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Hereditary thrombotic thrombocytopenic purpura (hTTP) in children is a rare but severe and fatal thrombotic microangiopathy. The etiology of the disease is the persistent severe deficiency of the enzyme ADAMTS13 gene mutation, resulting in microangiopathic hemolytic anemia, thrombocytopenia, neuropsychiatric symptoms, fever, and renal involvement. Different from adults, children with hTTP present earlier onset of the disease and are more likely to develop long-term complications in brain and kidney, so that the need for preventive replacement therapy is more urgent. This article reviews the research progress of hTTP in children.
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Objective:To develop an total knee arthroplasty(TKA) enhanced recovery after surgery information management system based on "cloud + terminal" data-drivene technology, which can be used in the perioperative interaction between doctors and patients and rehabilitation management of TKA, and evaluate its implementation effect.Methods:The clinical data of 96 patients with unilateral TKA from Qilu Hospital Shandong University were analyzed retrospectively. Forty eight patients in the control group from February to August, 2018 were treated with traditional management. From February to August, 2019, 48 patients in the research group adopted the accelerated rehabilitation information management system based on data-driven technology, which can automatically match, interact and push the information between medical and patient.Results:The Self-rating Anxiety Scale(SAS) score of the intervention group on hospitalization day was 41.52±4.65, which was better than that of the control group (52.60±4.84). The difference between the two groups was statistically significant ( F value was 11.446, P<0.05). The scores of Hospital for Special Surgery(HSS), Visual Analogue Scale(VAS) and SAS in the intervention group 3 days after operation were 56.31±5.81, 2.79±0.68, 38.69±3.81, which were better than those of the control group (51.31±5.20, 4.31±0.78, 46.02±3.86). The difference between the two groups was statistically significant ( F value was 4.44, 10.19, 9.37, all P<0.05); the HSS, VAS, and SAS scores of the intervention group one week after operation were 71.35±3.59, 1.98± 0.67, 36.81±4.03, better than the control group (67.15±3.67, 2.60±0.87, 39.48±4.04), the difference between the two groups was statistically significant ( F value was 5.683, 3.951, 3.237, all P<0.05); the postoperative hospital stay in the intervention group was (3.98±0.84) days, which was less than (6.00±0.80) days in the control group, the difference was statistically significant ( F value was 146.286, P<0.05); The patients′ satisfaction score in the intervention group during medical treatment was 97.46±1.73, which was higher than the control group (94.35±2.04), the difference was statistically significant ( F value was 64.911, P<0.05). Conclusions:TKA enhanced recovery management system based on "cloud + terminal" data-drivene can improve the management level of TKA patients, reduce the length of stay and improve satisfaction of patients.
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The clinical and laboratory data of a patient with chronic refractory immune thrombocytopenia (ITP) who had a significant increase in the proportion of inhibitory T cells in the hematological oncology center of Beijing Children′s Hospital Affiliated to Capital Medical University in February 2018 and regularly followed up in the outpatient department received a retrospective analysis.This 8-year-old patient′s clinical features were 6 years of skin and mucous membrane bleeding spots and petechiae recurring, with occasionally nasal epistaxis.Physical examination: cardiopulmonary abdomen and nervous system examinations are normal, and no superficial lymphadenopathy is touched.Blood routine indicated that the platelets were 2.00×10 9/L, and white blood cell count and hemoglobin level were normal.Bone marrow suggested that hyperplasia was significantly active, with more than 300 megakaryocytes.The patient was diagnosed with ITP, and he was treated with first-line treatment with gamma globulin and oral Corticosteroids.The first-line treatment with high-dose Dexamethasone therapy was repeated, and the second-line treatment was low-dose Rituximab combined with high-dose Dexamethasone.Evaluations had been conducted with every relapsed course, and Cyclosporine was administered orally on the basis of the highly suppressive T cells.After half a month, the child achieved partial remission and continued for 3 months, and then reached a complete remission of 6 months (till this paper). It is suggested that, with ITP as an immunological disease with high heterogeneity, the immune abnormality index is expected to become a breakthrough in the development of precise treatment.
