Purpose To explore the clinical application value of dual-layer detector spectral CT combined with half volume contrast agent in detecting the liver metastases in portal vein phase.Materials and Methods Ninety-six patients with liver metastases diagnosed by liver enhanced CT in the First Affiliated Hospital of Fujian Medical University from November 2021 to June 2022 were prospectively studied.They were randomly divided into two groups by using the contrast agent administration calculation method based on body surface area.The conventional polyenergetic image(CI)of patients in the conventional contrast agent group(48 cases with 18.6 gI/m2 body surface area iodine contrast agent)was constructed in the portal vein phase,and the 40-70 keV(5 kiloelectron voltage)virtual monoenergetic imaging(VMI)was reconstructed for patients in the semi contrast agent dose group(48 case with 9.3 gI/m2 body surface area iodine contrast agent).The image noise,CT value and contrast noise ratio in the two groups were measured and compared.Besides,the image quality was scored with a 5-point method,and the consistency of subjective scores between two radiologists were evaluated.Results The VMI noise at 40-70 keV was(10.26±1.38)Hu,(9.59±1.35)Hu,(9.15±1.31)Hu,(8.80±1.32)Hu,(8.58±1.31)Hu,(8.40±1.31)Hu and(8.27±1.33)Hu;while the CI noise was(10.86±1.13)Hu.It revealed that the VMI noise at 45-70 keV was lower than the CI noise(t=-3.885,-5.343,-6.449,-7.180,-7.756 and-8.132,P<0.001).There was no significant difference between VMI and CI noises at 40 keV(t=-1.800,P>0.05).With the decrease of energy level,CT value of normal liver parenchyma,and contrast noise ratio values of liver parenchyma and liver metastases gradually increased,at the 40 keV level,the values of which were(148.31±21.34)Hu,8.81±2.83 and 8.18±2.85;at the 45 keV level,the values were(130.10±16.95)Hu,7.66±2.46 and 7.48±2.55;at the 50 keV level,the values were(116.19±13.55)Hu,6.60±2.08 and 55.40±13.77,reaching the highest at 40 keV level,while the CI values were(118.02±7.02)Hu,5.69±1.11 and 5.77±1.41.Therefore,the value of 40 keV and 45 keV VMI was significantly higher than CI(t=3.804,4.883,4.462,all P<0.05;t=2.854,3.465,3.378,all P<0.05),respectively.There was no significant difference between 50 keV VMI and CI(t=-0.612,1.739,2.208,P>0.05).The subjective score in the overall quality of VMI images was higher than that of CI at 40-50 keV(t=9.628,7.508,3.514,P<0.05).The subjective scores of the two physicians were good consistent among image contrast,image noise and overall image quality(Kappa=0.766,0.749 and 0.661,P<0.05),respectively.Conclusion VMI at 40-50 keV can provide better image quality and display ability of liver metastases than CI images at portal vein phase with a 50%reduction in contrast agent dose.

OBJECTIVE: To explore the diagnosis, treatment and genetic characteristics of a neonate with severe pulmonary hypertension and respiratory failure. METHODS: Perinatal history, clinical manifestations, laboratory finding and diagnosis and treatment data of the child were collected. Whole exome sequencing was carried out for the child, and Sanger sequencing was used to verify the candidate variants. RESULTS: The female neonate has developed progressive respiratory failure and refractory pulmonary hypertension shortly after birth. Conventional treatment such as mechanical ventilation, vasoactive drugs, and inhaled nitric oxide were ineffective. She has developed sustained pulmonary hypertension after weaning from extracorporeal membrane oxygenation therapy, and had died after the treatment had ceased. Whole exome sequencing revealed that she has harbored a heterozygous de novo variant of c.682_683insGCGGCGGC (p.G234Rfs*148) of the FOXF1 gene, which was predicted as pathogenic based on guidelines from the American College of Medical Genetics and Genomics (ACMG), with evidence items of PVS1_Strong+PM2_Supporting+PS2. Based on her clinical manifestations and result of genetic testing, the child was diagnosed with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). CONCLUSION Discovery of the c.682_683insGCGGCGGC (p.G234 Rfs*148) variant of the FOXF1 gene has expanded the mutational spectrum of the FOXF1 gene, which has facilitated implementation of specific treatment and provided a basis for clinical diagnosis and genetic counseling.
Female ; Humans ; Child ; Infant, Newborn ; Pregnancy ; Persistent Fetal Circulation Syndrome/therapy* ; Hypertension, Pulmonary ; Pulmonary Veins ; Forkhead Transcription Factors/genetics*

