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Objective To observe the value of shear wave elastography(SWE)for evaluating hepatic iron overload in children with β-thalassemia major(β-TM),as well as the correlations of relative parameters with MR T2*value and serum ferritin.Methods Totally 96 children with β-TM and 100 healthy children(control group)were retrospectively enrolled.Children with β-TM were divided into hematopoietic stem cell transplantation(HSCT)group(n=41)or non-HSCT group(n=55)according to underwent HSCT or not.SWE parameters were compared among groups.Spearman correlation was performed to observe the correlations of liver shear wave velocity with MR T2*value and serum ferritin,as well as Young's modulus with MR T2*value and serum ferritin in children with β-TM.Results Liver shear wave velocity(LSWV)and Young's modulus in HSCT group and non-HSCT group were all higher than those in control group(all P＜0.001).No significant difference of LSWV nor Young's modulus was found between HSCT group and non-HSCT group(both P＞0.05).SWE parameters of children with β-TM were moderately and negatively correlated with MR T2*value(r=-0.501,P＜0.05;r=-0.514,P＜0.05),while weakly and positively correlated with serum ferritin(r=0.488,P＜0.05;r=0.470,P＜0.05).Conclusion SWE was helpful for evaluating hepatic iron overload in children with β-TM,with parameters being negatively correlated with MR T2*value and positively correlated with serum ferritin.
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Objective:To assess the altered right atrial(RA) function using two-dimensional speckle-tracking imaging(2D-STI) in patients with persistent pulmonary hypertension of the newborn(PPHN), and investigate the diagnostic value of different right heart strain parameters in PPHN.Methods:Ultrasound images of 42 newborns with clinically confirmed diagnosis of PPHN in the neonatal intensive care unit of Shenzhen Children′s Hospital (median gestational age 37 + 1 weeks, median age 6 d) and 30 normal newborns (control group, median gestational age 37 + 3 weeks, median age 6.5 d) from January 2020 to January 2023 were retrospectively analyzed, all with gestational age ≥34 gestational weeks. 2D-STI was applied to evaluate RA function: RA strain(RA LS) and area change fraction(RA FAC), where RA LS included RA reservoir strain (εs), RA conduit strain (εe) and RA active contract strain (εa), while evaluating right ventricle(RV) function: RV global longitudinal strain(RV GLS), RV FAC, etc. And the degree of pulmonary artery pressure(PAP) was assessed by tricuspid regurgitation velocity(TRV). The above parameters were compared between the PPHN group and the control group, and the correlations between RA function parameters, RV function parameters and PAP in PPHN group were analyzed. The area under ROC curve (AUC) was used to compare the accuracy of each parameter in the evaluation of impaired cardiac function in PPHN patients. Results:Compared with the control group, RA function (εs, εe, εa and RA FAC) were impaired in PPHN patients (all P<0.05). εs was positively correlated with RV GLS, RA FAC ( r=0.494, 0.356, both P<0.05) and negatively correlated with minimum right atrial area (RAA min), pulmonary artery diameter (PAD), and tricuspid annular internal diameter ( rs=-0.285, r=-0.495, -0.396; both P<0.05); εe was negatively correlated with PAD ( rs=-0.256, P<0.05); εa was positively correlated with RV GLS ( r=0.499, P<0.05) and negatively correlated with PAD and tricuspid annular internal diameter ( r=-0.390, -0.380; both P<0.05); RA FAC was positively correlated with RV GLS ( r=0.365, P<0.05) and negatively correlated with PAD and tricuspid annular internal diameter ( r=-0.439, -0.328; both P<0.05). RA LS and RA FAC had no correlations with TRV-estimated PAP ( P>0.05). ROC analysis showed that the sensitivity and specificity of εs<40.50% for diagnosing PPHN was 0.905 and 0.800, respectively, with an AUC of 0.929; the sensitivity and specificity of RV GLS<18.55% for diagnosing PPHN were 0.905 and 0.900, respectively, with an AUC of 0.963; εs combined with RV GLS was the best indicator for early detection of right heart functional impairment in PPHN, with sensitivity and specificity of 0.905 and 1.000, respectively, and AUC was 0.985. Conclusions:RA function and RV function are both impaired in PPHN patients. εs combined with RV GLS is the best indicator for early assessment of right heart function impairment and diagnosis of PPHN.