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Objective:To analyze the clinical characteristics and diagnosis and treatment of children with Kasabach-Merritt phenomenon (KMP).Methods:A retrospective analysis was conducted on the clinical data and follow-up data of 8 patients diagnosed KMP in Beijing Children′s Hospital, Capital Medical University from January 2016 to January 2019.The clinical data included laboratory examination, diagnosis, treatment and prognosis.Results:Among the 8 children with KMP, 6 cases were male and 2 cases were female.The median onset age was 4 (0-17) months, 2 cases of neonatal onset.The median onset to the diagnosis time was 59 (34-140) days; 6 cases with bone destruction; 6 cases had misdiagnosis and mistreatment history, they were misdiagnosed as idiopathic thrombocytopenic purpura, Evans syndrome, abnormal bone and joint development; 4 cases were Kaposiform hemangioendothelioma; 8 cases were used alone or combined with the application of hormones, Sirolimus, and Vincristine, 7 patients underwent interventional therapy.All patients survived with a median follow-up period of 487 (112-1 033) days.Median time of platelet count returned to normal was 24.5 (7-60) days, and median time of fibrinogen returned to normal was 20 (7-30) days.Median time of D-dimer dropped to a normal was 105 (40-240) days.Conclusions:Children with concurrent platelet count and coagulation abnormalities should be considered with KMP.Doctors need to identify the potential visceral vascular lesions.Early diagnosis and treatment are important, which can improve the clinical prognosis of patients.
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Objective:To study the effect of pidotimod combined with montelukast sodium in the treatment of patients with asthma, and its influence on the levels of inflammatory factors and immune function.Methods:From February 2019 to February 2020, 102 patients with bronchial asthma admitted to Zhuji People's Hospital were randomly divided into the observation group and the control group, with 51 cases in each group.The control group was treated with montelukast sodium, and the observation group was given pidotimod combined with montelukast sodium.The clinical effect, lung function index, inflammatory factors, T lymphocyte subsets and the incidence of adverse reactions were compared between the two groups.Results:The total effective rate of the observation group was 90.20%(46/51), which was higher than that of the control group [74.51%(38/51)](χ 2=4.317, P=0.038). After treatment, FEV 1, FVC, PEF in the observation group were (82.89±6.94)%, (3.89±0.45)L, (3.49±0.49)L/s, respectively, which were higher than those in the control group [(77.89±6.79)%, (3.58±0.43)L, (3.21±0.45)L/s] ( t=7.370, 5.753, 4.239, all P<0.05). After treatment, the serum levels of procalcitonin (PCT), interleukin-6 (IL-6), C-reactive protein (CRP) in the observation group were (1.45±0.49)μg/L, (9.87±2.65)mg/L, (5.79±1.48)mg/L, respectively, which were lower than those in the control group [(1.79±0.55)μg/L, (12.34±2.97)mg/L, (7.23±1.68)mg/L] ( t=4.624, 6.542, 8.090, all P<0.05). After treatment, the CD 3+ , CD 4+ , CD 4+ /CD 8+ in the observation group were (53.43±5.43)%, (46.34±5.31)%, (1.54±0.23), respectively, which were higher than those in the control group [(48.09±5.17)%, (41.89±5.15)%, (1.26±0.22)], while the CD 8+ in the observation group[(30.65±3.54)%] was lower than that in the control group[(33.72±3.69)%], the differences were statistically significant ( t=3.901, 4.080, 4.072, 5.967, all P<0.05). During the treatment period, the incidences of adverse reactions in the observation group and the control group were 15.69%(8/51) and 9.80%(5/51), respectively, there was no statistically significant difference between the two groups (χ 2=0.793, P=0.373). Conclusion:Pidotimod combined with montelukast sodium has significant therapeutic effect on bronchial asthma.It can improve the pulmonary function, reduce the levels of inflammatory factors, improve the immune function of the body, and has good therapeutic safety.It has a high clinical application value.
