Objective:To investigate the clinical features and prognosis of testicular relapse in pediatric acute lymphoblastic leukemia (ALL).Methods:Clinical data including the age, time from initial diagnosis to recurrence, relapse site, and therapeutic effect of 37 pediatric ALL with testicular relapse and treated in Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences between November 2011 and December 2022 were analyzed retrospectively. Patients were grouped according to different clinical data. Kaplan-Meier analysis was used to evaluate the overall survival (OS) rate and event free survival (EFS) rate for univariate analysis, and Cox proportional-hazards regression model was used to evaluate the influencing factors of OS rate and EFS rate for multivariate analysis.Results:The age at initial diagnosis of 37 pediatric testicular relapse patients was (5±3) years and the time from initial diagnosis to testicular recurrence was (37±15) months. The follow-up time was 43 (22, 56) months. Twenty-three patients (62%) were isolated testis relapse. The 5-year OS rate and EFS rate of the 37 relapsed children were (60±9) % and (50±9) % respectively. Univariate analysis showed that the 2-year EFS rate in the group of patients with time from initial diagnosis to testicular recurrence >28 months was significantly higher than those ≤28 months ((69±10)% vs. (11±11)%, P<0.05), 2-year EFS rate of the isolated testicular relapse group was significantly higher than combined relapse group ((66±11)% vs. (20±13) %, P<0.05), 2-year EFS rate of chimeric antigen receptor T (CAR-T) cell treatment after relapse group was significantly higher than without CAR-T cell treatment after relapse group ((78±10)% vs. (15±10)%, P<0.05). ETV6-RUNX1 was the most common genetic aberration in testicular relapsed ALL (38%, 14/37). The 4-year OS and EFS rate of patients with ETV6-RUNX1 positive were (80±13) % and (64±15) %, respectively. Multivariate analysis identified relapse occurred≤28 months after first diagnosis ( HR=3.09, 95% CI 1.10-8.72), combined relapse ( HR=4.26, 95% CI 1.34-13.52) and CAR-T cell therapy after relapse ( HR=0.15,95% CI 0.05-0.51) were independent prognostic factors for 2-year EFS rate (all P<0.05). Conclusions:The outcome of testicular relapse in pediatric ALL was poor. They mainly occurred 3 years after initial diagnosis. ETV6-RUNX1 is the most common abnormal gene.Patients with ETV6-RUNX1 positive often have a favorable outcome. Early relapse and combined relapse indicate unfavorable prognosis, while CAR-T cell therapy could significantly improve the survival rate of children with testicular recurrence.

Objective:To investigate the clinical characteristics and long-term prognostic factors of relapsed pediatric acute lymphoblastic leukemia (ALL).Methods:Clinical data including the age, time from initial diagnosis to relapse, relapse site, and molecular biological features of 217 relapsed ALL children primarily treated by the Chinese Children's Leukemia Group (CCLG)-ALL 2008 protocol in Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences between April 2008 and April 2015 were collected and analyzed in this retrospective cohort study. Kaplan-Meier analysis was used to evaluate the overall survival (OS) rate and event free survival (EFS) rate for univariate analysis, and Cox proportional-hazards regression model was used to evaluate the influencing factors of OS rate and EFS rate for multivariate analysis.Results:The age at initial diagnosis of 217 relapsed patients was 5 (3, 7) years. There were 135 males and 82 females. The time from initial diagnosis to relapse of 217 children was 22 (10, 39) months. After relapse, 136 out of 217 children (62.7%) received treatment and the follow-up time was 65 (47, 90) months. The 5-year OS rate and EFS rate of the 136 relapsed children were (37±4) % and (26±4) %, respectively. The predicted 10-year OS rate and EFS rate were (35±5) % and (20±4) %, respectively. Univariate analysis showed that the 5-year OS rate in the group of patients with late relapse (43 cases) was significantly higher than those with very early (54 cases) and early relapse (39 cases) ((72±7)% vs. (16±5)%, (28±8)%, χ2=35.91, P<0.05), 5-year OS rate of the isolated extramedullary relapse group (20 cases) was significantly higher than isolated bone marrow relapse group (102 cases) and combined relapse group (14 