Objective To investigate the clinical manifestations,laboratory features,methods of diagnosis and treatment and prognosis outcome of intrahepatic cholestasis caused by citrin deficiency (NICCD).Methods Four patients in two months ages diagnosed as NICCD were investigated from February 2014 to December 2014 in the Children's Hospital of Wuhan.The diagnosis and treatment and prognosis were analyzes.Results All patients were in hospital due to the skin with yellow dye admission.The direct bilirubin elevated anomalies associated with different levels of blood lipid,blood ammonia and lactate metabolism were characterized by liver function tests.The hot spot of NICCD SLC25A13 gene mutations in the regional common mutations IVS13 + 1G/A,1638ins23,IVS11 + 1G/A,851del4,S225X,1800ins1,R605X,IVS6 + 5G ＞ A were detected by genscaning and genotyping.Genotyping of SLC25A13 gene in 4 cases were 1638ins23 and 851del4 two heterozygous mutations,851del4 homozygous mutation,851del4 heterozygous mutation,and IVS6+ 5G＞A heterozygous mutation,respectively.So these patients were confirmed as NICCD.The patients were cured by antiinfective and gallbladder back yellow symptomatic treatments.The liver function,blood ammonia,lactic acid and blood lipid were exanimated at the time of admission,1 weeks and 2 weeks after admission,respectively.The results indicated that the treatment effect were good.The liver function were restored to normal after two weeks of outpatient.Conclusion The diagnosis of NICCD needs a comprehensive analysis of clinical,biochemical,metabolic genomics,imaging and pathology.SLC25A13 gene analysis is the reliable basis for the diagnosis of the disease.It is the key for NICCD to diagnose early.It is important to distinguish bile deposition caused by biliary atresia and other related diseases from NICCD.The diet early replacement is good for the prognosis.

Objective:To explore the effect of Nimodipine on synaptophysin in diabetic rat.Methods:38 Wistar rats were divided into the normal control group, diabetic model group, the Nimodipine treatment group randomly. Streptozotocin-diabetic rat model was established. The diabetic rats were treated with Nimodipine for 12 weeks. Then, the learning and memory abilities of rats were tested with the Morris water maze.The mRNA and protein level of synaptophysin in hippocampus were detected by RT-PCR and immunohistochemical method. Results:Compared with diabetic model group, the Nimodipine treatment group showed a significant decrease in the mean time of escape latencies(P

Objective:To evaluate the values of the endoscopic argon plasma coagulation (APC) in the treatment of gastrointestinal polyps. Methods:The gastrointestinal polyps confirmed by endoscopy and pathology were treated with argon plasma coagulation generated by ERBE APC 300 made in German ERBE Company. Results:A total of 78 polyps (59 flat and thin sessile polyps and 19 pedunculated polyps) in 23 patients were all cured with APC. 59 flat, thin and sessile polyps were only treated with APC, whereas 19 pedunculated polyps were treated with snared polypectomy and APC ablation of residual tissue. Time of coagulation for every polyp was determined by its size and location. Polyps became flat and thin after(1～7)times of coagulation. As many as 10 polyps could be treated at one time, but for patients with more than 10 polyps, several times of treatment were needed. Conclusion:Treatment of gastrointestinal polyps by APC is an effective and safety method with low side effects. It can be the first choice of methods for the treatment of flat and thin sessile gastrointestinal polyps or polypoid projection.

Children who recovered from hematological malignancies have different degrees of secondary immunodeficiency.They are prone to acquire various infectious diseases, thus affecting their health and even endangering their life and may become the source of infection that affects the health of the surrounding people and residents of co-mmunities.Preventive vaccination is the easiest and most effective measure to prevent infectious diseases.However, in addition to the limited qualification of vaccination, due to the lack of the understanding to the safety, effectiveness and contraindications in the vaccination for this type of patients, there still exists blankness in the preventive vaccination work for this group in China.This paper summarizes some consensus on the vaccination for discharged patients of hematological malignancies in light of providing reference for the implementation of this clinical work in China.

Objective　To study the visual electrophyological changes in　patients with pregnancy-induced hypertension syndrome(PIH).Methods　The visual evoked potentials (VEP) and electroretinogram (ERG) of 28 patients with PIH in 56 eyes were recorded.Results　The patients with PIH had pathologic visual electrophysiologic abnormalities. The VEP N75 peak latency significantly increased(P<0.05), and the ERG b wave latency in patients with PIH showed remarkable difference(P<0.05).Conclusion　Visual electrophysiological examination may be valuable in early diagnosis of retinal disfunction in patients with PIH.

