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Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.
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In this paper, the clinical case, pathological and immunohistochemical analyses were performed on 10 patients with intrathyroidal thymic carcinoma admitted to our hospital.Combined with relevant literature, it was found that tumour cells expressing CD5 and CD117 had differential diagnostic significance. Surgical resection of the tumour was the first choice for treatment, whether radiotherapy, chemotherapy and monotherapy were administered remained controversial.
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Objective:To explore the clinical characteristics, therapeutic efficacy and prognosis of congenital coronary artery fistula (CAF) in children.Methods:Clinical data of 71 pediatric patients diagnosed with congenital CAF at Department of Cardiology and Department of Cardiac Surgery, Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from January 2013 to June 2019 were retrospectively analyzed.The median age was 2.2 years (0.1-14.0 years), and the median body weight was 18.3 kg (3.2-55.8 kg), including 37 males (52.1%) and 34 females (47.9%). They were divided into the transcatheter closure group (30 cases) and surgical repair group (41 cases). The therapeutic effect and follow-up data of the 2 groups were compared by the Fisher′ s exact test. Results:Among the 71 congenital CAF children, 70 had heart murmurs, 2 had chest tightness after activity, and 5 were prone to recurrent respiratory tract infection.Transcatheter closure and surgical repair were successfully performed in 22/30 (73.3%) and 41/41 (100.0%) cases with a statistically significant difference ( P=0.001). However, in the surgical repair group, 1 (2.4%) case died after operation and 2(4.9%) needed further transcatheter closure due to large residual shunt during the follow-up period.At the last follow-up, there were 2 cases with minimal or small residual shunt in both groups ( P=0.567). There were 2/71(2.8%) cases suffering from postoperative thrombosis. Conclusions:Cardiac murmur is the main sign of congenital CAF in children, and some of them may have frequent respiratory tract infection due to increased lung blood caused by a large amount of shunt.A few children have chest tightness and chest pain due to myocardial ischemia caused by coronary steal.Both percutaneous closure and surgical repair are safe and effective with few complications.
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Objective:To explore the role and safety of endomyocardial biopsy (EMB) in the diagnosis of pediatric myocardial disease.Methods:Demographic, clinical and histopathological data of all children receiving EMB in Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine between January 2016 and August 2020 were collected.The pathological results and the procedure-related complications were retrospectively analyzed.Results:A total of 22 patients with the mean age of (10.2±3.1) years underwent EMB.Among them, 13 cases (59.1%) underwent right ventricular EMB, 5 cases (22.7%) underwent left ventricular EMB, and 4 cases (18.2%) underwent biventricular EMB.Among the 12 patients with clinically suspected myocarditis or unexplained heart failure, 4 cases were diagnosed with lymphocytic myocarditis, 2 cases were dilated cardiomyopathy, and 1 case was inflammatory cardiomyopathy.Seven patients presented ventricular diastolic dysfunction, including 6 cases of restrictive cardiomyopathy and 1 of constrictive pericarditis.Three patients exhibited hypertrophic cardiomyopathy with pre-excitation syndrome, involving 2 cases were diagnosed with glycogen storage cardiomyopathy.EMB was successfully performed in all patients.No patient died, and procedure-related complications were not reported.Conclusions:EMB assists the diagnosis and treatment of pediatric patients with selected myocardial disease, which is relatively safe with less complications if performed by experienced interventionalists in qualified pediatric cardiovascular medical centers.
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Alstr?m syndrome is a rare multisystem genetic disease caused by mutations in the ALMS1 gene.Both of its clinical diagnosis and treatment are very difficult.In 2020, the Consensus Clinical Management Guidelines for Alstr?m Syndrome, developed with the participation of many countries, was published in the Orphanet Journal of Rare Diseases.A systematic literature review on Alstr?m syndrome of the last 45 years until October 2019 was carried out and then the clinical management guideline for Alstr?m syndrome was proposed.In this report, the contents of the 2020 European guideline for Alstr?m syndrome would be introduced briefly with appropriate interpretation in order to provide reference.