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Objective To compare the efficacy and safety of short course and high-dose Dexamethasone (HDD) and conventional Prednisone as first-line strategy for children newly diagnosed as primary immune thrombocytopenia (ITP).Methods This study analyzed pre-experimental data of a single center prospective randomized controlled clinical trial.Newly diagnosed but untreated ITP patients enrolled at the Department of Blood and Cancer Center,Beijing Children's Hospital,Capital Medical University from November 2016 to May 2017 were randomized into HDD group[Dexamethasone 0.6 mg/(kg · d),intravenous injection for 4 days] and Prednisone group [Prednisone 2 mg/(kg · d) for 14-28 days and then tapered within 1-2 months,the course of treatment less than 3 months].Initial response,sustained response and adverse effects after therapy were observed in 2 groups.Results Sixty-six children with ITP were included in the study:32 patients were in the HDD group and 34 patients were in the Prednisone group.Two groups were matched in the baseline characteristics including gender,age,platelet counts and disease course before therapy and bleeding assessment (all P ＞ 0.05).The initial response (the response of HDD group within 10 days of treatment and Prednisone group within 28 days of treatment):overall initial response had no statistical difference between the HDD group and the Prednisone group[90.6％ (29/32 cases) vs.100.0％ (34/34 cases),x2 =1.528,P ＞ 0.05].HDD group had a lower incidence of complete response compared with that in the Prednisone group [54.4％ (19/32 cases) vs.94.1％ (32/34 cases),x2 =11.330,P =0.001];median time of response in two groups showed no statistically difference (2 d vs.1 d,Z =-0.149,P ＞ 0.05).There was no significant difference in the recovery of skin and mucosal bleeding after treatment between the Dexamethasone group and the Prednisone group (Z =-1.413,-1.031,all P ＞ 0.05).The sustained response (the response lasted for up to 6 months and above):overall and complete sustained response had no statistically difference between the HDD group and the Prednisone group [92.9％ (26/ 28 cases) vs.85.3％ (29/34 cases),P =0.594;78.9％ (15/19 cases) vs.81.3％ (26/32 cases),P=1.000].Log-rank test showed no significant difference in the duration of response between 2 groups (P =0.341).The side effects in the Prednisone group included weight gain or Cushing sign (94.1％) and mental and emotional changes (23.5％);in the HDD group 15.6％ of children had infection,without other glucocorticoid-related side effects.There was no significant difference in the incidence of infection between two groups[15.6％ (5/32 cases) vs.26.5 ％ (9/34 cases),P =0.281].All of the above infections were of respiratory tract infections and mild gastrointestinal infections.Conclusions Efficacy of the HDD group in the initial and sustained responses is similar,but side effects were apparently lower compared with that in the Prednisone group.However,a large multicenter randomized controlled clinical study is necessary to confirm this result.
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Objective To know the detection rate of hereditary thrombocytopenia (HT) in children with chronic thrombocytopenia and its clinical and laboratory characteristics for an early clinical identification and diagnosis of HT in future.Methods Data of the children with thrombocytopenia,who had been treated in Beijing Children's Hospital from April 2016 to May 2018 and whose present history lasted for more than 1 year and had poor response to immunotherapy were retrospectively collected.HT was screened in these patients by adopting next generation sequencing (NGS).Finally,clinical and laboratory characteristics of these children with HT were summarized and analyzed.Results A total of 161 children with chronic thrombocytopenia were included.Forty-three cases (26.7％) were found to have gene mutations.The genetic rules of the mutant gene,the family verification and the clinical manifestations of the proband and some related laboratory tests were analyzed and 24 cases (14.9％) can be diagnosed as HT.Among the HT patients,the proportion of males and females was 15 ∶ 9,and the median onset of age was 0.58 years,which was significantly lower than that of non-HT cases (the median onset of age was 4.36 years),and the difference was statistically significant (P ＜ 0.001);the proportion of mucosal hemorrhage and visceral hemorrhage (31.8％ and 13.7％) of HT was significantly higher than non-HT cases (15.3％ and 0.6％),and the difference was statistically significant (P ＜ 0.001).Fifty percent of (12/24 cases) cases of HT had positive family history;according to the average platelet volume and platelet morphology in peripheral blood smear,HT could be divided into small platelet HT,positive platelet HT and large platelet HT.Some cases had well response to immunotherapy but seemed easy to relapse during the withdrawal period,while the others responded poorly to therapy.Different clinical manifestations of HT suggest different pathogenesis,which can be divided into the related types of megakaryocyte differentiation defect,megakaryocyte maturation defect,platelet release defect and platelet survival time shortening.Conclusions The pathogenesis and clinical phenotype of HT was different.Some of them were effective for immunotherapy,which were easily confused with immune thrombocy-topenla(ITP).It is clinically necessary to perform NGS in children with thrombocytopenia at early onset,abnormal platelet morphology,prolonged disease course or severe mucosal/visceral hemorrhage,in order to recognize HT timely to avoid delay in diagnosis and poor prognosis.
