Lipodystrophies represent a heterogeneous group of diseases characterized by varying degrees of body fat loss and predisposition to insulin resistance and metabolic complications such as diabetes mellitus,hypertrigly-ceridemia and hepatic steatosis. The lipodystrophies can be divided into generalized,partial or local,depending on the degree and locality of the observable fat loss;moreover,the generalized and partial divisions can be partitioned further into congenital or acquired forms. Until now,11 genetic factors including AGPAT2,BSCL2,CAVI,PTRF,PPARG, LMNA,ZMPSTE24,AKT2,CIDEC,PLINI and WRN were reported to be involved in congenital lipodystrophies. The most prevalent subtype of acquired lipodystrophy currently occurs with prolonged duration of protease inhibitor - contai-ning,highly - active antiretroviral therapy in human immunodeficiency virus(HIV)- infected patients. Other types of acquired lipodystrophies are mainly autoimmune in origin and display complement abnormalities. The current manage-ment includes cosmetic surgery and early identification and treatment of metabolic and other complications with diet, exercise,hypoglycemic drugs,and lipid - lowering agents. Metreleptin treatment demonstrated remarkable clinical effect and good tolerance.

ObjectiveTo investigate the species of wild Chinese medicinal herbs for treating liver diseases in Quanzhou City of Fujian Province.Methods Based on the field investigation combined with literature reference, induction and analysis of the species of wild Chinese medicinal herbs for treating liver diseases in Quanzhou City of Fujian Province have been conducted.ResultsIt was found that there are 59 families, 112 genera, and 132 species of wild Chinese medicinal herbs for treating liver diseases in Quanzhou City of Fujian Province, most of which belong to angiosperm. 15 families, 21 genera, and 21 species are recorded in the Chinese Pharmacopoeia (2010 edition). Compositae has the largest number of genera and species (16 genera &19 species), followed by leguminosae (8 genera & 8 species) and labiatae (6 genera & 8 species). Chinese medicinal herbs for heat-clearing and promoting urination and leaching out damp amount to 69 and 20 species respectively. Over 88% of these wild medicinal herbs can be used to treat various liver diseases, and 46 species are used with high frequency. They are mainly taken by water decoction and oral administration.ConclusionWild Chinese medicinal herbs for treating liver diseases in Quanzhou City of Fujian Province are rich in species and resources, and are worth further development and utilization.

Objectives: To explore the influence of exercise training on the arterial baroreflex sensitivity （BRS）and correlation between blood pressure and BRS in spontaneously hypertensive rats (SHR). Methods: Male SHR（n=20）and normotensive Wistar rats（n=20）were randomly assigned to normality group and exercise group, n=10 in each group. Rats in two exercise groups received treadmill training at a speed of 20 m/min for 60 min/d, 6 d/w for eight weeks. Systolic blood pressure (SBP) and heart rate (HR) were measured using a tail-cuff method in a conscious state. Intravenous injections of phenylephrine (PE) and sodium nitroprusside (NP) were used to induce depressor and pressor reflex respectively. The ratio of HR over mean arterial pressure (MAP) (HR /MAP) after administration of PE or NP was regarded as an index of depressor reflex sensitivity (BRS-PE) and pressor reflex sensivity (BRS-NP). Results: After eight-week exercise training, compared with SHR normality group, there were significant reduction in resting SBP [(180±8.5) mmHg vs. (163.6±10.7) mmHg] and in HR [(368.4±13.3) beats/min vs. (345.0±9.8) beats/min] in SHR exercise group, P<0.01 both. However, there was no significant difference in resting SBP between Wistar exercise and normality groups (P>0.05), compared with Wistar normality group, there was significant reduction in HR [(362.2 ± 13.0) beats/min vs. (343.9 ± 10.2) beats/min, P <0.05] in Wistar exercise group. Compared with SHR normality group, there were significant rise in BRS [BRS-PE: (0.89 ± 0.13) bpm/mmHg vs. (1.32 ± 0.22) bpm/mmHg, BRS-NP： (0.60± 0.09) bpm/mmHg vs. (1.21± 0.26) bpm/mmHg, P<0.01] in SHR exercise group, but still lower than those of Wistar normality group [BRS-PE: (1.96±0.23) bpm/mmHg, BRS-NP: (1.32±0.17) bpm/mmHg]. Pearson linear correlation analysis indicated that MAP was significantly inversely correlated with BRS (r=-0.734, P<0.01) in SHR normality and exercise group. Conclusion: Exercise training may significantly decrease SHR blood pressure; it is related to improved baroreflex sensitivity induced by exercise, indicating that enhanced baroreflex may be an important mechanism of exercise therapy in hypertensive patients.