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BACKGROUND: Patients with peritoneal dialysis commonly have severe disorders of lipid metabolism, with particularly severe changes in serum lipoprotein(α) [Lp(α)]. Serum Lp(α) may play a role in the risk of mortality in peritoneal dialysis patients. The aim was to investigate the correlation between high serum Lp(α) levels and all-cause mortality and death from cardiovascular events and infection in peritoneal dialysis patients. METHODS: Three hundred and ninety-two patients with end-stage kidney disease who started peritoneal dialysis treatment between March 1, 2007 and May 31, 2020, were selected. Clinical data of all enrolled patients after 3 months of peritoneal dialysis were collected. Based on the median value of serum Lp(α) level, all enrolled patients were divided equally into a high serum Lp(α) level group (> 275.95 mg/L, n = 196) and a low serum Lp(α) level group (< 275.95 mg/L, n = 196). SPSS25.0 statistical software was used to analyze the factors affecting serum Lp(α) levels and the correlation between high serum Lp(α) levels and all-cause mortality and death from cardiovascular events and infection in peritoneal dialysis patients. RESULTS: Binary multivariate logistic regression analysis showed that higher low-density lipoprotein (LDL) levels (OR = 1.614, 95% CI: 1.261 - 2.068, p = 0.000) and high Body Mass Index (BMI) levels (OR = 1.063, 95% CI: 1.004 - 1.126, p = 0.036) were the risk factors for the high serum Lp(α) levels. High serum albumin levels (OR = 0.959, 95% CI: 0.927 - 0.991, p = 0.014) and high parathyroid hormone levels (OR = 0.999, 95% CI: 0.997 - 1.000, p = 0.010) were protective factors for the high serum Lp(α) levels. The cumulative survival of patients in the high serum Lp(α) level group was lower in death from cardiovascular events as shown by Kaplan-Meier survival analysis (Log-rank test χ2 = 4.348, p = 0.037). Multivariate Cox regression analysis showed that high serum Lp(α) levels were an independent risk factor for death from cardiovascular events in peritoneal dialysis patients (HR = 1.002, 95% CI: 1.001 - 1.003, p = 0.001). CONCLUSIONS: The occurrence of high serum Lp(α) levels in peritoneal dialysis patients was positively associated with LDL and BMI, and negatively associated with serum albumin and parathyroid hormone levels. High serum Lp(α) levels were related to the risk of death from cardiovascular events in peritoneal dialysis patients.
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Cardiovascular Diseases , Kidney Failure, Chronic , Lipoprotein(a) , Peritoneal Dialysis , Cardiovascular Diseases/etiology , Humans , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/therapy , Lipoprotein(a)/blood , Parathyroid Hormone , Peritoneal Dialysis/mortality , Risk Factors , Serum Albumin/analysisABSTRACT
Objective:To quantify the left ventricular myocardial work in patients with repaired tetralogy of Fallot (TOF), and to evaluate the changes in left ventricular systolic function after TOF repair by pressure-strain loops (PSL).Methods:Seventy-six cases of children after TOF complete surgery in Shenzhen Children′s Hospital from September 2015 to September 2021 were analyzed retrospectively. There were 41 cases in the ≤4-year group and 35 cases in the >4-year group. Seventy-six healthy children with matched body surface area in the same period were selected as the control group. All subjects underwent complete echocardiography and quantitative analysis of left ventricular myocardial work, including global longitudinal strain (GLS), global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency (GWE). Compared with the normal control group, the changes of left ventricular myocardial work after TOF repair were analyzed. For patients with complete echocardiographic data before and after surgery, the correlation between postoperative left ventricular myocardial work and preoperative degree of right ventricular outflow tract (RVOT) obstruction was analyzed.Results:①In the ≤4-year group, compared with control subjects, GLS, GWI, GCW, GWE decreased, and GWW increased in patients with repaired TOF. All the differences were statistically significant [(-20.00±1.52)% vs (-21.59±1.73)%, (1 349.37±133.63)mmHg% vs (1 553.51±246.09)mmHg%, (1 589.39±167.85)mmHg% vs (1 749.12±249.45)mmHg%, 94.0%(94.0%, 95.0%) vs 96.0%(95.0%, 97.0%), (78.80±20.53)mmHg% vs (62.27±21.44)mmHg%; all P<0.05]. ②In >4-year group, compared with the control group, GLS, GWI, GWE decreased, and GWW increased in patients with repaired TOF. All the differences were statistically significant [(-19.89±1.66)% vs (-21.31±1.60)%, (1 486.09±172.42)mmHg% vs (1 713.14±227.05)mmHg%, 96.0%(94.0%, 96.0%) vs 97.0%(96.0%, 97.0%), 75.00(65.00, 95.00)mmHg% vs 55.00(42.00, 71.00)mmHg%; all P<0.05]. ③GWW was negatively correlated with preoperative RVOT diameter and RVOT-Z score( r=-0.422, -0.433; both P<0.05). GWE was positively associated with preoperative RVOT diameter and RVOT-Z score( r=0.441, 0.540; both P<0.05). ④GLS, GWI, GCW, GWW, GWE had good repeatability within and between observers.All the differences were not statistically significant (all P>0.05). Conclusions:Left ventricular systolic function in patients with repaired TOF is lower than that in healthy children of the same age by echocardiography PSL, although traditional indicators are still within the normal range. Patients with more severe RVOT obstruction before surgery have worse left ventricular systolic function after operation. Quantification of left ventricular myocardial work by echocardiography PSL is helpful for long-term follow-up of children after TOF repair.