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Objective@#To understand the research status of occupational asthma and provide information for related research on occupational asthma in the future.@*Methods@#Papers on occupational asthma published from January 1998 to December 2017 had been retrieved in Web of Science core collection database on 9 October 2018. The data retrieval strategies were set as follows: #1 TS=(occupational AND asthma), #2 TS=(occupational AND asthmas), #3 TS=(occupational asthma OR occupational asthmas), #1 OR #2 OR #3. Three thousand two hundred and twelve publications were analyzed by bibliometric and visualizer.@*Results@#Yearly output of articles in this field had been at a stable high level and annual total citations had been increasing. A significant positive correlation was found between the year and annual total citations (r=0.97, P<0.05). The most productive countries were European countries except the United States and Canada. Our country had few literatures accounting for 1.21 percent of the total and the research on occupational asthma in our country started relatively late which were published mainly from 2013 to 2017. The most studied category and journal were public environmental occupational health and Am J Ind Med respectively. "occupational exposure", "allergy" and "rhinitis" were key words with high frequency.@*Conclusion@#Yearly output of publications of occupational asthma has been at a stable high level. Our country should do more research to provide a scientific basis for further prevention and management of occupational asthma.
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Objective@#To compare the efficacy and safety of short course and high-dose Dexamethasone (HDD) and conventional Prednisone as first-line strategy for children newly diagnosed as primary immune thrombocytopenia (ITP).@*Methods@#This study analyzed pre-experimental data of a single center prospective randomized controlled clinical trial.Newly diagnosed but untreated ITP patients enrolled at the Department of Blood and Cancer Center, Beijing Children's Hospital, Capital Medical University from November 2016 to May 2017 were randomized into HDD group[Dexamethasone 0.6 mg/(kg·d), intravenous injection for 4 days]and Prednisone group[Prednisone 2 mg/(kg·d) for 14-28 days and then tapered within 1-2 months, the course of treatment less than 3 months]. Initial response, sustained response and adverse effects after therapy were observed in 2 groups.@*Results@#Sixty-six children with ITP were included in the study: 32 patients were in the HDD group and 34 patients were in the Prednisone group.Two groups were matched in the baseline characteristics including gender, age, platelet counts and disease course before therapy and bleeding assessment (all P>0.05). The initial response (the response of HDD group within 10 days of treatment and Prednisone group within 28 days of treatment): overall initial response had no statistical difference between the HDD group and the Prednisone group[90.6%(29/32 cases) vs.100.0%(34/34 cases), χ2=1.528, P>0.05]. HDD group had a lower incidence of complete response compared with that in the Prednisone group[54.4%(19/32 cases) vs.94.1%(32/34 cases), χ2=11.330, P=0.001]; median time of response in two groups showed no statistically difference (2 d vs.1 d, Z=-0.149, P>0.05). There was no significant difference in the recovery of skin and mucosal bleeding after treatment between the Dexamethasone group and the Prednisone group (Z=-1.413, -1.031, all P>0.05). The sustained response (the response lasted for up to 6 months and above): overall and complete sustained response had no statistically difference between the HDD group and the Prednisone group [92.9%(26/28 cases) vs.85.3%(29/34 cases), P=0.594; 78.9% (15/19 cases) vs.81.3%(26/32 cases), P=1.000]. Log-rank test showed no significant difference in the duration of response between 2 groups (P=0.341). The side effects in the Prednisone group included weight gain or Cushing sign (94.1%) and mental and emotional changes (23.5%); in the HDD group 15.6% of children had infection, without other glucocorticoid-related side effects.There was no significant difference in the incidence of infection between two groups[15.6%(5/32 cases) vs.26.5%(9/34 cases), P=0.281]. All of the above infections were of respiratory tract infections and mild gastrointestinal infections.@*Conclusions@#Efficacy of the HDD group in the initial and sustained responses is similar, but side effects were apparently lower compared with that in the Prednisone group.However, a large multicenter randomized controlled clinical study is necessary to confirm this result.
