Objective:To explore the clinical characteristics,molecular characteristics,treatment and prognosis of pediatric Philadelphia chromosome-like acute lymphoblastic leukemia(Ph-like ALL)with a therapeutic target.Methods:A total of 27 patients of Ph-like ALL with targeted drug target were initially diagnosed in Children's Hospital of Soochow University from December 2017 to June 2021.The data of age,gender,white blood cell(WBC)count at initial diagnosis,genetic characteristics,molecular biological changes,chemotherapy regimen,different targeted drugs were given,and minimal residual disease(MRD)on day 19,MRD on day 46,whether hematopoietic stem cell transplantation(HSCT)were retrospective analyed,and the clinical characteristics and treatment effect were summarized.Survival analysis was performed by Kaplan-Meier method.Results:The intensity of chemotherapy was adjusted according to the MRD level during induced remission therapy in 27 patients,10 patients were treated with targeted drugs during treatment,and 3 patients were bridged with HSCT,1 patient died and 2 patients survived.Among the 24 patients who did not receive HSCT,1 patient developed relapse,and achieved complete remission(CR)after treatment with chimeric antigen receptors T cells(CAR-T).The 3-year overall survival,3-year relapse-free survival and 3-year event-free survival rate of 27 patients were(95.5±4.4)％,(95.0±4.9)％and(90.7±6.3)％respectively.Conclusion:Risk stratification chemotherapy based on MRD monitoring can improve the prognosis of Ph-like ALL in children,combined with targeted drugs can achieve complete remission as soon as possible in children whose chemotherapy response is poor,and sequential CAR-T and HSCT can significantly improve the therapeutic effect of Ph-like ALL in children whose MRD is continuously positive during induced remission therapy.

Objective:To analyze the surveillance data of Kashin-Beck disease (KBD) in Shandong Province, master the changes of KBD, and provide scientific basis for formulating prevention and control strategies of KBD and adjustment of the prevention and control focus of KBD.Methods:According to the requirements of the "National KBD Monitoring Plan" and the "Shandong KBD Monitoring Plan", from 2016 to 2020, the disease monitoring was carried out in the villages of KBD areas in Shandong Province, including the number of clinical positive cases, the number of X-ray positive cases, and the number of metaphyseal positive cases. The detection rate was calculated, and the dynamic changes of the disease was analyzed macroscopically.Results:From 2016 to 2020, a total of 1 816 children aged 7 - 12 years were clinically examined in Shandong Province, and no child with KBD was found. A total of 1 224 children were examined by X-ray, among which 1 positive patient was found in 2016 and 2017, respectively, both of whom were metaphyseal changes, with a total detection rate of 0.16%. There were 367 adult patients with KBD in Shandong Province, including 304 patients with grade Ⅰ, 47 patients with grade Ⅱ and 16 patients with grade Ⅲ. All villages in the KBD areas in Shandong Province had reached the elimination standard, with the elimination rate of 100%.Conclusion:No clinical case of KBD is detected in children aged 7 - 12 years in Shandong Province from 2016 to 2020, which has reached the elimination standard and is in a continuous elimination state.

This study reports the diagnosis and treatment of 2 children with isolated testicular recurrence (ITR) of acute B lymphoblastic leukemia (B-ALL) treated with CD 19 targeted chimeric antigen receptor T (CD 19 CAR-T) cells in May and December 2019 in the Department of Hematology and Oncology, Children′s Hospital of Soochow University, and explores the efficacy of CD 19 CAR-T cells therapy versus conventional radiotherapy and chemotherapy through literature review.Both cases were diagnosed as B-ALL by the morphologic, immunologic, cytogenetic and molecular biology methods.ITR was diagnosed by testicular biopsy at 60 months and 38 months after initial diagnosis in 2 cases, respectively.After infusion of CD 19 CAR-T cells at 7.0×10 6/kg and 1.5×10 7/kg, respectively for 7-10 days, testicular leukemia cell infiltration disappeared and complete remission was obtained.Among them, case 2 developed cytokine release syndrome and immune effector cell-related neurotoxicity syndrome after treatment, which was improved after drug intervention.It is suggested that CD 19 CAR-T cells are effective in the treatment of ITR in children, which may be an alternative to orchiectomy or local radiotherapy for ITR in children with B-ALL.