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Objective:To investigate the risk factors related to extrathyroidal extension (ETE) of differentiated thyroid carcinoma (DTC) and the specific effects on the prognosis.Methods:The clinical data of 592 patients with newly diagnosed DTC admitted to our hospital from Jun. 2015 to Jun. 2016 were retrospectively analyzed. The data including the maximum tumor diameter and lymph node metastasis were collected, and the survival data were followed up. Chi-square test and Logistic regression were used to analyze the risk factors associated with ETE. Kaplan-Meier method and Cox proportional risk model were used to analyze the effect of ETE on disease-free survival.Results:There were 100 ETE of 592 DTC patients (16.9%) . Univariate analysis showed that the risk factors for ETE were the largest tumor diameter ≥2 cm, multiple lesions, and lymph node metastasis in the lateral cervical region ( P<0.01) . The follow-up time was 29 to 64 months, and the median follow-up time was 60 months. The 3-year and 5-year DFS rates of patients with DTC and ETE were 86.9% and 83.2% respectively, which were significantly lower than those of patients without ETE ( P<0.001) . Univariate analysis showed that ETE ( P<0.001) was a risk factor for postoperative recurrence in patients with DTC. Cox multivariate regression showed that ETE (HR: 10.564, 95% CI 3.712-30.063, P<0.001) was an independent risk factor for postoperative recurrence in patients with DTC. Conclusions:DTC accompanied by ETE is associated with risk factors such as large tumor diameter and multiple lesions, which has a lower 5-year DFS. Patients with the above characteristics should consider the possibility of ETE before surgery, and focus on long-term follow-up after surgery.
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Objective:To assess the efficacy and the safety of the radiofrequency catheter ablation (RFCA) for the septal accessory pathway (AP) in children.Methods:From September 2013 to March 2019, 626 patients plan to underwent RFCA for paroxysmal supraventricular tachycardia (PSVT) in Shanghai Children′s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine.Among them, 74 consecutive patients with right or left septal APs were included in the study and their clinical and RFCA data were analyzed.Results:The age of these 74 children (45 males, 29 female) was (7.8±3.5) years, ranging from 10 months to 13 years.The body weight (BW) was (27.7±14.4) kg, with 3 patients BW<15 kg.A discordant ventricular wall motion (DVWM) was found in 5 patients, and the combined congenital heart diseases were discovered in 2 patients.A three dimensional mapping system was applied in 69 ablations, and 3 ablations were performed only with the fluoroscopy monitor of 5 cases.According to the AP location, the number of cases located in the anteroseptal, the midseptal, the mouth of coronary sinus, the left posteroseptal and the right posteroseptal, were 28, 18, 10, 10 and 8, respectively.The ablation operations were applied in 72 patients.The initial acute success reached in 67 (93.1%) patients.The ablation energy was (18.0±1.8) W, the fluoroscopy time during the ablations was (4.7±2.7) minutes, and the procedure duration was (151.5±58.6) minutes.One inadvertent complete atrioventricular block (AVB) was noted as the ablation-related complication.All 5 children with the pre-DVWM were recovered after ablations.During a follow-up of (23.8±10.8) months, 4 patients experienced the recurrence of preexcitation syndrome atrioventricular reentrant tachycardia.Conclusions:With the 3D-mapping system, the RFCA of septal APs can be performed safely and effectively in pediatric patients of paroxysmal supraventri-cular tachycardia.However, as the ablation-related complication, AVB should not be ignored.