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Objective@#To know the detection rate of hereditary thrombocytopenia (HT) in children with chronic thrombocytopenia and its clinical and laboratory characteristics for an early clinical identification and diagnosis of HT in future.@*Methods@#Data of the children with thrombocytopenia, who had been treated in Beijing Children′s Hospital from April 2016 to May 2018 and whose present history lasted for more than 1 year and had poor response to immunotherapy were retrospectively collected.HT was screened in these patients by adopting next generation sequencing (NGS). Finally, clinical and laboratory characteristics of these children with HT were summarized and analyzed.@*Results@#A total of 161 children with chronic thrombocytopenia were included.Forty-three cases (26.7%) were found to have gene mutations.The genetic rules of the mutant gene, the family verification and the clinical manifestations of the proband and some related laboratory tests were analyzed and 24 cases (14.9%) can be diagnosed as HT.Among the HT patients, the proportion of males and females was 159, and the median onset of age was 0.58 years, which was significantly lower than that of non-HT cases (the median onset of age was 4.36 years), and the difference was statistically significant (P<0.001); the proportion of mucosal hemorrhage and visceral hemorrhage (31.8% and 13.7%) of HT was significantly higher than non-HT cases (15.3% and 0.6%), and the difference was statistically significant (P<0.001). Fifty percent of (12/24 cases) cases of HT had positive family history; according to the ave-rage platelet volume and platelet morphology in peripheral blood smear, HT could be divided into small platelet HT, po-sitive platelet HT and large platelet HT.Some cases had well response to immunotherapy but seemed easy to relapse du-ring the withdrawal period, while the others responded poorly to therapy.Different clinical manifestations of HT suggest different pathogenesis, which can be divided into the related types of megakaryocyte differentiation defect, megakaryocyte maturation defect, platelet release defect and platelet survival time shortening.@*Conclusions@#The pathogenesis and cli-nical phenotype of HT was different.Some of them were effective for immunotherapy, which were easily confused with immune thrombocy-topenla(ITP). It is clinically necessary to perform NGS in children with thrombocytopenia at early onset, abnormal platelet morphology, prolonged disease course or severe mucosal/visceral hemorrhage, in order to recognize HT timely to avoid delay in diagnosis and poor prognosis.
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BACKGROUND:Rat models of complete spinal cord transection are common models for neural tissue engineering. After transecting the spinal cord by the previous methods, gap length of broken end cannot keep relatively uniform, so we cannot objectively evaluate effects of various treatments or tissue engineering materials. OBJECTIVE:The spinal cord transection models were established by using double edged micro scissors, andthe feasibility of this new model was explored by comparing with the conventional method. METHODS: A total of 42 adult female Sprague-Dawley rats were divided randomly into group A (n=6), group B (n=18) and group C (n=18). Group A only received laminectomy. In the group B, the spinal cord was transected with a sharp-pointed knife. Knife point should touch anterior wal of spinal canal and sidewal bone surface. Complete spinal cord transection models were prepared by repeated cutting. In group C, complete spinal cord transection models were established by using self-made double edged micro scissors. RESULTS AND CONCLUSION: (1) At 1 week after model establishment, in the groups B and C, complete paralysis of the hind limbs was found, and BBB scores were similar. However, significant differences in the spacing of broken end were detected. (2) At 4 weeks after model establishment, hind limb functions could restore to different degrees in both groups, but no significant difference in BBB scores was found. (3) At 8 weeks after model establishment, significant differences in hindlimb motor function scores were detectable between both groups. Biotin glucosamine tracer display: In group B, a few labeled axon fibers were observed at the caudal side of the injured spinal cord. In group C, spinal cord was completely transected, and labeled axon fibers cannot be found at the caudal side. (4) Results suggested that the modeling method of self-made double edged micro scissors could effectively eliminate individual differences, contribute to quantitative analysis and comparative study of therapeutic effects.