Portal vein tumor thrombus (PVTT) influence the prognosis of hepatocellular carcinoma.The development of PVTT is a multi-factor,multi-part process.According to anatomic features of the portal vein in the liver and tumor thrombus of HCC developing modes,a uniform tumor thrombus types system (typesⅠ -Ⅳ) is recommended.Multi-modal therapy based on surgery,interventional therapy and radiotherapy can improve the curative effect enormously.

Objective To investigate the incidence of the common respiratory viruses in children with respiratory tract infection and to compare the results among different ages and to analyze the relationship between wheezing of children and various viruses.Methods Nasopharyngeal secretions of 618 children with respiratory infection hospitalized were assayed for 7 respiratory viruses, including respiratory syncytial virus ,adenovirus, influenza A virus, influenza B virus, parainfluenza virus Ⅰ 、 Ⅱ、 Ⅲ.Results (1) A total of 166 cases, affected with viral etiology,were detected in 618 samples,and the total positive rate was 26.86％.The most common virus was RSV (17.96％), followed by PIV Ⅲ (5.99％), IFA (1.62％), ADV (0.81％), PIV Ⅰ (0.49％).Moreover, 1 case infected with RSV and PIVⅠ was included, with the positive rate 0.16％.(2)The positive rates were different in various ages, with 14d ～ ＜ 1-year 35.04％, 1 ～ ＜ 3-year 18.25％, 3 ～ ＜ 6-year 17.11％ and ≥ 6-year 9.26％.(3) There were differences of positive rate of viruses among different respiratory infectious diseases, and capillary bronchitis occupied the highest percentage(47.37％).(4)Among all the detected children, infants were more likely to whee in auscultation of lung, and RSV was the primary etiology.Conclusion RSV,which was the primary viral etiology in children with wheezing, was the most common virus leading to respiratory infection of children, followed by PIV Ⅲ.In the children with respiratory tract infection, the viral infection tends to occur in younger children.

Circulating tumor cells (CTCs) play pivotal roles for monitoring the tumor metastasis and prognosis.The nanotechnology provides a favourable platform for CTCs detection,and enables CTCs to be more promising for practical application.Meanwhile,the nanoscale device by virtue of nanotechnology has broad application prospects in eliminating CTCs and offers a new direction in the field of anti-cancer.

Objective To explore the effects of enalapril on spatial learning and memory,and to identify synaptophysin expression in hippocampus of streptozotocin-diabetic rats.Methods Forty rats were randomly divided into control group,diabetic model group and the enalapril treatment group.Streptozotocin-diabetic rat model was developed.The diabetic rats were treated with enalapril for 12 weeks.Then,the learning and memory abilities of rats were tested with Morris water maze;mRNA and protein expression of synaptophysin in hippocampus was detected by RT-PCR and immunostaining.Results Compared with diabetic model group,the enalapril treatment group showed a significant decrease in the mean time of escape latencies(P

Objective To compare and analyze the clinical effects of two surgical methods in the treatment of multilevel cervical myelopathy.Methods The data of 86 patients with multilevel cervical spondylosis myelopathy were retrospectively analyzed from June 2011 to June 2015.48 patients treated with anterior decompression and bone graft fusion were set as A group.38 patients underwent posterior single open -door laminoplasty were set as B group. The changes of nerve function indexes were compared between the two groups before operation,6 months after opera-tion and the last follow -up.Results 6 months after operation,the JOA score of the two groups was significantly higher than before the operation,and the difference was statistically significant(tA =-6.438,tB =-7.425,P <0.05).After 6 months and the last follow -up,there was no significant difference in the JOA score between the two groups(tafter 6 months =-0.892,tlast fol ow -up =0.160,P >0.05).At the last follow -up,the excellent and good rate of A group was 70.83%,that of B group was 71.05%,and there was no significant difference between the two groups (χ2 =0.00,P >0.05).Two groups of patients were successfully completed surgery,the average operation time of A group was (148.75 ±40.68)min,that of B group was (109.34 ±35.61)min,the difference between the two groups was statistically significant(t =4.711,P <0.05).The amount of bleeding in A group was (157.82 ±51.40)mL,that of B group was (235.76 ±61.55)mL,the difference was statistically significant(t =-6.399,P <0.05).Conclusion Anterior subtotal decompression and bone graft fusion and posterior single open door surgery in the treatment of multi segment cervical spondylosis myelopathy can obtain satisfactory neurological function.According to the patients'clini-cal symptoms and complications to develop a reasonable surgical plan is the key to treatment.