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Objective:To investigate the effects of miR-497 on cytobiology behaviors of papillary thyroid carcinoma (PTC) by targetingly regulating the expression of Yes-associated protein 1 (YAP1) .Methods:Human TPC-1 cells were divided into control group, miR-497 group, si-YAP1 group and miR-497+si-YAP1 group. The liposome transfection was conducted by LipofectamineTM3000. The targeted relationship between miR-497 and YAP1 was validated by Luciferase Reporter Assay. The cell proliferation activity in each group was detected by MTT method. The apoptosis rates were analyzed by flow cytometry. The number of invasion cells was detected by Transwell. The cell migration rates were detected by scratch assay. The expression of Cyclin D1, matrix metalloproteinase 2 (MMP-2) , MMP-9, matrix metalloproteinase inhibitor-1 (TIMP-1) and activated caspase 3 (cleaved Caspase-3) was detected by Western blot. SPSS 22.0 was used to analyze data, and normally distributed measurement data were expressed as ( ± s) . One-way ANOVA was analyzed for the difference between multiple groups, and SNK-q were analyzed for the difference between two groups. Results:Compared with the control group, the expression of YAP1 mRNA and protein was decreased in miR-497 group and si-YAP1 group ( q=14.682, 14.597; 23.743, 23.571; P<0.05) , cell proliferation activity, number of invasion cells and migration rate were decreased ( q=4.724, 4.568, 3.841; 4.216, 3.952, 3.274; P<0.05) , apoptosis rate was increased ( q=3.783; 4.336; P<0.05) , expression of CyclinD1, MMP-2, MMP-9 and cleared Caspase-3 proteins was decreased ( q=5.823, 5.981, 6.036, 6.485; 5.934, 6.110, 6.573, 6.614; P<0.05) , and expression of TIMP-1 protein was increased ( q=6.071; 6.148; P<0.05) . Compared with si-YAP1 group, miR-497 level was increased in miR-497+si-YAP1 group ( q=14.726, P<0.05) , the expression of YAP1 mRNA and protein was decreased ( q=3.089, 3.126; P<0.05) , cell proliferation activity, number of invasion cells and migration rate were decreased ( q=2.654, 2.537, 2.246; P<0.05) , apoptosis rate was increased ( q=2.875, P<0.05) , expression of CyclinD1, MMP-2, MMP-9 and cleared Caspase-3 proteins was decreased ( q=4.371, 4.365, 4.383, 4.368; P<0.05) , and expression of TIMP-1 protein was increased ( q=4.275, P<0.05) . Conclusion:MiR-497 can negatively targetingly regulate the expression of YAP1, inhibit proliferation, invasion and migration of TPC-1 cells.
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OBJECTIVE@#To explore the genetic basis for 7 patients with Alström syndrome.@*METHODS@#DNA was extracted from peripheral blood samples of the patients and their parents. Whole exome sequencing was carried out for the patients. Suspected variant was verified by Sanger sequencing and bioinformatic analysis.@*RESULTS@#Genetic testing revealed 12 variants of the ALMS1 gene among the 7 patients, including 7 nonsense and 5 frameshift variants, which included c.5418delC (p.Tyr1807Thrfs*23), c.10549C>T (p.Gln3517*), c.9145dupC (p.Thr3049Asnfs*12), c.10819C>T (p.Arg3607*), c.5701_5704delGAGA (p.Glu1901Argfs*18), c.9154_9155delCT (p.Cys3053Serfs*9), c.9460delG (p.Val3154*), c.9379C>T (p.Gln3127*), c.12115C>T (p.Gln4039*), c.1468dupA (p.Thr490Asnfs*15), c.10825C>T (p.Arg3609*) and c.3902C>A (p.Ser1301*). Among these, c.9154_ 9155delCT, c.9460delG, c.9379C>T, and c.1468dupA were unreported previously. Based on the standards and guidelines of American College of Medical Genetics and Genomics, the c.9379C>T and c.12115C>T variants of the ALMS1 gene were predicted to be likely pathogenic (PVS1+PM2), whilst the other 10 variants were predicted to be pathogenic (PVS1+ PM2+ PP3+PP4).@*CONCLUSION@#ALMS1 variants probably underlay the Alström syndrome in the 7 patients, and genetic testing can provide a basis for the clinical diagnosis of this syndrome. The discovery of four novel variants has expanded the mutational spectrum of Alström syndrome.
