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Objective:To develop a scale to assess motivation and use of learning strategies in the course of biochemistry for medical students in the theoretical frame of Zimmerman's cognitive theory.Methods:A total of 62 items was developed in the scale based on the revised motivated strategies for learning questionnaire (MSLQ), and 323 medical students from Shanghai Jiao Tong University School of Medicine were selected as subjects. The collected data were analyzed by item correlation, reliability and exploratory factor analysis.Results:Results of reliability analyses showed that Cronbach's α of the scale and two sub-scales were robust (all greater than 0.8), demonstrating good internal consistency. Results of exploratory factor analysis revealed that three factors in the motivation sub-scale were intrinsic value, self-efficacy and learning anxiety, and eight factors in the strategy sub-scale were critical thinking, organization, rehearsal, learning monitoring, learning planning, learning summarizing, time and study environment management, and peer learning.Conclusion:The reliability and validity of the scale are considered to be good, and the scale is of certain application value to understand self-regulated learning of biochemistry for medical students.
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Griscelli syndrome type Ⅱ (GS2) is a rare disease, and patients with GS2 are susceptible to secondary haemophagocytic lymphohistiocytosis (HLH). GS2 accompanied by secondary HLH has a dangerous clinical course, high mortality, and a high miss-diagnosis rate.In this paper, the pathogenesis and prognosis of a case confirmed as GS2 with secondary HLH by gene screening were reported, so as to improve diagnosis and treatment of this disease.The patient had clinical manifestations of silver hair and eye lashes, recurrent pulmonary infection, contiuning high fever, significantly increased ferroprotein levels and decreased fibrinogen levels.Besides, RAB27A gene homozygous mutations were found in the patient, originating from her parents (p.P126Qf3*3 frameshift mutation). This finding confirmed the diagnosis of GS2.The patient underwent transplantation of marrow stem cells from her father since the father-daughter HLA was 7/10.The follow-up results showed that the patient was still alive and healthy 2 years after transplantation.
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investigate the serum angiopoietin-Ⅱ (AngⅡ) levels in acute pancreatitis (AP) patients and the relationship between AngⅡlevels and the disease severity of AP based on the new Atlanta classification criteria. Methods Seventy-seven patients with AP who were treated in the Affiliated Baiyun Hospital of Guizhou Medical University were included in this prospective study. According to the revised Atlanta classification, the modified Mashall scoring system was used to divided patients into AP with the persistent organ failure(POF) group and without POF group, and the CECT was used to divided patients into AP with pancreatic necrosis(PN) group and without PN group. The levels of AP 72 h after admission of two groups were compared with t test, and Spearman analysis was used to analyze the correlation between common severity indicators and Ang Ⅱ. The receiver operating characteristic (ROC) was performed to analyze the area under curve(AUC), sensitivity and specificity of Ang Ⅱ in predicting POF and PN. Results According to the modified Mashall scoring system, there were 18 patients (23.38%) in POF group and 59 patients (76.62%) in without POF group. According to CECT, 22 patients (28.57%)were in PN group and 55 patients(71.43%) in without PN group. The AngⅡof the POF and PN groups was significantly higher than those without POF and PN groups (P＝0.001, 0.011) on day 1 on admission. The cut-off value was 126.44 μg/L, and the AUC, sensitivity and specificity for predicting POF were 0.739, 83.3% and 68.4%, which was significantly better than that of traditional predictive indicators, such as C-reaction protein (CRP), procalcitonin (PCT), blood urea nitrogen (BUN), bedside index for severity in AP (BISAP) score and Ranson score. When the cut-off value was 130.90 μg/L, the AUC, sensitivity and specificity for predicting PN were 0.703, 77.3% and 73.6%, respectively, which was significantly better than that of all traditional single indicator. The predicted value was less than PCT and CRP 2 or 3 day after admission. In addition, the Ang Ⅱlevel 1 day after admission was closely related to the Ranson score, BISAP score, and PCT level which was commonly used indicators of AP severity. Conclusions Under the new Atlanta classification criteria, Ang Ⅱ is superior to the traditional predictive indicators commonly used for predicting POF, and is a better single biochemical indicator for predicting PN in clinic.
