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

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

In this paper, five different methods were carried out for DNA extraction directly from soil. The result shows that all five methods could generate DNA with more than 15 kb in size. They were subsequently used as templates for PCR amplification with success, using primers of the bacterial 16S rRNA gene and Shiva-1 gene encoding an antibacterial peptide. However, method 5 is more suitable for DNA extraction directly from a small amount of soil sample as it produced a good yield of DNA in high integrity with reliable reproducibility.

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

OBJECTIVETo study the effect of Bushen Wenyang Huayu Recipe (BSWYHYR) on nerve growth factor (NGF) and transient receptor potential vanilloid receptor I (TRPV1) in experimental endometriosis (EMT), and to explore its mechanism for treating EMT-induced pain.

METHODSTotally in-bred line BALB/c 75 female mice were divided into five groups, i.e., the sham-operation group, the model group, the high dose BSWYHYR group, the low dose BSWYHYR group, the gestrinone group, 15 in each group. Writhing response was observed in each group. Serum contents of NGF were detected using ELISA. Expression levels of NGF and TRPV1 in uterus and ectopic foci were detected using immunohistochemical staining SP and Western blot. mRNA expression levels of NGF and TRPV1 in uterus and ectopic foci were detected by Real-time PCR.

RESULTSThe serum NGF content in the model group was higher than that in the sham-operation group (P < 0.01), and there was positive correlation between NGF and the writhing frequency (r = 0.574, P < 0.01). Compared with the model group, serum levels of NGF significantly decreased in the 3 treatment groups (P < 0.05). Compared with the sham-operation group, mRNA and protein expression levels of NGF and TRPV1 increased significantly in the model group (P < 0.01). Protein expression levels of NGF and TRPV1 decreased significantly in the 3 treatment groups, when compared with the model group (P < 0.01). mRNA expression levels of NGF and TRPV1 decreased most in the high dose BSWYHYR group (P < 0.01). NGF in uterus and ectopic foci was positively correlated with protein and mRNA expression levels of TRPV1 (P < 0.01).

CONCLUSIONSNGF and TRPV1 participated in the occurrence of pain in EMS. BSWYHYR played an important role in inhibiting EMT-induced pain through reducing the up-regulation of NGF on TRPV1.

Animals ; Drugs, Chinese Herbal ; therapeutic use ; Endometriosis ; Female ; Humans ; Mice ; Mice, Inbred BALB C ; Nerve Growth Factor ; metabolism ; Pain ; RNA, Messenger ; TRPV Cation Channels ; metabolism ; Up-Regulation ; Uterus

In this paper, the popular domestic varieties and quality standard of Scrophulariaceae plants used in Tibetan medicine were analyzed. The results showed that there were 11 genera and 99 species (including varieties), as well as 28 medicinal materials varieties of Scrophulariaceae plants were recorded in the relevant literatures. In relevant Tibetan standards arid literatures, there are great differences in varieties, sources, parts, and efficacies of medicinal plant. Among them, about 41.4% (including 41 species) of endemic plants, about 15.2% (including 15 species) of the original plants have medicinal standard legal records, except the medicinal materials of Scrophalaria ningpoensis, Lagotis brevituba, Picrorhiza scrophulariiflora, Veronica eriogyne general, most varieties have not completed quality standard. Consequently it is necessary to reinforce the herbal textual, resources and the use present situation investigation, the effects of the species resources material foundation and biological activity, quality standard, specification the medical terms of the plants, and promote Tibetan medicinal vareties-terminologies-sources such as the criterion and quality standard system for enriching the varieties of Tibetan medicinal materials and Chinese medicinal resources.
Drugs, Chinese Herbal ; chemistry ; pharmacology ; Humans ; Medicine, Tibetan Traditional ; standards ; Plants, Medicinal ; chemistry ; classification ; Scrophulariaceae ; chemistry ; classification