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Humans , Alstrom Syndrome/genetics , Cell Cycle Proteins/genetics , Mutation , Pedigree , Exome SequencingABSTRACT
Objective:To observe the relationship between the occurrence of symptomatic hypocalcemia (SH) and various potential influencing factors in patients after thyroidectomy, stratify according to the scope of thyroidectomy, and explore the predictive value of intact parathyroid hormone (iPTH) for postoperative SH.Methods:Among 3 379 patients with thyroidectomy who admitted into the First Affiliated Hospital of Zhengzhou University from January 2020 to February 2021, 122 patients with SH after thyroidectomy were collected retrospectively and set as SH group. 100 patients of the remaining 3 200 patients who did not suffer from SH in the same year were selected by systematic sampling method and set as control group. Pearson correlation analysis was used to analyze the potential influencing factors such as age, preoperative calcium, postoperative calcium, preoperative iPTH, postoperative iPTH, central lymph node number, blood loss, operation duration, gender, lymph node dissection method, thyroidectomy range, postoperative pathological type and other. Among them, the measurement data of normal distribution were expressed by mean±standard deviation( Mean± SD), t-test was used for the comparison between the two groups, and Chi-square test was used for count data. By drawing the receiver operating characteristic curve (ROC), the iPTH levels in patients with and without SH before/after operation (different surgical methods) were studied, and the diagnostic threshold, sensitivity and specificity of iPTH were predicted. Results:Among 3 379 patients, 122 patients suffered from SH after thyroidectomy, with the incidence rate of 3.6%. There were significant differences in gender (8 males and 114 females in SH group; 27 males and 73 females in control group), whether lateral area dissection was performed (58 cases with dissection and 64 cases without dissection in SH group; 7 cases with dissection and 93 cases without dissection in control group), thyroidectomy range (14 cases with one side and 108 cases with both sides in SH group; 73 cases with one side and 27 cases with both sides in control group), age (40.1 years old vs 43.2 years old), dissection number of central lymph nodes (8.6 vs 4.6), dissection number of cervical lymph nodes (12.3 vs 0.7), blood loss (22.8 mL vs 11.0 mL), operation duration (1.7 h vs 0.8 h), postoperative iPTH (16.4 pg/mL vs 41.9 pg/mL), preoperative iPTH (39.4 pg/mL vs 47.8 pg/mL) in SH group; and postoperative calcium level (1.9 mmol/L vs 2.2 mmol/L). There was significant differences between the two groups ( P<0.05). However, there was no significant differences between them with postoperative pathological type (4 cases with toxic goiter, 3 cases with medullary thyroid carcinoma, 1 case with thyroid follicular carcinoma, 114 cases with papillary thyroid carcinoma in SH group; 1 case with medullary thyroid carcinoma, 1 case of thyroid follicular carcinoma, 98 cases with papillary thyroid carcinoma in control group, P=0.25) and preoperative calcium (2.3 mmol/L vs 2.3 mmol/L, P=0.10). For patients with bilateral thyroidectomy, SH was easy to occur when postoperative iPTH < 20.08 pg/mL, and its sensitivity and specificity were 74.07% and 96.30%; however, for patients with unilateral thyroidectomy, SH was easy to occur when iPTH < 24.00 pg/mL after operation. Conclusions:Gender, age, postoperative calcium, preoperative iPTH, postoperative iPTH, central lymph node number, blood loss, operation duration, lymph node dissection method and thyroidectomy range are important factors affecting the occurrence of SH after thyroidectomy. With the expansion of surgical range, the postoperative iPTH level gradually decreases, which predicts the occurrence of symptomatic hypocalcemia. In order to avoid the occurrence of symptomatic hypocalcemia after operation, it is necessary to supplement calcium in time according to the range of operation and postoperative iPTH level.
