Objective To explore the diagnosis and curative effect of phenylketonuria(PKU) in Gansu.Methods Fifty-nine thousand nine hundred infants and 118 borderline cases were screened from Oct.1999 to Dec.2006.Three drops of venous blood was collected from the exterior or interior part in the heel,and trickled the venous blood onto the specialized filter paper,which could be formed into 3 bloodstains with the diameter of 0.8-1.0 cm.Phenylalanine(phe) was detected by chemiluminescence method,and PKU was screened by the PKU standard of diagnosis.Infants with PKU had been given low phe food.During the treatment,phe level,physical development and intellectual development were measured regularly.Patients′ body weight,height and head circumference were measured once 1 month.Patients′ intellectual quotient(IQ) were measured every half year(IQ was analyzed by Gesell test).Results were analyzed by SPSS 11.0 software for t test.Results In total,93 patients with PKU were diagnosed and treated.Thirty-six cases were identified by neonatal screening before 3 mouths old.Mental retardation was found in 57 cases before 6 years old.After treatment with low-phe diet,the follow-up for early-treated patients revealed that their physical and mental development were normal,and height,weight and head circumference were in the normal amplitude,and the results of IQ was(92?12)scores.In later treated patients,abnormal behaviors were significantly improved and their developmental quotients were elevated.Before treatment,Gesell value was(57?20)scores;after 12-18 months treatment Gesell value was(70?20)scores.The difference was significant(t=1.705 P

Aim To investigate the combined effect of mangiferin and ephedrine as well as explore its underlying mechanism in mouse model of allergic asthma.Methods The mouse model of asthma was sensitized and challenged with ovalbumin(OVA).The behaviors of mice during the challenging period were observed.Continuous autonomic activities of mice after 1 h of drug treatment were monitored with the autonomic activity recorder for 14 days.The total and differential cells in peripheral blood were counted.Histological study of lung sections on airway inflammation was carried out with haematoxylin and eosin(HE) staining.The levels of ovalbumin-specific immunoglobulin E(OVA-sIgE) and(Cyclic Adenosine monophosphate) cAMP in serum were detected by enzyme-linked immunosorbent assay(ELISA).The mRNA expression of cytokines mainly produced by Th1/Th2 lymphocytes such as IFN-γ and IL-4, IL-5 were semi-quantitatively analyzed with reverse transcription polymerase chain reaction(RT-PCR).Results Combination treatment and mangiferin could reduce the aberrantly autonomic activity caused by ephedrine in mice.Compared with the model group, asthmatic symptoms in all treatment groups could be significantly relieved, the pathological changes of lung tissue were improved and airway inflammation was reduced.The effect of combined treatment was better than that of mangiferin treatment alone.Compared with the model group, the total number of white blood cells(WBC) and the eosinophils(EOS) ratio in peripheral blood in all treatment groups decreased, the level of OVA-sIgE declined and the level of cAMP increased in serum.Combination treatment could lower mRNA expressions of IL-4 and IL-5 from Th2 lymphocytes and increase the mRNA expressions of IFN-γ released by Th1 lymphocytes.Conclusions The effect of combined therapy of mangiferin and ephedrine is better than that of mangiferin treated alone, meanwhile it can reduce the side effects caused by ephedrine.The underlying mechanism is mainly associated with attenuating Th1/Th2 cytokine imbalance and increasing the level of cAMP.

