A 54-year-old female presented with indurated nodules and plaques on the left chest and back for 32 years.Skin examination showed little finger- to soybean-sized,slightly whitish indurated nodules with little mobility arising on skin-colored indurated plaques following Blaschko's lines on the left chest and back.The lesional skin was sclerotic and atrophic,and the lesions were distributed unilaterally.Serum levels of calcium,phosphate,alkaline phosphatase and parathyroid hormone were normal.Histopathological examination revealed fibrous scar with flake-like calcification.The diagnosis was idiopathic calcinosis cutis following Blaschko's lines.

Objective To investigate clinical usefulness of intracranial lesions in pre-and post-operation by functional MRI with blood oxygen level dependent(BOLD)technique.Methods Common MRI and Motor hand area functional MRI examinations were performed in 45 patients (fMRI group)with intracranial lesions adjacent to the motor cortex before and after operation. As contrast group, 20 patients with occupying position lesions were made a routine MRI examination without fMRI before surgery. A 1.5 T signa super conducting system was employment (GE company ,America). Results The focal transactivation domain of cerebration was seen on fMRI in all cases of fMRI group. The cortical motor hand area shown displacement in 22 cases with abnormal size and shape and found not displacement in 23 cases. The distribution of transactivation domain was not changed in different disease . There was positive correlation between the distance from lesions to the domains and myodynamia in preoperation(r=0.553,P0.05). Our data indicated that there was negative correlation between the distance and the difference of myodynamia (r=-0.570,P

Objective To evaluate the efficacy and safety of tacrolimus in patients with generalized myasthenia gravis (MG).Methods A total of 69 cases admitted to our hospital were given 2-6 mg/day tacrolimus (FK506) for 12 months.The MG absolute and relative clinical scores were used to monitor the efficacy of tacrolimus.Clinical evaluation was conducted at month 1,3,6,and 12,while the serum concentration of FK506 was measured at one month after administration of tacromus for one month.Results The therapeutic response presenting as improved muscular strength showed within one month after administration of tacrolimus.The overall response rates (MG relative clinical score≥25％) at month 1,3,6 were 81.2％,87.6％,92.2％ respectively.It reached 93.8％ by the final visit at month 12.MG score to evaluate disease severity decreased significantly as the subjects continued to take tacrolimus.Statistic analysis suggested that the serum concentration of FK506 was correlated with its therapeutic effect.Serum trough levels in remission and response groups [(7.1 ± 3.9) μg/L and (6.3 ± 3.8) pg/L,respectively] were significantly higher than that of no response group [(3.4 ± 1.3) μg/L].The most common adverse effects included hyperglycemia (5 cases),myelosuppression (3 cases),and dizziness tinnitus (3 cases),majority of which were temporary and manageable.Conclusions Our study has shown that tacrolimus significantly improved muscular strength of generalized MG patients.The treatment is well tolerated.The therapeutic effect of tacrolimus is observed within 1 month after initial use.Adverse events were manageable and not common.

Objective Using MRI-T2 * method to quantify the cardiac iron overload in patients with β-thalassemia major and to evaluate the correlation between cardiac T2 * values and serum ferritin (SF),liver iron concentration(LIC).Methods Fifty-eight over 10 years old transfusion-dependent patients with β-thalassemia major were underwent MRI heart measurement to obtain T2 * values.Spearman rank correlation was used to analyze the relationship between cardiac T2*,SF,and LIC.Patients were divided into two groups based on standard setting (SF ＞2500 μg/L or LIC ＞ 15 mg/g of dry tissue).Differences of cardiac T2 * values between two groups were evaluated by Wilcoxon rank sum test with cardiac T2 * ＜ 20 ms as diagnosis standard.The sensibilities and specificities of prediction for cardiac iron deposition with the index of SF ＞ 2500 μg/L or LIC ＞ 15 mg/g dry tissue were calculated,and receiver operating characteristic (ROC) curve analysis was performed.Results The range (median) of cardiac T2 * values,SF and LIC in 58 patients were 4.7-51.1 ms (14.0 ms),1345-23 640 μg/L (5741 μg/L),9.0-＞43.0 mg/g dry tissue (41.4 mg/g),respectively.There was no linear correlation between cardiac T2 * values and SF (r =-0.240,P =0.070).Cardiac T2 * values and LIC was weakly correlated (r =-0.420,P =0.002).The range (median) of cardiac T2 * values was 6.1-47.6 ms (23.7 ms) in 7 patients of SF ≤ 2500 μg/L group.The range (median) of cardiac T2 * values was 4.7-51.1 ms(13.5 ms) in 51 patients of SF ＞2500 μg/L group.There was no statistically significant difference between two groups (Z =-0.489,P =0.625).The range (median) of cardiac T2 * values was 24.4-51.1 ms (44.8 ms) in 5 patients of the LIC ≤15 mg/g dry tissue group.The range (median) of cardiac T2 * values was 4.7-45.5 ms (13.2 ms) in 53 patients of HIC ＞ 15 mg/g dry tissue group.There was significant difference between T2 * values of the two groups(Z =-2.895,P =0.004).To predict cardiac iron deposition,the sensibilities and specificities were 90.9％ (30/33) and 16.0％ (4/25) for the index of SF ＞2500 μg/L,100.0％ (33/33) and 20.0％(5/25) for LIC ＞ 15 mg/g dry tissue respectively.The areas under the ROC curve were 0.652 with the index of SF and 0.775 with the index of LIC.Conclusions MRI-T2 * method can directly quantify the cardiac iron overload in patients with β-thalassemia major.There is no linear correlation between cardiac T2 * values and SF.Cardiac T2 * values and LIC is weakly correlated.Using SF or LIC as an indirect index to predict cardiac iron deposition is not reliable in clinical.