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Medullary thyroid carcinoma(MTC) is a rare neuroendocrine neoplasm caused by the malignant hyperplasia of thyroid parafollicular cells, accounting for 4% of all diagnosed thyroid cancer. Patients with MTC have a relatively poor outcome. Distant metastasis, especially in liver, is usually found at the time of diagnosis and the effect of chemotherapy is unsatisfactory in most cases. Due to its highly progressive characteristic and poorer prognosis compared with other subtypes of thyroid cancer, MTC has always been the focus and difficulty of clinical research. In the era of precision therapy, the classification of MTC is more accurate with the application of genetic testing technology and various kinds of individual treatments, such as targeted therapy and peptide receptor radionuclide therapy have been developed. This review will describe the latest development in MTC in the era of precision therapy.
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Objective To investigate the incidence and prognosis of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects in pediatric patients as well as the risk factors of regurgitation occurrence or aggravation.Methods Clinical data of 1 108 patients who underwent percutaneous closure in Shanghai Children's Medical Center,School of Medicine,Shanghai Jiaotong University from January 2011 to January 2017 was analyzed retrospectively,and the prognosis and risk factors of postoperative tricuspid regurgitation and aggravation were also analyzed.Results Mild tricuspid regurgitation occurred in 24 cases after surgery with the incidence of 2.1％.Among 5 cases with mild or above preoperative tricuspid regurgitation,the regurgitation was alleviated after surgery in 4 cases.No severe tricuspid regurgitation requiring surgical intervention occurred in any patient during follow-up.Univariate analysis showed that intervention time (P ＜ 0.05) and residual shunt (P ＜ 0.05) were risk factors for mild or above tricuspid regurgitation after intervention.Binary regression analysis indicated that the size of the occluder (mm) (OR =1.48,95％ CI:1.13-1.90) and residual shunt (OR =6.53,95％ CI:1.69-25.30) were risk factors for tricuspid regurgitation after intervention (all P ＜ 0.05).Conclusions There is a certain incidence of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects,but most tricuspid regurgitation do not need surgical intervention.The intervention time,size of occluder and residual shunt are risk factors of intraoperatively or postoperatively tricuspid regurgitation.
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Objective@#To investigate the incidence and prognosis of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects in pediatric patients as well as the risk factors of regurgitation occurrence or aggravation.@*Methods@#Clinical data of 1 108 patients who underwent percutaneous closure in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University from January 2011 to January 2017 was analyzed retrospectively, and the prognosis and risk factors of postoperative tricuspid regurgitation and aggravation were also analyzed.@*Results@#Mild tricuspid regurgitation occurred in 24 cases after surgery with the incidence of 2.1%.Among 5 cases with mild or above preoperative tricuspid regurgitation, the regurgitation was alleviated after surgery in 4 cases.No severe tricuspid regurgitation requiring surgical intervention occurred in any patient during follow-up.Univariate analysis showed that intervention time (P<0.05) and residual shunt (P<0.05) were risk factors for mild or above tricuspid regurgitation after intervention.Binary regression analysis indicated that the size of the occluder (mm) (OR=1.48, 95%CI: 1.13-1.90) and residual shunt (OR=6.53, 95%CI: 1.69-25.30) were risk factors for tricuspid regurgitation after intervention (all P<0.05).@*Conclusions@#There is a certain incidence of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects, but most tricuspid regurgitation do not need surgical intervention.The intervention time, size of occluder and residual shunt are risk factors of intraoperatively or postoperatively tricuspid regurgitation.
