Objective:To evaluate the efficacy and safety of obinutuzumab combined with glucocorticoid-based therapy in patients with relapsed immune thrombotic thrombocytopenic purpura (iTTP).Methods:This study analyzed the efficacy and adverse reactions of four patients with relapsed iTTP who were treated with a combination of obinutuzumab and glucocorticoids to assess the effectiveness and safety of the treatment.Results:All four patients had a history of multiple relapses and had previously undergone treatment with rituximab and bortezomib. Three patients exhibited additional autoantibodies. Following the combined therapy, all patients achieved clinical remission, with ADAMTS13 activity returning to normal levels and inhibitors testing negative. During a median follow-up period of 11 months (range: 3–17 months), all patients maintained sustained remission. No severe adverse events were reported during treatment or follow-up.Conclusion:The combination of obinutuzumab and glucocorticoid-based therapy is effective and safe for treating relapsed iTTP.

Objective:To evaluate the efficacy and safety of obinutuzumab combined with glucocorticoid-based therapy in patients with relapsed immune thrombotic thrombocytopenic purpura (iTTP).Methods:This study analyzed the efficacy and adverse reactions of four patients with relapsed iTTP who were treated with a combination of obinutuzumab and glucocorticoids to assess the effectiveness and safety of the treatment.Results:All four patients had a history of multiple relapses and had previously undergone treatment with rituximab and bortezomib. Three patients exhibited additional autoantibodies. Following the combined therapy, all patients achieved clinical remission, with ADAMTS13 activity returning to normal levels and inhibitors testing negative. During a median follow-up period of 11 months (range: 3–17 months), all patients maintained sustained remission. No severe adverse events were reported during treatment or follow-up.Conclusion:The combination of obinutuzumab and glucocorticoid-based therapy is effective and safe for treating relapsed iTTP.

Objective:To review the diagnosis and treatment process of a patient with relapsed immune thrombotic thrombocytopenic purpura (iTTP) secondary to systemic lupus erythematosus (SLE) and conduct a review of relevant literature, so as to provide a reference basis for the diagnosis and treatment of this disease.Methods:We retrospectively analyzed the diagnosis and treatment process of a patient with relapsed iTTP secondary to SLE who was admitted to the First Affiliated Hospital of Soochow University, and conducted a review of literature.Results:When the patient's iTTP relapsed, it was accompanied by moderate lupus activity, hypofibrinogenemia, renal insufficiency, and pulmonary infection. After adopting therapeutic plasma exchange (TPE), hormone pulse therapy, bortezomib, and comprehensive diagnosis and treatment by a multidisciplinary team, both the patient's iTTP and SLE were relieved.Conclusion:A comprehensive examination should be completed when diagnosing iTTP to rule out the possibility of combining with other autoimmune diseases. For relapsed and refractory cases, treatments targeting plasma cells, such as bortezomib, can be attempted.

Objective:To investigate the etiological distribution of hydronephrosis caused by upper uri-nary tract obstruction in adult patients and to improve the diagnostic accuracy for this condition.Me-thods:The clinical information of adult patients with newly diagnosed hydronephrosis in Upper Urinary Tract Repair Outpatient Clinic of Peking University First Hospital from May 2020 to May 2021 were pro-spectively and continuously collected.Patients with ureteral calculi or upper urinary tract tumor were ex-cluded.A total of 767 patients were involved.The underlying causes of upper urinary tract obstruction were identified by senior urological surgeons according to symptoms,medical history,physical examina-tion,and a range of diagnostic imaging techniques including ultrasound,computed tomography(CT),magnetic resonance imaging(MRI),retrograde pyelography,antegrade pyelography,radionuclide reno-gram and ureteroscopy.Results:Among the 767 patients,359(46.8％)were male and 408(53.2％)were female.The median age of these patients was 37 years(range,14-84 years).Hydronephrosis was observed at left-sided in 357 cases(46.6％),right-sided in 251 cases(32.7％),and bilateral in 159 cases(20.7％).The causes of hydronephrosis were classified as follows:(1)Non-iatrogenic factors were found in 464 cases(60.5％).These included urinary malformations in 355 cases(76.5％),infec-tion in 29 cases(6.3％),pelvic lipomatosis and/or cystitis glandularis in 23 cases(5.0％),ureteral en-dometriosis in 18 cases(3.9％),retroperitoneal fibrosis in 15 cases(3.2％),trauma in 7 cases(1.5％)and other non-iatrogenic factors in 12 cases(2.6％).Some of these patients had multiple non-iatrogenic causes.Among the 355 cases with urinary system malformations,252 cases(71.0％)had ureteropelvic junction obstruction.(2)Iatrogenic ureteral injuries accounted for 210 cases(27.4％),including 112 cases(53.3％)of urological surgical injuries,51 cases(24.3％)of radiotherapy for malignant tumor re-lated injuries,34 cases(16.2％)of gynecological and obstetrical surgical injuries,and 13 cases(6.2％)of general surgical injuries.(3)The cause of hydronephrosis remained unknown in 93 cases(12.1％).Conclusion:Hydronephrosis in adults due to upper urinary tract obstruction has a diverse range of cau-ses,with urinary malformations and iatrogenic ureteral injuries being significant contributors.Urological surgeon involved in upper urinary tract reconstruction should be familiar with these potential causes to fa-cilitate accurate diagnosis and effective treatment.

