A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

Homocysteine (Hcy) has attracted more and more attention in the medical field since it was found to be closely associated with cardiovascular and cerebrovascular diseases, hypertension, diabetes, kidney diseases, skeletal system diseases, and even mental illnesses. In recent years, the application of Hcy in patients with liver fibrosis and liver cirrhosis has been taken more seriously by clinicians and researchers. This article reviews the recent research advances in the metabolic mechanism and pathophysiologic significance of Hcy in China and foreign countries and highlights the association of Hcy with liver fibrosis and liver cirrhosis and its possible mechanism.

Objective:To establish a rapid HPLC testing method for chloroquine phosphate,hydroxychloroquine sulfate and amodiaquine hydrochloride.Methods:The chromatographic separation was performed on a GRACE prevail C18(53 mm×7 mm,3 μm)column,and the column temperature was maintained at 30℃.Acetonitrile-0.3% triethylamine acetonitrile solution (adjusting pH to 3.0 with phosphoric acid) (12∶88) was used as the mobile phase,the flow rate was 1.0 ml· min-1 and the UV detection wavelength was 254 nm.The qualitative research was performed using relative retention time and spectral similarity as the double indicators.The relative correction factor in the quantification analysis was used for the content determination.Results:Three anti-malarial drugs showed good behavior in one chromatographic system.The rapid HPLC testing analysis could be achieved.The qualitative research was more accurate by using the double indicators (UV spectral similarity and relative capacity factor).The HPLC qualitative accuracy was increased.The relative correction factor method for the quantification could effectively reduce the use of reference substances and speed up the analysis of HPLC.Conclusion:The method is rapid and simple,and suitable for the rapid determination of drugs.

Objective: To establish a content determination method for ferric iron and ferrous iron in ferrous succinate tablets.Methods: Using a Dionex RFICTM protection column (4 mm×50 mm)and a Dionex RFICM Ion PacRCS5A analytical column (4 mm×250 mm), the eluent solution consisted of 7 mmol·L-1 dipicolinic acid, 66 mmol·L-1 potassium hydroxide, 5.6 mmol·L-1 potassium phosphate monobasic and 74 mmol·L-1 formic acid.The flow rate was 1.5 ml·min-1, the column temperature was 30.0℃ and the injection volume was 1.3 μl.Results: Fe3+ showed good linearity within the range of 0.5-15 μg·ml-1(r=1.000 0), Fe2+ showed good linearity within the range of 25-200 μg·ml-1(r=1.000 0), and the average recovery was 103.6%(RSD=2.7%, n=9)and 98.3%(RSD=1.9%, n=9), respectively.Conclusion: The method is simple, reliable and accurate, and can be used for the determination of Fe3+and Fe2+ in ferrous succinate tablets.

Objective To evaluate the prognostic accuracy of the score of toxic epidermal necrolysis (SCORTEN) scoring system in patients with toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS).Methods Clinical data were collected from 39 patients with SJS/TEN hospitalized in Peking Union Medical College Hospital during April 1992 and March 2014,and retrospectively analyzed.Among the 39 patients,13 had died,and the other 26 patients,who were matched to the dead patients in a ratio of 2:1 for age,all had a definite diagnosis and were discharged with improved conditions.The SCORTEN scoring system was used to evaluate the 39 patients with SJS/TEN and calculate expected mortality.The expected mortality and actual mortality were compared between different groups stratified by age in the 39 patients.The receiver operating characteristic (ROC) curve was drawn to assess the prognostic accuracy of the SCORTEN scoring system.Results According to the SCORTEN scoring system,15 out of the 39 patients scored 1 point,14 scored 2 points,6 scored 3 points,and 4 scored 4 points.The total number of expected deaths was 6.808,while that of actual deaths was 13.There was no significant difference between the expected mortality and actual mortality in every SCORTEN score-based group.The area under curve (AUC) was 0.832 8,indicating a good predictive ability of the SCORTEN scoring system.Conclusion The SCORTEN scoring system can predict mortality in TEN/SJS patients at early stage.

Objective:To establish a method to determine ferrous succinate in ferrous succinate tablets .Methods:Ion chromatog-raphy was performed on an IonPac AG19(4 mm ×250 mm)anion exchange chromatography column with a guard column of Ion Pac TM AG19( 4 mm ×50 mm);30.00 mmol· L-1 KOH was used as the isocratic eluent at a flow rate of 1.00 ml· min-1 with suppressed conductivity detection ( Curb:75 mA) at the temperature of 30℃.The sample size was 25 μl .Results:The linear range of succinic acid was 2.005 1-25.064 0μg· ml-1(r=0.999 5).The detection limit and quantitation limit was 0.12ng and 0.42ng, respectively. The recovery was 101.45%(RSD=0.43%, n=6).Conclusion:The method is simple, rapid, accurate, reproducible and sensitive , and can be used to determine the ferrous succinate content in ferrous succinate tablets .