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Objective@#To describe the clinical characteristics, treatment and outcomes of Staphylococcus aureus infective endocarditis (SAIE) in children.@*Methods@#One hundred and fifty-one children with infective endocarditis (IE) and treated in Shanghai Children′s Medical Center of Shanghai Jiaotong University School of Medicine were retrospectively analyzed, from January 2007 to December 2017.There were 88 boys and 63 girls, the age of IE onset ranged from 1 month to 17 years [(6.27±4.80) years]. There were 23 cases (15.2%) with infection of staphyloco-ccus aureus, 128 cases with non-Staphylococcus aureus infective endocarditis (NSAIE). The clinical characteristics, treatment and prognosis of patients between the SAIE group and the NSAIE group were analyzed and compared.@*Results@#Among the SAIE group, there were 15 boys and 8 girls, the age of IE onset ranged from 3 months to 16 years [(6.2±5.6) years]; 10 cases of them had no underlying heart disease.There was no significant difference in gender, age and previous cardiac surgery between the SAIE group and the NSAIE group (all P>0.05). The number of patients without underlying heart disease in the SAIE group was more than that in the NSAIE group, and the difference was statistically significant[43.5%(10/23 cases) vs.20.3%(26/128 cases), χ2=5.762, P=0.016]. Compared with the NSAIE group, the SAIE group was associated with significantly higher levels of C-reactive protein [60.0(128.0) mg/L vs.25.0(58.0) mg/L, Z=-2.033, P=0.042], higher erythrocyte sedimentation rate [(59.3±43.2) mm/h vs.(39.4±31.5) mm/h, t=-2.283, P=0.024], and lower levels of serum albumin [30.1(12.7) g/L vs.34.3(8.4) g/L, Z=-2.282, P=0.022]. The proportion of severe sepsis in the SAIE group was also significantly higher than that in the NSAIE group [21.7%(5/23 cases) vs.7.0%(9/128 cases), Z=5.014, P=0.045]. Twelve cases (52.2%) of the SAIE group had systemic embolic events, including cerebral events (6 cases), pulmonary (2 cases), limbs (1 case), splenic (1 case), limbs and mesenteric (1 case), limbs and cerebral events (1 case). Eighteen cases in the SAIE group were treated with antibiotics combined with surgery and 1 case died, while 5 cases were treated with antibiotics alone, 4 cases died and 1 case abandoned.The SAIE patients had a significantly higher systemic embolic rate [52.2%(12/23 cases) vs.20.3%(26/128 cases), χ2=7.978, P=0.016], neurological events rate [30.4%(7/23 cases) vs.10.9%(14/128 cases), χ2=4.669, P=0.031] and in-hospital mortality [21.7%(5/23 cases) vs.6.3%(8/128 cases), χ2=4.139, P=0.042] than those in the NSAIE group.@*Conclusions@#SAIE was most common in those without heart disease.Compared with NSAIE, SAIE is characterized by a higher prevalence of severe sepsis, major neurological events, and inpatient mortality.Timely surgery is recommended in these cases, when possible, before the occurrence of complications.
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Exosomes are tiny vesicles produced in cells which sizes between 40 to 100 nm. Exosomes are carriers of cell signal transduction. Thyroid cancer is the common malignant tumor in endocrine system. Exosomes show the regulatory role in thyroid cancer about occurrence, development and metastasis. This review focuses on the relevant characteristics of exosomes and the research progress of exosomes in thyroid cancer.
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Objective@#To explore the effectiveness and safety of ultrasound-guided radiofrequency ablation (RFA) in treatment of thyroid benign nodules.@*Methods@#We analyze 573 patients with thyroid benign nodules from June 2014 to September 2017 treated by RFA at Department Ⅱof Thyroid Surgery, the First Affiliated Hospital of Zhengzhou University. Among these patients, there were 75 males and 498 females, with a median age of 45 years old. All patients were diagnosed as thyroid benign nodules by ultrasound-guided fine needle aspiration biopsy before RFA. A total of 750 benign tumors were treated. To evaluate the thyroid function of the patients before RFA and 3 months after it, and to observe the changes of thyroid benign nodules by ultrasound at 3, 6, 12 months after RFA. The paired t-test was used to compare the measurement data with normal distribution, and Wilcoxon's signed rank test was used to compare the measurement data with non-normal distribution. To calculate the volume change and reduction rate of thyroid benign nodules.@*Results@#RFA was successfully completed in all patients, the volume reduction rate was 67%(48%, 83%) in the 3rd month after RFA, in the 6th month was 81%(67%, 91%), in the 12th month was 89%(80%, 95%). Eighteen patients felt pain during RFA, but the pain was alleviated after stopping ablation. Three patients′ tone decreased, but recovered in a week. Hoarseness occurred in 6 patients and recovered in 3 months. Three patients had neck hemorrhage, which was managed with simple compression of the neck.@*Conclusions@#RFA is an effective and safe treatment for thyroid benign nodules and has obvious advantages such as less invasiveness, having no influence in thyroid functions. It is clinically prospective for application.