Objective To investigate the value of MRI and~1H-MRS in diagnosis of early stage of diabetic encephalopathy by detecting regional metabolite in cynomolgus diabetes models. Methods Five pathogen-free male adolescent cynomolgus were made type 1 diabetes mellitus models (T1DM) by intravenous injection of streptozotocin (STZ) (100 mg/kg), and the reliability and stability of the modes were assessed with long term follow-up of blood glucose and intravenous glucose tolerance tests. MRI and ~1H-MRS were performed to evaluate the volume, signal intensity and metabolic ratios of NAA/Cr, mI/Cr and Cho/Cr at hippocampus, lateral temporal lobe and occipital lobe 3 years after model establishment. Cortisol in serum was detected with immunoradiometric assay. In addition, 5 normal adult cynomolgus monkeys were selected in the control group and accepted the same examination above. Results ①Intravenous administration of STZ could made stable T1DM monkey model. ②Only mI/Cr ratio increased at hippocampus of diabetic monkeys compared to the control group (P<0.05). ③There was no statistical difference of cortisol in serum between the diabetic group and the control group (P＞0.05). Conclusion ~1H-MRS may detect the metabolic changes of the hippocampus in STZ-induced diabetic adolescent cynomolgus monkeys and may contributes to the early diagnosis of diabetic encephalopathy.

Objective To evaluate the therapeutic effects of thymectomy in myasthenia gravis (MG) patients with thymoma.Methods A total of 178 MG patients with thymectomy between July 2008 and December 2010 were included.All the subjects were received either cyclophosphamide alone or in combination with radiotherapeutic treatment after surgery.The MG absolute and relative clinical scores were used to assess the effectiveness of long-term trcatments.Clinical evaluations were conducted before,and at 1,6,12 and 24 months after operation.A comparative analysis on the inter-relationships among MG clinical presentation,WHO histology aud Masaoka stage was also conducted.Results (1) Of the 178 thymomapatients,103 were male and 75 were female,with a mean age of (43.7 ± 12.5)-years old.One hundred and twelve cases were taken cyclophosphamide,and 58 cases with invasive thymoma (stages Ⅱ,Ⅲ and Ⅳ or WHO type B3) were taken cyclophosphamide in combination with radiotherapy.Five patients refused cyclophosphamide or radiotherapy and 3 did not finish treatment.(2) The muscular strength improved obviously in 32.8％ (58/177) of the patients after 1 month after thymectomy,and 59.8％ (101/169),69.7％ (115/165) and 81.5％ (132/162) after 6 months,1 year and 2 years,respectively with MG score for disease severity decreased significantly with time.(3) No significant differences of the improvement rates were observed in patients within different WHO histology category.However,the rates were much higher in patients with Masaoka stage Ⅰ (91.4％) and stage Ⅱ (89.8％) than those in patients with stage Ⅲ (45.5％) and Ⅳ(28.6％) (all P＜0.001).Conclusions The remission rate of MG patients with thymomas increase after thymectomy plus cyclophosphamide or in combination with radiotherapy and reached 81.5％ after 2 years.The remission rate is associated with Masaoka stagc,but not with WHO histology.

