Objective To investigate the expression of Fas/FasL in children with aplastic anemia(AA) and its clinical significance.To explore the pathogenesis of AA.Methods CD34+ cell counts and the expression of Fas/FasL in 12 children with severe AA(SAA),18 with chronic AA(CAA)and 10 normal children were detected by flow cytometry combining monocolon antibody.Results The expression of Fas in children with AA were significantly higher than that in control group(P0.05).Conclusions Fas/FasL systeam take part in inducing CD34+ cell apoptosis.This may contribute to understanding the decrease number of stem cells and bone marrow failure.

Objective To study the correlation between blood glucose variability and TOAST type of patients with acute ischemic stroke and provide new evidence which can improve the prognosis of ischemic stroke.Methods One hundred and forty-three hospitalized patients with acute ischemic stroke from August 2013 to August 2014 were included.All patients were estimated about the general state of health and performed laboratory examinations and other auxiliary examinations.Then the TOAST type of all cases was classified.The 24-hour blood glucose monitoring was done to the patients to calculate the blood glucose variability.One hundred and forty-three cases were divided into blood glucose variability group ( standard deviation of glucose ( GluSD )≥1.4 mmol/L, 85 cases ) and control group ( GluSD <1.4 mmol/L, 58 cases) according to glucose variability.We estimated the neurologic impairment of the patients with the US National Institutes of Health Stroke Scale ( NIHSS) and the activities of daily living with Barthel index.The relation between blood glucose variability and the prognosis of ischemic stroke was analysed with multivariate analysis.Results Differences were significant in age, history of diabetes and NIHSS score between blood glucose variability group and control group.The degree of neurologic impairment of large artery atherosclerosis (LAA) group (moderate and severe neurologic impairment:43/53(81.0%)) and cardio-aortic embolism ( CE) group ( moderate and severe neurologic impairment:15/17 ) was more serious than that in small artery occlusion ( SAO) group ( moderate and severe neurologic impairment:6/71 ( 8.5%) ) , especially in CE group.The differences were significant (χ2 =7.043,P<0.05).Blood glucose variability in patients with LAA was more obviously than that in other patients.NIHSS score and activities of daily living of the patients estimated on admission and after 2 weeks were not different in the blood glucose variability group, but the difference was significant in control group.The poor prognosis in blood glucose variability group was 2.821 times that in control group ( 95% CI 1.880 -4.233 ).Conclusions The people sufferd ischemic stroke with old age, diabetes or severe case are more vulnerable to abnormal blood glucose variability.Abnormal blood glucose variability is an independent risk factor for acute ischemic stroke and induces difficulty to recovery and poor prognosis.

Abnormalities, Multiple ; genetics ; pathology ; Child, Preschool ; Chromosome Aberrations ; Diagnosis, Differential ; Diarrhea ; diagnosis ; etiology ; therapy ; Exocrine Pancreatic Insufficiency ; complications ; Fever ; diagnosis ; etiology ; Fingers ; abnormalities ; Humans ; Male ; Pancreatic Diseases ; etiology ; Syndrome

AIM: To explore the neuroprotective action of progesterone(PROG), which has been proved to be a "neuroactive steroid". METHODS: The model of focal cerebral ischemia was established in rats by reverserble inserting a nylon thread with a diameter of 0.2 mm into the anterior cerebral artery through the internal carotid artery. The effect of PROG was assessed by determining water,sodium, potassium, and calcium contents in striatum of rats subjected to 2 h ischemia followed by 22 h reperfusion. RESULTS: The water,sodium,and calcium contents of middle cerebral artery occlusion(MCAO) striatum were obviously higher,the potassium content was obviously lower than those of non-MCAO striatum in I/R and DMSO groups,but there was no significant difference between these two groups.Compared with the result in I/R and DMSO groups , water, sodium and calcium contents significantly decreased, but potassium(P0.05). CONCLUSION: Treatment with PROG can significantly reduce the striatal injury of rats with cerebral ischmia-reperfusion.

0.05),but the expression in controls were negative.The expression levels of PRAME gene at remission was decreased obviously,but increased again when the patients relapsed.Conclusions Expression of PRAME gene has a high level in childhood acute leukemia.The dynamic changes are closely related with the prognosis.It can be regarded as a candidate for detecting minimal residual disease in acute leukemia,and may have important implications for estimating the prognosis and guiding the chemical therapy.

