Objective To explore the effect of the thyroid morphologic change,the initiating treatment time and the thyroid function(serum FT_4,TSH)on mental development in children with congenital hypothyroidism (CH).Methods After diagnosed,52 cases of CH were orally administrated with L-T_4,then their mental and physical development were monitored and thyroid morphologic changes were observed by thyroid imaging(~(99m)TcO_4) and type B ultrasonography.The Bayley test was performed at the age of 10 to 30 months and expressed as mental developmental index(MDI)and psychomotor developmental index(PDI).Analysis was conducted upon treatment timing and thyroid morphologic changes.Results The MDI in those cases with morphologic abnormalities treated within 90 days was significantly lower than that with normal morphology(91?20 vs 105?15,P

Genetic Diseases, Inborn ; economics ; prevention & control ; therapy ; Humans ; Metabolic Diseases ; economics ; prevention & control ; therapy

Adrenal Hyperplasia, Congenital ; complications ; metabolism ; Body Height ; Growth Disorders ; etiology ; Humans ; Risk Factors ; Steroid 21-Hydroxylase ; metabolism

Processed Chinese medicine is the core of traditional Chinese medicine (TCM) industry chain,which directly affects the clinical efficacy and. safety of Chinese patent medicine and clinical formula Decoction pieces. Studied the variation of effective substance in vivo Chinese medicine processing before and after processed, clarifying the effective substance and processing principle is a top priority of the development of Chinese medicine processing. The traditional research method chiefly focus on the variation about chemicals in vitro of processed Chinese medicine, it cannot reveal that the integrity and complexity of processed Chinese medicine efficacy changes, so the change process is the focus of future research in vivo on the base of effective substance of TCM This paper described the research on the base of effective substance of TCM and Processed Chinese medicine research status in vitro, discussed the analytical methods (plasma chemistry, pharmacokinetics, metabonomics) of the dynamic process in vivo about processed Chinese medicine, and pointed out development and related problems in process in vivo on the base of effective substance of TCM, which could provided research ideas and methods for in-depth interpretation of Chinese medicine processing mechanism.
Animals ; Drugs, Chinese Herbal ; analysis ; pharmacokinetics ; Humans ; Medicine, Chinese Traditional

Gardeniae Fructus contains volatile ingredients, however, the species and proportions in different processed products of Gardeniae Fructus are different. In this experiment, volatile ingredients were separated by steam distillation with content of 1.2, 1.0, 0.9, 0.7 µL · g(-1) in Gardeniae Fructus, fried Gardeniae Fructus, stir-baked Gardeniae Fructus, Gardeniae Fructus fried into carbon respectively. One hundred and twenty-four kinds of volatile components were identified by GC-MS. Fifty-three kinds of volatile ingredients consisted in Gardeniae Fructus accounting for 93.85%, 54 kinds in fried Cardeniae Fructus accounting for 92.01%, 32 kinds in stir-baked Cardeniae Fructus accounting for 91.59% and 43 kinds in Gardeniae Fructus fried into carbon accounting for 90.81%. In this paper, analysis of Gardeniae Fructus by GC-MS provides a scientific basis for elucidating the mechanism of different processed products.
Chemistry, Pharmaceutical ; Drugs, Chinese Herbal ; chemistry ; Gardenia ; chemistry ; Gas Chromatography-Mass Spectrometry ; Molecular Structure ; Volatile Organic Compounds ; chemistry

OBJECTIVETo evaluate the efficacy and safety of a phenylalanine-free amino acid-based enteral formula (AA-PKU2) in the treatment of children with phenylketonuria (PKU) aged 1-8 years.

METHODSA prospective, open, self-controlled, multi-center trial was performed, enrolling 121 PKU children (1-8 years in age) consecutively between July, 2009 and May, 2011. Enteral nutrition therapy was administered for 32 weeks. The data on blood phenylalanine (PHE) levels, metal development, weight, height, head circumference, serum nutritional biomarkers (total protein, pre-albumin, albumin, total cholesterol, total triglyceride, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol), and measurements from routine blood and urine examinations and from renal and hepatic function tests were collected before the therapy and at 8 weeks and 32 weeks after the therapy and were comparatively analyzed.

