Fanconi Syndrome ; complications ; diagnosis ; therapy ; Female ; Humans ; Infant, Newborn ; Potassium ; therapeutic use ; Prognosis ; Proteinuria ; etiology ; Treatment Outcome ; Vitamin D ; therapeutic use

Congenital Hyperinsulinism ; diagnosis ; Humans ; Infant, Newborn ; Male

Objective To explore the cause, clinical characteristic and the relation to the alterations of bone metabolism and bone mineral density (BMD) in senile male patients with chronic obstructive pulmonary disease (COPD). Methods Fifty senile male patients with simple stable COPD were divided into moderate and severe groups based on the diagnostic criteria of pulmonary function. Thirty senile male health volunteers were considered as control group. Blood gas analysis, BMD, bone mineral content (BMC), biochemical indexes relative to the bone formation and bone absorption in blood and urine were measured and analyzed. Results Reductions in BMD and BMC were more significant in two COPD groups than those in control group (P

To obtain single chain variable fragment (scFv) and bivalent single chain variable fragment (bsFv) against transferrin receptor, up-stream and down-stream primers were designed according to the complementary sequences of FR1 region of variable heavy (VH) and FR4 of variable light (VL), respectively, which contained inter-linker G4S and the restriction endonuclease SfiI, AscI and NotI. Two pieces of scFv fragments were first amplified through PCR and then inserted into plasmid pAB1, which could express scFv protein once induced by IPTG in the host bacteria. To express scFv and bsFv, E. coli TG1 was cultured in LB broth and was induced by IPTG. The restriction enzyme digestion map and DNA sequencing demonstrated that scFv and bsFv genes were successfully inserted into the expression plasmid. SDS-PAGE and Western blotting revealed the protein band at 35kD and 60kD, which were consistent with the molecular weight of scFv and bsFv respectively. Flow cytometry showed that scFv and bsFv harbored the specific binding activity with TfR expressed in various tumor cells, and the avidity of bsFv was higher than that of the parent scFv.
Base Sequence ; Cloning, Molecular ; Escherichia coli/genetics ; Escherichia coli/metabolism ; Genetic Vectors/genetics ; Hep G2 Cells ; K562 Cells ; Molecular Sequence Data ; Receptors, Transferrin/*immunology ; Recombinant Fusion Proteins/biosynthesis ; Recombinant Fusion Proteins/genetics ; Single-Chain Antibodies/*biosynthesis ; Single-Chain Antibodies/genetics

ObjectiveTo investigate the correlation between any two of Capsule Endoscopy ScroringIndex (Lewis score),simplified Crohn Disease Activity Index (CDAI) and C-reactive protein (CRP) in small bowel Crohn disease (CD).MethodsA total of 58 consecutive patients with known small bowel CD were enrolled. We evaluated disease activity with Lewis score and simplified CDAI. Correlations among CRP,simplified CDAI and Lewis score were calculated with Spearman's rank order correlation coefficient.The optimal CRP cut-off value was calculated using the ROC curve.ResultsThe Lewis score showed inactive,mild and moderate-severe patients were 13,21 and 24,respectively.CRP of moderate-severe group was significantly higher than that in mild and inactive groups ( P ＜ 0.05 ).The optimal CRP cut-off value that differentiated patients with moderate to severe disease from the others was 13.50 mg/L with sensitivity of 87.5％ and specificity of 82.4％.The area under the ROC curve to analyze the cut-off was 0.849.Lewis score was moderately correlated with CRP (r =0.58,P ＜ 0.01 ),and weakly correlated with the simplified CDAI (r =0.40,P ＜ 0.01 ).ConclusionSerum CRP and the simplified CDAI cannot replace Lewis score for capsule endoscopy in the assessment of disease activity in small bowel CD.However,CRP may be considered as an inflammatory marker for evaluating the moderate to severe capsule endoscopic activity.

