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

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

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

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.

OBJECTIVETo study the correlation between TCM syndrome type and liver tissue pathological changes in patients with chronic hepatitis B (CHB) in order to provide evidence for syndrome differentiation.

METHODSSyndrome typing as well as liver pathological grading and staging of liver biopsy were performed on 260 patients with CHB, then the relationship between them was analyzed.

RESULTS(1) The grade of liver inflammation was mainly G1 and G2 in patients of Gan-qi stagnation and Pi-deficiency type (type 1); G2 in patients of inner damp-heat retention type (type II); G3 in patients of Gan-Shen yin-deficiency type (type lII) and Pi-Shen yang-deficiency type (type IV); while G4 occurred mainly in patients of blood stasis blocking collateral type (type V), showing significant difference as compared with other syndrome types. (2) The liver pathological stage in patients of type I and II was mainly S1 and S2, while S3 and S4 occurred mainly in patients of type III and type IV. (3) The pathological change was mainly G3-G4 and S3-S4 in blood stasis syndrome, while it was mainly G1-G2 and S1-S2 in non-blood stasis syndrome.

CONCLUSIONThe TCM syndrome type is correlated with liver tissue pathological change to certain extent, among them, syndrome with or without blood stasis showed the closest correlation. The syndrome type of CHB patients developed, along with the aggravating of liver pathological injury, from sthenia to asthenia, from qi to blood, and finally to the blood stasis blocking collateral. So the treatment should be lay stress on activating blood circulation to remove stasis, and be implemented by 3 stages.

Adult ; Diagnosis, Differential ; Female ; Hepatitis B, Chronic ; diagnosis ; pathology ; Humans ; Liver ; pathology ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Syndrome ; Yang Deficiency ; pathology ; Yin Deficiency ; pathology ; Young Adult

OBJECTIVETo investigate the expression of zinc finger E-box binding homebox 1 (ZEB1) in the prepuce of hypospadias children and its relationship to the incidence of hypospadias.

METHODSPrepuce tissues were collected from 37 children aged 6-15 months undergoing hypospadias repair and 11 age-matched controls receiving circumcision. Based on the position of the urethral meatus, the hypospadias cases were classified as severe (n = 13) and mild-moderate (n = 24). The mRNA and protein expressions of ZEB1 were determined by immunohistochemistry and RT-PCR.

RESULTSThe expression of the ZEB1 protein was remarkably higher in the severe (100% [13/13]) and mild-moderate hypospadias patients (75.0% [18/24]) than in the controls (9.1% [1/11]), with statistically significant differences between any two groups (P < 0.05). RT-PCR showed the integrated density value (IDV) of the ZEB1 mRNA expression to be (0.67 ± 0.21), (0.81 ± 0.24), and (1.55 ± 0.29) in the control, mild-moderate, and severe hypospadias patients, respectively, significantly higher in the severe hypospadias than in the control and mild-moderate hypospadias groups (P < 0.05), but with no significant difference between the latter two (P = 0.64).

CONCLUSIONThe expression of ZEB1 is significantly increased in hypospadias patients, and its upregulation is positively correlated with the severity of hypospadias, which suggests that the overexpression of ZEB1 may contribute to the development of hypospadias.

Biomarkers ; metabolism ; Case-Control Studies ; Circumcision, Male ; Foreskin ; metabolism ; Homeodomain Proteins ; genetics ; metabolism ; Humans ; Hypospadias ; classification ; etiology ; metabolism ; Immunohistochemistry ; Infant ; Male ; Penis ; RNA, Messenger ; metabolism ; Transcription Factors ; genetics ; metabolism ; Up-Regulation ; Urethra ; Zinc Finger E-box-Binding Homeobox 1

Adolescent ; Cord Blood Stem Cell Transplantation ; adverse effects ; Humans ; Hypertensive Encephalopathy ; etiology ; Male ; Transplantation, Homologous

OBJECTIVETo discuss the clinical effects and superiority of applying external fixation and bone transport to treat osteomyelitis and bone defect of femoral bone.

METHODSFrom August 2008 to December 2013,16 patients with osteomyelitis and bone defect of femoral bone were treated including 11 males and 5 females with an average age of 42 years old ranging from 13 to 62 years old. The average course of disease was 18 months ranging from 2 months to 4.5 years, and the average length of bone defect was 7.8 cm ranging from 4.5 to 15 cm. The bone defect of all cases were treated by external fixation and bone transport, the bone transport began at 1 week after operation, 1 mm per day and 4 times per day.

RESULTSAll patients were followed up for 10 to 36 months (means 22.5 months). One patient did not cooperate with treatment leads to the failure, then took the amputation. The remaining 15 cases of osteomyelitis were under control, including 12 cases of bone transport achieved one stage bone union, 3 cases achieved bone union via bone graft from iliac bone. The bone union time was 5 to 13 months(means 7.9 months). Thirteen patients almost obtained the same length of two lower extremities,2 patients had shortening of 1.5 to 2 cm. The time of moving the external fixation was from 6 to 16 months (means 9.3 months).

CONCLUSIONApplication of external fixation and bone transport is an effective method in treating the osteomyelitis and bone defect that can control the infection, eradicate wounds, and be the equalization of limb length.

Adolescent ; Adult ; Bone Transplantation ; External Fixators ; Female ; Femur ; surgery ; Humans ; Male ; Middle Aged ; Osteomyelitis ; surgery