RESULTSThe mean blood PHE level at 8 and 32 weeks of AA-PKU2 treatment was 353±253 and 361±280 µmol/L respectively, significantly lower than that before the treatment (487±327 µmol/L; P<0.01). The difference in intelligence quotient scores before and after AA-PKU2 treatment was not significant (P>0.05) when assessed by the Gesell tests in children aged 1-4 years but significant (P<0.01) when assessed by WPPSI or WISR-R tests in children over 4 years. The average height, weight and head circumference at 8 and 32 weeks after treatment were significantly increased as compared to these measurements before treatment (P<0.01) with absolute levels similar to those in the control children. In contrast, the mean values of total protein, pre-albumin, albumin, total cholesterol, total triglyceride, low-density lipoprotein cholesterol and high-density lipoprotein cholesterol at both time points were not different either from those prior to the treatment or from those in the control children. Mild diarrhea was the adverse events associated with AA-PKU2 treatment, which occurred in 3 (2.5%) cases. All these 3 patients fully recovered without treatment.

CONCLUSIONSThe phenylalanine-free amino acid-based formula, AA-PKU2, is effective and safe in controlling blood PHE levels and improving mental development with adequate nutritional support in PKU.

Child ; Child, Preschool ; Enteral Nutrition ; Female ; Humans ; Infant ; Intelligence ; Male ; Phenylalanine ; blood ; Phenylketonurias ; diet therapy ; psychology ; Prospective Studies

Objective Data on the leukapheresis from 26 pediatric patients with hematologic or solid malignancies was retrospectively evaluated to screen predictive factors affecting the efficacy of peripheral blood stem cell(PBSC) collection from donors,as well as hematopoietic recovery in recipients.Methods We present our experience with 49 apheresis from 26 granulocyte-colory Stimulating factor mobilized donors and analyzed the correlations between the mobilization,the leukocyte count in the donor peripheral blood and the MNC and CD_(34)~+ cell yields in collecting products and the neutrophil and platelet recovery of recipients.Results The process of mobilization and apheresis were well tolerated by our pediatric donors.The median numbers for harvested MNCs and CD_(34)~+ cells were 4.5?10~8/kg and 1.9?10~6/kg of recipient body weight,respectively.Mobilizing dose positively affected the number of mononuclear ceus(MNC) but not CD_(34)~+ cells in the apheresis products.The CD_(34)~+ cell number in the apheresis product was influenced significantly by donor circulating MNC on the day of harvest and correlated with recipient′s engraftment after PBSC was reinfused.Conclusions The MNC yield was stable and met with the demand for autologous stem cell transplantation while the CD_(34)~+ cell number varies obviously from each donor.Since a rapid engraftment was associated with a high number of CD_(34)~+ cells collected,which was in turn predicted by the level of the pre-apheresis CD_(34)~+ cells in the peripheral blood of donors,it is necessary to monitor the donors′ CD_(34)~+ cell during mobilization to determine the optimal time for apheresis.J Appl Clin Pediatr,2006,21(3):148-150

OBJECTIVETo report the results of clinical characteristics, enzyme activity determination and mutation analysis of GLB1 gene in a Chinese patient with mucopolysaccharidosis (MPS) type IVB (Morquio B disease).

METHODA 14-year-old Chinese boy with MPS type IVB was firstly diagnosed by blood leucocytes galactosamine-6-sulfate sulfatase (GALNS) and β-galactosidase (GLB1) determination, who was characterized by short stature, multiplex skeletal abnormalities, difficulty in walking. PCR-sequencing analysis was applied to detect the mutations in GLB1 of the patient.