Objective To investigate the relationship between apoptosis of bone marrow cells and tumor necrosis factor-?(TNF-?),Fas/FasL expression in children with aplastic anemia.Methods The concentration of TNF-? in supernatant of cultured bone marrow mononuclear cell(BMMC) was measured by enzyme linked immunosorbent assay.The expression of Fas/FasL,apoptosis ratio of bone marrow CD34+ cell were evaluated by flow cytometry.Results The concentration of TNF-? in supernatant of cultured BMMC in AA group[SAA (27.9?8.20) ng/L;CAA (23.6?6.78) ng/L] increased significantly compared with control group(t=3.432 P0.05).Apoptosis ratio of CD34+ cell in SAA group[(24.6?9.56)%] and CAA group[(20.9?7.32)%] significantly increased compared with control group(t=3.492,3.458 Pa0.05).The concentration of TNF-? was positively correlated with the expression of CD34+ Fas+ in all types AA children(r=0.542 P0.05).Conclusions TNF-? and Fas/FasL system can be involved in inducing CD34+ cell apoptosis.This may contribute to understanding the decreased number of stem cells and bone marrow failure.

OBJECTIVETo study the relationship between collgen (Col) IV, VEGF secreted by the tumor cells and vasculogenic mimicry (VM).

METHODS158 bi-phase differential malignant tumor specimens were alloted and made into tissue microarray. These tissue microarray sections were stained with CD31, periodic acid-Schiff (PAS) and Col IV. Subsequently, distributive trait of Col IV and the difference of VEGF expression were analyzed.

RESULTSThe basement membrane of VM was PAS and Col IV positive. The expression of VEGF in bi-phase differential malignant tumor with VM was less than that in those without VM. The difference of VEGF expression in malignant melanoma and alveolar rhabdomyosarcoma was significant (P < 0.05).

CONCLUSIONCollgen IV and periodic acid-Schiff positive material take part in constructing the basement membrane of vasculogenic mimicry. The difference of the VEGF expression proves that vasculogenic mimicry can sustain the tumor blood supply.

Collagen Type IV ; analysis ; Humans ; Immunohistochemistry ; Neoplasms ; blood supply ; chemistry ; pathology ; Periodic Acid-Schiff Reaction ; Vascular Endothelial Growth Factor A ; analysis

OBJECTIVETo explore the clinical effects of total vertebral column resection combined with anterior mesh cage support in treating severe congenital kyphoscoliosis.

METHODSFrom April 2008 to April 2012,21 patients with severe congenital kyphoscoliosis were treated with total vertebral column resection and internal fixation through posterior approach combined with anterior mesh cage support. There were 8 males and 13 females with an average age of 19.4 years old (ranged from 10 to 35). And 6 cases were thoracic segments deformity,13 cases were thoracolumbar segments and 2 cases were lumbar segments, of them, 2 cases were accompanied with Chairs deformity, 6 cases with diastematomyelia, 4 cases with syringomyelia,and 1 case with neurofibromatosis. According to the Frankel grade system, 3 cases were grade C, 5 cases grade D and 13 cases grade E. Blood loss, operative time, and perioperative complications were recorded. Coronal and sagittal Cobb angle, apical vertebral offset distance, sagittal offset, the relative height of shoulders, razor back deformities were measured and analyzed before and after operation.

RESULTSThe average operative time was 5.2 h (3.5 to 6.5 h) and blood loss was 2,500 ml (1,400 to 4,900 ml). The 2nd day after operation, apical vertebral offset distance, sagittal offset, the relative height of shoulders, razor back deformities had obviously improved than preoperative (P < 0.05). There was no significant difference in above items between postoperative on the 2nd day and final follow-up (P > 0.05). The corrective rate of kyphosis and scoliosis were (60.97 +/- 6.30)% and (62.24 +/- 5.82)%, respectively. On the first day after surgery,2 cases of Frankel grade E aggravated to grade D, and obtained recovery at 2 week after conservative treatment. And 1 case palinesthesia later,grade D aggravated to grade C and obtained recovery after revision surgery in time. One case complicated with permanent blindness of left eye, 1 case occurred injury of pleura and 2 cases had cerebrospinal fluid leak during operation. All patients were followed up from 9 to 31 months with an av- erage of 18.6 months. At final follow-up,all patients obtained bone union, Frankel grade D in 4 cases and grade E in 17 cases, no correction loss and internal fixation loosening was found.