cases) ((69±11)% vs. (31±5)%, (29±12)%, χ2=9.14, P<0.05), 5-year OS rate of high-risk group (80 cases) was significantly lower than standard-risk group (10 cases) and intermediate-risk group (46 cases) ((20±5)% vs. (90±10)%, (54±8)%, χ2=32.88, P<0.05). ETV6::RUNX1 was the most common fusion gene (13.2%, 18/136). The predicted 10-year OS rate of relapsed children with positive ETV6::RUNX1 was significantly higher than those without ETV6::RUNX1 (118 cases) ((83±9)% vs. (26±5)%, χ2=14.04, P<0.05). The 5-year OS for those accepted hematopoietic stem cell transplantation (HSCT) after relapse (42 cases) was higher than those without HSCT (94 cases) ((56±8)% vs. (27±5)%, χ2=15.18, P<0.05). Multivariate analysis identified very early/early relapse ( HR=3.91, 95% CI 1.96-7.79; HR=4.15, 95% CI 1.99-8.67), bone marrow relapse including isolated bone marrow relapse and combined relapse ( HR=6.50, 95% CI 2.58-16.34; HR=5.19, 95% CI 1.78-15.16), with ETV6::RUNX1 ( HR=0.23, 95% CI 0.07-0.74) and HSCT after relapse ( HR=0.24, 95% CI 0.14-0.43) as independent prognostic factors for OS (all P<0.05). Conclusions:Relapsed pediatric ALL mainly occurs very early and often affects bone marrow, which confer poor outcome. ETV6::RUNX1 is the most common genetic aberration with a favorable outcome. HSCT could rescue the outcome of relapsed children, though the survival rate is still poor.

Objective:To evaluate the upper airway dimension changes in mandibular prognathism accompanied with maxillary retrognathism patients treated with bimaxillary orthognathic surgery by spiral CT.Methods:The data of patients with mandibular prognathism accompanied with maxillary retrognathism who underwent bimaxillary surgery in the Department of Oral and Maxillofacial Surgery of Nanjing Stomatological Hospital, Medical School of Nanjing University from May 2017 to October 2020 were retrospectively analyzed. The surgical method was LeFort Ⅰ osteotomy and bilateral sagittal split mandibular osteotomy. The patients were divided into 2 groups based on the sagittal setback distance of the supramentale(in group A, the setback distance of the supramentale was more than 5 mm; in group B, the setback distance of the supramentale was less than or equal to 5 mm). Spiral CT data were collected 1 week before surgery(T0), 1 month after surgery(T1), and 6-12 months after surgery(T2). Three-dimensional model reconstruction was performed. The airway cross-sectional measurements, length and volumes in T0, T1 and T2 were measured. The effect of bimaxillary orthognathic surgery on upper airway of the patients was evaluated quantitatively. We performed repeated measures analysis of variance to compare the differences of upper airway among T0, T1 and T2 in the same group. The Bonferroni method was used for multiple comparisons if the difference was statistically significant(α=0.017). We used two-factor repeated measures analysis of variance to compare the differences of the upper airway change trend between the two groups.Results:A total of 30 patients were included, including 15 patients in group A, 5 males and 10 females[aged: (21.2±2.3) years]; and 15 patients in group B, 7 males and 8 females[aged: (23.6±2.4) years]. The cross-section area and sagittal diameter of lower velopharyngeal plane(Lvp), the glossopharynx airway volume and total upper airway volume: group A decreased significantly at T1, and did not return to T0 level at T2(all P<0.017); group B decreased significantly at T1(all P<0.017), and returned to T0 level at T2(all P>0.017); the change trend of these indexes between the two groups was statistically significant(all P<0.05). The airway length of the glossopharynx and the total upper airway: group A increased significantly at T1 and T2(both P<0.017); group B had no significant changes at T1 or T2(both P>0.017); the change trend of these two indexes between the two groups was statistically significant(both P<0.05). Conclusions:The glossopharynx airway volume and total upper airway volume decreased in mandibular prognathism accompanied with maxillary retrognathism patients after bimaxillary orthognathic surgery. If the setback distance of the mandible is less than or equal to 5 mm, they can probably return to the preoperative level 6-12 months after surgery. If the setback distance of the mandible is more than 5 mm, they can not return to the preoperative level 6-12 months after surgery.