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BACKGROUND: This study is to explore the predictive value of peripheral blood neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) on the prognosis of patients with peritoneal dialysis (PD). METHODS: A total of 378 patients who underwent PD from July 2004 to November 2019 were selected as the research subjects. According to whether death occurred during the follow-up period, they were divided into death group (86 cases) and survival group (292 cases). The differences in clinical indicators between the two groups were compared, and the multivariate Cox regression model and receiver operating characteristic curve (ROC) were used to analyze and summarize the factors affecting the prognosis of PD patients. RESULTS: Compared with the survival group, there were significant differences in age, lymphocytes, NLR, PLR, and combined cerebrovascular disease between the death group and the survival group (p < 0.05). Multivariate Cox regression analysis showed that advanced age (HR = 1.055, 95% CI: 1.038 - 1.072), increased NLR (HR = 1.136, 95% CI: 1.067 - 1.210), and increased PLR (HR = 1.184, 95% CI: 1.018 - 3.026) were risk factors for all-cause death in PD patients. The results showed that the area under the ROC curve (AUC) of NLR and PLR for predicting all-cause death of PD patients were 0.698 and 0.659, respectively, the sensitivity was 69.77%, and the specificity was 66.78% and 58.56%, respectively. The optimal critical values were NLR ≥ 3.71 and PLR ≥ 149.28. Taking the best cutoff value of the ROC curve as the threshold, it showed that the cumulative survival rate of patients with NLR ≥ 3.71 was significantly lower than that of patients with NLR < 3.71 (Log rank ï£2 = 37.551, p = 0.000). It also showed that the cumulative survival rate of patients with PLR ≥ 149.28 was lower than that of patients with PLR < 149.28 (Log rank ï£2 =23.686, p = 0.000). CONCLUSIONS: NLR and PLR have a good predictive effect on the prognosis of PD patients.
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Neutrophils , Peritoneal Dialysis , Blood Platelets , Humans , Lymphocytes , Prognosis , Retrospective StudiesABSTRACT
OBJECTIVE: This study is to investigate the correlation between serum triiodothyronine (T3) levels and all-cause and cardiovascular mortality in PD patients. METHODS: A total of 376 end-stage renal disease (ESRD) patients who started maintenance PD treatment in the Department of Nephrology in our hospital and stable treatment for ≥3 months were selected, and the total T3 (TT3) and free T3 (FT3) levels were determined. Among them, 168 cases with FT3 <3.5 pmol/L and/or TT3 <0.92 nmol/L were divided into the low serum T3 level group, and the remaining 208 cases were divided into normal serum T3 level group. The Cox survival analysis method was used to analyze the correlation between low serum T3 levels and all-cause and cardiovascular mortality in PD patient. RESULTS: Compared with the normal serum T3 level group, patients with low serum T3 levels had higher systolic blood pressure and a higher proportion of heart disease, and lower levels of total T4, free T4, hemoglobin, serum albumin, blood calcium, serum total bilirubin, alanine aminotransferase, and 24-h urine volume (all P < 0.05). Binary Logistic regression analysis showed that heart disease (P = 0.003, OR: 2.628, 95% CI: 1.382-4.997) and high TT4 level (P < 0.001, OR: 0.968, 95% CI: 0.956-0.979) were related to low serum T3 levels in PD patients. Multivariate Cox regression analysis showed that low serum FT3 level was an independent risk factor for all-cause death in PD patients (HR = 0.633, 95% CI = 0.431-0.930; P < 0.020). CONCLUSION: Low serum T3 levels in PD patients were associated with heart disease and TT4 levels. Low serum FT3 levels were associated with the risk of all-cause death in PD patients.