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Objective To investigate the clinical prognostic features of pediatric differentiated thyroid carcinoma (DTC).Methods A retrospective analysis of clinical data of 56 cases of pediatric differentiated thyroid carcinoma under 14 years old admitted in our hospital from Aug.2009 to Oct.2018 was performed.KaplanMeier method and COX proportional hazards model were used to analyze the factors affecting the disease-free survival of patients.Results The follow-up period was from 6 months to 82 months,and the median follow-up time was 28 months.Recurrence occurred in 15 patients,with a survival rate of 100％.The 3-and 5-year disease-free survival rate was 67.4％ and 63.5％.Univariate analysis showed that tumor diameter ＞2 cm (P=0.046),multiple lesions (P＜0.001),cervical lymph node metastasis (P=0.008),and extra thyroidal extension (ETE) (P=0.035) were related factors affecting postoperative recurrence,while gender,age,Hashimoto's thyroiditis,tumor TNM stage,central lymph node metastasis and surgical approach were not related to postoperative recurrence.COX multivariate analysis showed that multiple lesions (HR:8.815,P=0.010) was the independent factor influencing the prognosis of pediatric differentiated thyroid carcinoma.Conclusions Multiple lesion is an independent influencing factor for the recurrence of pediatric differentiated thyroid carcinoma patients after surgery.Meanwhile,total thyroidectomy and long-term follow-up should be followed.Although the metastasis rate of lymph node and distant organ are high,overall prognosis is good in pediatric DTC.
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Objective@#To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) .@*Methods@#A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t-test and a Chi-square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient.@*Results@#(1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54) , whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0±7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ2=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow-up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function.@*Conclusions@#Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long-term follow-up treatment.
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Objective@#To access the feasibility, effectiveness and safety of transcatheter closure of ventricular septal defect (VSD) with right coronary cusp bulge.@*Methods@#The study population consisted of 40 children (22 boys and 18 girls) undergoing transcatheter intervention for VSD with right coronary cusp bulge in Shanghai Children′s Medical Center from August 2013 to June 2017. All patients were examined using transthoracic echocardiography and cardiovascular angiography before occlusion and received transcatheter closure of VSD by placing an occlude. During the operation, angiography and transthoracic echocardiography were used to detect residual shunts, new-onset or aggravation of aortic regurgitation, and surface electrocardiogram was used to assess the impact of occlusion on the conduction system. The children were followed up to evaluate the degree of aortic regurgitation and the presence of heart block by transthoracic echocardiography and electrocardiogram in outpatient department. Comparative analysis between two groups was used by t test.@*Results@#The average age of the patients was (5.1±3.2) years, weight(20.6±10.7) kg. The diameter of defect shunt was (3.3±0.9) cm. The VSD of all 40 patients were successfully occluded immediately, and three patients (8%) with filament residual shunt were observed during the operation. No major surgical complication such as death, pericardial tamponade, embolism of important organs, infective endocarditis occurred during the perioperative period. During the follow-up period, the positions of all the occluders were good, the residual shunt in three patients disappeared, and no new-onset or aggravated aortic regurgitation occurred. Electrocardiogram did not reveal any atrioventricular block, and one patient suffered from an incomplete right bundle branch block. There were significant differences between pre- and post-occlusion regarding the left ventricular end diastolic diameter ((3.8±0.5) vs. (3.7±0.5) cm, t=2.092, P=0.043), the left ventricular end systolic diameter ( (2.4±0.3) vs. (2.2±0.4) cm, t=2.068, P=0.045), and the QRS interval ((75.4±10.4) vs. (79.8±11.4) ms, t=-2.277, P=0.028). No significant differences were found between pre- and post-operation regarding the left ventricular ejection fraction (67.8%±4.7% vs. 68.1%±4.6%, t=-0.447, P=0.657) and the PR interval ((125.6±14.7) vs. (122.6±14.2) ms, t=1.540, P=0.132).@*Conclusions@#Interventional closure of VSD with right coronary cusp bulge has small impact on aortic valve activity in children. With appropriate indications and methods, transcatheter interventional occlusion should be considered because it is effective for children diagnosed with VSD combined with right coronary cusp bulge.
