Neonatal intrahepatic cholestasis caused by Citrin deficiency(NICCD) is one of phenotypes of Citrin deficiency.It's an autosomal recessive disorder which was mainly seen in East Asia,including China.Case of NICCD was reported firstly by Japanese in 2001.In south area of China,the morbidity of NICCD is higher than that in north area of China.Most of the patients with NICCD has benign prognosis.Symptoms resolve within the first year of life,thus making a diagnosis difficult after this time.But few of patients will develop liver failure,even be fatal to life.Early diagnosis,regular follow-up and proper management may improve the prognosis.

In order to study on the properconcentrations for patch test(PT),and the availability of PT w ith the suspected cosm etic products used by the patients,PT ofvarious cosm etic products w as per- form ed on 35 healthy subjects and 38 patients w ith non-contactderm atitis and non-eczem a by using dif- ferent concentrations of cosm etic products and their vehicles in cosm etic products.In 62 patients w ith cosm etic derm atitis PT w as done using their ow n suspected causative cosm etic products.The results show ed thatm ost products could be tested “w ith originalproducts”,w ithout dilution,such as skin care products(cream ),lipsticks,hair care products(hair cream ,hair oil,m ousse),others had to be diluted, such as hair dyes,hotoiland soap,to 2% solution,sham poos to 5% solution,perm anent-w ave solutions and perfum es to 5% or 10% in distilled w ater,nailvarnish to 5% or 10% in acetone.The totalpositive rate of PT by using suspected cosm etics w as 95.16% .Our results indicate that PT concentrations and vehicles vary w ith different products in suspected cosm etics,PT of the suspected causative cosm etic products is an im portant m eans for the diagnosis of cosm etic derm atitis.Our study provides necessary data for the further study ofChinese standard cosm etic screening series allergens for PT.

Animals ; Arrestins ; metabolism ; Liver ; pathology ; Liver Cirrhosis, Experimental ; metabolism ; pathology ; Male ; Mice ; Mice, Inbred ICR ; beta-Arrestins

Adult ; Burns, Inhalation ; diagnostic imaging ; Explosions ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Radiography

OBJECTIVE: To explore different conditions and buffers of antigen retrieval which affect the CK of SNIP on immunohistochemical staining results. METHOD: Dividing paraffin tissue sections of 11 patients into four groups. Using the Image-Pro Plus Image analyzer and taking five horizons for each section to calculate an average of 200 areas, measured standard optical density of the positive reaction areas. RESULT: It is divided into four groups: high temperature and high pressure citrate buffer retrieval, microwave EDTA buffer retrieval, microwave citrate retrieval, high temperature and high pressure EDTA buffer retrieval. The standard optical density of positive reaction areas respectively express: 0.324 ± 0.051, 0.325 ± 0.056, 0.303 ± 0.061, 0.365 ± 0.023. The rates of CK positive expression with high temperature and high pressure EDTA buffer retrieval is batter than other repairing groups in the same paraffin tissue sections (P < 0.05). CONCLUSION For the Pan of Sinonasal inverted papilloma, the method of high temperature and high pressure EDTA buffer antigen retrieval can achieve the ideal staining results? which is worth while to promote and maybe as a bet? ter guide of clinic work.
Antigens ; analysis ; Buffers ; Humans ; Immunohistochemistry ; methods ; Microwaves ; Nose Neoplasms ; immunology ; Papilloma, Inverted ; immunology ; Paranasal Sinus Neoplasms ; immunology ; Staining and Labeling ; methods

Coptidis Rhizoma-Euodiae Fructus has been widely used for the treatment of digestive diseases since Song Dynasty, and therapeutic efficacy is very obvious. Modern research found that alkaloids are the main bio-active constituents, and some of their contents have striking difference after compatibility of the two herbs. The Chinese medicine pair (CMP) has extensive biological activities, such as the effect of gastrointestinal effect, anti-tumor, lowering the blood pressure and blood fat and so on. And some action mechanism of CMP also got partial demonstration. This paper mainly summarized the bio-active constituents, compatibility effects, action mechanism and clinical applications of the CMP, which can provide a basis for further research and development of the CMP.
Animals ; Drug Interactions ; Drugs, Chinese Herbal ; chemistry ; pharmacology ; Evodia ; chemistry ; Humans ; Medicine, Chinese Traditional ; methods

Objective:To evaluate the feasibility and safety of total aortic arch surgery under mild hypothermicvia single upper hemisternotomy approach.Methods:From January 2019 to July 2019, 35 patients(31 male and 4 female) with Stanford A type aortic dissection were diagnosed, who were(43.7±5.7)years old. Aortic arch surgeries were carried out under mild hypothermic via single upper hemisternotomy approach and the perioperative mortality, time of cardiopulmonary bypass(CPB), aortic cross clamp(ACC), circulation arrest(CA) and morbidity of neurological dysfunction were respectively were recorded.Results:All patients were finished aortic arch surgery under mild hypothermic single upper hemisternotomy approach, with 8.6% of mortality(3 patients died perioperation). The time of CPB, ACC and CA were respectively(202±53)min, (128±28)min and(8±3)min. There were 6 cases of transient neurological dysfunction(17.1%) and 1 case of permanent neurological dysfunction(2.9%).Conclusion:Aortic arch surgery under mild hypothermic for Standford A dissectionvia single upper hemisternotomy approach is safe and feasible.