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Recombinant human erythropoietin (rHuEPO) has been used worldwide for treatment of renal anaemia due to its good curative effect. However, rHuEPO treatment is associated with a rare but severe complication because of the development of anti-EPO antibodies, which are difficult to treat. Currently, the main treatments for the anti-EPO antibodies include withdrawing the rHuEPO, providing blood transfusions and administrating steroid-based immunosuppressive agents. Although the above methods can alleviate anti-EPO-related anaemia, there are obvious side-effects such as decreased immunity and an increased risk of infection. Therefore, accurately identifying anti-EPO-related anaemia and effectively treating this complication is worth exploring. This current case report describes a 49-year-old female patient with chronic kidney disease that received rHuEPO subcutaneously and then developed anti-EPO antibody-mediated renal anaemia with her haemoglobin levels dropping to 37 g/l. The patient refused to be treated with steroids, so she received 120 mg roxadustat administered orally every 72 h and her Hb level increased to 110 g/l over a few months. This current case report demonstrates that roxadustat can be used to successfully treat anti-EPO antibody-mediated renal anaemia without the use of steroid-based immunosuppressants.
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Anemia , Erythropoietin , Anemia/drug therapy , Erythropoietin/therapeutic use , Female , Glycine/analogs & derivatives , Humans , Isoquinolines , Middle Aged , Recombinant ProteinsABSTRACT
Objective:To evaluate the effectiveness and safety of ultrasound-guided hydrostatic reduction for pediatric acute intussusception.Methods:One thousand eight hundred and thirty patients with acute intussusception diagnosed by ultrasound in Shenzhen Children′s Hospital from September 2017 to July 2020 were treated with ultrasound-guided hydrostatic reduction method. The therapeutic effects, complications and ultrasonic features were observed.Results:Among 1 830 cases, 1 791 cases were diagnosed as primary intussusception, and 39 cases were secondary intussusception. The overall rate of successful ultrasound enema reduction were 1 780/1 830(93.7%) patients. All 50/1 830(2.7%) patients underwent surgery after unsuccessful enema reduction, including 42 cases of primary intussusception, and 8 cases of secondary intussusception. The complication of intestinal perforation occurred in 3 cases (0.16%), and there were no deaths.Conclusions:Ultrasound-guided enema reduction for pediatric acute intussusception is an effective and safe method without radiation exposure, and can be used as the preferred method for non-operative treatment of intussusception.
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Objective:To compare the diagnostic accuracy of lung ultrasonography and radiography in detection of acute traumatic intrathoracic injuries in children.Methods:A retrospective analysis was performed in 46 cases of children with chest trauma in Shenzhen Children′s Hospital from January 2017 to January 2021. The diagnostic efficiency of lung ultrasound and radiography in children with acute traumatic hemopneumothorax and lung contusion were compared. Computed tomography scan was used as gold standard.Results:The sensitivity and specificity of lung ultrasound were 0.79 and 0.98 for pneumothorax, 0.86 and 0.75 for hemothorax, 0.86 and 0.80 for pulmonary contusion, respectively. Area under the ROC curve of ultrasonography in detection of pneumothorax, hemothorax, and pulmonary contusion was 0.889 (95% CI=0.798-0.979, P<0.001), 0.804 (95% CI=0.707-0.901, P<0.001), and 0.831 (95% CI=0.623-1.000, P=0.013), respectively. Area under the ROC curve of radiography was 0.674 (95% CI=0.544-0.803, P=0.008) for detection of pneumothorax, 0.645 (95% CI=0.517-0.772, P=0.026) for hemothorax, and 0.547 (95% CI=0.289-0.805, P=0.724) for pulmonary contusion. Comparison of area under the ROC curve declared the significant superiority of ultrasonography in detection of pneumothorax, hemothorax and pulmonary contusion (all P<0.05). Conclusions:Lung ultrasound has higher sensitivity and accuracy than chest radiography in the initial evaluation of chest trauma. For children with acute chest trauma, lung ultrasound should be the first choice.
