Objective To investigate MRI value in diagnosing and differential diagnosing intrahepatic peripheral cholangiocarcinoma(IHPCC).Methods Twelve cases with IHPCC proved by pathology underwent plain MRI,and contrast-enhanced MR imaging including arterial,portal and delayed phase.MRI features were analyzed.Results All of 17 leisons in 12 cases,12 lesions exhibited hyperintensity at edge and hypointensity at center on T2WI,5 appeared as hyperintensity.2 lesions showed no enhancement and 15 lesions were circular enhancement in arterial phase,all of 17 lesions showed gradual fill in the center of lesions.The diagnostic accuracy was 100%.Conclusion MRI plays an important role in detecting and diagnosing IHPCC.

Objective To analyze the imaging features and follow-up changes of high-resolution CT(HRCT) in Goodpasture syndrome.Methods HRCT imaging features and follow-up findings of 15 cases Goodpasture syndrome confirmed by clinical were analyzed retrospectrively.The imaging features included extent,forms and follow-up changes.Results The lung lesion of Goodpasture syndrome involved two lobes(n=1), three lobes(n=2), four lobes(n=5) and five lobes (n=7).Upper lobe of the right lung was the most common involved region.Centered on the hilum of lung consolidations confused ground glass opacity (GGO) were showed in 7 cases, GGO distribution of pulmonary leaflets in 5 cases.On follow-up observation, lobar or segmental consolidation could change into GGO,GGO could disappear in short times.Conclusion Multiple lobar or segmental consolidations confused GGO without the lung bottom and periphery involvement is the imaging characteristics of Goodpasture syndrome patients with anemia and hemoptysis.HRCT is a helpful method for the diagnosis and following up of Goodpasture syndrome.

Objective To analyze the mutation characteristics of GBA gene in one patient with Gaucher disease and platelet transfusion refractoriness.Methods A female patient with anemia and thrombocytopenia showed platelet transfusion refractoriness,and then the proband and her family were performed bone marrow smear,β-glucocerebrosidase activity in leukocytes (dried blood spot assay),Bultrasonography and gene sequencing examination and pedigree investigation.Results Pedigree investigation showed that the heterozygous mutation of GBA gene existed in the father,mother,son,daughter and sister of the proband.Bone marrow cytomorphologic examination showed that Gaucher cells accounted for 6.0％ in the female patient.The β-glucocerebrosidase activity in leukocytes was 3.78 nmol/(h · mg Pro).B-ultrasonography showed slightly splenomegaly.Gene sequencing found that the homozygous mutation of GBA gene,c.484A ＞ G,existed in the female patient.Conclusion The patients with Gaucher disease may appear platelet transfusion refractoriness due to hypersplenism.The mutation of GBA gene is the main pathogenic factor of the family with Gaucher disease.

Objective To evaluate the serum pyridinoline cross-linked carboxyteminal telopeptide of type Ⅰ collagen ( ICTP) in the early diagnosis potency,efficacy and prognosis of tumor bone metastasis. Methods According to emission computed tomography(ECT) ,MRI and X-ray results,336 cases of tumor were divided into higher ICTP (5. 98 ± 1. 95μg/L ) than normal values. Twenty-two cases were identified bone metastasis through PET/CT examination. 26 cases were identified bone the effective cases decreased from( 13. 22 ± 4.65)μg/L (before treatment) to (7. 18 ±3. 54)μg/L (after treatment) (t = 10. 076,P = 0. 000). Conclusions Serum ICTP is helpful for the early diagnosis, screening and efficacy evaluation of tumor bone metastasis. It could be used for monitoring the occurrence of tumor bone metastasis and its prognosis.