Objective To summarize the nursing experience during removing the upper gastrointestinal foreign bodies in special patients by painless endoscopy. Method Retrospective analysis was done to investigate the clinical records on endoscopy for removing the upper gastrointestinal foreign bodies in 69 special patients. Result The foreign bodies in 67 patients were removed by endoscopy successfully, without severe complications such as bleeding and perforation; one patient was removed with duodenum lateral telescope; one patient turned for sugery . Conclusion Sufficient preoperative preparation and skilled surgical nursing cooperation are promising for the successful removal of upper gastrointestinal foreign bodies in special patients.

Objective To assess the clinical value of high frequency ultrasound diagnosis for little bone fracture of ankle. Methods Thirty-seven ankle wound patients with negative X-ray examination underwent high frequency ultrasound. The second X-ray examination was performed on patients with hinted little bone fracture of ankle, and then CT/MR examination was performed on patients whose second X-ray examination was negative. The consequence of the second X-ray examination was counted and the sonographic features were analyzed. Results ①Little fracture of fibula (lateral malleolus) was in 16, of tibia (medial malleolus) in 12, of talus in 2, of scaphoid bone in 4, of metatarsale in 2 and of calcaneus in one patients. ②The second X-ray examination was positive in 26 patients (26/37, 70.27%), indicating that the detection rate of the second X-ray is higher than that of the first X-ray. ③Sonographic features of little bone fracture of ankle included incomplete bone surface of bone was rough and soft tissue swelling and thickening. No malposition of bone fracture was found. Conclusion High frequency ultrasound is supplementary for X-ray examination, being able to possess important clinical priority in the diagnosis of little bone fracture.

A computerized stabilometer was used to evaluate the balance function of 31 subjects with healthy backs and 3l subjects with chronic low back pain. According to the difference of age and sex,they were divided into 2 groups equally. They were told that the feet stood side by side and stood separately with eyes opened and closed. The sensitivity and applying value of all of the test parameters were analyzed. The result showed that the stabilization of the subjects with chronic low back pain was poorer than the healthy subjects. The parameters, such as Sx, Sy, MS, PL, CA and AS, were sensitive, and feet stood side by side was more sensitive than feet stood separately.

Objective To understand the clinical and molecular characteristics of children with Prader-Willi syndrome (PWS) in South China.Methods Clinical and molecular data of children diagnosed as PWS by Methylation-specific PCR(MS-PCR) and/or Array Comparative Genomic Hybridization(Array-CGH)in Guangzhou Women and Children's Medical Center from November 2012 to November 2014 were analyzed.Results A total of 27 children diagnosed as PWS were included in this study,including 21 cases diagnosed by Array Comparative Genomic Hybridization (Array-CGH) and 13 cases diagnosed by methylation-specific PC R (MS-PCR).Within the 27 cases,13 cases were male(48.1％) and 14 cases were female(51.9％).The age on diagnosis was from 16 days to 16 years old.MS-PCR was performed in 13 cases,7 cases of them also performed Array-CGH,both of them showed a 174 bp fragment from the methylated allele and a 100 bp fragment from the unmethylated allele.Array-CGH analysis was performed in 21 cases,paternal deletion in 18 cases and mean interstitial deletions measure (5.48 ± 0.51) Mb in size,paternal duplication in 2 cases,loss of heterozygosity measure approximately 79.58 Mb in 1 case.Eighteen simple chromosome deletion cases were divided into 6 Del Ⅰ and 12 Del Ⅱ according to the location of Array-CGH and query the database to DECIPHER(Database of Chromosomal Imbalance and Phenotype in Humans Using Ensembl Resources).The major phenotype included central hypotonia and feeding difficulty in all cases (100.0％),hypogonadism in 25 cases (92.6％),weak crying in 22 cases(81.5％),and hypopigmentation in 22 cases(81.5％).Fourteen cases beyond 1 year old had varied degrees of development disability and behavioral and psychiatric disturbance:speech articulation defects in 13 cases(92.9％),hyperphagia and weight gain too fast in 13 cases(92.9％) when they were between 1 to 6 years old[(2.80 ± 1.32) years old],and obesity in 12 cases (85.7％).Conclusions For PWS children in South China,there is no statistically significant difference in the clinical manifestation between Del Ⅰ and Del Ⅱ.PWS children in South China have typical clinical characteristics,which can be used as a further screening indication to implement molecular diagnostics.