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BACKGROUND AND OBJECTIVE: Elevated pulse wave velocity (PWV) has been associated with higher mortality in the general population, chronic kidney disease (CKD) patients, and hemodialysis patients. However, in peritoneal dialysis (PD) patients, this association has received little attention. The aim of this study was to evaluate the association between baseline brachial-ankle PWV (baPWV) levels and total mortality in PD patients. METHODS: In this single-center retrospective cohort study, 254 incident PD patients with baseline baPWV were followed up from 1 January 2013 to 31 July 2016. Collected baseline data included demographic characteristics and clinical and laboratory measurements. The association of baseline baPWV levels with total mortality was assessed using multivariable-adjusted Cox models. RESULTS: Of 254 patients, mean age was 61.4 (±15.3) years, 61.4% of patients were men, and 22.4% of patients were diabetic. The mean baseline baPWV level was 17.6 (±5.84) m/s. During a mean 31.5 (±20.8) month follow-up period, 45 patients died, of which 13 deaths were caused by cardiovascular disease. After adjusting for sex, age, blood pressure (BP), body mass index (BMI), and hemoglobin, as well as serum albumin, calcium, phosphorus, intact parathyroid hormone (iPTH), uric acid, and medication use, the highest baPWV tertile was significantly associated with a hazard ratio (HR) for total mortality of 2.97 (95% confidence interval [CI], 1.23 - 7.16; p = 0.02). Each 1 m/s higher baseline baPWV level was associated with a 7% (95% CI, 1.03 - 1.12; p = 0.001) higher risk of total mortality. In sensitivity analyses, individuals with baPWV > 21.0 m/s had the highest adjusted risk for total death (HR, 3.22; 95% CI, 1.71 - 6.08; p < 0.001). CONCLUSION: Higher baseline baPWV levels at the commencement of PD were independently associated with total mortality, which suggests that clinicians could use baPWV as a predictor of higher risk of mortality in PD patients.
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Ankle Brachial Index , Peritoneal Dialysis , Pulse Wave Analysis , Renal Insufficiency, Chronic/mortality , Renal Insufficiency, Chronic/physiopathology , Adult , Aged , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Renal Insufficiency, Chronic/therapy , Retrospective Studies , Survival RateABSTRACT
Objective To analysis the change of hip joint in healthy infants by ultrasound,and establish the normal reference value of the developmental dysplasia of the hip(DDH). Methods A total of 8 000 healthy infants from 0 to 24 weeks were collected from the Multi-center study of 10 children′s medical centers. Among them,3 855 infants(2 065 females and 1 790 males) with complete data and follow-up were included in this study. All subjects were divided into 6 groups ( ＜4,4～7,8～11,12～15,16～19 and≥20 weeks group). α angle,femoral head length and width,femoral head coverage ratio by acetabulum ( FHC) were measured in the coronal view on the neutral position;distance from pubis to femoral head ( P-H) and distance from ischium to femoral head ( I-H ) were measured in the transverse view on neutral position;distance from femoral head topubis ( H-P) was measured in the posterolateraltransverse view on the flexion position. The results of each group changes with age were analysised. Results ① The α angle of healthy infants from 0 to 20 weeks were increased with age,the difference among the groups were statistically significant( P ＜0.05),but there was no significant difference between 16～19 and ≥20 weeks group( P ＞0.05). ②The femoral head length and width of all age groups were increased with age,the difference among all the groups was statistically significant( all P ＜0.05). ③ FHC from 0 to 20 weeks were increased with age,the difference among the groups were statistically significant( P ＜0.05) except between 16～19 and ≥20 weeks group( P ＞0.05). ④ The P-H and I-H in all age groups showed no statistically significant ( all P＞0.05). ⑤The H-P of all age groups were increased with age,the difference between the groups were statistically significant(all P ＜0.05).Conclusions The development of hip joints have the certain regular developmental pattern in healthy infants less than 5 months of birth and are relatively constant after birth more than 5 months. The ultrasound normal reference value of the hip joints can be used for the early diagnosis of the DDH.