ABSTRACT
Objective To describe the clinical characteristics,treatment and outcomes of Staphylococcus aureus infective endocarditis (SAIE) in children.Methods One hundred and fifty-one children with infective endocarditis (IE) and treated in Shanghai Children's Medical Center of Shanghai Jiaotong University School of Medicine were retrospectively analyzed,from January 2007 to December 2017.There were 88 boys and 63 girls,the age of IE onset ranged from 1 month to 17 years [(6.27 ±4.80) years].There were 23 cases (15.2％) with infection of staphylococcus aureus,128 cases with non-Staphylococcus aureus infective endocarditis (NSAIE).The clinical characteristics,treatment and prognosis of patients between the SAIE group and the NSAIE group were analyzed and compared.Results Among the SAIE group,there were 15 boys and 8 girls,the age of IE onset ranged from 3 months to 16 years [(6.2 ±5.6) years];10 cases of them had no underlying heart disease.There was no significant difference in gender,age and previous cardiac surgery between the SAIE group and the NSAIE group (all P ＞ 0.05).The number of patients without underlying heart disease in the SAIE group was more than that in the NSAIE group,and the difference was statistically significant [43.5 ％ (10/23 cases) vs.20.3 ％ (26/128 cases),x2 =5.762,P =0.016].Compared with the NSAIE group,the SAIE group was associated with significantly higher levels of C-reactive protein [60.0 (128.0) mg/L vs.25.0(58.0) mg/L,Z =-2.033,P =0.042],higher erythrocyte sedimentation rate [(59.3 ±43.2) mm/h vs.(39.4 ±31.5) mm/h,t =-2.283,P =0.024],and lower levels of serum albumin [30.1 (12.7) g/L vs.34.3 (8.4) g/L,Z =-2.282,P =0.022].The proportion of severe sepsis in the SAIE group was also significantly higher than that in the NSAIE group [21.7％ (5/23 cases) vs.7.0％ (9/128 cases),Z =5.014,P =0.045].Twelve cases (52.2％) of the SAIE group had systemic embolic events,including cerebral events (6 cases),pulmonary (2 cases),limbs (1 case),splenic (1 case),limbs and mesenteric (1 case),limbs and cerebral events (1 case).Eighteen cases in the SAIE group were treated with antibiotics combined with surgery and l case died,while 5 cases were treated with antibiotics alone,4 cases died and 1 case abandoned.The SAIE patients had a significantly higher systemic embolic rate [52.2％ (12/23 cases) vs.20.3％ (26/128 cases),x2 =7.978,P =0.016],neurological events rate [30.4％ (7/23 cases) vs.10.9％ (14/128 cases),x2 =4.669,P =0.031] and in-hospital mortality [21.7％ (5/23 cases) vs.6.3％ (8/128 cases),x2 =4.139,P =0.042] than those in the NSAIE group.Conclusions SAIE was most common in those without heart disease.Compared with NSAIE,SAIE is characterized by a higher prevalence of severe sepsis,major neurological events,and inpatient mortality.Timely surgery is recommended in these cases,when possible,before the occurrence of complications.