Objective:To investigate the reproducibility of whole-heart and volume-targeted balanced steady-state free precession (bSSFP) non-contrast MR coronary angiography (CMRA) for displaying coronary trunks.Methods:From February and September 2021, the whole-heart and volume-targeted CMRA examinations of 58 volunteers were prospectively and consecutively acquired in The First Affiliated Hospital of Fujian Medical University. Each volunteer underwent CMRA twice within a week. The subjective score, vessel-to-myocardium ratio (VMR), vessel-to-fat ratio (VFR), signal-noise ratio (SNR), and coronary corresponding coordinate was analyzed and extracted. Inter-observer, intra-observer and inter-scan consistency were evaluated by intraclass correlation coefficient (ICC), Bland-Altman analysis, Hausdorff Distance (HD), and Dice Similarity Coefficient (DSC).Results:The inter-observer and intra-observer consistencies of subjective scores, VMR, and VFR of the whole-heart and volume-targeted coronary artery images were excellent (ICC>0.76, P<0.001). The inter-scan VFR consistencies of RCA, LM, and LCX of whole-heart coronary scans were moderate (ICC=0.235, 0.264, 0.380, all P<0.05), while the consistencies of the remaining variables were good, (all ICCs>0.49, P<0.001). Bland-Altman method showed that most VMR, VFR, and SNR of two CMRA imaging were within the 95% limits of agreement. Whole-heart CMRA inter-and intra-observer mean HD was 1.79 (1.35, 3.25), 1.68 (1.09, 4.10), mean DSC was 0.96±0.04, 0.97±0.03. Volume-targeted CMRA inter-and intra-observer mean HD were 1.74 (1.63, 3.11), 1.74 (1.63, 1.98), and the mean DSC was 0.91±0.10, 0.95±0.05. The subjective score of raw images of the total artery trunk of volume-targeted CMRA [3.86 (3.68,4.00) vs. 3.80 (3.58,3.96) ], VMR [1.45 (1.27,1.58) vs. 1.22 (1.13,1.41) ], and VFR [7.36 (6.44,8.60) vs. 5.97 (4.97,6.64) ] were better than those of whole-heart CMRA (all P<0.05). The overall subjective score of whole-heart CMRA coronary trunk curved projection reformation was better than volume-targeted CMRA [3.75 (3.57, 3.88) vs. 3.63 (3.44, 3.71)] ( P<0.001). Conclusions:Whole-heart and volume-targeted bSSFP non-contrast CMRA represent good reproducibility and image quality in the main coronary artery of healthy volunteers. Both of the two methods have their advantages and complement each other.