Objective To measure the peripheral dose distributions of the mobile head cone beam computed tomography (CBCT) and evaluate the impact of CBCT on the surrounding personnel and environment, and provide data support for clinical radiation protection management. Methods Combined with the structural characteristics of CBCT, AT1123 was used in the direction of 0° (counterclockwise), 45°, 90°, 135°, 180°, 225°, 270° and 315° in front of CBCT to measure the ambient dose equivalent rate of 30 cm, 80 cm and 130 cm away from the ground when the equipment was normally out of the beam, and the boundary of the temporary control area was drawn. At the same time, the dose level behind the lead screen 1 m away from the external surface of the equipment was measured and analyzed. Results The dose field around CBCT was symmetrically distributed with the dividing line of 0° and 180°, and the radiation dose level of 5.5 m in the direction of 0°, 3.5 m in the direction of 45°, 0.5 m in the direction of 90° and within 1.0 m in the direction of 180° (inside the "spoon" type) was higher than 2.5 μSv/h. The radiation dose levels of CT aperture 0° (straight forward), 45° and 315° behind the lead screen 1 m away from the equipment surface were 0.37 μSv/h, 0.22 μSv/h and 0.54 μSv/h, respectively. Conclusion The results show that the radiation dose around the mobile head cone beam CT is in a low dose level, the distribution of the dose field can provide necessary reference for the administrative and medical personnel to strengthen the radiation safety management. At the same time, it is suggested that lead screens should be set up in the clinical use of mobile CT to ensure the health and safety of the surrounding people and the environment.

To establish the experts consensus on the management of delirium in critically ill patients.A special committee was set up by 15 experts from the Chinese Critical Hypothermia-Sedation Therapy Study Group.Each statement was assessed based on the GRADE (Grading of Recommendations Assessment,Development,and Evaluation) principle.Then the Delphi method was adopted by 36 experts to reassess all the statements.(1) Delirium is not only a mental change,but also a clinical syndrome with multiple pathophysiological changes.(2) Delirium is a form of disturbance of consciousness and a manifestation of abnormal brain function.(3) Pain is a common cause of delirium in critically ill patients.Analgesia can reduce the occurrence and development of delirium.(4) Anxiety or depression are important factors for delirium in critically ill patients.(5) The correlation between sedative and analgesic drugs and delirium is uncertain.(6) Pay attention to the relationship between delirium and withdrawal reactions.(7) Pay attention to the relationship between delirium and drug dependence/ withdrawal reactions.(8) Sleep disruption can induce delirium.(9) We should be vigilant against potential risk factors for persistent or recurrent delirium.(10) Critically illness related delirium can affect the diagnosis and treatment of primary diseases,and can also be alleviated with the improvement of primary diseases.(11) Acute change of consciousness and attention deficit are necessary for delirium diagnosis.(12) The combined assessment of confusion assessment method for the intensive care unit and intensive care delirium screening checklist can improve the sensitivity of delirium,especially subclinical delirium.(13) Early identification and intervention of subclinical delirium can reduce its risk of clinical delirium.(14) Daily assessment is helpful for early detection of delirium.(15) Hopoactive delirium and mixed delirium are common and should be emphasized.(16) Delirium may be accompanied by changes in electroencephalogram.Bedside electroencephalogram monitoring should be used in the ICU if conditions warrant.(17) Pay attention to differential diagnosis of delirium and dementia/depression.(18) Pay attention to the role of rapid delirium screening method in delirium management.(19) Assessment of the severity of delirium is an essential part of the diagnosis of delirium.(20) The key to the management of delirium is etiological treatment.(21) Improving environmental factors and making patient comfort can help reduce delirium.(22) Early exercise can reduce the incidence of delirium and shorten the duration of delirium.(23) Communication with patients should be emphasized and strengthened.Family members participation can help reduce the incidence of delirium and promote the recovery of delirium.(24) Pay attention to the role of sleep management in the prevention and treatment of delirium.(25) Dexmedetomidine can shorten the duration of hyperactive delirium or prevent delirium.(26) When using antipsychotics to treat delirium,we should be alert to its effect on the heart rhythm.(27) Delirium management should pay attention to brain functional exercise.(28) Compared with non-critically illness related delirium,the relief of critically illness related delirium will not accomplished at one stroke.(29) Multiple management strategies such as ABCDEF,eCASH and ESCAPE are helpful to prevent and treat delirium and improve the prognosis of critically ill patients.(30) Shortening the duration of delirium can reduce the occurrence of long-term cognitive impairment.(31) Multidisciplinary cooperation and continuous quality improvement can improve delirium management.Consensus can promote delirium management in critically ill patients,optimize analgesia and sedation therapy,and even affect prognosis.

