Objective To analyze the clinical features and diagnostic experiences of retroperitoneal neuroendocrine carcinoma (NEC).Methods Clinical data of primary retroperitoneal NEC from January 2000 to January 2012 were retrospectively collected and analyzed.Results Five candidates were included 4 male and 1 female.The median age was 53 years old.3 cases initially suffered from abdominal pain and radiative back pain,2 cases were confirmed by normal physical check.The CT scan and B type ultrasound were performed among all patients,1 case was diagnosed as pancreas head neoplasm,1 case pancreas insulinoma,which was closely associated with other tissues and the intervational therapy was performed twice preoperatively.All 5 cases were selected surgery as the main treatment,2 cases performed radical tumor resection,3 cases received bypass operation.2 cases were 3 years survival.Conclusion Retroperitoneal NEC is a rare carcinoma,characterized by seriously invasion and high malignant degree.Surgery still remains the mainstay of selections,early diagnosis can significantly improve NEC patients’ overall survival rate.But the early diagnosis rate of this rare kind of patients still need to be improved.

Objective To report the clinical, myopathological and genetic features in myofibrillar myopathy (MFM) with numerous cytoplasmatic-spheroid bodies. Methods Ten patients in 5 successive generations began to present progressive proximal limbs weakness at 35 to 40 years old. Additionally, 4 cases manifested diarrhea and 6 cases accompanied with cardiorespiratory symptoms. An open biopsy was performed on the proband. In addition to histological, enzymhistochemical staining and ultrastructural examination, immunohistochemical staining with antibody against tau, desmin, ubiquitin, dysferlin, dystrophin-C', dystrophin-N' and dystrophin-R were done. All the exons of the MYOT, CRYAB, DESMIN, LDB3, LMNA, SEPNI gene and the FLNC exon 48 were analysed. Results Cytoplasmatic bodies and spheroid bodies were found in the fibers. The deposited material were positive for tau, desmin, ubiquitin, dysferlin and dystrophin-R, dystrophin-C'. Electron microscope showed granular dense Z-disc material in the inclusions which were surrounded by thin filament. There was no mutation in the above exons of the 7 candidate genes. Conclusions Myofibrillar myopathy involves multiple system impairment. Cytoplasmatic and spheroid bodies contain microtubule and membrane associated protein. The disease might be induced by some unknown genetic abnormities.

BACKGROUND:Vascular smooth muscle is the main cell component of constructing vascular wall tissue structure and maintaining vascular tension.Studies have shown that smooth muscle progenitor cells obtained from peripheral blood can differentiate into vascular smooth muscle cells.OBJECTIVE:To compare smooth muscle progenitor cells dedved from human peripheral blood with mature vessel wall smooth muscle cells in cell morphology and proliferation potential.DESIGN,TIME AND SETTING:Cell culture and observation trials were performed at the Key Laboratory of Molecular Medicine in Jiangxi province from October 2008 to April 2009.MATERIALS:Peripheral blood 20mL were adopted from 6 healthy volunteers,and great saphenous vein were sourced from 6 patients with varicose vein.METHODS:Mononuclear cells were isolated from human peripheral blood and cultured in fibronectin,platelet derived growth factor-BB and EGM-2 medium.At the same time,the smooth muscle cells were obtained from great saphenous vein with the collagenase digestion method.High-glucose DMEM medium was used for the culture.MAIN OUTCOME MEASURES:Cellular morphology;Cellular immumofluorescence method and RT-PCR method were used to detect the expression of α-SMA and calponinl.MTT method was used to analyze the proliferation activity.RESULTS:The smooth muscle progenitor cells denved from human peripheral blood exhibited biological characteristics of vascular smooth musc Je cells.they were found to express α-SMA and calponin1.The proliferation activity of smooth muscle progenitor cells was also greater than vascular smooth muscle cells.CONCLUSlON:Peripheral blood mononuclear cells in the stimulus of platelet-derived growth factor can differentiate into smooth muscle progenitor cells.Compared to smooth muscle cells,smooth muscle progenitor cells proliferate better,and are not easy to age in a long time of cultivation.Smooth muscle progenitor cells are a ideal ceil source of tissue engineered vessel.