Objective To evaluate application feasibility of Array CGH in genetic diagnosis of clinical complex chromosomal abnormalities.Methods Two patients of genetic counseling and two patients of prenatal diagnosis were selected from Xiamen Maternity & Child Health Care Hospital during the period of December 2010 to December 2011.Under aseptic conditions 2-4 ml peripheral blood was collected in EDTA and 2-3 ml Cord Blood was collected through cordocentesis after genetic counseling and preoperative examination.G-banded chromosome analysis and genome DNA extraction were carried out on the four cases.The whole genome of four cases were scanned and analyzed by Array CGH.The results of Array CGH were confirmed by FISH.Results Array CGH detected different kinds of duplications and deletions in several chromosomes.Most of these duplications and deletions were not detected by karyotype analysis.The results of Array CGH showed duplication of 4p16.3-4p15.31,deletion of 4p16.3 in the first case,duplication of Xp11.22-Xq11.1 in the second case,duplication of 4p16.3-4p15.32,deletion of 2q37.3 in the third case and duplication of 2q21.2-2q32.1,deletion of 2q14.3-2q21.1 in the fourth case.These duplications and deletions were confirmed by FISH.Conclusions Compared with conventional cytogenetic analysis,Array CGH can not only accurately detect micro deletion and micro duplication with high resolution and sensitivity but also identify breakpoints precisely.Array CGH can provide the basis for clinical genetic diagnosis.

Objective To explore the impact of breast conservation surgery and modified radical mastectomy combined with intensity modulated radiation therapy respectively on the living quality and sexual life for patients with early-stage breast cancer.Methods From January 2011 to December 2016, 89 patients with breast cancer were admitted and divided into two groups according to different surgical methods.Patients in the conservation group (n=24) received breast conservation surgery combined with intensity modulated radiation therapy, while patients in the modified radical group (n=65) received breast radical surgery combined with intensity modulated radiation therapy.The quality of life and sexual satisfaction of patients were measured by questionnaire investigation.And the data were calculated by SPSS 16.0.Results Different surgical methods combined with radiotherapy affectted the score of living quality a lot.The average score was (87.500±7.940) points in the conservation group, while it was (65.350±8.490) points in the modified radical group, the difference was statistically significant (P<0.01).At the same time,the degree of self acceptance and sexual satisfaction in the conservation group after surgery was better than that in the modified radical group,and the difference was statistically significant (P<0.01).Conclusion Breast conservation surgery combined with intensity modulated radiation therapy for breast cancer patients could receive better quality of life and sexual satisfaction compared with modified radical mastectomy combined with intensity modulated radiation therapy.

Objective: To investigate the effects of taurine on blood glucose, number of platelets in plasma, pletelet aggregation and metabolism of oxygen free radicals in diabetic rats. Methods: Diabetes was induced by a single injection (ip) of streptozotocin (STZ,70 mg/kg body weight). The rats were maintained on drinking water with or without taurine (1.5 g/kg body weight per day). The concentration of blood glucose, maximal aggregation rate of platelets induced by adenosine 5 diphosphate(ADP) or thrombin(Thr) and the number of platelets in plasma were measured. Metabolism of oxygen free radicals in platelets was assessed as superoxide dismutase(SOD) activity and content of malondialdehyde(MDA) in platelets. [WT5FZ]Results: [WT5BZ]The concentration of blood glucose and maximal aggregation rate of platelets induced by ADP or Thr were significantly higher in diabetic rats than in non diabetic rats. The diabetic rats showed decreased platelet number and SOD activity with higher MDA level. In taurine treated diabetic rats concentration of blood glucose was obviously decreased, but no significant change was found in platelet number, SOD activity, MDA content and aggregation of platelets induced by ADP or Thr. [WT5FZ] Conclusion: [WT5BZ]There is a correlation between the increase in platelet aggregability and the abnormal metabolism of oxygen free radicals in platelets and taurine can decrease the blood glucose level but has no significant effect on platelet aggregability and metabolism of oxygen free radicals in diabetic rats.