Objective:To review the diagnosis and treatment process of a patient with relapsed immune thrombotic thrombocytopenic purpura (iTTP) secondary to systemic lupus erythematosus (SLE) and conduct a review of relevant literature, so as to provide a reference basis for the diagnosis and treatment of this disease.Methods:We retrospectively analyzed the diagnosis and treatment process of a patient with relapsed iTTP secondary to SLE who was admitted to the First Affiliated Hospital of Soochow University, and conducted a review of literature.Results:When the patient's iTTP relapsed, it was accompanied by moderate lupus activity, hypofibrinogenemia, renal insufficiency, and pulmonary infection. After adopting therapeutic plasma exchange (TPE), hormone pulse therapy, bortezomib, and comprehensive diagnosis and treatment by a multidisciplinary team, both the patient's iTTP and SLE were relieved.Conclusion:A comprehensive examination should be completed when diagnosing iTTP to rule out the possibility of combining with other autoimmune diseases. For relapsed and refractory cases, treatments targeting plasma cells, such as bortezomib, can be attempted.

Objective:To explore the safety and efficacy of adenosine triphosphate (ATP) in terminating paro-xysmal supraventricular tachycardia (PSVT) in children and the correlation between its efficacy, age and dose.Methods:A retrospective analysis was performed on the clinical data, efficacy and adverse effects of intravenous ATP in 120 children who had received ATP emergency cardioversion among 1 488 children with PSVT hospitalized in the Department of Pediatric Cardiology, Heart Center, First Hospital of Tsinghua University from September 2014 to November 2019.There were 80 boys and 40 girls with the age of (3.50±3.66) years (25 d-15 years). As for the group comparison, the measurement data was subject to the independent samples t test and Mann- Whitney U test; the enumeration data was subject to χ2 test. Results:Among the 120 children with PSVT, there were 42 cases (35.0%) <1 year old, and 24 cases(20.0%) combined with congenital heart disease.There were 8.3% of them (10/120 cases) suffering from tachycardiomyopathy (TCM) secondary to PSVT, whose LVEF increased from (32.70±11.69)% to (40.50±11.63)% after successful control of PSVT ( t=-3.647, P=0.005). The complete termination of PSVT by intravenous ATP was achieved in 53 of 120 cases (44.2%). ATP was given at 0.3 mg/kg, 0.2 mg/kg and 0.1 mg/kg in dose, and the significant effective rate was 56.5%(13/23 cases), 36.4%(32/88 cases) and 0, respectively, which showed that there was a significant difference in the therapeutic effect between different dose groups ( χ2=10.058, P=0.007). There was a significant difference regarding the complete termination rate between children <1 year old and those ≥1 year old [31.0%(13/42 cases) vs.51.3%(40/78 cases), χ2=4.575, P=0.032]. For refractory PSVT, the intravenous ATP was performed based on the absence of cardioversion with continuous pumping of other antiarrhythmic drugs, which achieved a significantly complete termination rate of 55.6% (10/18 cases). The rate of ATP adverse reactions was 2.5%(3/120 cases), presented with sinus arrest in children >1 year old with the dose of 0.2-0.3 mg/kg. Conclusions:It was relatively safe and effective to terminate PSVT in children with ATP, which was related to dose and age.For refractory PSVT, ATP can be intravenously pushed on the basis of continuous pumping of other antiarrhythmic drugs, which can achieve a higher complete termination rate.

