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 To study the clinical characteristics which are related to malignancy in pancreatic intraductal papillary mucinous neoplasm (IPMN) with an aim to provide evidence for clinical practice.Methods Using PubMed,all pancreatic IPMN related articles with positive pathologic results before July 30th,2011 were studied.A pooled analysis was carried out on the morphological features of the disease.The analysis included gender,diameter of main pancreatic duct,diameter of cystic lesion,mural nodules and histological types (benign/malignant) of the neoplasm.Results 98 articles (including 1902 cases) were collected and analyzed.1025 cases were benign (53.89％) and 877 cases (46.11 ％) were malignant.Morphologically,there were a correlation between main pancreatic duct dilatation (≥5 mm),cystic lesion of large size (≥30 mm),presence of mural nodules and malignancy.The OR (95％ CI) were 5.591 (3.657-8.548),3.633 (2.626-5.027) and 4.983 (3.872-6.412) respectively.Conclusions A main pancreatic duct dilatation (≥5 mm),cystic lesions of large size (≥30 mm) and presence of mural nodules prompt the tumor to be malignant.In clinical work,the management of pancreatic IPMN should be made prudently based on comprehensive analysis of clinical features and the patient's status and intent.

Objective To investigate the relationship between the immunohistochemical types and the pathological types of pancreatic intraductal papillary mucinous neoplasms (IPMN).Methods Literatures on the diagnosis and treatment of pancreatic IPMN published before June 30,2011 in the PubMed database were retrieved.The literatures adopted were scored by the case reports quality assessment list.The correlation analysis between the immunohistochemical types and the gender,pathological types,characteristics of benign or malignant tumor and morphological types were analyzed.All data were analyzed using the Pearson chi-square test or multiple regression analysis.Results Thirteen literatures were adopted,the scores were 31-45 (full mark:50),and the mean score was 37.The clinical data of 826 pancreatic IPMN patients who were comfirmed by pathological examination were collected,and there were 4 immunohistochemical types:(1) The gastric type (363 patients).Of the 271 patients who underwent gender analysis,there were 195 males and 76 females,with the median age of 65.6 years.Of the 225 patients who underwent pathological types analysis,there were 146 cases of adenoma,34 cases of borderline tumor,24 cases of carcinoma in situ,21 cases of invasive carcinoma,the benign tumor was accounted for 86.68％ (293/338).Of the 215 patients who underwent morphological type analysis,there were 34 cases of main pancreatic duct type,151 cases of branch duct type and 30 cases of mixed type.(2) The intestinal type (327 patients).Of the 269 patients who underwent gender analysis,there were 184 males and 85 females,with the median age of 64.5 years.Of the 262 patients who underwent pathological types analysis,there were 28 cases of adenoma,43 cases of borderline tumor,91 cases of carcinoma in situ,100 cases of invasive carcinoma,the benign tumor was accounted for 29.21％ (85/291).Of the 151 patients who underwent morphological type analysis,there were 63 cases of main pancreatic duct type,54 cases of branch duct type and 34 cases of mixed type.(3) The pancreatobiliary type (92 patients).Of the 78 patients who underwent gender analysis,there were 41 males and 37 females,with the median age of 69.2 years.Of the 81 patients who underwent pathological types analysis,there were 4 cases of adenoma,1 case of borderline tumor,21 cases of carcinoma in situ,55 cases of invasive carcinoma,the benign tumor was accounted for 5.75％ (5/87).Of the 34 patients who underwent morphological type analysis,there were 7 cases of main pancreatic duct type,18 cases of branch duct type and 9 cases of mixed type.(4) The oncocytic type (44 patients).Of the 37 patients who underwent gender analysis,there were 24 males and 13 females,with the median age of 60.3 years.Of the 33 patients who underwent pathological types analysis,there were 18 cases of carcinoma in situ,15 cases of invasive carcinoma,and all of them were with malignant tumors.Of the 36 patients who underwent morphological type analysis,there were 9 cases of main pancreatic duct type,16 cases of branch duct type and 11 cases of mixed type.The immunohistochemical type of pancreatic IPMN was correlated with gender,pathological type and characteristics of maglignant or benign tumors (x2=10.626,281.839,333.212,r =0.097,0.569,0.625,P ＜ 0.05).The result of the chi-square test between the immunohistochemical types and the morphological types was statistically significant (x2 =50.732,P ＜ 0.05),but there was no correlation between them (r =0.010,P ＞ 0.05).Conclusion The immunohistochemical type of pancreatic IPMN is correlated with gender,pathological type and the characteristics of maglignant or benign tumors,which provide references for the diagnosis and prognosis evaluation 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.

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 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 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.

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.