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Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.
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Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.
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Not only does autophagy play a vital role in maintaining kidney cell survival and homeostasis,but al-so it is involved in the pathophysiology of several kidney diseases. Recent studies have indicated that autophagy was ac-tivated in kidney cells during acute kidney injury and its regulatory mechanism was unclear. Autophagy activation in kidney may be associated with oxidative stress, endoplasmic reticulum stress, hypoxia inducible factor-1α, p53 and Bcl-2 family. The role of autophagy in acute kidney injury is still controversial. Most believe that it plays a protective role during acute kidney injury. Therefore autophagy will become a novel and potential target for the prevention and treatment of acute kidney injury.
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Objective To explore the effect of evidence-based medicine combined withcase based learning(CBL) in pediatric residency training.Methods Totally 52 residents were equally divided into two groups:experiment group and control group.Method of CBL combined with the evidencebased medicine (Taking two residents as one group,firstly teacher posed the questions and then residents searched for references when off duty and made evidence-based case presentation at fixed time when doing teaching ward around,after wards all personnel in the department made case discussion,finally teacher made the summary) was used in experimental group while traditional teaching method (teacher guided the residents to analyze the case by clinical experiences,residents took notes and sometimes asked questions,teacher directly gave the answers)was used in control group.The teaching effect is evaluated by questionnaire,theory test and clinical skills assessment.Statistical analysis was carried out on appraisal results with SPSS 16.0 software,test results and appraisal results were expressed as ((x-) ±s) and t test was performed,P ＜ 0.05 was taken as statistically significant.Results Residents in experimental group had more advantages in self-study ability,literature retrieval ability,physician teamwork spirit,clinical thinking,PowerPoint making and language expression after receiving CBL combined with evidence-based medicine.Theory examination showed that average score of experimental group was better than that of control group (75.46 ± 6.646) vs.(71.38 ±4.758),with statistically significantly differences (P =0.014),especially in subjective items.Clinical skills assessment showed that experimental group was better than control group in writing medical records and doing clinical thinking.Conclusions Teaching method of evidence-based medicine combined with CBL is significantly better than traditional indoctrination teaching in improving the abilities of self-study,cultivating clinical thinking,literature retrieval,language performance and other skills.This teaching method is more suitable for pediatric residency training.
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This review focuses on the current literatures on the therapeutic potential of non-renal derived adult stem cell transplantation for kidney disease.Although the therapeutic mechanism remains debatable,some reports suggest that hematopoietic stem cell transplantation holds potential for treatment of renal diseases such as renal ischemia reperfusion injury,IgA nephropathy and lupus nephritis.Mesenchymal stem cell(MSC) are capable of homing to injured kidney and contribute to activate intrinsic kidney cells,promote angiogenesis,inhibit oxidative stress and reduce apoptosis,inflammation and renal fibrosis.These renoprotective effects are likely mediated by paracrine/autocrine mechanism via cytokine and microvesicles from MSC.Pluripotent stem cells may be induced from fibroblast,mesangial cells,renal tubular epithelial cell and urine epithelial cell.Induced pluripotent stem cell(iPSC) can differentiate into renal lineage cells.The derivation of kidney disease-specific iPSC opens new avenues for the resolution to limited donor availability in kidney transplantation and for treating kidney diseases such as diabetic nephropathy and autosomal dominant polycystic kidney disease.
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Neonatal diabetes mellitus ( NDM ),which was often misdiagnosed as type 1 diabetes in the past,is a heterogenous single-gene genetic disease.Permanent neonatal diabetes( PNDM )is mainly associated with mutation in KCNJ11,ABCC8,and insulin associated gene instead of immunity.The most common manifestation includes diabetic ketoacidosis,intrauterine growth retardation and dehydration.Gene examination contributes to the classification of NDM and corresponding targeted therapy.Oral sulfonylurea may be used in treating patients with gene mutation of ATP-sensitive K+ channel.
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Objective To analyze the clinical and pathological features of Wegener granulomatosis (WG) in children in order to improve the understanding of this disease. Methods Nine cases of WG,including two cases from our hospital and seven cases throughout the country,were analyzed in aspects of their clinical characteristics,laboratory examinations,X-ray and treatments. Results Of the nine cases,six cases were male and three cases were female with average age of twelve years old. The average time from the initiation of clinical symptom to diagnosis was 8.3 months. Patients had multiple systems and organs involvements. Lung and kidney involvement were the most common. Six out of seven were positive in c-ANCA and anti-PR3 antibody. Biopsy was conducted in eight patients,and pathological manifestations involved parenchymal necrosis,granulomatosis,vasculitis and so on. Most patients improved significantly after being treated with glucocorticoids and cyclophosphamide. Conclusion WG is a syndrome which involves many organs without distinctive clinical manifestation. An ANCA test should be performed to make an early diagnosis.