Objective:To identify the risk factors for cardiac events like recurrent syncope and fatal events in children with long QT syndrome (LQTS).Methods:A retrospective review involving 69 children with LQTS with the age of (7.6±4.2) years (43 male patients, 26 female patients) admitted in the Heart Center, Division of Pediatric Cardiology, First Hospital of Tsinghua University from August 2013 to March 2019 was conducted.Medical history, surface electrocardiogram, Doppler echocardiography and screening results of pathogenic genes of each patient were analyzed.Patients were divided into cardiac event group (32 cases) and non-cardiac event group (37 cases) according to the presence or absence of cardiac events like syncope or sudden death.Differences between 2 groups were compared using the independent sample Student′s t test and Chi- square test. Results:There were 32 cases among 69 children (46.4%) had recurrent syncope, involving 2 deaths, 14 cases among 69 children (20.3%) had a positive family history and 8 cases among 69 children (11.6%) had family history of sudden death.There were 52 cases among 69 children (75.4%) had arrhythmias.Among them, 32 patients (46.4%) with ventricular arrhythmia, including 11 cases (15.9%) with torsades de pointes (TdP). Ten LQTS-associated pathogenic or pathogenic pathologic mutations were found in 53 cases among 69 children (76.8%). Children with cardiac events had a younger mean age of onset [(4.7±4.1) years vs.(7.4±3.7) years, t=-2.856, P=0.006], a longer mean corrected QT (QTc) [(529±66) ms vs.(478±52) ms, t=3.537, P=0.001] and a higher incidence of TdP (34.4% vs.0%, P<0.001) compared with those who did not have cardiac events.Pathogenic mutations of KCNQ1 and KCNH2 were more frequent in children with cardiac events (62.5% vs.18.9%, χ2=14.178, P=0.001). Conclusions:Children with LQTS are prone to cardiac events.Children with a younger onset age, a longer QTc duration, malignant ventricular tachycardia and mutations of the KCNQ1 and KCNH2 genes are more frequently complicated by cardiac events.

Objective:To investigate clinical electrophysiological characteristics of idiopathic ventricular arrhythmias (VAs) and outcomes of radiofrequency catheter ablation (RFCA) in pediatric patients.Methods:A total of 328 consecutive pediatric patients with VAs and treated with RFCA in the First Hospital of Tsinghua University from January 2014 to December 2019 were recruited, involving 205 males and 123 females with the mean age of (7.8±3.9) years and the mean body weight of (32.8±17.7) kg.Their clinical electrophysiological characteristics, RFCA outcomes of different origins of VAs and complications were analyzed.Results:Among the 328 patients with the mean onset age of (5.4±4.1) years, 57.6% had frequent premature ventricular complex (PVC), 28.7% had paroxysmal ventricular tachycardia (VT) and 13.7% had incessant VT.A total of 38/328 cases (11.6%) VAs children were complicated with tachycardia-induced cardiomyopathy.Except for 13 cases of non-induced VAs, among 315 cases there were 152/328 cases (46.4%) originated from the ventricular outflow tract (including 46.1% of the origination of the right ventricular outflow tract septum, 27.6% of the origination of the left coronary cusp, 18.4% of the origination of the right coronary cusp, and 7.9% of the origination of the right ventricular outflow tract free wall), 55/328 cases (16.5%) originated from the tricuspid valve, 54/328 cases (17.4%) originated from the left posterior fascicle, 39/328 cases (11.9%) originated from the left posterior papillary muscle, 5/328 cases (1.5%) originated from multi-origin VAs, 3/328 cases (0.9%) originated from the left anterior fascicle, and 7/328 cases (2.1%) originated from other origins.Among 307/328 cases (93.6%) VAs patients receiving RFCA, 271/307 cases (88.3%) were instantly successful, 14/307 cases (4.6%) were effectively treated and 22/307 cases (7.2%) were invalid.During the follow-up time of 3 to 36 months, there were 42/271 cases (15.5%) recurrent cases.The mean radiation time and dose were (3.2±5.8) min, and (1.4±2.6) mGy, respectively.The mean dose-area product (DAP) was (384.2±42.6) mGy·cm 2.A total of 4/328 cases (1.2%) reported perioperative vascular complication.In 20 infant patients younger than 3 years, only 1 case was not induced during procedure of RFCA, and the remaining 19 cases were performed with RFCA, including 18/19 cases (94.7%) with an instant success and 4/18 (22.2%) recurrent cases during follow-up.There were 1/20 case (5.0%) had perioperative vascular complication caused by vascular puncture.No serious complications, such as myocardial perforation, cardiac tamponade and atrioventricular block were reported. Conclusions:The right ventricular outflow tract septum is the most common origin of the idiopathic VAs in pediatric patients.VAs originated from the ventricular outflow tract and tricuspid valve usually have an acceptable outcome following ablation.The recurrent rate is high following ablation in VAs cases originated from the left ventricular fascicle and posterior papillary muscle.RFCA is safe and effective for drug resistant or intolerant VAs in infants, but the surgical indications should be strictly mastered and operated by experienced pediatric electrophysiologists.The radiation dose of RFCA can be limited in a safe range under the guidance of the 3-dimensional mapping system.

