Glycogen storage diseases (GSD) are a group of inherited disorders characterized by enzyme defects that affect the glycogen metabolism. Hypoglycemia, hepatomegaly, splenomegaly and growth retardation are the main clinical manifestations. The enzymes affecting glycogen synthesis and degradation are varied.Consequently, the clinical manifestations are different because of different enzyme defection. Most patients treated regularly can obtain the normal growth and grow to adults, but still can be accompanied long-term complications by adulthood. Now some scientists find new modified cornstarch is superior to standard therapy in maintaining blood glucose levels. Gene therapy is still in the experimental animals.

Triple negative breast cancer(TNBC) is defined by the lack of estrogen receptor (ER) , progesterone receptor (PR) and human epidermal growth factor receptor-2 ( Her-2) expression. It is a subtype of tumor with distinct clinical and pathologic features and lacks effective targeted therapies. It is non - sensitive to endocrine therapy and targeted therapy drug herceptin, leaving chemotherapy as the mainstay of treatment The feature and the treatment of TNBC is the hotspot of resent research.

C-termini of proteins often play an important role in various biological processes, such as the transcription and translation from DNA to protein and also participating in various biological regulations. The determination of protein C-terminus is so crucial because it provides not only distinct functional annotation, but also a way to monitor the proteolysis-modified proteins. Based on the biological mass spectrometry, a series of novel methods and technologies were developed both for qualitative and quantitative analyses of protein C-terminus. These methods or technologies can be applied to accurate and effective protein C-terminus profiling, including the sequences and quantitative information of C-termini, which reveals the biological function of C-termini in life's activities and provides a better understanding of the degradation of mature proteins. Combined with our research, this review highlights the improvements in C-terminal proteomics study in the past decades, including the methodologies for recognition and identification of C-terminus, as well as the enrichment strategies for protein C-terminus.
Mass Spectrometry ; Protein Processing, Post-Translational ; Proteins ; chemistry ; Proteolysis ; Proteomics

Objective To study the mechanism of toxicity of hexane. Methods SD rats inhaled 15 g/ma n-hexane statically for 8 hours. Results The levels of GSH in whole blood of rats declined significantly (t-test,P＜0. 01). The levels of MDA in serum of rats revealed increasing trend but without statistical significance (t-test,P＞0. 05). The activities of SOD and GSH-Px in liver of rats decreased significantly (t-test,P＜0.01). Conclusion n-Hexane could induce or enhance the reaction of oxygen free radical in organism,and result in damages of lipid peroxidation,which might be one of the mechanisms of the toxicity of alkane.

OBJECTIVETo explor the curative effect of double pedicle sliding skin flap for correction of cryptotia.

METHODSFrom January 2011 to October 2014, 7 cases with cryptotia (12 ears) were treated. We designed a double pedicle sliding skin flap above the helix. The flap was slided downward to form a new auriculocephalic sulcus. The other wound was covered by skin graft.

RESULTSAll wounds healed very well with no complication. The follow-up period ranged from 3 months to 1 year, with an average of (5.67 ± 3.38) months. The ear had stable appearance with no recurrence. The result was satisfactory.

CONCLUSIONSThe method is simple with reliable flap survival rates. The new auriculocephalic sulcus is stable and resistant. It's a good choice for correction of cryptotia.

Ear Auricle ; abnormalities ; surgery ; Ear Cartilage ; abnormalities ; surgery ; Follow-Up Studies ; Graft Survival ; Humans ; Reconstructive Surgical Procedures ; methods ; Surgical Flaps ; transplantation ; Time Factors ; Wound Healing

Objective To understand the endemic situation of schistosomiasis after its transmission interruption in Baoying County,so as to provide the evidence for adjusting the prevention and control strategy. Methods The data of schistosomiasis surveillance in Baoying County from 1988 to 2015 were collected and retrospectively analyzed. Results The schistosomiasis surveillance has been established since it was interrupted in Baoying County in 1987. The total surveyed area with Oncomelania hupensis snails was 21 269.95 hm2,but no living snails were found. The number of schistosomiasis examinations of residents was 163 196 person?times,and 270 person?times were positive in serum test,26 person?times were positive in stool test. No positive cases were found since 1997. The number of schistosomiasis tests of livestock was 2 180 cattle?times but no infection was found. Conclusion No schistosome infected snails or livestock are founded in Baoying County for last 28 years,and no patients are founded for last 11 years. The effect of schistosomiasis control is steady.