In this paper, the domestic varieties and quality standard of Leguminosae medicinal plants used in Tibetan medicine were analyzed. The results showed that there were 36 genera and 142 species (including varieties), as well as 64 medicinal materials varieties of Leguminosae plants were recorded in relevant literatures. In relevant Tibetan standards and literatures, there are great differences in varieties, sources, used parts, and efficacy of medicinal plants. Among them, about 38.0% (including 54 species) of the endemic plants, about 25.4% (including 36 species) of the original plants have medicinal standard legal records, except 9 kinds of traditional Chinese medicine general quality standard more fairly completed, the most varieties have only description about characters, identification, etc. Therefore it is necessary to reinforce study for the herbal textual, resources and the use present situation, chemical components and biological activity, quality standard, medicinal terms specification, to promote establishment of quality standard system for variety-terminologies-sources of Tibetan medicinal plants.
Fabaceae ; Medicine, Tibetan Traditional ; standards

This paper is in order to discussion with the composition and characteristics of Tibetan medicine plant resources, and promote the reasonable protection and utilization of the resources of Tibetan materia medica. Statistical analysis of species, distributions, and others of Chinese endemic seed plant from Tibetan medicine plants and usually used in the clinic of Tibetan medicine. The results showed that there are 523 species (25%) of Chinese endemic seed plant, belonging to 65 families and 162 genera, in about 2 000 varieties of Tibetan medicine plants recorded in relevant literatures. There are 180 Chinese endemic seed plant species (28%) belonging to 42 families and 72 genera from 625 medicine plants usually used in the clinic of Tibetan medicine. Specifically, the most of these Chinese endemic seed plant species are characteristic crude drug used in Tibetan medicine, and mainly or only distributed in Qinghai-Tibet Plateau. And a few species of them were intersected with traditional Chinese medicines (TCM) and other ethnic medicines. In addition, about 10% are listed in China Species Red List. The Qinghai-Tibet Plateau is the most abundant areas of Areal-types of the Chinese endemic seed plant. This is the biological and ecological reason formation the characteristics of Tibetan medicine plant resources. Therefore, strengthen the research of Chinese endemic seed plants used in Tibetan medicine is great significance for the reasonable protection and utilization of Tibetan medicine plant resources.
Drugs, Chinese Herbal ; chemistry ; Medicine, Tibetan Traditional ; Plants, Medicinal ; chemistry ; classification ; growth & development ; Seeds ; chemistry ; classification ; Tibet

Objective To identify mutations site and clinical characteristics of a familial hypercholesterolemia(FH) proband diagnosed clinically through DNA sequencing and family analysis in the proband and his family members of 3 generations.Methods Blood samples and clinical data of the kindred of total 29 from 3 generations members were collected.Proband had a physical examination electrocar-diogrom and vascular ultrasound.The proband and his family members took routine clinical exams,and genomic DNA was isolated.The promoter region and the 18 exons of low density liporotein receptor(LDLR) gene were screened by Touch down polymerase chain reaction -single strand conformation polymorphism(PCR-SSCP) and DNA sequencing.The result of sequencing were matched gene sequence published in the BLAST database.Results 1.Increased intima-media thickness and plaque were detected in the common carotid artery,right subclavian artery of the proband.Aortic valve regurgitation was found by echocardiography.2.No mutation R3500Q of ApoB100 was observed.3.Two heterozygous mutations in exon 10 and 13 of LDLR gene (W462X and A606T) were identified.The proband and 5 members of paternal relatives showed W462X heterozygosis mutation in exon 10 of LDLR gene which introduced the change from tryptophone to a new stop codon.The proband's mother and grandmother harboured A606T heterozygous mutation in exon 13 of LDLR gene due to a single base pair substitution of G for A in the codon for residue 1 879.Conclusions Disease causing mutations of proband are W462X and A606T compound heterozygosis mutation in exon 10 and 13 of LDLR gene inherited from mother and father.Proband shows homozyous phenotype though the genotype analysis indicates heterozygous mutations.