Caroli's disease is a rare autosomal recessive hereditary disease. The low mobidity, insufficient awareness of doctor and complex clinical manifestations of the disease are likely to result in misdiagnosis and missed diagnosis.The pathogen, typing, pathology, clinical manifesation and therapy of this disease are discussed to recognise the disease better.

BACKGROUND:In recent years, great progress has been achieved in the mechanical and viscoelastic properties of arterial blood vessels of normal human corpses and animals. OBJECTIVE: To quantitatively analyze the tensile mechanical properties of thoracic aorta in normaly fed spontaneously hypertensive rats and salt-loaded spontaneously hypertensive rats. METHODS: Twenty spontaneously hypertensive rats were obtained and randomly divided into experimental and control groups (n=10/group). Rats in the experimental group were subjected to continuous salt loading intervention for 16 weeks. Rats in the control group were fed with normal diet and ordinary tap water. At the 16th week, 10 specimens of thoracic aorta of rats from these two groups were harvested to conduct stress relaxation experiments.RESULTS AND CONCLUSION: The decrease in stress at 7 200 seconds and the decrease in normalized stress relaxation function value at 7 200 seconds of thoracic aortic specimens of rats in experimental group were both lower than those in the control group (P< 0.05). These results confirm that the stress relaxation properties of thoracic aorta of normaly fed and salt-loaded spontaneously hypertensive rats change, wherein the changes in salt-loaded spontaneously hypertensive rats are more obvious.

To identify the isolated suspicious strain of Campylobacter jejuni from the blood of bacteremia patient in Guizhou Province ,China ,conventional and molecular techniques (specific mPCR and NAP-mPCR) were used to identify suspi-cious bacteria strains .Results showed that Campylobacter jejuni suspicious colonies were cultured in bacteremia patient blood samples .The strain was identified as Campylobacter jejuni ssp . jejuni by conventional tests and was identified as Campy-lobacter jejuni by genus specific mPCR .Then the strain was classified as Campylobacter jejuni ssp . jejuni by subspecies NAP-mPCR .The strain was identified as Campylobacter jejuni ssp .jejuni isolated from the blood of bacteremia patient and Campylobacter jejuni can be identified subspecies by NAP-mPCR .

Objective To construct short hairpin RNA (shRNA) recombinant expression vector for herpes simplex virus typeⅡ(HSV-2) UL29 gene and observe its inhibitory effect on HSV-2. Methods Four interference target sites of HSV-2UL29 gene were selected to construct 4 groups of small hairpin RNA respectively,named shRNA recombinant expression vector. The expression vectors were transfected into HEK293 cells with liposome. HEK293 cells were infected with HSV-2 after expression vector being transfected. The viral titer was estimated by end-point titration assay. The level of transcription was estimated by Real-Time PCR method. The expressing effect of protein was detected by Western-blot. Results Recombinant expression vector pGPU6/GFP/Neo-shRNA was constructed successfully. The result of end-point titration assay showed that the viral titer was reduced comparing with blank control (P<0.05). The result of RT-PCR showed that inhibition rates were respectively 28.80%, 59.95%, 66.08%and 36.27% comparing with blank control, and there were significant differences (P < 0.05). The effect of UL29shRNA1461 group was the best one. The result of Western-blot showed that the expressing quantity of ICP8 was reduced. Conclusion Recombinant expression vector pGPU6/GFP/Neo-shRNA can interfere HSV-2 UL29 gene expression from different cell level in vitro, which can inhibit the replication of HSV-2 genome in HEK293 cells. Thus, RNA interference (RNAi) is conducive to the further exploration of viral therapy.

Objective: To explore the effect of oxidative stress injury on the mechanism of vascular smooth muscle cell (VSMC) calciifcation induced by calcium and phosphorus in experimental rats.
Methods: The VSMC calcification was induced by incubating the cells with calcium chloride (CaCl2) andβ-sodium glycerophosphate (β-GP) for 8 days, and the cells were divided into 4 groups: ① Control group, ② Calcification group,③ Calciifcation+H2O2 group, ④ Calciifcation+catalase group. The calcium nodule formation and calcium deposition in VSMC were detected by Alizarin red staining and o-cresolphthalein complexone method, the reactive oxygen species (ROS) was detected by DCFH-DA probe staining and the protein expression of Runx2 was examined by Western blot analysis.
Results: Compared with Control group, Calciifcation group showed the higher ROS production, more calcium nodule and calcium deposition, higher Runx2 protein expression;while compared with Calciifcation group, the above indexes were even higher in Calciifcation+H2O2 group, P<0.05. The ROS production, calcium nodule, calcium deposition and Runx2 protein expression were lower in Calciifcation+catalase group than those in Calciifcation group and Calciifcation+H2O2 group, but still higher than that in Control group. The protein expression of Runx2 was similar between Calciifcation+catalase group and Control group, P>0.05.
Conclusion: CaCl2 andβ-GP treatment may induce VSMC calciifcation via activating ROS-Runx2 signal pathway in experimental rats.