AIM: To evaluate the clinical outcomes of management in younger patients with primary chronic angle-closure glaucoma (PCACG).METHODS: Thirty-eight patients (50 eyes) aged 40 or younger with confirmed diagnosis of PCACG in advanced or late stage who received surgical treatment in Zhongshan Ophthalmic Center from January 2000 to December 2005were retrospectively investigated. All patients underwent trabeculectomy. The mean follow-up was 23.6±7.5 months.Full ophthalmic examinations were performed. The clinical outcomes including clinical presentations, surgical results and complications were evaluated.RESULTS: The mean age of patients was 33.5±6.1 years old. There was a female preponderance (60.5%). The mean axial length was 22.4±3.5mm with 18.0% short axis of eyeball and 14% nanophthalmos. There was 60.0% fiat anterior chamber depth (＜1.9mm). Ultrasonic Biomicroscopy identified that plateau iris was the most common underlying etiology (80.6%). There was a statistically significant difference in intraocular pressure (IOP) reduction postoperativelyvs preoperatively (P＜0.001). Four eyes failed to control IOP and received second filtration surgery. The main postoperative complications included shallow anterior chamber (20.0%) and malignant glaucoma (12.0%).CONCLUSION: The younger PCACG patients in advanced or late stage can be effectively managed by trabeculectomy.They have more frequency of postoperative sustained shallow anterior chamber and malignant glaucoma. Careful ophthalmic examinations, delicate surgical procedures and well-managed technique of complications were suggested on younger PCACG patients.

Objective To investigate the effect of neutral amino acid on preventing Parkinson disease.Methods Mice were injected with L-Valine,L-Pheylalanine,D-Valine or L-Lysine before or after paraquat administration,by which prakinsonian mouse model was constructed.The paraquat immunoreactivity was observed within nigral cell bodies.Then neurodegeneration and ?-synuclein aggregation were observed by immunohistochemistry and Western blot.Results Paraquat immunoreactivity was abolished by the administration of L-Valine,L-Pheylalanine before paraquat exposure.Pre-treatment with these two amino acids also protected the paraquat-induced loss of nigrostriatal dopaminergic cells and formation of thioflavine S-positive aggregates.In contrast, paraquat-induced toxicity was unaffected if animals were injected with these two amino acids after paraquat exposure or pre-treated with D-Valine or L-Lysine.Conclusions L-type neutral amino acids such as L Valine and L-Pheylalanine can prevent paraquat-induced neurodegeneration and a synuclein pathology through a competitive inhibition mechanism with stereospecificity in the central nervous system (CNS).Neutral amino acid could protect the dopaminergic neuron in substantia nigra and may prevent Parkinson disease.

An HPLC method for the determination of geniposide concentration in mouse plasma was developed and the pharmacokinetics after intranasal administration of Xingnaojing microemulsion (XNJ-M) and mPEG2000-PLA modified Xingnaojing microemulsion (XNJ-MM) were investigated. Eighty mice were treated by XNJ-M and XNJ-MM nasally. The plasma samples were collected at different times and the drug in samples was detected by HPLC. The pharmacokinetic parameters were calculated by the software of Kinetica. The pharmacokinetic parameters of geniposide of XNJ-M were C(max) (4.36 +/- 2.69) mg x L(-1), t(max) 1 min, MRT (29.73 +/- 4.54) min, AUC (53.63 +/- 14.03) mg x L(-1) x min. The pharmacokinetic parameters of geniposide of XNJ-MM were C(max) (9.75 +/- 4.14) mg x L(-1), t(max) 1 min, MRT(22.34 +/- 2.90) min, AUC (131.87 +/- 40.13) mg x L(-1) x min. Geniposide can be absorbed into blood in a higher degree after intranasal administration with XNJ-MM compared to XNJ-M, which maybe caused by its less irritating and more absorption.
Animals ; Drugs, Chinese Herbal ; administration & dosage ; chemistry ; Emulsions ; Iridoids ; blood ; pharmacokinetics ; Lactic Acid ; chemistry ; Male ; Mice ; Polyesters ; Polyethylene Glycols ; chemistry ; Polymers ; chemistry

OBJECTIVETo observe the effect of Bushen Wenyang Huayu Recipe (BWHR) on hypoxia inducible factor-1α (HIF-1α), proline hydroxylase2 (PHD2), von Hippel Lindau disease (VHL) suppressor gene expressions in endometriosis (EM) rats with Shen yang deficiency blood stasis syndrome (SYDBSS), and to explore the pathogenesis of EM and the mechanism of BWHR for treating EM.