RESULTThe patient was characterized by dwarfism, pectus carinatum, kyphosis, normal intelligence, and no neurologic damage of spasms, linguistic capacity and so on. The patient had normal GALNS enzyme activity and very low GLB1 enzyme activity [5.03 nmol/(h·mg) vs. normal value 118 - 413 nmol/(h·mg) ] in leukocytes. A compound heterozygous missense mutations c.442C > T(p.R148C)/c.1454A > G(p.Y485C) in GLB1 gene were detected in this patient. The mutation p.Y485C is a novel variant. With the method of gene analysis of new variant, the mutation p.Y485C was considered to be a pathogenic mutation.

CONCLUSIONThe MPS IVB patient showed severe multiple skeletal deformities, normal intelligence, no neurologic damage and very low GLB1 enzyme activity, who carries compound heterozygous mutations p.R148C/p.Y485C. The mutation p.Y485C in GLB1 gene may be a novel pathologic mutation of MPS type IVB.

Adolescent ; Amino Acid Sequence ; Asian Continental Ancestry Group ; genetics ; Chondroitinsulfatases ; genetics ; metabolism ; DNA Mutational Analysis ; Humans ; Joints ; pathology ; Male ; Molecular Sequence Data ; Mucopolysaccharidosis IV ; enzymology ; genetics ; pathology ; Mutation, Missense ; Pedigree ; Polymerase Chain Reaction ; Radiography ; Spine ; diagnostic imaging ; pathology ; beta-Galactosidase ; genetics ; metabolism

OBJECTIVETo observe the effect of acupoint injection by astragalus injection on local SIgA and pathomorphologial changes in rats with chronic pelvic inflammatory disease (CPID).

METHOD50 female Wistar rats were randomly devided into 6 groups, in which CPID model was made except the normal group and sham operation group. The astragalus injection group and the 0.9% NaCl injection group were treated by acupoint injection in Guanyuan (RN4) and Zusanli (ST36). The group was fed Qianjinpian solution into stomach. The histopathologic changes of rats' uterus of each group were observed and SIgA in vagina flushing was detected.

RESULTThe model group showed inflammatory changes, and astragalus injection group and Qianjinpian group showed little histopathologic changes. The levels of SIgA in astragalus injection group were significantly higher than those in other groups, but that in the model group was the lowest.

CONCLUSIONThe deficiency of local SIgA lead to repeatedly attack of CPID. The treatment of acupoint injection by astragalus injection can improve the excretion of SIgA, reinforce the local immunity, and prevent the repeatedly attack.

Acupuncture Points ; Animals ; Astragalus membranaceus ; chemistry ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; Female ; Immunoglobulin A, Secretory ; blood ; Injections ; Pelvic Inflammatory Disease ; blood ; pathology ; Plants, Medicinal ; chemistry ; Random Allocation ; Rats ; Rats, Wistar ; Uterus ; pathology

Objective To investigate the occurrence and reporting of sharp injuries among health care workers (HCWs)at all levels of hospitals in Xuzhou City,provide evidences for formulating protective measures against sharp injuries and improving the reporting system.Methods From July to August 2016,13 hospitals in Xuzhou City were randomly selected by multi-stage stratified random sampling method,the general information,occurrence of sharp injuries,and reporting situation was performed questionnaire survey.Results A total of 2 694 valid question-naires were collected,incidence,case incidence,and reporting rate of sharp injuries were 10.32%,12.84%,and 30.64% respectively.Case incidence of sharp injuries among HCWs in primary,secondary,and tertiary hospitals were 44.83%,11.53%,and 12.52% respectively,case incidences of sharp injuries in different levels of hospitals were significantly different (χ2＝55.148,P＜0.001).The main opportunity for sharp injuries was when HCWs re-turned needle cap (79 cases,22.83%),the main device involving sharp injuries was hollow-bore needle (297 cases, 85.84%).Incidences of sharp injuries among HCWs receiving different training were significantly different (χ2＝66.760,P＜0.001).Conclusion Current situation of sharp injuries among HCWs in this region is not optimistic, there are some problems such as poor training efficacy,low reporting rate and low use rate of safety devices,effec-tive measures should be taken to establish an effective monitoring and tracking system for sharp injuries,so as to re-duce the occurrence of sharp injuries.