Objective To compare the performance of Mandarin screening version of hearing handicap inven-tory in elderly (HHIE-S)and the single question of hearing loss in the hearing screening in the old adults.Methods A total of 570 participants,aged from 50~85 years,of native Mandarin speakers were tested with standard pure tone audiometry (PTA),HHIE-S and single questionAre you now having difficulty in hearing?.HHIE-S>8 was taken as an indicator of hearing handicap.Sensitivity,specificity,negative predictive values and positive predic-tive values of both HHIE-S and single question inquiry when 0.5~4 kHz PTA average > 25 dB,>40 dB and >60 dB were compared.Results The prevalence was 83.9% (478)measured by PTA.The prevalence was 55.3%and 52.5% for HHIE-S and positive answer for single question,respectively.The sensitivity of HHIE-S when the participants grouped with 0.5~4 kHz PTA average > 25 dB,>40 dB and > 60 dB was 61%,85% and 96%, respectively,whereas the sensitivity of single question of the above 3 conditions was 58%,81% and 100%,respec-tively.The specificity of HHIE-S decreased with greater hearing loss.It was 72%,88% and 47% respectively for HHIE-S and 75%,61% and 50% for the single question.There was no significant difference either for sensitivity and specificity between the HHIE-S and the single question.Conclusion Both the Mandarin version of HHIE-S and single question are effective in the hearing screening of old adults.Depending on the purpose and availability of resources,one or both of them can be adopted.

OBJECTIVE:To establish a method for the uncertainty measurement evaluation of the content determination of pred-nisolone API. METHODS:HPLC internal standard method was adopted to determine the content of prednisolone API,establish mathematical model for uncertainty evaluation,analyze influential factors and evaluate the factors. RESULTS:HPLC internal stan-dard method showed the contont was 98.8%,and expanded uncertainty of prednisolone API was 1.6%,and the determination result was(98.8±1.6)%,k=2. CONCLUSIONS:The method is suitable for the uncertainty measurement evaluation of the content deter-mination of prednisolone API by HPLC internal standard method.

Objective To investigate the expression of β-catenin and PCNA,and its relationship with clinical parameters and prognosis.Methods Tissue microarray technology and immunohistochemical method were used to detect the expression of β-catenin and PCNA in gastric carcinoma and normal tissue collected from January 2000 to December 2006.Data were analyzed by SPSS 19.0.Results The percentage of expression of β-catenin and PCNA in gastric carcinoma is 96.7％,99.2％,respectively,and higher than the normal gastric tissue (x2 =41.082,P =0;x2 =97.692,P =0).ROC curve was used to find that PCNA was better than β-catenin to discriminate gastric carcinoma from normal gastric tissue in sensitivity,specificity,coincidence rate(84.4％,87.5％,84.6％).The expression of β-catenin is related to lymph node metastasis in gastric cancer (x2 =6.107,P =0.04).The expression of PCNA is related to pathological type (x2 =9.71,P =0.02).The expression of β-catenin and PCNA in gastric carcinoma were positively correlated (r =0.24,P =0.005).Multivariate analysis showed that TNM staging and both positive β-catenin and PCNA were independent factors impacting on the overall survival rate of patient with gastric cancer.Conclusions The expression of β-catenin and PCNA in gastric carcinoma is signifrcandy high.PCNA is more important than β-catenin in discriminating gastric carcinoma from normal gastric tissue.Combined detection of the two kinds of protein have some clinical significance in predicting the prognosis of patients with early gastric cancer.

Objective:To study the difference in dissolution behavior of clozapine tablets in four different media between the do-mestic preparations and original preparation to compare the internal quality of the tablets from various manufacturers and provide refer-ence for the drug control. Methods:Referring to the methods of in vitro dissolution test, the dissolution profiles of 46 batches of sam-ples from 18 pharmaceutical enterprises were determined in four kinds of dissolution media with different pH values, and the results were compared with that of the original drug by the method of f2 similarity factor. Results: Totally 46 dissolution profiles were drawn out. The profiles of two batches of samples from one pharmaceutical enterprise were similar to that of the original drug, which account-ing for 4%, and that of the other samples showed notable difference from that of the original drug. Conclusion:The preparation tech-nology of the tablets from domestic has great difference, which leads to significant difference in dissolution behavior. The screening and optimization of the production process in domestic pharmaceutical enterprises should be strengthened to improve the preparation technol-ogy of clozapine tablets.