The main function of interstitial cells of Cajal(ICCs) is to produce the gastrointestinal slow-wave electric potential,to impel the propagation of electric potential,and to participate in the transmission of neural signal.The decrease or abnormal distribution of ICCs would lead to gastrointestinal inflammation easily.Meanwhile,as the gastrointestinal inflammation happens,inflammatory cells and mediators also can reduce the number of ICCs.

Airway problems in foetus that may cause airway obstruction can be safely managed by using an ex utero intrapartum technique(EXIT).The EXIT procedure maintains a period of uteroplacental circulation for fetal oxygenation during delivery to provide time to explore and secure the airway of the fetus or to perform fetal operations before the umbilical cord is clamped.The common indication for the EXIT procedure is the presence of foetal airway obstruction,which is usually caused by neck mass,congenital diaphragmatic hernia,congenital high way obstruction syndrome,thoracic abnormality,congenital heart and lung dysfunction,conjoined twins,and so on.It avoids the acute airway obstruction in a neonate that has disastrous consequences.

Humans ; Integrative Medicine ; Medicine, Chinese Traditional ; methods

Objective To increase the preoperative diagnosis rate,reform the operation and improve the prognosis in children with ganglio -nauroma.Methods Clinical data of 20 children with ganglioneuroma hospitalized in Tongji hospital of Huazhong university of science and technology from Nov.1986 to May.2006 were reviewed(male 9,female 11).The biological,clinical and pathological characteristics of patients were analyzed.Results Among these 20 patients,15 cases were discovered to have no clinical symtom.The B ultrasound showed low-echo in all 16 cases.Under CT scan,20 cases showed low to moderate density shadows,and the distinct enhancement in these tumors was noted with enhanced CT scan.Fourteen cases undergoing MRI all showed low signals in T1W1 and inhomogeneous high signals in T2W1.All patients underwent surgical operations,completely removed in 14 cases,partly in 4 cases, and biopsy was performed only in 2 cases.Fifteen cases were followed up from 7 months to 6 years,tumor recurrence happened only in 1 case.Conclusions Ganglioneuroma is mostly found in chest-abdominal sympathetic chain,it grows up slowly and seems to be innocent.The imaging data of B ultrasound,CT and MRI can give helpful information in the diagnosis of ganglioneuroma.We shall pay attention to the choice of incision and avoidance of injuring the important blood vessels during the operation.Children who have neuroblastoma cells should receive prophylactic chemoprophylaxis treatment and the result of long-time follow up is satisfactory.

Objective To observe the effect of intra-articular injection of dehydroepiandrosterone (DHEA)on the experimental osteoarthritis in rabbits and study the mechanism.Methods Forty rabbits un derwent unilateral anterior cruciate ligament transection(ACLT)and then divided into two groups randomly. 100?mol/L DHEA resolved in the dimethylsulphoxide were injected into the knees of experimental rabbits 4 weeks after transection,once a week for five weeks.Rabbits in the control group were treated under the same schedule using dimethylsulphoxide.All rabbits were killed 9 weeks after ACLT and the knee joints were evalu- ated by gross morphology and histology.The mRNA expression of metalloproteinases-3(MMP-3),tissue in- hibitor of metalloproteinases-1(TIMP-1)and interleukin-lbeta(IL-1?)in the cartilage and synovium was analyzed using reverse transcription polymerase chain reaction(RT-PCR).Results Gross morphologic in- spection and histological evaluation showed that the extent and grade of cartilage and synovium damage in the experimental group were less severe than the control group.The mRNA expression of MMP-3 in cartilage and synovium decreased significantly in the experimental group(both P＜0.05).The mRNA expression of TIMP-1 in cartilage and synovium increased significantly in the experimental group compared with that in the control group(both P＜0.05).No significant difference of IL-1?mRNA expression in cartilage was found between the experimental and the control groups(P＞0.05).The mRNA expression of IL-1?in the synovium was signifi- cantly suppressed in the experimental group compared with that in the control group(P＜0.01).Conclusion DHEA protects against cartilage degradation,alleviates synovium inflammation and inhibits the progression of osteoarthritis in the experimental model.Down-regulation of MMP-3 and up-regulation of TIMP-1 in cartilage and synovium and IL-1?in the synovium may be the mechanism of the protective effect of DHEA on os- teoarthritis.