Objective To investigate the underlying neural mechanism of left hemiparalexia and left hemialexia in reading Chinese characters. Methods A patient with reading disorders caused by brain infarctions at the left ventralis medialis occipitotemporal lobe and the splenium of the corpus callosum was studied. A series of neuropsychological tests, such as reading Chinese characters presented in the central foveal field or in the left and right half of the foveal field, were conducted with the patient, and neuroimaging techniques including high spatial resolution 3D-MRI and diffusion tensor tractography (DTT) were used to examine whether or not there were lesions of the neural pathway. Results The patient showed left hemiparalexia, which was characterized by making substitution or omission mistakes, mostly in the left parts of Chinese characters, and also left hemialexia(alexia for characters presented in left visual field). 3D-MRI demonstrated infarctions in the left ventral mesial occipitotemporal area and in the left side of the splenium of the corpus callosum. The left lateral mid-fusiform cortex, which has been identified as the visual word form area(VWFA), was almost intact. DTT indicated the major forceps fibers running through the splenium were all disconnected due to the infarction of the left splenium. Conclusion As a result of disruption of the splemium-major forceps pathway, visual character information in the left visual field which is initially projected to the right occipital cortex cannot be transferred from the right visual cortex to the left VWFA. This mechanism of left hemiparalexia and left hemialexia in reading Chinese characters is similar to that in reading English words.

Objective: To explore the effects of Vitamin K2 (VK2) on theaortic artery calciifcation and oxidative stress injury in experimental rats.
Methods: A total of 24 rats were divided into 4 groups:①Control group,②6-week calciifcation group,③12-week calciifcation group and④6-week calciifcation + 6-week VK2 group;n=6 in each group. The arterial calciifcation was induced by warfarin (WFN) treatment. The calcium nodule and deposition in rat’s theaortic artery were detected by Alizarin red staining and o-cresolphthalein complexone method, the reactive oxygen species (ROS) were measured by DHE probe staining and the morphological changes of mitochondria in smooth muscle cells were detected by transmission electron microscopy.
Results: Calciifcation nodule formed in both 6-week and 12-week calciifcation groups, the calciifcation deposition and ROS were higher than Control group,P<0.01. Compared with both calcification groups, the above indexes were decreased in 6-week calciifcation + 6-week VK2 group,P<0.01. Both calciifcation groups showed mitochondria swelling with unclear structure and cytoplasm vacuoles degeneration in vascular smooth muscle cells. The vascular smooth muscle cell volumes were similar between Control group and 6-week calcification + 6-week VK2 group, and no cytoplasm vacuoles degeneration was observed.
Conclusion: Warfarin induced aortic calciifcation is related to oxidative stress injury which may cause the ultra-micro structural damage in smooth muscle cells; VK2 may reduce the oxidative stress injury and improve the condition of vessel calciifcation in experimental rats.