Objective:To understand the change trend and influencing factors of fecal calprotectin(FC) in very low birth weight(VLBW) infants, and to explore the application value of FC detection in the diagnosis of necrotizing enterocolitis(NEC) in VLBW infants.Methods:VLBW infants hospitalized in the neonatal department at Quanzhou Children′s Hospital from June 2018 to May 2019 were selected as research object for a prospective study.Fecal samples from the 1st, 7th, 14th, 21st, 28th and 35th days after birth and fecal samples from the acute and recovery stages of NEC were collected continuously.The content of FC was determined quantitatively by immunofluorescence assay.Results:(1) The FC level of non NEC VLBW infants from 1 to 35 days after birth was 143.5(47.8, 391.2) μg/g.Univariate analysis showed that the level of FC fluctuated with the postnatal age, the level of FC was the highest at 21 days, and then decreased.The level of FC increased significantly in formula feeding, premature rupture of membranes, neonatal sepsis, feeding intolerance and pregnant mothers without glucocorticoid before delivery( P< 0.05). (2) Multivariate covariance analysis showed that prenatal application of glucocorticoid( F=10.550, P=0.001), premature rupture of membranes( F=13.311, P<0.001), neonatal sepsis( F=8.001, P=0.005), feeding intolerance( F=4.751, P=0.030) and NEC( F=54.566, P<0.001) had significant effects on FC level.After controlling the effects of prenatal corticosteroid, premature rupture of membranes, neonatal sepsis and feeding intolerance, the levels of FC in NEC group and non-NEC group were 3 162.3(1 412.5-7 244.4)μg/g and 141.3(125.9-162.2)μg/g, respectively.In NEC group, the levels of FC in acute stage and recovery stage were 3 166.9(1 745.1, 6 806.4)μg/g and 130.9(97.4, 273.9)μg/g, respectively, with significant difference( t=10.304, P<0.001). While the levels of FC were 2 347.9(1 404.4, 5 893.4)μg/g in the mild NEC and 4 114.7(2 764.5, 9 208.4)μg/g in the moderate or severe NEC, respectively, with no significant difference( t=1.131, P=0.280). Conclusion:The levels of FC fluctuate with postnatal age and it is affected by multiple factors.FC maybe a useful marker for the diagnosis and evaluation of efficacy of NEC in VLBW infants.

Objective To explore the value of amplitude integrated electroencephalography (aEEG) in cerebral function monitoring in preterm infants younger than 30 weeks of gestation. Methods A total of 165 cases of preterm infants younger than 30 weeks of gestation were prospectively enrolled in the study from September 2015 to February 2017,including 20 preterm infants with severe brain injury ( severe brain injury group) and 145 with non-severe brain injury ( non-severe brain injury group). Five aspects of each tracing, such as continuity(Co),sleep-wake cycling( Cy),amplitude of the lower border(LB),bandwidth( B) and total maturation scores,were evaluated and compared between two groups by applying a preterm infants aEEG scoring system. The neuromotor development of preterm infants survivors was assessed by using the 0 to 6 years old children′s neurological and psychological development scale of China Capital Institute of Pediatrics. Clinical data were collected and compared with the results of aEEG. Results The scores for Co,Cy,LB,B as well as total maturation scores of preterm infants with severe brain injury at different gestational age were lower than those of infants with non-severe brain injury at the same gestational age. The differences were sta-tistically significant (all P<0. 05). There were no statistical differences in Co,Cy,LB,B scores and total maturation scores between each gestational ages in severe brain injury group (all P>0. 05). Scores for Co, Cy,B and total maturation scores progressively increased with advancing gestational age in non-severe brain injury group( all P <0. 05),but there were no statistical differences between each gestational ages in LB scores(all P>0. 05). Follow-up results of 16 cases with severe brain injury:15 cases of DQ≤69,1 case of DQ 70－84,and no cases of DQ≥85; 130 cases of non-severe brain injury group:78 case of DQ≥85,42 cases of DQ 70－84,10 cases of DQ≤69. By chi-square analysis and Spearman rank correlation analysis,the results of aEEG total maturation scores were correlated with outcome of these preterm infants younger than 30 weeks of gestation ( r ＝0. 702,P <0. 05). Conclusion aEEG can provide important information of the status of cerebral function in preterm infants younger than 30 weeks of gestation and help to predict their outcome.