Objective To investigate the prognostic factors of EGC patients,especially the relationship between prognosis and tumor size.Methods Clinical data of 119 EGC cases from 1998 to 2002 were analyzed retrospectively.Results The overall 5-year survival rate was 90.9%.and the survival rate of patients with tumor D＜2 cm.2 cm≤D＜4 cm and D≥4 cm respectively was 100%,92.0%and 80.8%(P=0.024).In this group,tumor stage,lymph node metastasis and tumor invasion depth did not affect the survival statistically.There was no correlation between tumor size and other prognostic factors.Conclusion Tumor size is an independent prognostic factor of patients with EGC,which may have some relevance to the surgical approach of EGC.

Objective To report filaminopathy with novel insertion mutation in a Chinese family.Methods Total 19 patients from successive 5 generations involved in an autosomal dominant family. The detailed clinical manifestations had been described (Chinese Journal of Neurology, 2008, 41:751-755).The filamin C gene sequencing was performed in 3 patients, 5 family members without symptoms and 50 normal persons. The amplified fragments of the exon 18 in filamin C gene were cloned into pBluesripts vectors, then sequenced and identified with capillary electrophoresis. Results 18-nucleotide deletion and 6-nucleotide insertion were identified in the exon 18 of filamin C gene. The mutation caused the disturbance of the seventh immunoglobulin-like domain in filamin C, leading to the instability of dimmers of filamin C.Another 2 patients in the family had same mutation while 5 family members without symptoms and 50 normal controls were normal. Conclusion The novel nucleotide deletion-insertion in exon 18 of filamin C gene causes filaminopathy. This disease can appear in non-Nordic race.

Objective:To investigate whether the mindfulness training can reduce the aggression level and im-prove the sleeping quality among incarcerated people.Methods:Fifty-four male criminals were recruited into the study.They were divided into mindfulness training group (n =25)and control group (n =29).The training group took mindfulness exercise once a week for 6 weeks.While the participants in waiting list control group waited for 6 weeks without intervention.After 6-week mindfulness training for training group and post-assessment for all partici-pants,the waiting list control group went into 6-week mindfulness training.The Five Facet Mindfulness Question-naire (FFMQ),Aggression Questionnaire (AQ)and Pittsburgh Sleep Quality Index (PSQI)were assessed before and after the intervention.Results:There were 19 valid data in training group,and 21 in control group.After 6-week training,the score difference between pre-and post-assessment of FFMQ (P <0.01 )was higher in the training group than in the control group,and the difference of AQ (P <0.01)and PSQI (P <0.01)was lower in training group than in the control group.Conclusion:The results suggest that 6-week mindfulness training could effectively reduce the aggression level and improve the sleep quality in the long-term incarcerated males.

Objective By simulating a high-glucose condition of peritoneal dialysis (PD)fluid,to explore the effect of high glucose on the expression of leptin and its relationship with peritoneal angiogenesis.Methods Adipocytes differentiated from 3T3-L1 were divided into high glucose group (139 mmol/l glucose) and high mannitol group.Leptin levels in supernatant collected at 0 h,12 h,24 h and 48 h were measured by ELISA.Endothelial cells (ECs) were respectively cultured with normal glouse,high glucose,high mannitol condition,supernatants of adipocyte induced by normal glouse,high glucose and high mannitol,high glucose supernatants+leptin antibody,and high mannitol supernatants + leptin antibody.Tubular structure formation and migration of ECs were detected.Results Adipocytes exposed to high glucose for 48 h produced more leptin as compared with control group,high mannitol group,12 h-high glucose group and 24 h-high glucose group (all P ＜ 0.05).Compared with ECs in normal group,ECs in high glucose had less tubular structure formation and increased migration (all P ＜ 0.01).Compared with those of ECs in high glucose,the tubular structure formation and the migration of ECs in adipocyte supernatants induced by high glucose had increased (all P ＜ 0.01),and these effects were reduced by leptin antibody (all P ＜ 0.01).Conclusion There is an up-regulation of leptin in adipocytes exposed to high glucose,which may be an alternative way to prevent peritoneal angiogenesis.

