ObjectiveTo study the clinical characteristics of the temperature instability in Prader-Willi syndrome (PWS). MethodsThe clinical characteristics of one case of PWS with recurrent fever were retrospectively analyzed.ResultsA 5-month-old boy who had such clinical characteristics as low central muscle tension, sucking weakness, feeding dififculties, cryptorchidism and white skin was diagnosed as PWS by chromosomal microarray analysis and methylation-speciifc PCR. In the course of the disease, the infant presented recurrent fever. The pathogenic bacteria culture and virus antibody detection showed negative, and the treatment of reducing fever, anti-infection and immune support were ineffective.ConclusionsThe cause of the recurrent fever is unknown and central fever is suspected. Whether the temperature instability is the clinical feature of PWS still needs a lot of clinical researches.

We carried out parallel design and development of two differently structured auto sampler based on a multi-axis and multi-mode high-precision closed-loop servo control system. An integrated embedded control drive module was developed based on the idea of compatibility and inter-changeability, so that DC motor and encoder were standardized into uniform models. Meanwhile, electric and mechanical interfaces were uniformed to a same standard. This allows the direct exchange of above-mentioned components between the two models. A 1-μL manual sample injection syringe was installed on both standard 110-sample and platform 40-sample liquid auto sample injectors connected with gas chromatographer. Approximately 0. 5μL of cetane-isooctyl was sampled for 6 consecutive times at six different positions in the sample bottle. The repeatability RSDs of the injection peak areas of the two systems were 1. 1% and 1. 5%, respectively. A linear correlation coefficient (0. 9947) of peak area with injection volume was achieved based on the gradient sampling volume of 0. 1, 0. 3, 0. 5, 0. 7 and 0. 9 μL.

Objective To summarize the experience in diagnosis and treatment of popliteal vein entrapment syndrome (PVES). Methods A total of 57 patients with PVES were selected from the Department of Vascular Surgery in Xinxiang Central Hospital from March 2009 to October 2015. Of which 43 patients were severe stenosis of popliteal vein (stenosis degree>90%), and another 14 cases were with stenosis less than 90%. All the patients underwent ascending venography of low limb to confirm the clinical classification after admission. Forty-three cases with severe stenosis of the popliteal vein were treated with releasing popliteal vein of entrapment and stripping varicose veins. The static pressure and dynamic pressure of popliteal vein and foot dorsal vein were measured before and after operation. Another 14 patients were treated with medical circulation driven sock and medical therapy. Results The degrees of popliteal vein stenosis were more than 75% in all patients. The patients were divided into bove-knee stenosis (n=9), knee stenosis (n=18), and below-knee stenosis (n=30) according to the different parts of stenosis. Forty-three patients treated with surgery showed relief of leg swelling and pain, and ulcer healing. And the imaging examination showed that there were no obvious compression and stenosis of popliteal vein, and vascular filling was well. The static pressure and dynamic pressure of the popliteal vein and dorsal vein were lower than those before surgery (P<0.05). The lower limb swelling and pain were relieved, and varicose veins of lower limbs were no longer continued to increase in 14 patients with conservative treatment. Conclusion PVES is easy to be misdiagnosed, which should be paid attention to, and satisfactory clinical results can be achieved by releasing popliteal vein of compression combined with stripping varicose veins in patients with serious symptoms .

Tanshinones and phenolic acids are two major classes of effective compounds in S.miltiorrhizae Radix et Rhizoma.Accumulative levels of these compounds directly determined its quality and efficacy.Presently,for the purpose of understanding the underlying the mechanism behind the biosynthesis and regulation of effective compounds in S.miltiorrhiza,a series of progresses have been pressed ahead.Benefiting from the boost and convenience of high-throughput genomic,transcriptomic,and metabolic analysis methods,remarkable achievements have been accessed in this field.In this paper,a summary for the recent progress have been made with the provision of a reference for better understanding over the quality of S.miltiorrhiza.