Objectives To investigate the effects of ethanol on neural development and kainate receptor expression in young mice. Methods Fetal alcohol spectrum disorder model was established by administration of 20% ethanol solu?tion to 7-day-old Kunming mice and control animals received physiological saline (The number of treatment and control were 80 and 40, respectively ). Body weight and general biological features were observed every day. Morris water maze was used to test learning and memory ability. Fluoro-Jade B was used to examine neural cells 24 hours after treatment in additional thirty 7-day-old Kunming mice which were further divided into two groups:a treatment group receiving 20%ethanol solution (n=15) and a control group receiving physiological saline (n=15). The development of neural cells and expression levels of kainite receptors were examined by using immunofluorescence staining. Results The body weight was significantly lighter in treatment group than in control group(control:21.13 ± 1.72g,treatment:13.96 ± 2.98g,P<0.05). Morris test showed that model group had longer latency than control group to find hidden platform(control:21.05± 5.31s,treatment:34.15±3.26s,P<0.05). Spatial probe test revealed that the number of passing through the platform were significantly smaller in model group than in control group(control:2.70 ± 1.25 times,treatment:0.93 ± 0.80 times,P<0.05). Astrocyte development anomaly was evident after ethanol treatment for 7 days. The expression levels of kainite re?ceptor GluR-6 and KA2 were up-regulated in the CA region of the hippocampus after ethanol treatment for 7 days. Con?clusion Kainite receptor GluR-6 and KA2 in CA region of the hippocampus may contribute to ethanol-induced hippo?campal neural development anomaly.

Alagille syndrome (AGS) is a multisystem disorder and caused by mutations in JAG1 or NOTCH2 gene.The diagnosis of AGS is hampered by its highly variable clinical manifestations.We performed a retrospective analysis on 16 children diagnosed as having AGS in recent five years in our hospital.Cholestasis was seen in 15 patients (93.8％),heart disease in 12 (75％),characteristic facies in 7 (43.8％),and butterfly vertebrae in 7 (43.8％).Ophthalmology examination was not performed on all the patients.Further,serum biochemical parameters were compared between AGS and 16 biliary atresia (BA) patients who were confirmed by surgery.Elevated liver enzymes were seen in all the patients.Serum total cholesterol (TC) (P=0.0007),alanine aminotransferase (ALT) (P=0.0056),aspartate aminotransferase (AST) (P=0.0114),gamma-glutamyl transferase (GGT) (P=0.035) and total bile acid (TBA) levels (P=0.042) were significantly elevated in AGS patients compared to those in BA cases.However,there were no significant differences in serum total bilirubin (TB),conjugated bilirubin (CB) and albumin (ALB) between the two groups.We identified 14 different JAG1 gene variations and 1 NOTCH2 gene mutation in 16 Chinese AGS patients.Our study suggested clinical features of AGS are highly variable and not all patients meet the classical diagnostic criteria.It was suggested that hypercholesterolaemia and significantly elevated GGT,TBA and ALT may be helpful to diagnose AGS.Genetic testing is integral in the diagnosis of AGS.