ObjectiveTo evaluate the treatment efifcacy of the treatment promotion of standardized management for chil-dren with asthma.MethodsMedical records of 150 children with asthma were reviewed and divided into management group or control group according to whether standardized management was accepted. Comprehensive asthma education for asthma pa-tients and their parents including asthma associated basic knowledge education, health education as well as follow-ups at deifned intervals was conducted in 78 cases. In the meantime, standardized asthma therapies were performed. Control group involved 72 cases who did not receive asthma education managements and only accepted regular clinical therapies. After 1-year observational follow-up, , clinical efifcacy of children with asthma, changes of knowledge-attitude-practice of parents, and compliance of med-ication were compared between the two groups.ResultsAfter promotion of standardized managements treatment, asthma con-trol rates in the management group were signiifcantly higher than that of the control group(χ2=54.68,P<0.01); In addition, the rate of asthma attacks, emergency visits as well as hospitalizations were obviously reduced in the management group than control group (both withP<0.01). Knowledge associated with asthma, therapy and management executions as well as knowledge-atti-tude-practice of parents also demonstrated apparent elevations in the management group (P<0.01); At the same time, management group has illustrated superior medication compliance over the control group (χ2=66.27,P<0.01).ConclusionPromotion of standardized treatment management among children with asthma can help to achieve effective control by raising levels of knowl-edge-attitude-practice of the parents as well as the patient’s compliance to the treatment.

Objective To study the methods and effect of the surgical treatment for intrahepaticolithiasis(IHL). Methods In recent 12 years, in our hospital,230 cases of IHL were treated by different operations companied with biliary fibrescopy to remove the residnal stones of biliary tract. Results (1) Hepatolobectomy combined with Y-type hepatocholangiojejunostomy was done in 103 cases,the success rate was 91.8%.(2)Y-type hepatocholangiojejunostomy was performed on 45 cases,the success rate was 81.2%.(3)Hepaticolithotomy combined with T-tube drainage was done in 72 cases,the success rate was 69.3%. Conclusions For intrahepaticolithiasis,combination of hepatolobectomy with other operation and with biliary fibrescopy during or after the operation shoul be done, which can effectively improve the curative effect.

Objective: To evaluate the efficacy and safety of Oryz-Aspergillus enzyme and pancreatin tablets (Combizym^®) in the treatment of postprandial distress syndrome (PDS) in the elderly, compared with gastrointestinal motility drugs. Methods: A prospective randomized controlled trial was designed and registered in the China Clinical Trials Registry (ChiCTR-IPR-16008185). The elderly patients with PDS were randomly divided into three groups, including Mosapride group with Mosapride citrate tablets 5 mg 3 times per day for 2 weeks; Combizym^® group with Combizym tablets 244 mg 3 times per day for 2 weeks; combined treatment group with both drugs and same doses for 2 weeks. The modified Nepean dyspepsia index (NDSI) score, discomfort intensity score and PDS score were calculated on patients before treatment, at the end of first and second week of treatment, as well as 4 weeks after treatment finished, respectively. Adverse effects were evaluated. Results: A total of 323 patients from 16 tertiary hospitals in China were enrolled in this study. Among them, 105 patients were in Mosapride group, 109 in Combizym^® group and 109 in combined treatment group. There were 148 males (45.8%) and 175 females (54.2%) with median age 71.4±9.0 years (60-100 years). Baseline characteristics of three groups were comparable. After treatment, the NDSI scores in three groups all decreased significantly (P<0.001), while they were similar between groups (P>0.05). The discomfort intensity score and PDS score in three groups showed a significant reduction after treatment (P<0.001), especially in the combined treatment group. Compared with Mosapride group, the scores in Combizym^® group decreased significantly after one or two weeks [discomfort intensity score: after one week, 4.0(2.5, 8.0) vs. 6.0(3.0, 10.0); after two weeks, 3.0(0.0, 5.0) vs. 4.0(2.0, 6.0); all P<0.05. PDS score: after one week, 6.0(3.0, 9.0) vs. 7.0(3.5, 10.5); after two weeks, 3.0(0.0, 5.0) vs. 4.0(2.0, 7.0); all P<0.05]. The efficacy rate in all patients after first week of treatment was over 15.0%. The efficacy rates after two weeks were 55.2%, 68.8% and 73.4% in Mosapride group, Combizym^® group and combined treatment group, respectively. After two week treatment, the efficacy rates in Combizym^® group (P=0.041) and combined group (P=0.006) were higher than that of Mosapride group. The recurrence rate of Mosapride group was 9.5%, which was significantly higher than that of Combizym^® group (1.8%, P<0.05) and combined treatment group (1.8%, P<0.05). There were no serious adverse effects in the three groups. Conclusions The efficacy of Oryz-Aspergillus enzyme and pancreatin tablets is comparable with that of Mosapride in elderly PDS patients, with fewer adverse effects and low recurrence rate. Combination regimen indicates better efficacy than that of Oryz-Aspergillus enzyme and pancreatin tablets or Mosapride alone.