Objective: To explore the clinical efficacy of S-1 single agent adjuvant chemotherapy for the patients undergoing radical resection of extrahepatic biliary carcinoma. Methods: The clinical data of 108 patients with extrahepatic biliary carcinoma receiving radical resection who were admitted from January 2014 to June 2017 were retrospectively analyzed. There were 62 males(57.4%)and 46 females(42.6%),with a median age of 59 years (range:26 to 79 years),10 cases(9.3%) in stage Ⅱ,85 cases(78.7%) in stage Ⅲ, and 13 cases (12.0%) in stage Ⅳ, 40 cases(37.0%) of hilar cholangiocarcinoma, 8 cases(7.4%) of middle cholangiocarcinoma, 25 cases (23.2%) of distal cholangiocarcinoma, 35 cases(32.4%) of gallbladder carcinoma.After radical resection of extrahepatic biliary carcinoma, 49 patients receiving S-1 single agent chemotherapy and 59 patients receiving non-special treatment were divided into the chemotherapy group and the operation group,respectively. All the dates of the patients were followed up and collected with the overall survival time,tumor-free survival time,1,2 and 3-year survival rate after operation,and the rate of major toxic reaction during chemotherapy of the chemotherapy group. Survival curve was drawn by the Kaplan-Meier method, and survival analysis was done using the Log-rank test. Results: There were no significant differences in the general date of two groups(sex, age, tumor size, tumor site, TNM stages, degree of differentiation). The median overall survival time and the median tumor-free survival time in the chemotherapy group were 27 months and 21 months,respectively,and in the operation group were 21 months and 17 months,respectively. There were differences between the two groups in the overall survival rates(χ²=3.967,P<0.05) and the 2 and 3-year survival rate(63.3%,36.6%;41.6%,20.4%;χ²=4.510,P<0.05;χ²=6.143,P<0.05),but the 1-year overall survival rate (83.4%,79.7%)was not statistically significant(χ²=0.286,P>0.05). There were no significant differences in the tumor-free survival time,1,2 and 3-year tumor-free survival rate(77.6%,41.4%,33.1%;62.7%,30.9%,21.2%)between the two groups(χ²=0.876,P>0.05;χ²=0.252,P>0.05;χ²=1.571,P>0.05;χ²=3.323,P>0.05,respectively). The main toxic reaction during chemotherapy were dyspepsia(28.6%, 14/49), anemia(26.5%, 13/49), and leukopenia(22.5%, 11/49), all of which were mild. Conclusion S-1 single agent chemotherapy after radical reseetion of extrahepatic biliary carcinoma could effectly improve the survival of patients and all of the main toxic reaction during chemotherapy were mild.

Objective: To evaluate the efficiency and safety of low intensity conditional regimen for children with Fanconi anemia (FA) receiving allogenic hematopoietic stem cells transplantation (allo-HSCT). Methods: Four patients diagnosed as Fanconi anemia were enrolled in this study. One patient received HLA-identical sibling donor hematopoietic stem cell transplantation, two patients underwent unrelated donor matched (UD) HSCT, and one patient received unrelated cord blood transplantation. The conditional regimen consisted of Busulfan with low dose of cyclophosphamide. Results: All 4 cases succeeded in allo-HSCT. The median time for neutrophils engraftment was 11(9-15) day, median time to platelets (PLT) engraftment was 12 (8-28) day. One case occurred with grade I of aGVHD, 1 case with hemorrhagic cystitis. No patient happened with hepatic veno-occlusive disease (VOD). Conclusion Low intensity of conditional regimen is efficient and safe which should be recommended for FA patients with HSCT.

Objective: To evaluate the efficiency and safety of low intensity conditional regimen for children with Fanconi anemia (FA) receiving allogenic hematopoietic stem cells transplantation (allo-HSCT). Methods: Four patients diagnosed as Fanconi anemia were enrolled in this study. One patient received HLA-identical sibling donor hematopoietic stem cell transplantation, two patients underwent unrelated donor matched (UD) HSCT, and one patient received unrelated cord blood transplantation. The conditional regimen consisted of Busulfan with low dose of cyclophosphamide. Results: All 4 cases succeeded in allo-HSCT. The median time for neutrophils engraftment was 11(9-15) day, median time to platelets (PLT) engraftment was 12 (8-28) day. One case occurred with grade I of aGVHD, 1 case with hemorrhagic cystitis. No patient happened with hepatic veno-occlusive disease (VOD). Conclusion: Low intensity of conditional regimen is efficient and safe which should be recommended for FA patients with HSCT.
Busulfan ; Fanconi Anemia ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Transplantation Conditioning

