Objective To observe the clinical, neuropathogical, neurophysiological characteristics of Charcot-Marie-Tooth disease, type 1A (CMT1A) which was similar to chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).Methods The clinical data, neuroelectrophysiological changes and pathological features of sural nerve biopsy were taken from 2 CMT1A patients who were proven to be 17p12 duplication with CIDP features, and analyzed comprehensively. Results Two CMT1A cases with classical chronic course showed both subacute course and clinical manifestations similar to CIDP, however, the changes of neuroelectrophysiology and pathological characteristics of the nerve biopsy in 2 cases were different from CIDP in some way. We confirmed the chronic inflammatory demyelinating type of the CMT1A patients whom the immunotherapy was effective on.Conclusion The classical CMT1A 17p12 duplication patients who obtained immunotherapy effects might resemble CIDP both in clinical course and manifestation.

Based on the anatomic data and fractal theory, the vascular tree model of forearms similar to actual vessel was established, and then the 3D heat transfer model of forearms based on the actual vessel anatomic structure was created. The model was analyzed and investigated with finite element method to explore the influential factors such as dimensional distribution of vessel, blood perfusion rate and external heat source for the 3D temperature field. The simulation results were consistent with the experimental data derived from Pennes. The model well reflects the regional difference of tissue temperature caused by the irregular distribution of vessel, and provides temperature guidance for tumor thermotherapy.

Adult ; Antigens, CD20 ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Ki-1 Antigen ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymph Nodes ; metabolism ; pathology ; Lymphoma, B-Cell ; pathology ; Pseudolymphoma ; metabolism ; pathology

Objective To investigate the relationship between the expression of DNA methyltransferases(DNMT)and clinical prognosis in children with acute leukemia(AL).Methods The mRNA expressions of DNMT1,DNMT3A,DNMT3B,p15,mdrl were measured in 56 AL children and 20 normal controls by semi-quantitative reverse transcriptionpolymerse chain reaction.Results In 56 cases of children with AL,the positive rate of DNMT1 was 73.2%(41/56);the positive rate of DNMT3A was 67.9%(38 /56);the positive rate of DNMT3B was 64.3%(36/56).Thirty-one cases showed positive expressions of the 3 DNMT simultaneously,4 cases with negative expressionss imultaneously,21 cases with at least 1 positive expression of the DNMT,positive rate of p15 was 19.6%(11/56);positive rate of mdrl was 28.6%(16/56),all 3 simultaneous expressions of the 3 DNMT in AL children were significantly higher than those in normal controls(P

Hematologic Diseases ; Humans ; Mesenchymal Stem Cell Transplantation ; Mesenchymal Stromal Cells

Objective To investigate the effectiveness and safety of minimal invasive surgery combined with ventriculoscope for the treatment of basal ganglia hemorrhage.Methods From June 2014 to June 2015 there were 57 patients with basal ganglia hemorrhage were selected into this study.These patients were signed into the minimal invasive surgery combined with ventriculoscope group and the craniotomy group according to the methods of surgery they received.And the clinical outcomes of the two groups were compared.Results Compared with the craniotomy group,patients in the minimal invasive surgery combined with ventriculoscope group got a significantly lower Glasgow coma scale at 7 days,14 days and 28 days after the operation (P =0.02,0.04,0.04);the hospital stays were significantly reduced in the minimal invasive surgery combined with ventriculoscope group [(21.45 ±5.67)d vs.(25.67 ±7.45)d,P =0.02];and the operation time were significantly reduced as well [(134.45 ±21.11)min vs.(178.65 ±45.32)min,P =0.000)].There was no significant difference in intra-cranial pressure,rate of hematoma clearance,rate of organ functional failure,rate of re-bleeding and mortality 28 days after operation (P >0.05).Conclusion The minimal invasive surgery combined with ventriculoscope is effective and safe for the treatment of basal ganglia hemorrhage,which is worthy of popularization.

Antigens, CD34 ; metabolism ; Female ; Humans ; Hypoglycemia ; etiology ; Immunohistochemistry ; Middle Aged ; Pleura ; metabolism ; pathology ; surgery ; Solitary Fibrous Tumor, Pleural ; complications ; metabolism ; surgery ; Treatment Outcome ; Vimentin ; metabolism

Objective To explore the significance of nuclear factor-?B(NF-?B)activation in peripheral blood mononuclear cells(PBMC)during acute Kawasaki disease(KD).Methods Peripheral blood was collected from children with acute KD(n=30)and healthy age-matched children(n=20).PBMC were cultured in vitro and divided into 3 groups:naturally cultured blank control group,protein kinase C(PKC)activator stimulated phorbol 12-myristate 13-acetate(PMA)group and PMA plus NF-?B inhibitor treated PMA plus pyrrolidine dithiocarbamate(PDTC)group.Percentages of NF-?B activation were detected by immunohistochemistry.Results Under natural culturing,the percentage of cells with activated NF-?B was significantly higher in acute KD blank control group than that in healthy blank control group.The percentage of cells with activated NF-?B was significantly higher in acute KD PMA group than that in acute KD blank group and that in normal control PMA group,respectively(Pa0.05).Conclusions NF-?B activation in PBMC during acute KD is markedly increased,which suggests that NF-?B activation plays an important role in the formation of vasulitis and CAL in this disease.NF-?B activation in PBMCs in children with KD is regulated by the PKC signaling pathway and PDTC obviously inhibits the activation of NF-?B.J Appl Clin Pediatr,2009,24(1):35-37

Objective To retrospectively analyze 45 cases of oral and maxillofacial malignant tumors and to assess the efficacy and feasibility of oral and maxillofacial malignant tumors implanted with 125I radioactive seeds under guidance of ultrasound.Methods A total of 47 focuses in these 45 patients were determined with the size of these tumors by imaging study,the section was planed by ultrasound,the number and distribution of radioactive seeds were determined with the help of the particle treatment planning system,and were percutaneously implanted particles under guidance of ultrasound.The number and the distribution of particles were assessed by CT.Efficacy endpoints were reexamined and evaluated regularly by ultrasonic and CT according to the standards of WHO.Results The total percentage of efficacy was 70.2% (including complete remission,partial remission).The treatment effect of metastatic carcinoma of lymph node is superior to the parotid tumor.There was no serious complication during the period of implanting and 2 patients with oral ulcers were found after operation.Conclusion The oral and maxillofacial malignant tumor treated implanted with 125I radioactive seeds under guidance of ultrasound is very effective and safe,which is deserved to popularize.The ultrasound is the first choice as a guided method for oral and maxillofacial malignant tumors.

Objective To explore the related diseases,main causes and clinical features of children with basal ganglia calcification(BGC).Methods Thirty cases with BGC detected by CT were studied retorspectively,and its clinical symptoms and image were summarized.Results Many factors and diseases were related to BGC,such as hypothyroidism,intrauterine infection,intrauterine hypoxia,epilepsy,posttraumatic cerebral lacunar infarction.Main clinical manifestations of BGC in children were twich,mental retardation,disorders of limb movements etc.The CT scan showed localized punctuate calcification in basal ganglia.Conclusions The main causes of BGC in children are hypothyroidism,intrauterine infection and intrauterine hypoxia,and the clinical manifestations are diverse.For children with CT-detected BGC should diagnose its causes;and for unknown causes cases should strengthen follow-up.