Objective To summarize and analyze the clinical feature,therapeutic mnethods and prognosis of colorectal small cell carcinoma.Methods From January 2000 to January 2012,15 patients of colorectal small cell carcinoma were analyzed retrospectively.Results There were 12 male cases,3 females.The age at diagnosis was between 39-71 years,with median age of 60.SCC located in the rectum in 12 cases,in the colon in 3 cases.The time from the onset of symptoms to final diagnosis was from 1 to 12 months.The diameter of tumors varied from 2.5 to 8.0 cm.13 cases received up-front surgery,including radical tumor resection in 6 cases,palliative resection in 7 cases,and neoadjuvant-chemotherapy followed by palliative resection in one case.The initial Ⅰ,Ⅲ B,Ⅳ B stage were 1 case,6 cases and 8 cases,respectively.The overall median survival time is 11 months,1,2 year's survival rate is 40.0％ and 20.0％,respectively.Conclusions Colorectal SCC is less common and the prognosis is poor.Multimodality management,with radical surgical resection of the primary lesion followed by standard adjuvantchemotherapy,affords good local disease control and a fair survival.

Objective To study the clinical features of gallbladder small cell carcinoma (GSCC),to improve the diagnosis and treatment of GSCC.Methods We retrospectively analyzed the clinical data of GSCC patients at our hospital from January 2000 to January 2012,and made a collective review of the literature.Results In this series,there were four female cases,one male case,the age at the first diagnosis was between 42-67,with the median age of 57.The main complain was pain and dis-comfort on the up and right abdomen.Tumor located in the bottom of gallbladder in 3 cases,and in the body in 2.Cholelithiasis was complicated in 4 cases.2 patients received radical resection of GSCC,followed by adjuvant chemotherapy of VP-16 and cisplatin,radioactive therapy in one.Postoperatively,these two were followed up for 45 and 32 mons with tumor free survival.3 cases received palliative resection,followed by adjuvant chemoradioactive therapy or intervention treatment,these three were followed up to 8,11,30 months respectively to their death for tumor recurrence.Conclusions GSCC is a rare disease,the initial symptoms are not often specific and easily misdiagnosed.The prognosis of GSCC is poor.

Objective To assess retinal arteriole stenosis in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and to evaluate the relationship between retinal arteriole stenosis with cranial MRI changes and clinical features. Methods Sixteen CADASIL patients (mean age was (43.4±8.1)years, mean duration was(4.7±3.4)years)and sixteen age matched healthy individuals were enrolled. Mini-Mental State Examination (MMSE) and modified Rankin scale scoring were performed in 16 patients. Cranial MRl with Coulthard scores was assessed on CADASIL patients as well. Retinal examinations for arteriole stenosis were done for each subject. Bivariate correlations (Kendall's tau-b) were used to assess the relationship among the grade of retinal stenosis, MMSE, modified Rankin seale, cranial MRI with Coulthard scores and age. Results Retinal arteriole stenosis presented in 15 cases. in whom 4 cases showed mild arterio-venous nicking. while it only presented in 2 controls. Grade 0.Ⅰ, and Ⅲ of retinal arteriole stenosis are noted in 1/16,4/16,7/16 and 4/16cases respectively. Coulthard scores of cerebral MRl were 6.0.19.0.25.1 and 29.8 respectively from Grade 0 toⅢ of retinal arteriole stenosis. The correlation coefficient of retinal arteriole stenosis and cranial MRI scores was 0.743(P＜0.001＝,MMSE-0.429(P＜0.05＝,modified Rankin scale 0.437(P＞0.05).and age 0.299(P＞0.05).Conclusion Retinal arteriole stenosis is significantly correlated with lesion in cranial MRI and dementia.