CONCLUSIONTotal vertebral column resection combined with anterior mesh cage support can effectively correct kyphosis and scoliosis in severe congenital kyphoscoliosis and can avoid injury of spine cord by spinal crispation, but intraoperative position and neurologic complications should still be considered.

Adolescent ; Adult ; Child ; Female ; Humans ; Kyphosis ; complications ; congenital ; diagnostic imaging ; surgery ; Male ; Retrospective Studies ; Scoliosis ; complications ; congenital ; diagnostic imaging ; surgery ; Spine ; surgery ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo gain new insights into the molecular pathogenesis of the 109(InsG) and 139(C--> T) mutations and their roles in familial oligodontia.

METHODSThe region of PAX9 paired domain (PAX9PD) was amplified and the expression plasmids were constructed in pGEXlambda -1T by PCR-based cloning. PAX9PD proteins were prepared on the basis of GST instruction. The binding of wild type and two novely mutant PAX9 paired domain to double-stranded DNA targets were analyzed by gel mobility shift assay.

RESULTSWild type PAX9PD protein bind to the high affinity paired domain recognition sequences, CD19-2(A-ins) and Pax6CON, the 109(InsG) and 139(C--> T) mutant PAX9PD protein were unable to bind to these cognate DNA-binding sites.

CONCLUSIONThe functional defects in DNA binding of mutant 109(InsG) PAX9 and 139(C--> T) PAX9, as well as loss-of-function of PAX9 most likely result in its haploinsufficiency during the patterning of dentition and the subsequent loss of posterior teeth.

Anodontia ; genetics ; Base Sequence ; Chromatography, High Pressure Liquid ; DNA Mutational Analysis ; Electrophoretic Mobility Shift Assay ; Family Health ; Humans ; Mutation ; PAX9 Transcription Factor ; genetics ; metabolism ; Polymerase Chain Reaction

OBJECTIVETo analyze and optimize the gene rearrangement primers of different frame regions (FR) of immunoglobulin heavy chain (IgH) genes by bioinformatic methods and explore the application of these primers in the detection of paraffin-embedded lymphoma tissues.

METHODSThree pairs of primers from IgH FR1, FR2 and FR3 regions (P1c, P2A and P31, respectively) were selected as the B cell gene rearrangement primers after comparison of the gene fragments in 44 IgH variable and 6 joining regions. Using one pair of T cell receptor (TCR) gamma primer as the T cell gene rearrangement primer, 101 histopathologically confirmed lymphoproliferative samples including 80 B cell lymphomas, 14 T cell lymphomas, and 7 reactive proliferative lymph nodes were examined by PCR for gene arrangement. The DNAs from DG75 and Jurkat cell lines were used as the positive controls for B and T cell lymphoma, respectively, with those from reactive proliferative lymph nodes as the negative control.

RESULTSThe positivity rates of IgH primers (P1c, P2A and P31) in the 80 B cell lymphomas were 37.5% (30/80), 52.5% (42/80) and 70.0% (56/80), respectively, and only one of the 14 T cell lymphoma cases was positive for the primers, suggesting significant differences in the detection rates of B cell lymphomas by the 3 primers. The detection rate was increased to 83.9% by combining the results by P31 and P2A primers. No positivity was found in the proliferative reaction tissues.

CONCLUSIONPrimers from IgH FR3 region genes are more sensitive than that from the FR1 and FR2 regions in the detection of gene rearrangement in paraffin-embedded lymphoma tissues. The detection rates can be increased by combining the results with the primers for IgH FR3 with that of FR2.

DNA Primers ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; genetics ; Humans ; Immunoglobulin Heavy Chains ; genetics ; Lymphoma, B-Cell ; diagnosis ; genetics ; pathology ; Lymphoma, Non-Hodgkin ; diagnosis ; genetics ; pathology ; Lymphoma, T-Cell ; diagnosis ; genetics ; pathology ; Male ; Paraffin Embedding