Objective To investigate the application value of multi-slice CT enterography (MSCTE)in diagnosing intestinal tuberculosis (ITB)and Crohn’s disease (CD).Methods MSCTE findings were retrospectively analyzed in 25 patients with ITB and in other 25 patients with CD diagnosed through endoscopy,pathologic examination and clinical follow-up.Statistical analysis was performed to find out the difference in CT findings between the ITB and CD.Results 25 patients with CD included the involved ileum in 23,involved duode-num and jejunum in 8,multiple segmental lesions in 20,asymmetrically thickened intestinal wall in 20,hierarchical reinforcement in 1 9,mesenteric vascular hyperplasia in 20,fibrofatty hyperplasia in 18,peritoneal abscess or fistula in 8,anal fistula in 1,and pseu-do-diverticulum formation in the intestinal wall on the opposite side of the mesentery in 2.Meanwhile,the other 25 patients with ITB included the involved terminal ileum in 25,symmetrically thickened intestinal wall in 23,annular enhancement of lymph nodes in 11,thickened peritoneum and omentum together with distinct enhancement,or intestinal adhesion,or ascites appeared in 15.The CD was more likely to represent multiple segmental lesions,asymmetrically thickened intestinal wall,hierarchical reinforcement,mesenteric vascular hyperplasia,fibrofatty hyperplasia,and peritoneal abscess or fistula formation (P <0.05).Meanwhile,the ITB was more likely to represent the lesion only in ileum,symmetrically thickened intestinal wall,and annular enhancement of lymph nodes (P <0.05).Conclusion MSCTE shows promising clinical application in diagnosis and differential diagnosis of CD and ITB.

Objective To investigate the relationship between cysteine-rich protein 61 ( Cyr61 ) and phosphatidylinositol 3-kinase ( PI3K ) signal pathway on cell proliferation and apoptotic in human ovarian carcinoma cells.Methods Recombinant human Cyr61 (rhCyr61) was pretreated with ovarian carcinoma cells.The expression of Cyr61 protein was detected by confocal spectral microscopy.Then treated the ovarian carcinoma cells with PI3K transduction inhibitors (LY294002) for 24 hours.Cell apoptosis was detected by flow cytometry (FCM).Cell viability was determined by methyl thiazolyl tetrazolium (MTT) method.The mRNA expressions of Cyr61,the protein levels of protein kinase B ( PKB),phospho-PKB and Cyr61 were assaved by real time-PCR and western blot analysis,respectively.Results The Cyr61 and phospho-PKB protein expression in two ovarian carcinoma cells (OV2008 and OVCAR-3 ) were increased in rhCyr61pretreated group.The decreasing of cell apoptosis [ ( 1.4 ±0.9)％,(2.1 ± 1.0)％ ] and increasing of cell proliferation [ ( 124.0 ± 1.8)％,( 133.0 ±2.2)％ ] was detected in the same time,compared with negative control group,there were significant difference ( P ＜ 0.05 ).After exposed to LY294002 for 24 hours,the apoptosis rate of OV2008 and OVCAR-3 in pretreated with rhCyr61 group exposed to LY294002 were (21.1 ± 1.6)％ and (26.4 ± 1.5 )％,respectively.Cells viability [ (59.0 ± 2.3 )％,(51.0 ± 2.0)％ ]was also significantly decreased in OV2008 and OVCAR-3 pretreated with rhCyr61 cells.Meanwhile,the mRNA expressions of Cyr61 (3.2 ± 0.8,6.2 ± 1.1 ) and the protein levels of phospho-PKB and Cyr61 were greatly decreased.Compared with negative control group,there were significant difference in OV2008 and OVCAR-3 cells (all P ＜ 0.0l ).Conclusions The activation of PI3K intracellular signaling pathways may lead to up-regulation of Cyr61 expression.Block PI3K signal pathway could significantly inhibit the expression of Cyr61,and may promote the apoptotic effects and inhibit the cell growth of ovarian carcinoma cells.

Objective To investigate the clinical characteristics and mutations of guanosine triphosphate eyclohydrolase (GCH) Ⅰ gene in patients with dopa-responsive dystonia (DRD). Methods Five families with 18 affected family members and 17 patients with sporadic DRD were examined. Patients were allocated into 3 groups according to onset time, either in childhood, or in adolescence or adult. Interview, physical examination, psychologic testings and CT/MR scan were performed. Mutation screening was performed on 26 patients and 1 normal family nember. Thirty-five healthy control subjects were matched for age and sex. Statistical analysis were conducted with the use of SPSS 13.0 computer software. Results(1)Most of patients started with dystonia. The main clinical manifestation was dystonia too. There was no difference among 3 groups.(2) There were significant differences in diurnal fluctuation among 3 groups(15/15,6/6,7/14, χ2=13.125,P=0.001). Diurnal fluctuation negatively correlated with age (r=-0.720, P<0.01).(3)The differences in postural tremor were also found among 3 groups (7/15,5/6,1/14, χ2=8.073, P=0.018). Postural tremor positively correlated with age (r=0.399, P=0.018).(4)There were differences in exaggeration of tendon among three groups(11/15,1/6,4/14, χ2=8.309, P=0.016). Exaggeration of tendon reflexes negatively correlated with age (r=-0.429, P=0.010).(5)The scores of Hamilton Depression Scale and Hamilton Anxiety Scale in patients were higher than those in controls.(6)DNA sequencing revealed a heterozygous A224G missense mutation(Tyr75Cys)located within exon 1 in one autosomal dominant inheritance family. Conclusions The manifestations of DRD varies. The clinical course is closely correlated with age. A missense mutation(A224G)in coding region of the GCH 1 gene probablyleads to the occurrence of DRD.