Objective To explore the value of MRI-R2 * and to compare clinical effect of two iron chelators(deferasirox and deferoxamine) in iron-overloaded patients.Methods By completely randomized balanced design,24 iron-overloaded patients were randomly divided into 2 groups,which consisted of 12 patients treated with deferasirox and 12 patients treated with deferoxamine.The planned deferasirox dose was 40 mg· kg-1 · d-1,and the deferoxamine dose was no less than 50 mg · kg-1 · d-1 All patients underwent quantitative MRI at the time points of the primary screening,6 months and 12 months.Pair Wilcoxon rank sum test was used to compare the differences of liver R2 * values of the 2 groups at various time points respectively.Wilcoxon rank sum test was used to compare the differences of change rate of liver R2 * values between the two groups at the time point of 6 months,12 months,respectively.Results Deferasirox group's liver R2 * values of primary screening,6 months and 12 months were 1081,889 and 712 Hz,while deferoxamine group's liver R2 * values were 1042,838 and 488 Hz.There was no statistically significant difference between liver R2 * values of two groups at primary screening (Z =-0.029,P ＞ 0.05).The change rate of liver R2 * of deferasirox group at 12 month was-32％,while it was-58％ for the deferoxamine group,and there was statistically significant difference between the two groups (Z =-3.060,P ＜0.01).The change rate of serum ferritin of deferasirox group at 12 month was-15％,while it was -55％ for the deferoxamine group,and there was statistically significant difference between the two groups (Z =-2.945,P ＜ 0.01).Conclusion By using MRI-R2*,it suggest that both deferasirox and deferoxamine can effectively remove liver iron and deferoxamine is superior to deferasirox.

ObjectiveTo analyse the clinical characteristics of pancreatic intraductal papillary mucinous neoplasm (IPMN) which coexists with extrapancreatic malignancy (EPM),with an aim to provide strategies for clinical diagnosis and treatment.MethodsThe PubMed was used to search for the pancreatic IPMN related articles with positive pathologic results.A pooled analysis was then performed.The ratio ofpancreatic IPMNs coexisting with EPMs and the locations (or the type) of EPMs were analyzed.ResultsAfter a strict process of screening,18 articles met the pre-determined standardsand were accepted.Of the 1327 patients,363 had coexisting EPMs (27.35％).There were 392 EPMs in these 363 patients.The EPMs occurred in almost all the systems of the body,especially in the digestive tract and its related organs,which accounted for 63.06％ of the EPMs. Conclusions There is a tendency for patients with pancreatic IPMN to have coexisting EPM. More than half of these EPMs are malignant tumors in the digestive system. When pancreatic IPMN is diagnosed,the clinician should be aware of the possible coexistence of an EPM and should look for the possibility of a new EPM developing in a patient after treatment of pancreatic IPMN.

Objective To evaluate the clinical value of laparoscopic cystogastrostomy for retrogastric pancreatic pseudocysts.Methods Five patients suffering from retrogastric pancreatic pseudocysts caused by severe acute biliary pancreatitis received conservative management for 2 ～ 6 months,and the sizes of pseudocysts were 8,10,12,14,15 cm.All the 5 patients received laparoscopic cystogastrostomy,and 4 ports methods was applied,through anterior gastric wall,the posterior gastric wall and pancreatic pseudocysts were incised by using harmonic scalpel,then cystogastrostomy was performed to drain the pseudocysts.Results Laparoscopic cystogastrostomy for retrogastric pancreatic pseudocysts was successful in all patients,theoperation time was 90,105,115,120,150 minutes.The blood loss was 100,150,150,200,300 ml.No intra-gastric bleeding occurred.After 1 month follow-up,all the pseudocysts disappeared,and there was no acute pancreatitis and local infection recurrence.Gastric leakage occurred 7 d after operation in one patient,and was healed after one month of conservative management.Conclusions Laparoscopic cystogastrostomy through gastric cavity for retrogastric pancreatic pseudocysts is simple and effective,mini-invasive,and it can be an alternative therapeutic method for pancreatic pseudocysts.

Objective To evaluate the clinical value of noninvasive positive-pressure ventilation (NPPV) treatment in acute respiratory distress syndrome caused by acute pancreatitis. Methods A retrospective study of 27 cases, with acute respiratory distress syndrome (ARDS) caused by acute paucreatitis,who were admitted to our department from Jan 2007 to May 2010 and treated with NPPV, was performed. The changes of heart rate, respiratory rate, PaO2, oxygenation index (OI) and PaCO2 before and after treatment were compared. Results The heart rate, respiratory rate of 25(92.6％ ) patients decreased from (118.4 ±13.4)/min, (32.1 ± 1.7)/min to ( 81.9 + 8.5 )/min, ( 19.9 ± 2.1 )/min; PaO2, OI and PaCO2 increased from (74.1 ±5.0)mmHg, (148.2 +10.0)mmHg, (28.7 ±1.6)mmHg to (110.4 ±20.8)mmHg, (204.5±71.1)mmHg, (38.4 +3.6)mmHg 48 h after NPPV, respectively, and they recovered and were transited to oxygen supply by Venturi mask. 2 (7.4％) patients deteriorated and were transited to invasive positive-pressure ventilation support. Conclusions NPPV could effectively improve oxygenation of patients with ARDS caused by acute pancreatitis. The procedure of NPPV is relatively easy to use and to learn with few complications, and worth of clinical application.