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]The paper summarizes the clinical experience of Professor Lu Su for treating menopausal syndrome.[Methods] By learning from Professor Lu Su, sum up the knowledge of etiology and pathogenesis, treatment characteristics and analyze cases. [Results]Professor Lu Su thought that the main etiological and pathogenesis of menopausal syndrome was that deficiency of the kidney-yin and hyperactivity of heart and liver fire. The kidney water cannot aid heart, the occurrence of heart(including liver) fire too strong, the heart is not calm, leading to hot flashes sweating, upset insomnia and other symptoms of menopausal syndrome. Therefore, she made a total therapeutic principle of invigoration of the kidney and nourishment of Yin, purging heart fire and soothing the nerves. Besides, the article introduces prescribing characteristics of other lesions relating to menopausal syndrome, such as blood stasis, phlegm, yang deficiency and disharmony between spleen and stomach. In treatment, Lu Su is good at syndrome differentiation and treatment, prescribing refinedly and simply, combining medication with psychotherapy. [Conclusion]Therapies of Professor Lu Su on treating menopausal syndrome have significant effect.

[ABSTRACT]OBJECTIVETo evaluate the efficacy of combined oral and intratympanic glucocorticoids treatment for severe to profound sudden sensorineural hearing loss(SSNHL).METHODSFifty-four patients with severe to profound SSNHL were retrospectively studied. They were treated with oral steroids, vasodilators, anticoagulants. The patients who accepted intratympanic methylprednisolone sodium succinate were included the therapy group, the others were included in the control group. Hearing improvements were analyzed respectively after two weeks.RESULTSHearing in both groups were improved significantly after treatment(P<0.01), but there was no difference in hearing improvements between two groups(P=0.194). The efficacy for severe SSNHL patients in therapy group was equal to those who were in control group (P=0.251), so was the efficacy for profound SSNHL patients in both groups(P=0.380).CONCLUSIONThe combined systemic and intratympanic glucocorticoids treatment was effective for patients with severe to profound SSNHL, but while with no better efficacy than oral glucocorticoid alone.

Objective To explore the main factors of hypokalemia in the patients with acute myocardial infarction after PCI.Method The clinical data of ninety-six patients with myocardial infarction after PCI were analyzed to look into the reasons for hypokalemia.Results Fifty-seven patients contracted hypokalemia,with a rate of 59.4%.The complication was cured by dietetic supplementation of potassium,oral supplementation of potassium,and potassium supplementation by atomization inhalation and intravenous infusion.The reasons for hypokelemia included inadequate input,over-ejectment,abnormal distribution of potassium and use of contrast agent.Conclusions Patients with myocardial infarction after PCI are more susceptible to hypokalemia.Therefore,it is a need to correct hypokelemia by means of potassium supplementation.

Objective To investigate the effects of carbon monoxide poisoning on the long-term cognition in elderly patients.Methods Totally 53 patients with carbon monoxide poisoning and 51 healthy persons at our Hospital from June 2005 to June 2008 were enrolled.Mini-mental state examination(MMSE) and activities of daily living(ADL) scale were utilized to evaluale the life quality of the elderly patients through a cross-sectional study on July 2011.Results MMSE score showed that the attention calculation score,delayed recall score,and total score were higher in control group (4.9±0.4,2.9±1.0,28.3±2.3,respectively) than in carbon monoxide poisoning group (4.5±0.8,2.6±0.6,25.5±3.5,respectively) (t＝3.138,3.607,4.802,all P＜0.05).ADL score of carbon monoxide poisoning group was 26.7±7.3,and that of the control group was 2.04±7.6 (t＝1.827,P＞0.05).Conclusions Elderly patients with acute carbon monoxide poisoning may cause long-term cognitive impairment with decreasing attention calculation and delayed recall.

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 develop a quality standard of Citrus limon oils.Methods Limonene in Citrus limon oils was identified by capillary gas chromatography and its content was determined by internal standard method.A polyethylene glycol elastic quartz capillary column (30 m of length,0.32 mm of internal diameter,0.25?m of film thickness) was used,column temperature being 90℃and split ratio being 5:1.Results In the range of 0.0627 mg/mL～4.0136 mg/mL for limonene,an equation was obtained:Y=1.53321X-0.05275,r=0.999 9.The average recovery of limonene was 97.2 % with RSD being 0.6 %.Conclusion The method is stable and reliable,and can provide evi- dences for the development of quality standard of Citrus limon oils.

AIM: To establish a new quality standard for Kangbindu Oral Solution (Radix Isatidis, Gypsum Fibrosum, Rhizoma Phragmitis, Radix Rehmannia, etc.). METHODS: Rhizoma Acori Tatarinowii and Fructus Forsythiae were identified by TLC. The forsythin content was determined by HPLC on Inertsil ODS column (4.6mm?250mm,5?m). The mobile phase was acetomitrile water(25∶75). The detection wavelength was set at 277nm. RESULTS: The linear range of forsythin was in the range of 4.24～21.2?g. The average recovery was 99.09%, and RSD was 0.4%( n =6). CONCLUSION: The method is simple and accurate, and can be used for the quality control of Kangbindu Oral Solution.