Objective:To investigate the efficacy and feasibility of transthoracic implantation of permanent left atrial and left ventricular dual-chamber pacemaker for synchronous treatment of cardiac dysfunction due to idiopathic complete left bundle branch block (CLBBB) in children.Methods:The clinical data of five children with cardiac dysfunction due to idiopathic CLBBB and accepting implantation of permanent left atrial and left ventricular epicardial dual chamber pacemaker from January 2015 to July 2019 at the Pediatric Cardiologic Department of the First Hospital of Tsinghua University were analyzed retrospectively. The effects of pacemaker implantation on patients′ cardiac function and cardiac synchrony were evaluated by echocardiogram.Results:Among 5 patients, 2 were males and 3 females. At the time of pacemaker implantation, the age of these patients was 0.5-5.7 years, the left ventricle ejection fraction (LVEF) was 29%-46%, the left ventricle end stage of diastolic diameter was 30-53 mm and the mean Z score was 4.0-34.0. Pacemaker was successfully implanted for all the patients. After the implantation, medications that can suppress atrioventricular node conduction were used and sensed atrioventricular delay (SAV) parameters were modulated until patients′ QRS duration became shortest and the percentage of left ventricular pacing increased to and maintained at 97% to 100%. Patients′ QRS duration was 120-160 ms before implantation and 90-120 ms after implantation. Patients′ cardiac function began to improve 1 day to 1 month after implantation. Patients′ cardiac function normalized after a mean of 1-12 months. LVEF increased from 29%-46% to 55%-67%. During the follow-up, interventricular mechanical delay, septal-to-posterior wall motion delay, and left ventricular systolic dyssynchrony index decreased significantly: IVMD decreased from 31-62 ms to 26-50 ms; SPWMD decreased from 40-63 ms to 10-50 ms and Ts-SD decreased from 34.3-50.3 ms to 16.3-31.4 ms. The global longitudinal strain of left ventricle decreased from -7.7%--13.8% to -13.5%--20.3%.Conclusion:Cardiac dysfunction due to CLBBB in children can be treated with transthoracic implantation of permanent epicardial left atrial and left ventricular dual chamber pacemaker which can substitute three chamber pacemaker to achieve the effects of synchronous therapy that lead to reversion and normalization of cardiac function.

Objective:To investigate the clinical characteristics of focal atrial tachycardia (FAT) and the efficacy of radiofrequency catheter ablation (RFCA) in pediatric FAT.Methods:A total of 125 children with FAT who were treated with RFCA in the First Hospital of Tsinghua University from January 2010 to July 2018 were involved in the study. The clinical characteristics, origin of FAT, success rate of RFCA and recurrence rate and complications post RFCA were retrospectively analyzed, and the efficacy and X-ray radiation were compared between two-dimensional (2D) and 3D mapping system by t test or Chi-square analysis. Results:Among the 125 children, 57 were males and 68 females, age of (7.6±3.5) years old and body weight of (27.7±13.3) kg; and 62 (49.6%) had paroxysmal FAT, and 63 (50.4%) incessant FAT. Twenty-seven patients (21.6%) presented with tachycardia-induced cardiomyopathy (TIC), and 22 of whom (81.5%) were secondary to the incessant FATs. Most of the FATs originated from atrial auricle (46/125, 36.8%). Ablation was successful in 111 children (88.8%), and the recurrence rate was 25.2% (28/111). No complications were identified in the whole group. The efficacy and safety of 3D mapping system (87 cases) was better than that of the 2D mapping system (38 cases), according to the lower recurrence rate ((19.0% (15/79) vs. 40.6% (13/32), χ 2= 3.849, P=0.049), shorter X-ray exposure time ((4.9±2.3) vs. (12.5±5.7) min, t=7.942, P<0.01) and lower radiation doses ((5.3±3.3) vs. (10.9±3.7) mGy, t=8.038, P<0.01). Conclusions:FAT in children is mainly originated from atrial auricle, and incessant FAT is prone to progress to tachycardia-induced cardiomyopathy. RFCA is safe and effective for drug-resistant or drug-intolerant FAT in children, and 3D mapping system should be preferred.