METHODSTotally 50 SD rats were randomly divided into five groups, i.e., the blank control group, the sham-operation group, the model group, the Chinese medicine (CM) group, and the Western medicine (WM) group, 10 in each group. Rats in the blank control group and the sham-operation group were fed routinely. Rats in the rest 3 groups received 30-day "extended refrigerator freezing and ice water immersion" and combined with " autotransplantation" to establish EM rat model with SYDBSS. One Milliliter BWHR at 3.33 g/mL was administered to rats in the CM group by gastrogavage. Gestrinone at the daily dose of 0. 5 mg/kg was administered to rats in the WM group by gastrogavage. Equal volume of normal saline was administered to rats in the model group, the blank control group, and the sham-operation group. The size and morphology of ectopic foci in rats were observed after 4 weeks of medication. Expressions of serum CA125, plasma cyclic adenosine monophosphate (cAMP), and plasma cyclic guanosine monophosphate (cGMP) were detected by radioimmunoassay. Morphological changes of eutopic endometrium and ectopic tissue were observed under the optical microscope by HE staining. Protein expressions and contents of HIF-lα, PHD2, and VHL were detected by immunohistochemical SABC method and Western blot. mRNA expressions of HIF-1α, PHD2, and VHL were detected by RT-PCR.

RESULTSThe ectopic foci grew significantly in the model group. Their volumes were obviously contracted after treated by CM and WM. Compared with the blank control group and the sham-operation group, serum CA125 and plasma cGMP obviously increased, cAMP obviously decreased (P < 0.05); expressions and contents of HIF-1α mRNA and protein all decreased (P < 0.05); mRNA and protein expressions and contents of PHD2 and VHL all decreased in the model group (P < 0.05). Compared with model group, levels of CA125 and cGMP obviously decreased; cAMP levels obviously increased, expressions and contents of HIF-1α mRNA and protein all increased, mRNA and protein expressions and contents of PHD2 and VHL all increased in the WM group and the CM group (P < 0.05). Compared with the CM group, PHD2 protein contents were higher in the WM group (P < 0.05). HIF-1α was negatively correlated with PHD2 (r = -0.799, P = 0.00). HIF-1α was negatively correlated with VHL (r = -0. 625, P = 0.003).

CONCLUSIONSBWHR could effectively treat EM. Its mechanism might be associated with reducing contents of HIF-1α, serum CA125, and plasma cGMP, and up-regulating expressions of PHD2, VHL, and cAMP.

Animals ; Cyclic AMP ; Drugs, Chinese Herbal ; therapeutic use ; Endometriosis ; drug therapy ; metabolism ; Female ; Hypoxia-Inducible Factor 1, alpha Subunit ; metabolism ; Proline ; metabolism ; RNA, Messenger ; Rats ; Rats, Sprague-Dawley ; Up-Regulation ; Yang Deficiency ; drug therapy ; metabolism

Objective To characterize the antibiotic resistance,homology and carbapenemase genotypes of imipenem resistant Acinetobac1ter baumannii isolated from our hospital,and analyze the clonal relatedness of the test strains.Methods Ninety five strains of imipenem resistant A.baumannii were isolated from August 2003 to December 2004 in the First Affiliated Hospital, College of Medicine,Zhejiang University.The MICs of 16 antimicrobial agents against these strains were determined by agar dilution and E-test method.The homology of these isolates was analyzed by pulse-field gel electrophoresis(PFGE).The coding gene of carbapenemases was amplified.PCR products were purified,cloned and sequenced.Plasmid DNA was extracted and purified.Conjugation and Southern blot were performed to locate the position of oxa 23 gene.Results The resistance rates to ampicillin-sulbactam and cefoperazone sulhactam were 67.9% and 30.2%.Polymyxin E had the lowest resistance rate of 17%. The resistance rate to other antimicrobial agents was higher than 90%.The 95 strains,isolated from 10 clinical units,were classified into 6 clones.Clones A and B were predominant clones.All strains produced carbapenemases which were confirmed as OXA 23 by PCR and sequencing analysis.No plasmid was extracted and conjugation was not successful.Southern bolt showed that oxa-23 gene was located on Apal-digested chromosomal segments about 220 kb and 200 kb in Clones A and B,re spectively.Conclusions OXA 23-producing A.baumannii has become one of the most important multi-resistant pathogens in our hospital.Clones A and B have widely spread in our hospital.Oxa-23 gene is located on chromosomal DNA.