Objective Multiple carboxylase deficiency(MCD) is an autosomal recessively inherited defect of organic acid metabolism.The underlying mechanism is biotinidase(BT) or holocarboxylase synthetase(HLCS) deficiency.The authors reported 15 cases of MCD(clinical characteristics,diagnosis and treatment) and outcomes of long-term follow-up.Methods The clinical data of 15 patients with MCD admitted to Guangzhou Women and Children's Medical Center between Aug.2001 and Feb.2013 were analyzed.The diagnosis was confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS),blood enzymatic determination and gene analysis.The patients consisted of 12 male and 3 female.The onset age ranged from newborn infants to 16 months.Results 1.Remarkable elevations of urinary lactate,3-oxy-butyric acid,3-OH-isovalerate,methylcitrate,3-methylcrontonylglycine,3-OH-propionate were detected in all of 15 cases.Fourteen cases with HLCS deficiency and 1 case with BT deficiency were confirmed by gene analysis.2.Most of patients with HLCS deficiency presented in the neonatal period or early infancy.The main clinical manifestations were skin rash (14 cases),tachypnea (9 cases),developmental retardation (8 cases),vomiting(5 cases),poor feeding (3 cases),developmental regradation (1 case),convulsion (1 case).Laboratory evaluation showed persistent metabolic acidosis and varied degree of ketosis,lactic acidosis,hyperuricacidemia,ammoniemia and hypoglycemia.Biotin was given to 13 patients in 10 mg/d and their metabolic disorders were corrected within 48 h,except one who gave up treatment and died.Treatment with Biotin in 5 mg/d provided clinical stability and normal neurodevelopmental outcome on follow-up for 3-11 (6.47 ± 2.70) years.3.One patient with BT deficiency presented with severe neurological symptoms(impaired consciousness,limbs trembling,tachypnea with irregular respiratory rhythm) at 16 months old.Demyelination of corpus callosum was evident on magnetic resonance imaging.Biotin treatment was given to him on the second of onset,and 1-year follow-up indicated significant improvement of his clinical symptoms,but the right limb weakness did not completely recover.Conclusions The main clinical features of HLCS deficiency include frequent skin rash,tachypnea,and metabolic disorders.BT deficiency may produce variable neurologic manifestations including demyelination of corpus callosum.Urinary organic acid analysis with GC/MS is critical to the early diagnosis of MCD.Prompt biotin treatment is recommended to correct metabolic derangements and continued therapy is essential to the improvement of long-term prognosis.Delayed commencement of therapy in BT deficiency can result in irreversible neurological damage.

Objective To approach on the expression pattern of MMP-2 and E-Cadherin in the effects of gastrin on gastric canc-er cells ,so as to investigate their role in the metastasis and invasion of gastric cancer .Methods Genomic DNA modified by sodium bisulfite was used as a template .RQ-MSP detect the methylation status of the gene promoter .The relative amount of Methylation status was performed with comparative threshold cycle (2- △ t ) method .Results MMP-2 and E-Cadherin genes in gastric cancer cells were at hemimethylated status in gastrin before and after the intervention .Gastrin can reduce the methylation levels of MMP-2 gene(P<0 .05) ,but the could be effected by proglumide receptor antagonist to some certain extent (P>0 .05) .Gastrin can pro-mote the methylation levels of MMP-2 gene ,but proglumide decrease that effect (P<0 .05) .Conclusion Gastrin plays a certain role in the biological behavior of gastric cancer impact by changeing on the expression pattern of MMP-2 and E-Cadherin in the effects of gastrin on gastric cancer cells .

Objective To investigate the clinical efficacy and adverse events of methimazole ( MMI ) treatment for children with hyperthyroidism, and to identify the predictors of remission and relapse. Methods A total of379children(260girlsand119boys)diagnosedwithhyperthyroidismandtreatedbyMMIinGuangzhouWomenand Children's Medical Center from March, 2004 to July, 2014 were retrospectively analyzed. The average age at diagnosiswas(9.3±2.3)years(range2.0~15.9years). Results AftertreatmentwithMMIfor3and6months, the thyroid functions of 96. 3%(365/379) and 98. 9%(375/379) patients returned to normal, respectively. By the end of this study, 256(67. 5%) patients continued to use MMI treatment and 44 patients(11. 6%) dropped out. 79 patients(20. 8%) achieved remission, 35 patients (44. 3%) of whom experienced a later relapse. Children who achieved constant remission had significantly lower FT3 and FT4 levels at diagnosis compared with the relapsed children(P<0. 05 or P<0. 01). It was more likely to remain long-term remission for children turned to be euthyroid within 3 months after initiating MMI treatment(P<0. 05). The relieved patients with family history of thyroid diseases weremorelikelytoberelapsed(P<0.05). Therewerenosignificantdifferencesinage,gender,exophthalmos, initial goiter size, thyroid peroxidase autoantibody, and thyroglobulin antibody levels between the relieved and relapsed patients. The overall incidence of adverse events associated with MMI was 27. 7%, mainly elevated alanine aminotransferase, bilirubin, and neutropenia. Most(66. 7%) of adverse events occurred within the first three months of MMI treatment. Conclusion MMI has a good effect on pediatric hyperthyroidism, with low remission and high relapse rate. The low thyroid hormone concentrations at diagnosis and normalization of thyroid function within three months seem to be useful predictors of remission. Vigilance is needed concerning MMI-associated adverse events throughout the MMI treatment period, especially during the first trimester of MMI initiation.