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Objective To assess long-term outcomes of coronary artery (CA) Z scores in children with Kawasaki disease (KD) with echocardiography.Methods Echocardiographic data of 100 KD children during 7-14 years interval follow-up were analyzed retrospectively.The children were divided into dilatation group (n =54,CA dilated) and non-dilatation group (n=46,CA not dilated) at the acute phase.Fifty one children were selected simultaneously as the controls (control group).Diameters and Z scores of left main coronary artery (LMCA),left anterior descending (LAD) and proximal right coronary artery (pRCA) were compared,and factors affecting CA diameter during the recovery phase were analyzed.Results CA diameters in dilatation group were larger than those in non-dilatation group and control group (all P＜0.05),whereas no statistical difference of CA diameter was found between non-dilatation group and control group (all P＞0.05).In dilatation group,Z score of LMCA,LAD and pRCA was 0.569 5 ± 1.061 6,0.420 (-0.029,1.078) and 0.640(0.283,1.250),while in non dilatation group,Z score of LMCA,LAD and pRCA was-0.0313±0.8467,-0.0662±0.6612 and 0.1887±0.5935,respectively.In control group,Z score of LMCA,LAD and pRCA was-0.1246±1.0167,-0.2558±1.0848 and 0.1943±0.6101,respectively.Z scores in dilatation group were larger than those in non-dilatation group and control group (all P＜0.05),while no statistical differences of Z scores was found between nondilatation group and control group (all P＞0.05).Dilation degree of CA at the acute phase was the factor affecting longterm CA dilation (odds ratio=39.146,P＜0.001).Conclusion During 7-14 years of follow-up,CA diameters and Z scores kept to increase in KD children with CA dilatation at the acute phase.The dilation degree of CA at the acute phase in KD children affects the long-term CA dilation.
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Objective To discuss the application of shear wave elastography and strain elasticity imaging diagnosis of infants with congenital muscular torticollis. Methods Children with congenital muscular torticollis in 50 patients and 100 healthy infants were recruited for the research at Shezhen Children′s Hospital in the period of September to December, analyzing factors in normal sternocleidomastoid two mode ultrasonic elastography, including the young′s modulus, shear wave velocity, shear modulus and the ratio of strain results, comparing the change of elasticity between the patients and normal. Results The elastic parameters of the sternocleidomastoid showed no statistically significant difference between the genders, the left side and the right side. The results of young′s modulus, shear wave velocity and shear wave modulus were higher in long axis section than the short axis section, and the difference was statistically significant (P<0.05). However, the results of the strain ratio and thickness was not statistically significant. The young′s modulus, shear wave velocity and shear wave modulus are higher in the stretch back position than symmetrical supine position, and the difference is statistically significant (P<0.05). But those of strain ratio and thickness was not statistically significant. The thickness of the sternocleidomastoid muscle and the young′s modulus, shear wave velocity, shear modulus and the ratio of strain results ultrasound elastic results were higher in the lesion side than the healthy side, and the difference is statistically significant (P<0.05). Conclusion The ultrasound elastic imaging of multimodal technique can quantify the elasticity of the normal infant and the patients sternocleidomastoid, be used for diagnose for the infants with the congenital muscular torticollis.
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Objective To evaluate the value of lung ultrasound score (LUS) in the quantitative assessment of the severity of neonatal respiratory distress syndrome (NRDS) and the value of clinical diagnosis and treatment.Methods Totally 74 NRDS cases and 30 normal neonates were studied.LUS was compared with X-ray examination,clinical data,ventilator assisted ventilation and ventilator parameters.ROC curve was used to calculate sensitivity and specificity of LUS to predict the severity of NRDS and application of invasive ventilator treatment.Results The main findings of the lung ultrasound in NRDS included diffuse distribution of dense B line,disappeared A line,pleural line abnormalities,decreased pulmonary slip sign and pulmonary consolidation.LUS in patients with NRDS was significantly correlated with X ray grades,clinical grades,assisted ventilation mode grades,number of days on ventilator and ventilator parameters (all P＜0.05).LUS value to predict mild,moderate and severe NRDS were 13.0,22.5,and 29.5,respectively.The best cutoff point for LUS to predict the adoptation of invasive assisted ventilation was 22.5,which had sensitivity of 86.0 ％ and specificity of 64.5 ％.Conclusion LUS can be used to diagnose and evaluate the severity of the desease,and to guide the clinical diagnosis and treatment.
