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 investigate the distribution of connexin 43 (Cx43) and interstitial cells of Cajal (ICC) in bowels of children with Hirschsprung's disease (HD) and explore their roles in the pathogenesis of HD.

METHODSForty-two children with HD diagnosed by histopathology (33 males and 9 females) aged from 2 months to 10 years were enrolled. Expression of Cx43 and ICC in the bowels was detected using immunohistochemistry. These cases were all sporadic, including 30 cases of common type and 12 cases of short segment type. Five samples from the children with intussusception (aged from 1 month to 8 years) were used as controls.

RESULTSCx43 and ICC were not expressed in the muscle layers of the aganglionic segment in children with HD, which was significantly different from the Cx43 and ICC expression in the ganglionic segment of HD patients and the control bowels. Moderate expression of Cx43 and ICC were observed in the migratory segment, which was significantly different from that in the ganglionic and aganglionic segments in patients with HD. Moderate or strong expression of Cx43 and ICC was observed in the circular muscle and the region between the circular and longitudinal layer in the ganglionic segment from patients with HD. There were no significant differences in the Cx43 and ICC expression between the ganglionic segment of HD patients and control bowels.

CONCLUSIONSThe absence of Cx43 and ICC expression in the aganglionic bowel and the destruction of the gap junction may induce the dysfunctions of intercellular substance exchange and communication transmission, which might partly be responsible for the pathogenesis of HD.

Child ; Child, Preschool ; Connexin 43 ; analysis ; physiology ; Female ; Hirschsprung Disease ; etiology ; metabolism ; pathology ; Humans ; Infant ; Intestines ; chemistry ; pathology ; Male ; Myocytes, Smooth Muscle ; chemistry ; Proto-Oncogene Proteins c-kit ; analysis

OBJECTIVETo investigate the relationship between the levels of antidesmoglein (DSG) 1, 3 antibodies in the sera of patients with paraneoplastic pemphigus (PNP) and alopecia.

METHODSSera from PNP patients, bullous pemphigoid patients, and normal healthy subjects were collected and 2 tissue samples from 2 healthy scalps were resected. Anti-DSG 1, 3 antibodies in the sera of PNP patients were detected by enzyme-linked immunosorbent assay (ELISA). Indirect immunofluorescent assay was used to detect whether the antibodies in the sera of PNP patients binds with the follicular epithelium of normal healthy scalp.

RESULTSAnti-DSG3 autoantibody was strongly positive and anti-DSG1 weakly positive in one patient, while both two antibodies were negative in the other patient. Their sera could bind to keratinocytes and follicular epithelium in human scalp. Immunofluorescent signals were found on the intercellular epidermal cell surface and outer root sheath of the follicular epithelium. However, the immunofluorescent signals in the section incubating with serum of bullous pemphigoid were only found on basal membrane zone. No signals were found in the section incubating with normal healthy serum.

CONCLUSIONAlopecia in PNP patients are correlated with the anti-DSG3.

Adult ; Alopecia ; etiology ; immunology ; Autoantibodies ; blood ; Desmoglein 1 ; immunology ; Desmoglein 3 ; immunology ; Female ; Humans ; Male ; Paraneoplastic Syndromes ; complications ; immunology ; Pemphigus ; complications ; immunology