OBJECTIVE :To determine the content of Troxerutin in Troxerutin injection by HPLC METHODS :The separation was performed on a Hypersil BDS C18 column The mobile phase was composed of acetontrile-tetrahydrofuran-0.05 mol/Lsodium dihydrogen phosphate(15∶8∶77V/V ) ,adjusted to pH4 0 with phosphoric acid The flow rate was 0 8ml/ min The ultraviolet wavelength was 254nm RESULTS :The calibration curve showed good li nearityinrange of (20～200) ?g/ ml(r=0 9998) ;The mean recovery was 100 7 % The within -day RSD was 1 61 %,and inter-day RSD was 2 46 % CONC_ LUSION :The method was simple ,convenient and accurate and can be used for the quality control of Troxerutin injection

Objective In order to study the role of APP SW mutation in the aetiology of Alzheimer′s disease(AD), the phenotypes of the PDAP SW transgenic mice were investigated. Methods Body weight, coat color and reproducibility of transgenic mice were observed;The pathological changes in the brain of the transgenic mice were examined using immunohistochemistry; Behavioral changes of transgenic mice were examined by Y-maze. Result At 3 month′s age, the mean body weight of the transgenic mice was (31.0?3.7) g, that of non-transgenic was (34.0?2.9) g;while the mean body weight of F1 transgenic mice was (32.0?3.3) g, of negative mice was (31.0?4.2) g. There were no significant difference between these two groups. There were three transgenic mice with abnormal coat and one male was infertile. The transgenic mice had amyloid deposit in their brains. In the Y-maze test, transgenic mice showed an increased number of arm entered (53?7 versus 37?4) and an impaired spontaneous alternation. The frequency of alternation was 48.2% in the transgenic mice and 76.4% in the non-transgenic mice,respectively.Conclusion The PDAP SW transgenic mice had typical pathological and behavioral changes similar to that of AD and could be used as an animal model for studying AD.

Present basic researches on hepatic stem cells have obtained great progress. It is believed that hepatic stem cells can differentiate into hepatic cells and biliary duct cells. Hepatic stem cells are isolated and cultured by two-step collagenase digestion method, density gradient centrifugation, centrifugal elutriation, fluorescent activation screening method and immunomagnetic beads screening method. Directional differentiation of hepatic stem cells is a complicated process, which is affected by exterior and interior cell factors under liver microenvironment where are beneficial to growth, differentiation and function of stem cells. Hepatocyte growth factor can promote the proliferation of hepatic stem cells, and accelerate the differentiation into mature hepatic cells. Transcription factor of hepatic cells play a crucial effect in regulating the differentiation of hepatic stem cells. Incomplete or abnormal differentiation of hepatic stem cells can be responsible for liver cancer. Hepatic stem cells are the potential carrier cells as a target gene for liver cancer.

Objective To compare the accuracy of full field digital mammography (FFDM) and digital breast tomosynthesis (DBT) in predicting size of ductal carcinoma in situ (DCIS).Methods Totally 30 women pateints with DCIS confirmed by histology were analyzed retrospectively.Two radiologists measured the size of the lesions on FFDM,DBT images respectively,and compared with pathological results.Two kinds of inspection methods correlation with pathology were compared with Pearson correlation analysis.Results Size of DCIS measured by doctor 1 and doctor 2 between FFDM and histology had correlation (r=0.857,0.818,both P＜0.01),and those between DBT and histology also had correlation (r=0.924,0.885,both P＜0.01).Intraclass correlation coefficient (ICC) of two doctors was 0.947 on FFDM,and 0.908 on DBT.Conclusion DBT provides a better estimation of the size of DCIS.Two physicians had high consistency for lesion size evaluation.For DCIS with microcalcifications,both FFDM and DBT provide smaller estimations compared with pathology,thus a bigger surgical resection of lesion is needed.