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Objective To evaluate the biological characteristics of hip joint in infants with developmental dislocation of the hip (DDH).Methods From January 2013 to June 2016,30 patients (age from 1 to 8 months,25 females and 5 males) who were diagnosed as DDH,underwent ultrasound examination in Shenzhen Children's Hospital,in these patients 20 cases were treated surgically,and 10 cases were treated with non-surgical treatment.There were 34 hips (26 unilateral dislocation and 4 bilateral dislocation) dislocation in 60 hips.All the patients were confirmed by X-ray,magnetic resonance imaging (MRI) examination or operation.All the patients were examined by ultrasound through the coronal and transverse plane of the hip joint.The α angle,femoral head coverage ratio by acetabulum (FHC),femoral head length and width,distance from pubis to femoral head (P-H) and distance from ischium to femoral head (I-H) were measured.The dislocation joints were compared with contralateral joints.Results The α angle in the hip dislocation group was smaller than the contralateral group [(50.5±3.75)° vs (64.8±3.38) °],and there was significant difference between the two groups (t=-15.181,P ＜ 0.001).The FHC,femoral head length and width in the hip dislocation group were all smaller than the contralateral group [(23.4t17.63)％ vs (64.3±6.45)％,(0.98 ±0.15) cm vs (1.19 ±0.11) cm,(1.38±0.21) cm vs (1.61 ±0.16) cm],and there were significant differences between the two groups (t=-12.469,-6.034,-4.568,all P ＜ 0.001).The P-H and I-H in the hip dislocation group were larger than the contralateral group [(0.97±0.45) cm vs (0.27±0.05) cm,(0.75±0.30) cm vs (0.17±0.05) cm],and there were significant differences between the two groups (t=8.805,10.696,both P ＜ 0.001).The α angle,femoral head length and width in bilateral dislocation of hip group were slightly smaller than the unilateral dislocation of hip group [(50.3±2.75)° vs (51.3±4.77)°,(0.90 ±0.15) cm vs (0.97 ±0.12) cm,(1.25±0.20) cm vs (1.37 ±0.17) cm],but there were no significant differences between the two groups.The P-H and I-H in bilateral dislocation of hip group were slightly larger than the unilateral dislocation of hip group [(0.97 ± 0.49) cm vs (0.80±0.31) cm,(0.92±0.26) cm vs (0.68±0.18) cm],but there were no significant differences between the two groups.The value of acetabular index in the ultrasound group was slightly larger than the X-ray group [(33.13 ± 7.82)° vs (31.20 ± 8.31)°],and there were no significant differences between the two groups.Conclusions The characteristics of DDH includes acetabulum and femoral head dysplasia,and femoral head and acetabulum position relationship abnormalities.Quantitative ultrasonography of the hip can be used to quantitatively evaluate the structural features of DDH,and it is helpful to the early diagnosis and follow-up of DDH.
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Objective To investigate the diagnostic value of ultrasonography in radial head subluxation (RHS).Methods The clinical data and ultrasonographic findings of 34 children with RHS were retrospectively analyzed.All patients were routinely treated by bilateral compared and multi-sectional elbow joints ultrasonography.And the X ray results were negative.Results Ultrasonographic manifestations of RHS included increased radiocapitellar distance in 29 cases (29/34,85.29％);widened joint space and enhanced echo in 25 cases (25/34,73.53％);hook sign,supinator muscle above the radial head in 32 cases (32/34,94.12 ％);annular ligament entrapment in 33 cases (33/34,97.06％).Conclusion Ultra sonographic manifestations of RHS have certain characteristics.Comparation of bilateral elbow joints and the application of continuous scanning ultrasound are helpful to make a quick and accurate diagnosis of X-ray negative RHS.
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Objective To summarize the clinical manifestations of 46, XX ovotesticular disorder of sex development (DSD) caused by a NR5A1 heterozygous mutation. Methods The first case of 46,XX ovotesticular DSD was caused by a NR5A1 heterozygous mutation in China and was reported with a review of 11 similar cases in the literatures since July 2016. Results A 5. 6-year-old child raised as female was born with ambiguous genitalia. The left gonad was palpable in the inguinal region while the right one was located in abdomen. Gonadal histology showed both ovotestis. Vaginoscopy revealed a short, blind-ending vagina. No uterine was detected by laparoscopy. Repeated karyotype results were 46, XX with SRY gene negative. A heterozygous de novo mutation ( p. Arg92Trp) in the accessory DNA-binding region of NR5A1 gene was found in that child. Conclusions We reported for the first time in China a new phenotype caused by a NR5A1 heterozygous mutation-46,XX ovotesticular DSD. According to the review of literatures, such mutation seemed with incomplete penetrance. It could cause both 46, XX DSD and 46, XY DSD with varied manifestations. The possible underlying mechanism might relate to the impairment of the binding between the mutant protein and target DNA which might lead to a decreased inhibition of the male developmental pathway through downregulation of female antitestis genes.