Objective:To evaluate the upper airway dimension changes in mandibular prognathism accompanied with maxillary retrognathism patients treated with bimaxillary orthognathic surgery by spiral CT.Methods:The data of patients with mandibular prognathism accompanied with maxillary retrognathism who underwent bimaxillary surgery in the Department of Oral and Maxillofacial Surgery of Nanjing Stomatological Hospital, Medical School of Nanjing University from May 2017 to October 2020 were retrospectively analyzed. The surgical method was LeFort Ⅰ osteotomy and bilateral sagittal split mandibular osteotomy. The patients were divided into 2 groups based on the sagittal setback distance of the supramentale(in group A, the setback distance of the supramentale was more than 5 mm; in group B, the setback distance of the supramentale was less than or equal to 5 mm). Spiral CT data were collected 1 week before surgery(T0), 1 month after surgery(T1), and 6-12 months after surgery(T2). Three-dimensional model reconstruction was performed. The airway cross-sectional measurements, length and volumes in T0, T1 and T2 were measured. The effect of bimaxillary orthognathic surgery on upper airway of the patients was evaluated quantitatively. We performed repeated measures analysis of variance to compare the differences of upper airway among T0, T1 and T2 in the same group. The Bonferroni method was used for multiple comparisons if the difference was statistically significant(α=0.017). We used two-factor repeated measures analysis of variance to compare the differences of the upper airway change trend between the two groups.Results:A total of 30 patients were included, including 15 patients in group A, 5 males and 10 females[aged: (21.2±2.3) years]; and 15 patients in group B, 7 males and 8 females[aged: (23.6±2.4) years]. The cross-section area and sagittal diameter of lower velopharyngeal plane(Lvp), the glossopharynx airway volume and total upper airway volume: group A decreased significantly at T1, and did not return to T0 level at T2(all P<0.017); group B decreased significantly at T1(all P<0.017), and returned to T0 level at T2(all P>0.017); the change trend of these indexes between the two groups was statistically significant(all P<0.05). The airway length of the glossopharynx and the total upper airway: group A increased significantly at T1 and T2(both P<0.017); group B had no significant changes at T1 or T2(both P>0.017); the change trend of these two indexes between the two groups was statistically significant(both P<0.05). Conclusions:The glossopharynx airway volume and total upper airway volume decreased in mandibular prognathism accompanied with maxillary retrognathism patients after bimaxillary orthognathic surgery. If the setback distance of the mandible is less than or equal to 5 mm, they can probably return to the preoperative level 6-12 months after surgery. If the setback distance of the mandible is more than 5 mm, they can not return to the preoperative level 6-12 months after surgery.

Objective:To analyze the risk factors of bronchopulmonary dysplasia(BPD)in very preterm infants(VPI), and to provide scientific basis for the prevention and treatment of BPD in VPI.Methods:A prospective multicenter study was designed to collect the clinical data of VPI in department of neonatology of 28 hospitals in 7 regions from September 2019 to December 2020.According to the continuous oxygen dependence at 28 days after birth, VPI were divided into non BPD group and BPD group, and the risk factors of BPD in VPI were analyzed.Results:A total of 2 514 cases of VPI including 1 364 cases without BPD and 1 150 cases with BPD were enrolled.The incidence of BPD was 45.7%.The smaller the gestational age and weight, the higher the incidence of BPD( P<0.001). Compared with non BPD group, the average birth age, weight and cesarean section rate in BPD group were lower, and the incidence of male infants, small for gestational age and 5-minute apgar score≤7 were higher( P<0.01). In BPD group, the incidences of neonatal respiratory distress syndrome(NRDS), hemodynamically significant patent ductus arteriosus, retinopathy of prematurity, feeding intolerance, extrauterine growth restriction, grade Ⅲ~Ⅳ intracranial hemorrhage, anemia, early-onset and late-onset sepsis, nosocomial infection, parenteral nutrition-associated cholestasis were higher( P<0.05), the use of pulmonary surfactant(PS), postnatal hormone exposure, anemia and blood transfusion were also higher, and the time of invasive and non-invasive mechanical ventilation, oxygen use and total hospital stay were longer( P<0.001). The time of starting enteral nutrition, cumulative fasting days, days of reaching total enteral nutrition, days of continuous parenteral nutrition, days of reaching 110 kcal/(kg·d) total calorie, days of reaching 110 kcal/(kg·d) oral calorie were longer and the breastfeeding rate was lower in BPD group than those in non BPD group( P<0.001). The cumulative doses of amino acid and fat emulsion during the first week of hospitalization were higher in BPD group( P<0.001). Multivariate Logistic regression analysis showed that NRDS, invasive mechanical ventilation, age of reaching total enteral nutrition, anemia and blood transfusion were the independent risk factors for BPD in VPI, and older gestational age was the protective factor for BPD. Conclusion:Strengthening perinatal management, avoiding premature delivery and severe NRDS, shortening the time of invasive mechanical ventilation, paying attention to enteral nutrition management, reaching whole intestinal feeding as soon as possible, and strictly mastering the indications of blood transfusion are very important to reduce the incidence of BPD in VPI.

