the AUC, sensitivity, and specificity were 0.662, 0.80, and 0.56, respectively; and when the cut-off value of LAScd-c was -17.5%, the AUC, sensitivity, and specificity were 0.706, 0.56, and 0.78, respectively. Conclusions:Left atrial remodeling occured in pregnant women with hypertensive disorders, their reserve and conduit function were impaired, and aggravated with the progress of the disease. The four-dimensional parameters LASr, LAVmax, and LAScd-c were relevant indicators for risk stratification of HDPs. In predicting the severity of HDPs, LASr has high diagnostic value and good specificity; LAVmax and LAScd-c can be considered as supplementary parameters to predict the risk stratification of HDPs.

Objective: To investigate the profiles of blood amino acid and acylcarnitine in early neonates with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and the sensitivity of newborn screening, and to explore potential biochemical metabolic markers for newborn screening program. Methods: Amino acid and acylcarnitine profiles in dried blood spots of newborn screening program were analyzed by tandem mass spectrometry (MS/MS). A total of 158 651 neonates born in Guangzhou from January 1, 2015 to June 30, 2019 were enrolled in this newborn screening program, and additionally 55 patients with NICCD confirmed by SLC25A13 gene analysis in Guangzhou Women and Children Medical Center were included in this study. NICCD screen-positive was defined as the cutoff value of citrulline (Cit) ≥ 30 μmol/L. The values of blood sampling time of the true positive group and those of the false negative group were compared by t-test. The levels of amino acid and acylcarnitine among different groups, including true positive group (Cit≥30 μmol/L), false negative group (Cit 21-<30 μmol/L and Cit<21 μmol/L) and the normal control group, were analyzed by F test, respectively. Results: Among 158 651 neonates, 39 neonates were positive for NICCD screening. Three of them were confirmed NICCD and 4 cases were found to be false negatives. The positive predictive value was 7.7% and the sensitivity was about 43.0%. Among 55 patients with NICCD, 18 cases (18/55, 32.7%) were true positives and 37 cases (37/55, 67.3%) were false negatives based on the cutoff value of citrulline in the dried blood spots for newborn screening. The blood sampling time was significantly different between true positive group and false negative group ((4.28±1.6) vs. (2.98±0.74) d, t=4.06, P<0.01). The increased levels of tyrosine((176.0±98.4) μmol/L), methionine ((37.0±26.9) μmol/L) and phenylalanine ((133.0±80.9)μmol/L) in Cit≥30 μmol/L group (n=18) were significantly different as compared with those in the other three groups, respectively (F=117.0, 58.5, 135.0, P<0.01). The levels of arginine ( (10.0±9.2) , (11.0±9.3) , (9.0±17.8) μmol/L), valine ( (119.0±29.8) , (107.6±14.1) , (102±68) μmol/L) and leucine ( (167.0±37.1) , (161.0±37.7) , (163.5±180.6) μmol/L) were not statistically significant among groups of Cit≥30 μmol/L(n=18), Cit21-<30 μmol/L(n=7) and Cit<21μmol/L(n=30,P>0.05), but they were significantly higher than those of the normal control group ((4±3), (78±21), (114.0±31.5) μmol/L, n=1 000), respectively(F=30.1, 23.0, 29.8, P<0.01). Alanine (Ala) ( (150±50) , (156.0±30.2), (168±105), (152±52) μmol/L) levels showed no significant difference (F=0.86, P>0.05) but the ratios of Ala/Cit (1.52±1.44, 6.82±1.56, 12.06±7.71, 19.42±6.27) decreased significantly among the four groups (F=69.0, P<0.05). The acylcarnitine levels showed no statistically significant results among the different groups (P>0.05). With Cit≥30 μmol/L and Ala/Cit<7.5 as cutoff values, the number of screen-positive cases reduced from 39 to 22 cases with no additional false negative case. With Cit≥21 μmol/L and Ala/Cit<7.5 as cutoff values the number of screen-positive cases increased to 117 cases with 1 additional true positive. Conclusions The profiles of blood amino acid in early neonates with NICCD present the increased levels of multiple amino acids including citrulline, tyrosine, methionine and phenylalanine, and decreased ratio of Ala/Cit. Taking citrulline and ratio of Ala/Cit as screening markers can improve the positive predictive value appropriately. The limited sensitivity of NICCD newborn screening may be related to early blood sampling time.