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Objective To summarize the clinical manifestations of 46, XX ovotesticular disorder of sex development (DSD) caused by a NR5A1 heterozygous mutation. Methods The first case of 46,XX ovotesticular DSD was caused by a NR5A1 heterozygous mutation in China and was reported with a review of 11 similar cases in the literatures since July 2016. Results A 5. 6-year-old child raised as female was born with ambiguous genitalia. The left gonad was palpable in the inguinal region while the right one was located in abdomen. Gonadal histology showed both ovotestis. Vaginoscopy revealed a short, blind-ending vagina. No uterine was detected by laparoscopy. Repeated karyotype results were 46, XX with SRY gene negative. A heterozygous de novo mutation ( p. Arg92Trp) in the accessory DNA-binding region of NR5A1 gene was found in that child. Conclusions We reported for the first time in China a new phenotype caused by a NR5A1 heterozygous mutation-46,XX ovotesticular DSD. According to the review of literatures, such mutation seemed with incomplete penetrance. It could cause both 46, XX DSD and 46, XY DSD with varied manifestations. The possible underlying mechanism might relate to the impairment of the binding between the mutant protein and target DNA which might lead to a decreased inhibition of the male developmental pathway through downregulation of female antitestis genes.
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Objective To investigate the toxic effects and mechanisms of sulphur mustard on DNA damage of human immortalized epidermal keratinocytes (HacaT cells).Methods The inhibitory effect of sulphur mustard on the proliferation of HacaT cells was detected by CCK-8 method.The apoptosis index of cells was measured by Annexin V-FITC method.The effects of sulphur mustard on DNA damage were detected by single cell gel electrophoresis.The expression levels of DNA damage and repair related proteins were detected by Western blot.Results The proliferation rate of HacaT cells was inhibited in a dose-dependent manner after treatment with sulphur mustard for 24 h (IC50 value was 121 mol/L).The apoptotic and comet tailing rates of cells treated with sulphur mustard also increased in a dose-dependent manner.The expression levels of DNA damage and repair related proteins were changed after treatment with sulphur mustard.Conclusion Sulphur mustard has significant cytotoxic effect on HacaT cells,and can induce apoptosis and DNA damage.In addition,ATM-P53-γH2AX-PARP signaling pathway plays an important role in the repair of DNA damage induced by sulphur mustard.
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Objective To evaluate the value and clinical application of cardiac structure Z scores in children with preoperative tetralogy of Fallot (TOF) by echocardiography. Methods Considering the result of 31 patients who underwent echocardiography in Shenzhen Children’s Hospital before TOF repair from January 2011 to September 2013, the median age was 10 months (range:3 days to 14 years). Other 31 normal children were selected as normal control group with age-and sex-matched, the median age was 10 months (range: 6 days to 13 years). Echocardiographic measurements included the diameter of right ventricular outflow tract (RVOT), pulmonary valve diameter (PVD), left pulmonary artery diameter (LPAD), right pulmonary artery diameter (RPAD), pulmonary valve peak velocity (PV-Vmax), tricuspid annulus diameter (TVD), mitral annulus diameter (MAD), left ventricular end-diastolic volume (LVEDV) and left ventricular mass (LVmass). Shenzhen Children’s Hospital pediatric echocardiography normal reference values and the regression equation are applied to obtain the forecast average, and using the Z-score formula into the standard value (Z=[M-y]/ MSE ). Results There was no significant difference in body surface area between the TOF group and control group (P>0.05). Compared with control group, the true value of RVOT reduced significantly in TOF group, its Z-score showed negative values increase (-4.87±1.86 vs 0.33±0.93, t=-13.90, P0.05), but the Z scores of TOF were significantly reduced than the control group (0.25±0.78 vs 0.76±0.65, t=-2.75;-0.92±1.94 vs 0.03±1.01, t=-2.41;-0.83±1.59 vs 0.67±0.69, t=-4.71; all P0.05). For the Z scores of PV-Vmax, there were no significant difference between transannular patch surgery and pulmonary valve-sparing repair (8.58±1.20 vs 8.12±1.16, t=1.07, P>0.05). Conclusions The Z scores value of cardiac structure can be used in quantitative analysis of RVOT obstruction and development of left ventricular backward of TOF. The Z score of transannular patch surgery is more lower than pulmonary valve-sparing repair patients. The normalized Z score by body surface area correction is important for the preoperative diagnosis and the surgical planning.