Objective:To evaluate the feasibility of three-dimensional measurement in the study of jaw stability in patients with skeletal Class Ⅲ malocclusion after orthognathic surgery, and to analysis the jaw relapse of risk factors.Methods:Patients with skeletal Class Ⅲ malocclusion who underwent bimaxillary surgery in Department of Oral and Maxillofacial Surgery, Nanjing Stomatological Hospital, from July 2019 to December 2019 were included. CT data at 1 week preoperative (T0), 3 days after surgery (T1), and 6-12 months after surgery (T2) were collected respectively. The 3D model was constructed by 3D Slicer, and the movement of maxilla and mandible after surgery was measured by Geomagic Qualify. The paired student t-test, Wilcoxon rank sum test and Pearson correlation was performed in this study. P<0.05 was considered statistically significant. Results:A total of 15 patients were included, including 5 males and 10 females. The age is from 18 to 25 year old. The average age is 21.3. In horizontal direction, the RGo coordinate has significant difference between T1[(-50.47±4.44) mm] and T2[(-50.06±4.66) mm] ( t=2.948, P=0.011), while all other landmarks have no significantly statistic difference. In the anteroposterior direction, there were significant differences for all maxillary landmarks between T1 with T2 ( P< 0.05). The relapse rates at point of A, Rp, Lp, RMF and LMF were 37.7 %(1.36/3.61), 35.7%(1.15/3.22), 25.4%(0.84/3.31), 26.9%(0.84/3.12), 14.0%(0.41/2.92), respectively. There were significant differences in all mandibular landmarks between T1 with T2 ( P<0.01). The relapse rates at point of B, Pog, Gn, Me, RGo and LGo were respectively 36.9%(1.75/4.74), 53.9%(2.45/4.55), 55.5%(2.72/4.90), 61.7%(2.90/4.70), 85.3%(2.20/2.58), 93.4%(2.40/2.57). The distance of skeletal relapse movement was significantly correlated with the surgery-induced distance ( r: 0.572-0.736, P<0.05). In the vertical direction, there was no significant difference of maxillary landmarks between T1 with T2 ( P > 0.05). For B, Pog, Gn and Me points, there were statistically significant differences ( P< 0.01). From T1 to T2, the mandible has tendency of counter-clockwise rotation. Conclusions:The three-dimensional measurement can accurately reflect the three-dimensional changes of jaw in patients with skeletal Class Ⅲ malocclusion after bimaxillary surgery. No significantly statistic relapse was in horizontal direction, while obvious relapse was occurred in anteroposterior directions. The counter-clockwise rotation of mandible was shown in vertical direction.

