Hirschsprung's disease (HD) is a conginental disorder of enteric nervous system (ENS) and is associated with the abnormal migration of gut neural cerst cell (GNCC).Recent studies showed that the regulation of this migration is a complex gene network.By the development of epigenetic and stem cells,this network is becoming much more clear.Transplantation of the modified gut neural crest stem cells/gut neural crest precursors(GNCSC/GNCP) acquired from the HD bowl is becoming the potential therapy of HD.This paper summerizes the recent advances and is to promote better understanding the pathogenesis of HD.

Child ; Humans ; Rhinitis, Allergic, Perennial ; diagnosis

Cardiovascular Diseases ; etiology ; prevention & control ; Disasters ; Earthquakes ; Humans

Objective To report the clinical effects of repairing skin defects in hand by reversed neu- ro-veno-fasciocutaneous flaps based on the superficial branches of radial nerve.Methods Eleven cases with soft tissue defects around the wrist and the dorsum of the hand were treated with superficial branches of radial nerve-cephalic vein neuro-veno-fasciocutaneous flaps.Results The flaps totally survived in the 9 cases,the others were small distal necrosis.Follow-up survey in the(6-48)months after the oporation showed the appear- ante and function were satisfactory.Conclusion Superficial branches of radial nerve neurofasciocutaneous flap is well supplied with blood and easy to perform.It is the ideal flap to repair the skin defects of the first finger web,radial palm and dorsum of hand.

Objective To investigate the relationship between flow-mediated dilatation of the brachial artery(FMD) and cardiovascular risk factors as well as target organ damage in elder patients with isolated systolic hypertension(ISH).Methods Ninety patients with ISH were divided into two groups: ISH group(n=51) and ISH with hyperlipidemia group(n=39).Color Doppler ultrasound for FMD was performed on all the patients,and carotid artery ultrasound and cardiac ultrasound were also conducted. Results Patiens with hyperlipidemia had lower FMD than those of ISH group(P=0.021).It was revealed by univariate analysis that FMD was negatively related to systolic blood pressure,carotid intima-media thickness and total cholesterol(P

Objective To discuss the genetic relationship between Chaoxian and the other populations with STR polymorphisms, and study the origination of Chaoxian with historical data, analyze the difference among them. Methods Samples were obtained from 91 unrelated individuals of Jilin province. PCR amplification was performed using PE9700, the results were analyzed with the GeneScan software and then using the Genetic Analyzer ABI377 and Genotyper2.5. Tests for Hardy Weinberg equilibrium (HWE) and the clustering map were carried out using SPSS11.5. The DA (genetic distance) according to Nei's was calculated and a phylogenetic tree based on the neighbor-joining method using Mega2 software package was constructed. Results 81 alleles and 196 genotypes were observed, with the corresponding frequencies ranging from 0.0055 to 0.4615 and 0.0110 to 0.2747, the observed and expected of genotypes were evaluated using χ2-test and all of the loci were in accordance with Hardy Weinberg equilibrium (P>0.05). Chaoxian was clustered with the other Chinese ethnic groups, then the American Black and the White; it was clustered with the Baishan Han, the Beijing Han of China first, then the South Korean. Conclusion The differentiation among races is larger than that among minorities, and, the differentiation among minorities is related to and is consistent with their geographic location.

Objective To establish a satisfactory method of isolating,culturing and determining gut neural crest stem cells,which may provide theoretical basis for clinical application.Methods The guts of embryonic mice removed and dissociated were plated into serum-free DMEM/F12 medium.The mitogen-free DMEM/F12 medium supplementd with 10% fetal bovine serum was used to induce differentiation of GNCSCs.Neurospheres and their derivations were determined with immunocytochemical and immunofluorescent staning.Results Neurospheres were generated in the simplified serum-free medium.The staining results showed that enteric neurospheres were GNCSCs and could differentiate into neurons,glial cells and smooth muscle cells by serum-induction.Conclusion GNCSCs have the capacity of self-renewal and proliferation,and give rise to neurons,glial cells and myofibroblasts.

OBJECTIVETo evaluate clinical outcomes of anterior cruciate ligament (ACL) and posterior cruciate ligament (PCL) reconstruction under arthroscopy and repair of the injured posteromedial complex structure of the knee joint in the treatment of posterolateral knee dislocation with multiple ligament injuries.

METHODSFrom March 2008 to August 2012,22 patients (16 males and 6 females, ranging in age from 20 to 53 years old, with an average of 30.5 years old) with posterolateral dislocation of the knee were treated with primary reconstruction of ACL and PCL, combined with the repair of injuries in the posteromedial complex and soft-tissue. Eight patients had injuries caused by sports,5 patients road accidents and 9 patients falling down. The ACL was reconstructed using the gracilis and semitendinosus tendons. The PCL was reconstructed using LARS artificial ligaments (14 cases), or gracilis and semitendinosus tendons (8 cases). Suture repair was performed in 17 patients with posteromedial ligament injuries,and self-semitendinosus strengthening operations were performed in 5 patients. Continuouspassive montion (CPM) and active exercises were executed after operation at early stage. The IKDC and Lysholm system were used to evaluate therapeutic effects.

RESULTSAll the patients were regularly followed up, and the duration ranged from 11 to 56 months (averaged, 39 months). According to the IKDC scale,9 patients got a grade A result, 10 got a grade B result, and 3 got a grade C result. The IKDC subject score was 89.6±3.1 and the Lysholm scores was 90.7±1.8 at the latest follow-up, which were both better than those before operation.

CONCLUSIONReconstructing the ACL and PCL and repairing injured posteromedial complex of the knee followed by an active rehabilitation is an effective method to treat posterolateral knee dislocation.

Adult ; Anterior Cruciate Ligament ; surgery ; Anterior Cruciate Ligament Injuries ; Arthroscopy ; methods ; Female ; Humans ; Knee Dislocation ; surgery ; Male ; Middle Aged ; Posterior Cruciate Ligament ; injuries ; surgery ; Reconstructive Surgical Procedures ; methods

Boronic Acids ; adverse effects ; Bortezomib ; Fatty Acids, Omega-3 ; administration & dosage ; therapeutic use ; Fish Oils ; administration & dosage ; therapeutic use ; Humans ; Male ; Middle Aged ; Peripheral Nervous System Diseases ; chemically induced ; drug therapy ; Pyrazines ; adverse effects