Objective: To explore the effect of influenza and 23 valent pneumococcal polysaccharide pneumococcal vaccinations on symptom-improvement among elderly with chronic obstructive pulmonary diseases (COPD). Methods: Data was gathered from 4 communities in 3 National Demonstration Areas set for comprehensive prevention and control of chronic non- communicable diseases in Chongqing city and Ningbo city respectively, from November 2013 to October 2014. The communities were selected by cluster sampling and divided into 4 groups: (1) injected influenza vaccines; (2) injected with pneumococcal vaccines; (3) received both of the two vaccines; (4) the control group that without any intervention measures. All the subjects aged from 60 to 75 were selected to fill in demographic information questionnaire and receive (COPD assessment test, CAT) scores twice, before intervention and 1 year after the vaccination. SAS 9.4 software was used to analyze the change of symptoms and CAT scores before and after the intervention program and comparing the improvement on symptoms among the elderly people under study. Results: A total of 1 244 subjects with nearly same baseline conditions after the propensity score matching, were involved in this study. CAT scores appeared as Median=21 (IQR: 17-26) at baseline. The CAT scores appeared as Median=18 (IQR: 14-24), decreasing in all the 3 vaccinated groups, one year after the intervention program (influenza vaccines, matching t test, t=-6.531, P=0.403; pneumococcal vaccines, Wilcoxon test, H=-9 623, P<0.001; combined vaccine vaccines, matching t test, t=-10.803, P<0.001). However, in the control group, no obvious change was observed (Wilcoxon H=1 167, P=0.403). Proportions of impacts at high or very high levels all decreased in the 3 intervention groups, while little change was observed in the control group. Outcomes from the Factorial analysis suggested that influenza vaccination could improve the general conditions and symptoms including cough, chest tightness, dyspnea, physical activities, and stamina. Pneumococcal vaccination appeared more effective on all of symptoms and indicators. Conclusion: Pneumococcal and influenza vaccination seemed helpful for elderly people suffering COPD to improve the general health condition.
Aged ; Humans ; Influenza Vaccines/immunology* ; Influenza, Human ; Pneumococcal Vaccines/immunology* ; Pneumonia, Pneumococcal/prevention & control* ; Pulmonary Disease, Chronic Obstructive/complications* ; Streptococcus pneumoniae ; Surveys and Questionnaires ; Vaccination/statistics & numerical data* ; Vaccine Potency

Objective To explore the curative effect and prognosis of umbilical cord blood in the treatment of hematological diseases in children. Method The clinical data of 51 children who underwent umbilical cord blood transplantation from January 2011 to June 2016 were analyzed retrospectively. Results In 51 children (34 males and 17 females) with median age of 62 months, 32 children had malignant hematologic diseases and 19 children had nonmalignant hematologic diseases. Two children died before the granulocytes were reconstructed, 4 children had primary implantation failure, and 45 children had successfully implantation. The median time of implantation was 16 d, and the median time of platelet implantation was 23 d. The incidence of peri-implantation syndrome was 46.94%. The 100 day survival rate and long-term overall survival (OS) in children with peri-implantation syndrome were (73.9±9.2)% and (50.2±11.7)% respectively, which were significantly lower than the OS (100%) in children without peri-implantation syndrome (P<0.01). The incidence of acute graft versus host disease (aGVHD) was 55.10%, among which Ⅱ-Ⅲ degrees of aGVHD was 28.57% and Ⅳdegrees of aGVHD was 26.53%. The 100 day OS in children with Ⅳ degrees of aGVHD was (61.5±13.5)%, and The OS in children with Ⅲ and Ⅳ degrees of aGVHD were (75.0±21.7)% and (44.9±14.1)% respectively, and the OS in children without aGVHD was (90.2±6.6)%. The difference was statistically significant (χ2=14.35,P=0.002). The incidence of chronic GVHD (cGVHD) was 28.57%. The long-term OS in children with cGVHD was (72.7±13.4)%, while OS in children without cGVHD was 100%. The 100 days OS was (86.0±4.9)%. Long-term OS in cord blood transplantation was (77.9±6.3)%, among which OS for malignant hematological diseases was (76.6±7.8)% and OS for nonmalignant hematological diseases was (79.5±11.3)%. Among malignant hematological diseases, the OS in acute lymphoblastic leukemia (ALL) was (87.5±11.7)%, OS in acute myeloid lymphocytic leukemia (AML) was (76.7±10.3)%, and OS in myelodysplastic syndrome (MDS) was (33.3±27.2)%. Conclusions Umbilical cord blood transplantation is an effective treatment for hematologic diseases in children. It is important to treat the peri-implantation syndrome. Prevention and treatment Ⅲ/Ⅳ degree of aGVHD and cGVHD are important strategies to improve the efficacy of umbilical cord blood transplantation.