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Objective To evaluate the effect of integrated service platform of extensional care based on the combined internet and extensional care model in the orthopedics nurse care. Methods Two hundred and eighty six hospitalized patients in the orthopedic department were randomly divided into two groups including experimental group (144 cases) and control group (142 cases) according to the random number table method. Patients in the control group received extensional care by traditional telephone follow-up, patients in the experimental group received extensional care by using the integrated service platform combing internet and extensional care. All the patients were evaluated using the self-made extensional care satisfaction questionnaire and Short Form 36 Questionnaire. Results The extensional care satisfaction scores of experimental group using internet plus extended care mode were respectively ( 74.4 ± 5.3) points and (76.2 ± 8.1) points at the time points of 3 months and 6 months after discharge from hospital;The extensional care satisfaction scores of control group were respectively (69.4±9.9) points and (70.1±12.2) points at the time points of 3 months and 6 months after discharge from hospital;The two groups had statistically comparative difference (t=7.40, 6.37, P<0.01). There was a statistically significant improvement of extensional care satisfaction and physical function score and role-physical score and social function score in the experimental group compared with the control group both at the time points of 3 months and 6 months after discharge from hospital. There was a statistically significant improvement of vitality score and role-emotion score in the experimental group compared with the control group both at the time points of 1 month and 3 months after discharge from hospital (t=2.366-8.211, P<0.05). Conclusions The effect of integrated service platform of extensional care based on the combined internet and extensional care model is better than traditional telephone follow-up, patients′satisfaction is higher. This new mode of service platform could greatly deepen the connotation of quality nursing, intensify the effect of extensional care, and reduce the working strength of nurse who did extensional care as while.
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[Objective] To emphasize the importance of early prevention and treatment, this article discusses on the theory and application of preventive treatment of disease from traditional Chinese medicine in prevention and treatment of carotid atherosclerosis in patients with diabetes. [Methods] With de-tailed research into related study of risk factors affecting carotid atherosclerosis in type 2 diabetic patients from recent years, comprehensive summary of tra-ditional risk factors and risk factors of the latest findings were demonstrated. The prevention and treatment of carotid atherosclerosis in type 2 diabetes mel itus was discussed in two aspects by theory of“preventive treatment of disease. [Result] In diabetic patients without carotid atherosclerosis, early and timely intervention can prevent the occurrence and development of the diabetes vascular lesions; In diabetic patients with carotid atherosclerosis, active treatment can reduce the incidence of stroke, myocardial infarction and other critical care. [Conclusion] In patients with type 2 diabetes, risk factors affect-ing carotid atherosclerosis should be control ed and intervened early, which can effectively reduce the occurrence and development of the diabetes vascular lesions to improve the quality of life, but early diagnosis technology and indicators need to be further researched.
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According to the related requirements of medical technology clinical application management, the specific implementation approaches of surgery physicians surgery permission management were made, including establishing surgery classification project directory library, developing surgery classification management system and the surgery permission declared, and audit program, and investigating to determine the steps of surgery permission owned by surgery physicians currently, and finding problems and solution measures in implementation surgery permission management. To prevent surgical risk, improve the quality of operation, and evaluate surgical doctors scientifically, it provides management experience and references.
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Objective To investigate the role of interleukin-6(IL-6), interleukin-8(IL-8) and tumor necrosis factor-?(TNF-?) in the pathogenesis of secondary syphilis, and their relationship with RPR titers and clinical manifestations in secondary syphilis. Methods The serum IL-6, IL-8 and TNF-? were tested by ELISA. RPR titers of the patients were determined simultaneously. Histopathology was performed if skin lesions were present. Results Thirty three cases of secondary syphilis were studied. The average levels of serum IL-6, IL-8 and TNF-? were 78.7?82.5 pg/mL, 171.9?117.9 pg/mL and 115.7?67.9 pg/mL, respectively, in syphilis group, which were significantly higher in comparison with those in controls (P