BACKGROUND:Retinal diseases are caused by damage or apoptosis of the retinal cel s and lack of ideal treatment. Induced pluripotent stem cel s have self-renewal ability and differentiation potential. A new potential source from which to produce stem cel s as a therapeutic platform for the treatment of retinal diseases and avoid ethics issues is the research focus currently.
OBJECTIVE:To review recent advancement of induced pluripotent stem cel s and the potential utility for retinal diseases.
METHODS:The first author searched related articles published from 2006 to 2011 by computer retrieval in PubMed database using the keywords of“induced pluripotent stem cel s, retinal, photoreceptor, retinal pigment epithelium, retinal ganglion cel”in English. Final y, total y 42 papers were involved.
RESULTS AND CONCLUSION:Induced pluripotent stem cel s can be induced to differentiate into retinal cel s which have a possibility to be used as donor cel s for transplantation therapies. Patient-derived induced pluripotent stem cel s can be used as disease models for mechanistic studies and drug screening. It is a promising therapy for retinal degenerative diseases. However, some problems stil remain, such as risk of teratoma formation and low efficiency of somatic cel s to be successful y induced into induced pluripotent stem cel s. It stil requires further more studies.

To observe the clinical outcome of implanting AcrySof lQ Toric intraocular lens to correct corneal astigmatism in cataract surgery, and to evaluate the result and rotational stability of AcrySof lQ Toric after cataract surgery.
●METHODS: A retrospective study of 26 eyes in 21 cataract patients with corneal astigmatism. All patients implanted AcrySof lQ Toric intraocular lens. The preoperative and postoperative uncorrected visual acuity ( UCVA ), best corrected visual acuity ( BCVA ), preoperative corneal astigmatism, anticipated residual astigmatism, total astigmatism, postoperative residual astigmatism and Toric lens axis were detected and measured.
●RESULTS: All patients' visual acuity and best corrected visual acuity improved significantly. The mean refractive cylinder decreased significantly after surgery from (2. 05± 0. 57)D to (0. 55±0. 33)D (t = 13. 574, P<0. 05). There was no significant difference between preoperative ( 0. 47 ± 0. 19)D and postoperative corneal astigmatism (t = 1. 149, P > 0. 05 ). Three months after surgery, there was no significant difference between preoperative (2. 01±0. 58)D and postoperative (-1. 89±0. 53) D corneal astigmatism (t =1. 908, P> 0. 05). The rotation of intraocular lens were <20°, the mean rotation was 3. 65°±2. 86°.
●CONCLUSlON: The AcrySof lQ Toric lens make cataract patients enjoy the better UCVA including good rotational stability in the correct of corneal astigmatism. The AcrySof lQ Toric implantation is an effective option for the correct of preexisting corneal astigmatism in cataract surgery.

AIM: To evaluate the efficacy of vitrectomy and pan-retinal photocoagulation followed with Ahmed implantation in management of neovascular glaucoma ( NVG) retrospectively.
METHODS: Vitrectomy combined with pan retinal photocoagulation and Ahmed implantation was performed on 15 cases ( 15 eyes ) with NVG. All patients were followed up for 12 ~ 36mo. The change of intraocular pressure (IOP), visual acuity, neovascularization of iris, complications were observed.
RESULTS: The visual acuity of 10 eyes was improved postoperative. IOP of postoperative 1, 6 and 12mo were decreased significantly compared with preoperative ( P<0.01 ) . Neovascularization of iris was reduced dramatically. No serious complications occurred.
CONCLUSION: Vitrectomy combined with pan retinal photocoagulation followed Ahmed implantation can manage NVG effectively.