Objective To study the effects of selenium deficiency,iodine deficiency and combined selenium and iodine deficiency on bone and cartilage growth in the parental and the first filial generation rats. Methods Forty-eight weanling healthy SD rats were randomly divided into selenium deficieney, iodine deficiency, combined selenium and iodine deficiency and control groups according to their body mass. These rats were fed with selenium deficiency, iodine deficiency, combined selenium and iodine deficiency, and normal fodder, respectively. The parental rats (about 3 months old) were mated in each group 8 weeks after the beginning of the experiment. Right tibias and left knee joints were collected when the parental generation rats were about 6 months and the first filial generation rats were about 3 months old. Tibial length, mid-shaft tibial diameter, and articular cartilage diameters of the right tibias were measured by vernier caliper. Left knee joints were embedded and cut into sections and the thickness of the growth plate cartilage, layers of proliferative and hypertrophic chondrocytes in growth plate cartilage were observed under the light microscope. Results The selenium deficiency had significant effect on serum selenium level of the parental and the first filial generation rats(F value were 239.56,232.68, P< 0.01), and also on serum T4 level of the first filial generation rats(F value were 6.95, P < 0.05). The iodine deficiency had significant effect on serum T3 and T4 level in the two generations rats(F value were 14.11,14.05,30.29,34.53, P < 0.01 ). There were interactions between selenium deficiency and iodine deficiency on serum T4 level in the first filial generation rats (F= 5.99, P< 0.05). The serum selenium of selenium deficiency group[ (30.28 ± 6.34), (43.95 ± 9.75)μg/L],combined selenium and iodine deficiency group[ (30.33 ± 5.18), (35.40 ± 3.16)μg/L] were significantly lower than iodine deficiency group[(345.83 ± 29.55), (245.24 ± 9.95)μg/L] and the controls[ (358.64 ± 30.50), (236.50 ±9.75) μg/L] in the two generations. The serum T3 of combined selenium and iodine deficiency group [(0.55 ± 0.05 ),(0.88 ± 0.14)nmol/L] were significantly lower than the controls[(0.75 ± 0.08), (1.26 ± 0.26)nmol/L] in the two generations. The serum T4 of iodine deficiency [ (24.11 ± 2.29), (42.10 ± 8.92) nmol/L ] and combined selenium and iodine deficiency group[ (20.66 ± 1.93), (26.55 ± 5.98)nmol/L] were significantly lower than the controls[ (36.15 ±2.74), (52.79 ± 8.84)nmol/L] and selenium deficiency group[ (28.12 ± 3.33), (52.02 ± ll.99)nmol/L] in the two generations. The selenium deficiency and iodine deficiency had significant effect on tibial length, thickness of the growth plate cartilage, layers of proliferative and hypertrophic chondrocytes in first filial generation rats(F values were 24.31,6.98,40.76,56.15,25.24,82.82, 10.07,5.57, P <0.05 or <0.01). There were interactions between selenium deficiency and iodine deficiency on tibial length, thickness of the growth plate cartilage, layers of proliferative and hypertrophic chondrocytes (F values were 5.68,24.86,41.82,9.12, P <0.05 or <0.01 ). The tibial length of the selenium deficiency group[ (33.17 ± 0.34)mm] and combined selenium and iodine deficiency group[ (31.30 ± 0.87)mm] were significantly lower than the controls[ (34.12 ± 0.32)mm, P< 0.05]. Thickness of the growth plate cartilage [ (1.60 ± 0.18)mm ], layers of proliferative chondrocyte (8.54 ± 0.81), and hypertrophic chondrocyte (4.95 ± 0.37)of the combined selenium and iodine deficiency group were significantly decreased when compared to the selenium deficiency group[ (3.03 ± 0.10)mm, 14.68 ± 0.84,6.60 ± 0.31], iodine deficiency group[ (2.90 ± 0.09)mm, 13.75 ±0.33,6.61 ± 0.84 ] and the controls [ (3.19 ± 0.09) mm, 14.94 ± 0.36, 6.64 ± 0.26, P <0.05]. Thickness of the growth plate cartilage, layers of proliferative chondrocyte of the iodine deficiency group were lower than the controls(P<0.05). Conclusions Selenium deficiency impair tibial growth in first filial generation rats, iodine deficiency retarded the chondroncyte proliferation and decreases the thickness of growth plate cartilage in first filial generation rats, and combined selenium and iodine deficiency significantly impair the growth of bone and cartilage in first filial generation rats.