Objective To investigate the resistant mechanism of imipenem-resistant K. pneumoniae.Methods The minimal inhibitive concentrations (MICs) of the antimicrobial agents were determined by Etest.Isoelectric focusing electrophoresis (IEF),plasmid extraction,conjugation, transformation,PCR amplification,cloning and sequencing were carried out for analyzing the encoding gene of ?-1actamases.Results Three kinds of ?-1actamases were detected with pIs of 7.2,6.7,and 5.4.in a clinical strain of K.pneumoniae.These ?-1actamases were TEM-I (pI,5.4),SHV-12 (pI,8.2) and KPC-2 ( pI,6.7 ) confirmed by sequencing of the PCR products.Only one band of ?-1actamase with pI 6.7 was displayed in the transformant.A 1500 bp segment,which contained the KPC-2 gene confirmed by nucleotide sequence analysis,was cloned from a 60 000 bp plasmid of the transformant.Conclusion The strain of K.pneumoniae resistant to imipenem produces a plasmid-mediated carbapenemase KPC-2 which belongs to Bush group 2f,class A ?-1actamase.

OBJECTIVETo observe the efficacy of treatment of acute leukemia by Shengfu Injection (SFI) in combination with chemotherapy and the effect of treatment on T-lymphocyte subsets (CD4, CD8, CD4/CD8), serum interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-alpha).

METHODSSixty-one patients with acute leukemia of initiatory treating were randomly divided into two groups, the 31 patients in the treated group were treated with SFI plus chemotherapy and the 30 patients in the control group treated with chemotherapy alone. The remission rate, changes of absolute number of peripheral mature neutrophils, T-lymphocyte subsets, serum levels of IL-6 and TNF-alpha before and after treatment were observed.

RESULTSThe remission rate was higher in the treated group than that in the control group but the difference was insignificant (P > 0.05). The restoring of peripheral mature neutrophils in the treated group was higher than that in the control group, from the 3rd week of treatment, the difference was significant (all P < 0.05). CD4 and CD4/CD8 ratio after treatment increased in both groups, the increment was more obvious in the treated group than that in the control group significantly (P < 0.05). Serum levels of IL-6 and TNF-alpha lowered in both groups after treatment significantly(all P < 0.01), but the decrement was greater in the treated group with significant difference to the control group (P < 0.05).

CONCLUSIONSFI could improve and regulate the immune function in acute leukemia patients undergoing chemotherapy and enhance the therapeutic effect.

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Interleukin-6 ; blood ; Leukemia, Myeloid, Acute ; drug therapy ; immunology ; Male ; Middle Aged ; Phytotherapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; immunology ; Tumor Necrosis Factor-alpha ; metabolism

Objective To understand the situation of congenital defects' in five counties/cities in Gansu province so as to provide scientific evidence for the development of effective interventions.Methods General imformaton was collected on all the neonates who were born in Dunhuang city,Jingchuan county,Hui county,Weiyuan county and Yongjing county in Gansu province between Oct.1st,2009 to Sep.304th,2010,with all of their gestational age above 28 weeks.Neonates would include live birth,dead fetus and still birth.Results The overall incidence of congenital defects was 7.49‰ in the five counties/cities in Gansu province in 2009.Ranking order in the top three showed as congenital heart disease,pigmented nevus and limb deformity.Disease with the highest mortality was congenital heart disease (0.79‰).The incidence of congenital defects was 8.35‰ in 2010 with the ranking order of the top three as congenital heart disease,neural tube defects/pigmented nevus and hydrocephalus.Diseases having the highest mortality was congenital heart disease (1.10‰o).Different incidence rates on congenital defects were seen in realted areas,with the highest incidence as 14.65‰ in Dunhuang city.Hui county had the lowest incidence—3.28‰.Conclusion Different incidence of congenital defects were seen in respective areas in Gansu province,with the change of ranking orders.Different strategies should be developed differently depending on the current states of congenital defects in respective areas,according to the three-grade prevention model,to reduce the occurrence of congenital defects.