Objective To investigate the epidemiological and clinical characteristics and prognosis of 31 children with hand foot and mouth disease(HFMD) complicated with acute flaccid paralysis(AFP).Methods Thirty-one HFMD children complicated with AFP served as study objective,who were admitted to Tangshan Maternity and Children Health Hospital from Jun 2011 to Dec 2014.The clinical manifestations and follow-up outcomes of 31 children with HFMD followed by AFP were retrospectively reviewed.Results Participants in this study consisted of 31 children(16 males,15 females,age from 3 months to 9 years) who met the criteria for HFMD with AFP.Among the 31 cases,27(87.1％)cases were less than 3 years old and 29(93.5％) cases were from countryside.Eleven cases (35.5％)were infected by enterovirus 71,1 case (3.2％) was infected with coxsackieviruses A16 and 19 cases(61.3％) were infected by other enteroviruses.AFP developed(7.1 ±2.9) days after the onset of fever and progressed to maximum severity within 1-2 days.All of the cases presented with fever and skin rash,38.7％ cases presented with limbs tremor and 87.1％ cases presented with startle and skip.Besides AFP of limbs, 100％ cases complicated with encephalitis.Thirty-one cases showed poliomyelitis-like syndrome(20 cases with one flaccid limb,5 cases with lower limbs,4 cases with upper limbs and 2 cases with left hemiplegia).In these cases, the muscle power varied from level 0 to level 4.The muscle strengthen and muscle tone in 21 patients were recovered within 2 weeks,and the other cases showed recovery 2 to 3 weeks later.After four weeks, the muscle strengthen in 19 cases recovered to 5 level(10 cases with single lower limb,5 cases with single upper limb,3 cases with two lower limbs and 1 case with two upper limbs).Conclusion HFMD complicated with AFP most commonly occurs in children aged less than 3 years old.The majority of cases were from countryside.Enterovirus 71 is still relatively common pathogen.All the 31 cases complicated with encephalitis.Cases clinically presented one flaccid limb, lower limbs, upper limbs and hemiplegia.AFP may be to some degree reversible in HFMD cases.In most cases described here, paralysis occurred in a single lower extremity and recovered more rapidly than those with two fimbs affected or with single upper extremity impairment.

The effects of Sonic hedgehog (Shh) signaling pathway activation on S-type neuroblastoma (NB) cell lines and its role in NB tumorigenesis were investigated. Immunohistochemistry was used to detect the expression of Shh pathway components-Patched1 (PTCH1) and Gli1 in 40 human primary NB samples. Western blotting and RT-PCR were used to examine the protein expression and mRNA levels of PTCH1 and Gli1 in three kinds of S-type NB cell lines (SK-N-AS, SK-N-SH and SHEP1), respectively. Exogenous Shh was administrated to activate Shh signaling pathway while cyclopamine was used as a selective antagonist of Shh pathway. S-type NB cell lines were treated with different concentrations of Shh or/and cyclopamine for different durations. Cell viability was measured by using MTT method. Apoptosis rate and cell cycle were assayed by flow cytometry. The xenograft experiments were used to evaluate the role of Shh pathway in tumor growth in immunodeficient mice. High-level expression of PTCH1 and Gli1 was detected in both NB samples and S-type NB cell lines. Cyclopamine decreased the survival rate of the three cell lines while Shh increased it, and the inhibition effects of cyclopamine could be partially reversed by shh pre-treatment. Cyclopamine induced the cell apoptosis and the cell cycle arrest in G(0)/G(1) phase, while Shh induced the reverse effects and could partially prevent effects of cyclopamine. Cyclopamine could also inhibit the growth of NB in vivo. Our studies revealed that activation of the Shh pathway is important for survival and proliferation of S-type NB cells in vivo and in vitro through affecting cell apoptosis and cell cycle, suggesting a new therapeutic approach to NB.

Objective To explore the application of "Work-Study Combination"(referred to "Institutions Join Forces with Hospitals" in this paper) in nursing education system of Shanghai Institute of Health Sciences. Methods Data of teaching plan and curriculum of students from 2005 and 2006 grades were compared.With construction of "National Model of Higher Vocational Institutions",transformation in professional curriculum reforming,teaching resources sharing,teachers staff constructing,in-school and out-school vocational training bases establishing,and students evaluation system were compared.Results Regulated and updated teaching contents and new curriculum reflected vocational education characteristics.The ratios of in-school vocational training,clinical teaching and practice in curriculum rose rapidly.Conclusion The results showed that "Institutions Join Forces with Hospitals" applying in higher nursing vocational education system was a direct expression of combination of work and study to the process of personnel cultivating.