Objective To study the clinical effects of continuous blood purification (CBP) in the treatment of newborns with severe sepsis and multiple organ dysfunction syndrome (MODS). Method From May 2013 to November 2018, the clinical data of infants with severe sepsis and MODS receiving CBP in the neonatal department of our hospital were retrospectively analysed. Changes of blood pressure, arterial partial pressure of oxygen/inhaled oxygen concentration (PaO2/FiO2), pH, serum potassium, sodium, urea nitrogen, creatinine, urine volume and maintenance dose of adrenaline at different time points before and after CBP were analysed. Result According to the inclusion and exclusion criteria, a total of 8 newborns with sepsis and MODS were enrolled in the study. One patient had the complication of perforated colon, and the other one had acute renal failure. The number of affected organs in these infants was 3～5. Six cases began CBP treatment within 1～5 days after admission, and the other two cases began CBP treatment on the 38th and 47th days after admission. The average treatment duration was (58.6±25.9) h. The effective rate of CBP in the treatment of severe sepsis with MODS was 75.0％(6/8). Blood pressure was increased at 6 h, 12 h, 24 h and 48 h after treatment and at the end of treatment. PaO2/FiO2 and blood pH were increased. The urine volume was increased at 24 h and 48 h after treatment and at the end of treatment (P<0.05). Serum potassium, urea nitrogen and creatinine were significantly decreased (P<0.05). The maintenance dose of adrenaline was also decreased significantly at 12 h after CBP (P<0.05), and withdrawn at 48 h after treatment. Only one case experienced membrane blockage during CBP. Dialysis was continued after the replacement of filtration membrane. No other complications existed. Conclusion CBP is effective in the treatment of neonatal severe sepsis with MODS. It can improve the circulation and renal function.

Objective To study the expression of intestinal intestinal fatty acid-binding protein (I-FABP) and its clinical significance in an experimental model of necrotizing enterocolitis (NEC) in neonatal rats. Method Twenty-four neonatal Sprague-Dawley (SD) rats were randomly assigned into 4 groups at 48 h after birth (6 rats in each group):group A (control group), group B (NEC group-1), group C (NEC group-2), and group D (NEC group-3). The neonatal rats were fed by the mother rats in the same cage within 48 h after birth. After 48 h, the NEC group received artificial feeding, hypoxia, cold stimulation and lipopolysaccharide (LPS) gavage (10 mg/kg). NEC group-1, 2 and 3 were sacrificed on an empty stomach at 1, 2 and 3 d after the modeling. The control group was sacrificed on an empty stomach 3 d after the modeling without special treatment. Intestinal tissue were obtained from each rats. The histological changes of ileal tissues were studied using hematoxylin-eosin (HE) staining. The expressions of intestinal I-FABP were detected using RT-PCR and ELISA methods. Result Compared with the control group, body weight of rats in NEC group-1, 2 and 3 were lower, and pathology scores in these three groups were higher (P<0.05). The levels of intestinal I-FABP mRNA in NEC group-1, NEC group-2 and NEC group-3 were 2.69±0.27, 2.12±0.09, 3.18± 0.22, respectively. The protein expression levels were 363.7 ± 11.4, 321.7 ± 45.8, 432.3 ± 50.3, respectively. The mRNA and protein levels were all significantly higher than the control group (mRNA: 1.00 ± 0.02, protein: 134.2 ± 24.0, P<0.05). Conclusion I-FABP was a useful marker for ischemic injury to the intestine. These findings may contribute to a better diagnosis of NEC in newborns.

Objective To analyze the role of intestinal fatty acid-binding protein (I-FABP) expression in a neonatal rat model of necrotizing enterocolitis (NEC).Methods A total of 24 newborn rats were randomly divided into two groups:control group (n=6) and NEC group (n=18).Rats in the NEC group were fed with formula and experienced hypoxia,reoxygenation,cold stress and sequentially Lipopolysaccharide (10 mg/kg) lavage for three consecutive days to establish NEC model,after which were respectively sacrificed on day 1,2 and 3 (six for each day).Those in the control group were all sacrificed on day 3.Ileocecal tissues were collected for morphological and histological analysis.I-FABP expression was detected using Western blot and immunohistochemistry (IHC).One-way analysis of variance,LSD-t test,Kruskal-Wallis H test,Mann-Whitney U test and Pearson's correlation analysis were used for statistical analysis.Results The NEC model (intestinal pathological score ≥ 2) was established successfully without causing death.Compared with the control group,the NEC group showed less body weight gain [M (P25-P75):1.00 (0.48-1.35) vs 1.74 (1.62-1.86),1.25 (0.75-1.40) vs 2.61 (2.53-2.99),1.35 (0.88-1.48) vs 3.60 (3.48-3.73);Z=-2.898,-2.903,-2.892;all P＜0.05] and higher intestinal pathological scores [(2 (2-3),3 (2-3),4 (3-4) vs 0 (0-1);all P＜0.05] on day 1,2 and 3.The intestinal pathological score on day 3 was significantly higher than that on day 2 and day 1 (both P＜0.05).Expression of I-FABP and the number of I-FABP positive enterocytes in the NEC model group were increased compared with those in the control group [Western blot:0.179 (0.179-0.186),0.231 (0.211-0.245),0.202 (0.192-0.225) vs 0.091 (0.086-0.093);IHC:59 (55-60),80 (83-86),80 (84-88) vs 44 (39-47);all P＜0.05].Moreover,the expression of I-FABP protein and the number of I-FABP positive enterocytes on day 2 and day 3 were significantly higher than those on day 1 (all P＜0.05).I-FABP expression was positively associated with intestinal pathological score (Western blot:r=0.932,95％CI:0.872-0.969;IHC:r=0.709,95％CI:0.484-0.872).Conclusions I-FABP is an efficient marker for NEC and correlates with the severity of intestinal injury.