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Objective@#To investigate the serum angiopoietin-Ⅱ (Ang Ⅱ) levels in acute pancreatitis (AP) patients and the relationship between AngⅡ levels and the disease severity of AP based on the new Atlanta classification criteria.@*Methods@#Seventy-seven patients with AP who were treated in the Affiliated Baiyun Hospital of Guizhou Medical University were included in this prospective study. According to the revised Atlanta classification, the modified Mashall scoring system was used to divided patients into AP with the persistent organ failure(POF) group and without POF group, and the CECT was used to divided patients into AP with pancreatic necrosis(PN) group and without PN group. The levels of AP 72 h after admission of two groups were compared with t test, and Spearman analysis was used to analyze the correlation between common severity indicators and Ang Ⅱ. The receiver operating characteristic (ROC) was performed to analyze the area under curve(AUC), sensitivity and specificity of Ang Ⅱ in predicting POF and PN.@*Results@#According to the modified Mashall scoring system, there were 18 patients (23.38%) in POF group and 59 patients (76.62%) in without POF group. According to CECT, 22 patients (28.57%)were in PN group and 55 patients(71.43%) in without PN group. The Ang Ⅱ of the POF and PN groups was significantly higher than those without POF and PN groups (P=0.001, 0.011) on day 1 on admission. The cut-off value was 126.44 μg/L, and the AUC, sensitivity and specificity for predicting POF were 0.739, 83.3% and 68.4%, which was significantly better than that of traditional predictive indicators, such as C-reaction protein (CRP), procalcitonin (PCT), blood urea nitrogen (BUN), bedside index for severity in AP (BISAP) score and Ranson score. When the cut-off value was 130.90 μg/L, the AUC, sensitivity and specificity for predicting PN were 0.703, 77.3% and 73.6%, respectively, which was significantly better than that of all traditional single indicator. The predicted value was less than PCT and CRP 2 or 3 day after admission. In addition, the Ang Ⅱlevel 1 day after admission was closely related to the Ranson score, BISAP score, and PCT level which was commonly used indicators of AP severity.@*Conclusions@#Under the new Atlanta classification criteria, Ang Ⅱ is superior to the traditional predictive indicators commonly used for predicting POF, and is a better single biochemical indicator for predicting PN in clinic.
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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.
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The safety of decitabine as bridging treatment before allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with refractory hematological malignancies was evaluated.All 11 cases succeeded in hematopoietic reconstitution.The main adverse reaction was hematological toxicity.Neither did infections occur,nor drug-induced liver damage and renal impairment during decitabine administration.Most cases showed grade Ⅰ-Ⅱ gastrointestinal adverse events.One case was diagnosed as severe acute graft versus host disease and died of intracranial hemorrhage on day 61 after allo-HSCT.The other 10 patients survived.Decitabine bridge is a safe regimen before allo-HSCT in children with refractory hematological malignancies.
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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.
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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.
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Objective To compare effect of three therapies( modified constraint-induced movement therapy (mCIMT), constraint-induced movement therapy (CIMT) and Bobath therapies)on the recovery of motor function in upper-extremities of patients with apoplexy hemiparalysis. Methods Fifty-seven patients with apoplexy hemiparalysis were randomly divided into CIMT treatment group ( n = 19), mCIMT group (n = 19) and Bobath group (n = 19). Patients in Bobath group exercised 2 h/d, 5 d/week for 6 weeks. CIMT group and mCIMT group had restriction of movement at the unimpaired upper extremity by placing it in a sling a whole day for training using impaired extremity. Patients in CIMT treatment group exercised 6 h/d,5 days a week for 2 weeks in a row; mCIMT treatment group exercised for 1 h/d, 3 days a week, and also exercised in daily life at least 2 hours every day, for ten consecutive weeks. The patients in all three groups were assessed for upper-extremities motor function using FMA of Fugl-Meyer, for recovery of upperextremities motor speed using STEF and for recovery of daily ability at respective time-points of pre-treatment and the second week, sixth week and tenth week of post-treatment. Results In 3 assessments in the 3 groups there was no obvious change before treatment, while the FMA score was 45. 2 ± 5.1 (F = 1. 107 ,P =0. 382), BI score was 63. 1 ± 10. 3 ( F = 0. 930, P = 0. 401 ), STEF score was 63.4 ± 8.6 ( F = 1. 843, P =0. 168). At the second week, sixth week and tenth week of the treatment, the difference of assessment in the three groups was significant. The improvement in CIMT group was better after 2 weeks treatment compared with Bobath and mCIMT group, while the Bobath group showed better results than mCIMT group did after 6 weeks treatment. After 10 weeks treatment, the FMA, STEF in mCIMT and CIMT groups were improved better than Bobath group, while the BI score in mCIMT group was better than that in Bobath and CIMT group. Conclusions The mCIMT and CIMT therapy are better in improving the upper limb function in stroke patients compared with Bobath, while the mCIMT therapy is more effective in improving the abilities of daily life in stroke patients compared with the other two treatments.