The aimed of this study was to prepare stabilized thiomers to overcome the poor stability character of traditional thiomers. Poly(acrylic acid)-cysteine (PAA-Cys) was synthesized by conjugating cysteine with poly(acrylic acid) and poly(acrylic acid)-cysteine-6-mercaptonicotinic acid (PAA-Cys-6MNA, stabilized thiomers) was synthesized by grafting a protecting group 6-mercaptonicotinic acid (6MNA) with PAA-Cys. The free thiol of PAA-Cys was determined by Ellmann's reagent method and the ratio of 6MNA coupled was determined by glutathione reduction method. The study of permeation enhancement and stabilized function was conducted by using Franz diffusion cell method, with fluorescein isothiocyanate dextran (FD4) used as model drug. The influence of polymers on tight junctions of Caco-2 cell monolayer was detected with laser scanning confocal fluorescence microscope. The results indicated that both PAA-Cys and PAA-Cys-6MNA could promote the permeation of FD4 across excised rat intestine, and the permeation function of PAA-Cys-6MNA was not influence by the pH of the storage environment and the oxidation of air after the protecting group 6MNA was grafted. The distribution of tight junction protein of Caco-2 cell monolayer F-actin was influenced after incubation with PAA-Cys and PAA-Cys-6MNA. In conclusion, stabilized thiomers (PAA-Cys-6MNA) maintained the permeation function compared with the traditional thiomers (PAA-Cys) and its stability was improved. The mechanism of the permeation enhancement function of the polymers might be related to their influence on tight junction relating proteins of cells.
Acrylic Resins ; chemistry ; Actins ; metabolism ; Animals ; Caco-2 Cells ; Cysteine ; chemistry ; Dextrans ; Fluorescein-5-isothiocyanate ; analogs & derivatives ; Glutathione ; Humans ; Intestinal Absorption ; Intestinal Mucosa ; drug effects ; Nicotinic Acids ; chemistry ; Rats ; Sulfhydryl Compounds ; chemistry

Objective To investigate clinical features of lower motor neuron lesion (LMNL) caused by the single level lower thoracic disc protrusion (LTDP),and to observe clinical outcomes of surgical treatment.Methods Between January 1997 and December 2009,17 patients with LMNL caused by single level LTDP underwent en bloc resection of the superior articular process,Cave-in 360° circumferential decompression and internal fixation in our hospital.MRI and CT scans were taken to confirm lesion levels:T10-11 in 4 patients of whom 3 had patellar clonus and ankle clonus,T11-12 in 5 patients of whom 4 had ankle clonus,and T12L1 in 8 patients who only had positive Babinski sign.The neurologic status was assessed using the Japanese Orthopaedic Association (JOA) scoring system.The muscle strength of the tibialis anterior was assessed using the Manual Muscle Test (MMT).Sagittal Cobb angle and cross-sectional area of the dural sac at the level of maximal compression in MRI were also observed.Results All patients were followed up for 22 to 76 months (average,48.6 months).The mean JOA score increased from preoperative 5.88±1.11 to 9.53±0.94 at final follow-up (t=16.143,P＜0.05).The muscle strength of the tibialis anterior recovered to more than grade 4 in all patients.Postoperative Cobb angle was unchanged compared with that before operation.MRI indicated that the cross-sectional area of the dural sac at the level of maximum compression increased from preoperative 35.8±7.3 mm2 to postoperative 132.9±6.5 mm2 (t=70.78,P＜0.05).Conclusion LMNL can be caused by LTDP.The eu bloc resection of the superior articular process,Cave-in 360° circumferential decompression and internal fixation can provide a satisfactory decompression effect and marked recovery of neurological function.