Objective: To investigate the effectiveness and safety of intraoperative sac coiling embolization among patients who are athigh-risk developing type-Ⅱ endoleak after endovascular aortic repair(EVAR). Methods: From Jan 2014 to Jan 2018, one hundred and twelve consecutive patients with infra-renal AAA were enrolled for this study, There were 76 patients undergoing standard EVAR (standard-group)and 36 patients doing aneurysmal sac coiling embolization (embo-group). Baseline characteristics, aneurysmal sac parameters, radiological intervention details and follow up results were recorded. Results: Mean follow-up time was 25.9 m for embo-group. During follow-up period, no coils-related complications were noted and no type-Ⅱ endoleak associated secondary interventions were reported. A mean of (2.72±1.16) coils (range 1-7) was used in the embo-group. The incidence of type Ⅱ endoleak was 30.3%(23/76) in standard-group and 11.1%(4/36) in embo-group(χ²=4.90, P=0.027). Logistic multivariate analysis revealed that the independent risk factors of type Ⅱ endoleak after endovascular aortic repair for high-risk patients were those EVAR without sac embolization and sac volume≥128 cm³. In the subgroup analysis (sac volume≥128 cm³), the incidence of type-Ⅱ endoleak was lower in embo-group compared to standard-group (χ²=6.07, P=0.014). Conclusion Intraoperative sac coiling embolization in high-risk patients is safe and effective in prevention of type Ⅱ endoleak. This preventive effect is more significant with large sac aneurysm compared to small sac aneurysm.

Objective To study the clinical value of transcutaneous O2 (TcPO2) and transcutaneous CO2 (TcPCO2) monitoring among infants with respiratory failure.Method From August 2017 to February 2018,neonates with respiratory failure treated with nasal continuous positive airway pressure (NCPAP) or mechanical ventilation (MV) in the neonatal department were prospectively enrolled.At four time points of 30 min,6 h,24 h after respiratory support and before discharged,TcPO2 and TcPCO2 were compared with PaO2 and PaCO2 using the correlation and consistency analysis methods.Result A total of 368 paired samples from 92 infants were collected.The correlations of TcPCO2 and PaCO2 at 30 min,6 h,24 h and before discharged were strong (r =0.790,95 ％ CI 0.656 ～ 0.884;r =0.827,95 ％ CI 0.710 ～ 0.908;r =0.901,95 ％ CI 0.867 ～ 0.932;r =0.905,95 ％ CI0.830 ～ 0.954,P ＜ 0.05).The correlations of TcPO2 and PaO2 at 30 min,6 h,24 h were weak (r =0.629,95％ CI 0.461 ～ 0.767;r =0.638,95％ CI 0.465 ～ 0.793;r =0.739,95 ％ CI 0.619 ～ 0.831),but strong before discharged (r =0.886,95 ％ CI 0.818 ～ 0.934).Conclusion When tissue well perfused,TcPCO2 is an accurate,continuous and noninvasive marker to evaluate the PaCO2 of neonates with respiratory failure.But TcPO2 cannot reflect PaO2 accurately,and the combination of other index of oxygenation should be used.