Aim To investigate the method of establishing the atopic dermatitis mice model induced by calcipotriol ( MC903 ) . Methods Induction dose exploratory experiment: since d 0, 0.33,1,3 nmol MC903 was smeared on the right ear of BALB/c mice in each dose group respectively , for 7 consecutive days . The ear swelling degree of the mice was observed every day and the bilateral ears thicknesses were measured .The materials were drawn and analyzed in d 3 and d 7.Induction days exploratory experiment: 2 nmol MC903 was smeared on the right ear of BALB/c mice, for 14 consecutive days .The ear swelling degree of mice was observed every day and the bilateral ears thicknesses were measured .The histopathological examination of the right ear was conducted in 3 d, 7 d, 11 d and 15 d respectively .The tissue homogenate of the mice right ear was prepared .The ex-pressions of thymic stromal lymphopoietin (TSLP),IL-33, IL-4 and IFN-γin the homogenate , CD40 +,CD86 +,the DC surface markers and ILC2 contents in the peripheral lymph nodes were detected.Results ①1,3 nmol MC903 induced ear swelling in mice was significantly increased in d 3, the levels of TSLP and IL-4 were significantly increased .The level of IL-33 in 3 nmol dose group was increased significantly in d 7.② The right ear swelling of 2 nmol MC903 induced atopic dermatitis mice was significant , the ear thickness was increased gradually and reached the peak in d 14.The histopathological examination of the mice ear tissue showed in d 7, the right ear tissue of the mice was swelling and red , the capillary vessels were dilated and the infiltration of inflammatory cells was obvious .The ear in-flammatory symptoms maintained and gradually aggravated for 15 days.Compared with the normal mice , MC903 increased the TSLP level in the right ear tissue homogenate significantly in d 3 and then decreased gradually .The levels of IL-4 and IL-33 were increased significantly in d 7 and then decreased gradually .The levels of ILC2, CD40 +, CD86 + in the peripheral lymph nodes were increased in d 7 and d 15.Conclusion The atopic derma-titis mice model can be successfully established using 2 nmol MC903 induced mice for 7 days.Appropriate testing point of TSLP is d 3.Appropriate testing point of IL-33 and IL-4 is d 7.

Objective Todiscuss the diagnosis and therapy of the pancreatoblastoma(PB).Methods The data of 2 cases of PB were analyzed retrospectively and related literatures were reviewed.Results Both cases were males,11 years old and 8 years old respectively.The 2 cases both had solid mass located in the tail of the pancreas.Alpha-fetal protein(AFP) was normal in case 1 and 2 903 ng/ml in case 2.The 2 cases underwent resection of the pancreas tail,and the postoperative pathological examination confirmed the diagnosis of PB.Followup of 26 months in case 1 and 10 months in case 2 showed that the survival was good.Conclusions PB is an extremely rare tumor of exocrine pancreas and often occurs in male children.The solid mass located in the pancreas with elevated AFP can be considered as PB.Our experience showed a pancreatic mass with normal AFP can also be PB.Surgery is the best management of PB.

Objective To investigate the voicing acoustic characteristics of sulcus vocalis patients with mini-mum glottal area(MGA) .Methods 60 normal subjects (30 male ,30 female) ,and 62 subjects with sulcus vocalis (33 male and 29 female)were recruited .They all had stroboscopic laryngoscopy ,and had MGA measured when say-ing /i:/using software supporting KIPS .The acoustic parameters consisted of vocal cord fundamental frequency (F0) ,jitter and shimmer ,normalized noise energy ( NNE) ,harmony to noise ratio (HNR) and signal noise ratio (SNR) ,maximum phonation time(MPT) and respectively make correlation analysis with voice MGA .Results The voicing MGA of male patients with sulcus vocalis was 434 .74 ± 112 .83 ,larger than the males with normal vocalis 298 .25 ± 93 .63 .This was statistically significant (P<0 .05) .The voicing MGA of females with sulcus vocalis was (484 .75 ± 143 .91) ,significantly larger than those of females with normal vocalis (293 .43 ± 93 .73) and the differ-ence was statistically significant (P<0 .05) .The voicing MGA on both males and females with sulcus vocalis was noticeably relation to the F0 (r=0 .972 ,P<0 .05) and (r=0 .928 ,P<0 .05) ,Jitter (r=0 .978 ,P<0 .05) and (r=0 .910 ,P<0 .910) and Shimmer (r=0 .973 ,P<0 .05) and (r=0 .921 ,P<0 .05) ,normalized noise energy(r=0 .883 ,P<0 .05) and (r=0 .960 ,P<0 .05) ;and negative relation to the signal noise ratio (r= -0 .947 ,P<0 .05) and (r = -0 .957 ,P<0 .05) ,signal noise ratio (r= -0 .959 ,P<0 .05) and (r= -0 .944 ,P<0 .05) ,max-imum phonation time (r= -0 .891 ,P<0 .05) and (r= -0 .936 ,P<0 .05) .Conclusion Patients with pathological sulcus vocalis have glottal areas larger than normal .Acoustic analysis of the voices of the sulcus vocalis can be used as an objective laboratory examination .