Objective:To evaluate the feasibility of three-dimensional measurement in the study of jaw stability in patients with skeletal Class Ⅲ malocclusion after orthognathic surgery, and to analysis the jaw relapse of risk factors.Methods:Patients with skeletal Class Ⅲ malocclusion who underwent bimaxillary surgery in Department of Oral and Maxillofacial Surgery, Nanjing Stomatological Hospital, from July 2019 to December 2019 were included. CT data at 1 week preoperative (T0), 3 days after surgery (T1), and 6-12 months after surgery (T2) were collected respectively. The 3D model was constructed by 3D Slicer, and the movement of maxilla and mandible after surgery was measured by Geomagic Qualify. The paired student t-test, Wilcoxon rank sum test and Pearson correlation was performed in this study. P<0.05 was considered statistically significant. Results:A total of 15 patients were included, including 5 males and 10 females. The age is from 18 to 25 year old. The average age is 21.3. In horizontal direction, the RGo coordinate has significant difference between T1[(-50.47±4.44) mm] and T2[(-50.06±4.66) mm] ( t=2.948, P=0.011), while all other landmarks have no significantly statistic difference. In the anteroposterior direction, there were significant differences for all maxillary landmarks between T1 with T2 ( P< 0.05). The relapse rates at point of A, Rp, Lp, RMF and LMF were 37.7 %(1.36/3.61), 35.7%(1.15/3.22), 25.4%(0.84/3.31), 26.9%(0.84/3.12), 14.0%(0.41/2.92), respectively. There were significant differences in all mandibular landmarks between T1 with T2 ( P<0.01). The relapse rates at point of B, Pog, Gn, Me, RGo and LGo were respectively 36.9%(1.75/4.74), 53.9%(2.45/4.55), 55.5%(2.72/4.90), 61.7%(2.90/4.70), 85.3%(2.20/2.58), 93.4%(2.40/2.57). The distance of skeletal relapse movement was significantly correlated with the surgery-induced distance ( r: 0.572-0.736, P<0.05). In the vertical direction, there was no significant difference of maxillary landmarks between T1 with T2 ( P > 0.05). For B, Pog, Gn and Me points, there were statistically significant differences ( P< 0.01). From T1 to T2, the mandible has tendency of counter-clockwise rotation. Conclusions:The three-dimensional measurement can accurately reflect the three-dimensional changes of jaw in patients with skeletal Class Ⅲ malocclusion after bimaxillary surgery. No significantly statistic relapse was in horizontal direction, while obvious relapse was occurred in anteroposterior directions. The counter-clockwise rotation of mandible was shown in vertical direction.

Objective:To compare the caries experience and the kinds of dental treatment between children with autism spectrum disorders (ASD) and children without systemic disease who were all treated under general anesthesia.Methods:Totally 103 children with ASD who received dental treatments under general anesthesia in 13 professional dental hospitals around China from April to November 2016 were included in the present study. A group of 97 children without systemic disease, according to the age, gender and application propensity score matching method, were chosen as controls, who received dental treatments under general anesthesia between January 2015 to November 2018 in the same hospitals as the children with ASD. Decay missing filling tooth (DMFT/dmft, DMFT for permanent teeth and dmft for primary teeth) indices of two groups of children and the contents of the dental treatments under general anesthesia were analyzed.Results:No significant difference of DMFT/dmft index ［ M( Q25, Q75)］ was found between children with ASD group ［0 (0, 3)/11(8, 14)］ and control group ［0 (0, 3)/9(7, 13)］ ( P>0.05). The average number of dental treatments under general anesthesia and the average number of endodontic treatment in children with ASD were 13 (11, 15) and 3 (2, 6) teeth respectively, while those in the control group were 12 (9, 14) and 2 (1, 4) teeth respectively, the differences were statistically significant ( P<0.01, P<0.05). Conclusions:No significant difference was found between children with ASD and the normal controls who receive dental treatments under general anesthesia in DMFT/dmft index, but the treatment needs of children with ASD is relatively higher, and their tooth decay is relatively severer.

Objective:To investigate the safety of inter-hospital referral and the prognosis of neonatal with critical congenital heart disease.Methods:The criticality score, transit distance and time, and the prognosis of 237 newborns with critical congenital heart disease in Guangdong Provincial People′s Hospital from July 2016 to July 2018 were retrospectively analyzed.Results:A total of 237 children were included (162 male and 75 female) with the median age of 6 days and the median body weight of 2.98 kg.The median transit distance was 90 km.The average value of neonatal critical illness score (NCIS) was (86.54±9.05) scores before transport; 136 cases were greater than 90 scores, 84 cases between 70 and 90 scores, 17 cases less than 70 scores; while the average NCIS was (87.05±8.19) scores when arrived at neonatal intensive care unit (NICU), 138 cases were greater than 90 scores, 82 cases between 70 and 90 scores, 17 cases less than 70 scores.There were no significant differences in the scores of critical cases before and after transfer according to the transfer time and distance ( t=0.346, P>0.05). There was no one death occurred during the transfer process.All over, 222 cases were cured and discharged from the hospital after surgery and or medical interventional treatments, 15 cases died after giving up treatment or losing the opportunity for surgery. Conclusions:It is safe and effective of the inter-hospital transport for the rescue of infants with critical congenital heart disease when followed the principles and transport rules and regulations, with trained workers and special equipments.

Objective: To evaluate the efficacy of the Chinese Children′s Leukemia Group (CCLG) acute lymphoblastic leukemia (ALL) 2008 protocol (CCLG-ALL 2008) in the treatment of children′s T-cell acute lymphoblastic leukemia (T-ALL). Methods: Clinical characteristics and outcomes of 84 newly diagnosed T-ALL children (63 males and 21 females) treated with CCLG-ALL 2008 protocol from April 2008 to April 2015 in the Department of Pediatric Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Kaplan-Meier analysis was used to evaluate the overall survival (OS) and event free survival (EFS), and COX regression was used to evaluate the influencing factors of OS and EFS. Results: (1) Baseline data: 84 children were included, 56 cases (67%) of children were younger than 10 years old. Patients whose white blood cell count≥50×10⁹/L ranked 70% (59/84). Karyotype: 58% (49/84) with normal karyotype, 10% (8/84) with abnormality of chromosome 11, 8%(7/84) with abnormality of chromosome 9, 2%(2/84) with abnormality in both chromosome 11 and chromosome 9, 8% (7/84) with other complex karyotypes. Fusion gene: 33%(28/84) were SIL-TAL1 positive. The patients were grouped by CCLG-ALL 2008 risk score, 40% (34/84) were in the intermediate risk group and 60% (50/84) in the high risk group. (2) Treatment efficacy: 84 cases were followed up until May 30, 2018. The follow-up time was 42.0 (0.3-120.0) months. The sensitivity rate of prednisone treatment was 56% (47/84); the complete response (CR) rate after the induction therapy of vincristine+daunoblastina+L-asparaginase+dexamethasone (VDLD)(d 33) was 88% (74/84); the total CR rate after VDLD induction combined with cyclophosphamide+cytarabine+6-mercaptopurine (CAM) treatment (d80) was 94% (79/84); the recurrence rate was 24% (20/84). Among the 20 recurrent cases, there were 13 cases (65%) with ultra-early recurrence (within 18 months after diagnosis), 6 cases (30%) with early recurrence (18 to 36 months after diagnosis); 1 patient (5%) with late recurrence (over 36 months after diagnosis). During the follow-up period, twenty-eight children (33%) died (22 cases with recurrence or suspending treatment without remission, 2 cases with infection, 1 case of sudden death in chemotherapy, 1 patient failed in transplantation, 1 patient with severe cirrhosis, and 1 patient with unknown cause). (3) Kaplan-Meier analysis: the 5-year OS and EFS of the 84 children were (63±6)% and (60±6)% respectively. (4) Efficacy in different risk groups: prednisone sensitivity rates in the two different risk groups were 100% (34/34) and 26% (13/50), respectively (χ²=3.237, P<0.05). The CR rates at the end of VDLD induction therapy (d 33) were 100% (34/34) and 80% (40/50), respectively (χ²=2.767, P<0.05). The recurrence rate of children in the two groups was 12% (4/34) and 32% (16/50), respectively (χ²=4.245, P<0.05).The mortality rates of the two groups were 21% (7/34) and 42% (21/50), respectively (χ²=3.198, P<0.05). Kaplan-Meier analysis showed that the 5-year OS of the two groups were (77±7)% and (53±8)%; and the 5-year EFS of the two groups were (75±8)% and (49±8)% (χ²=4.235, 3.875, both P<0.05) . (5) COX multivariate regression analysis showed that the classification of risk according to CCLG-ALL 2008 was an important factor influencing the prognosis of children with T-ALL (OR=3.313, 95% CI 1.165-9.422, P=0.025). Conclusions The results of the risk group treatment according to the CCLG-ALL 2008 protocol showed that the long-term survival of children with middle risk was significantly better than that of children at high risk.