Objectives To summarize the characteristics,differential diagnosis and management of incomplete 17 alpha-hydroxylase/17,20-1yase deficiency(17 OHD)of Chinese patients.Methods Six cases of incomplete 17 OHD from Peking Union Medical College Hospital were studied retrospectively through analyzing their clinical data,and the molecular pathogenic mechanism was discussed after literature review.Results Four cases of 46,XX incomplete 17 OHD were reported.The clinical characteristics included female phenotype,various degrees of breast development and absent or sparse axillary/pubic hair, oligomenorrhea or secondary amenorrhea,recurrent luteinized ovarian cysts,hypogonadism with persistent hyperprogesteronemia or high serum 17 alpha-hydroxyprogesterone level,with or without hypokalemic hypertension.There were also 2 cases of 46,XY incomplete 17 OHD,in which ambiguous genitalia were present besides hypokalemic hypertension.Conclusions Incomplete 17 OHD is a very rare form of congenital enzymatic deficiencies of steroid synthesis,which should be included in the differential diagnosis when there are menstrual disorders,sexual infantilism,recurrent ovarian cysts or ambiguous genitalia.Under such circumstances,hyperprogesteronemia offers a valuable clue for further investigation.

BACKGROUNDCurrently, all the diagnostic indicators for endometriosis lack perfect sensitivity and specificity. According to the characteristic of endometriosis, we analyzed the new biomarker neutrophil-to-lymphocyte ratio (NLR) and the combination of NLR and serum CA-125 to investigate their diagnostic value for identifying stages III and IV endometriosis.

METHODSThe values of serum CA-125 and routine blood tests were collected from 197 patients with endometriosis, 102 with benign tumors and 112 healthy individuals. We investigated the sensitivity and specificity of NLR and its combination with serum-CA-125 for diagnosing stages III and IV endometriosis by using receiver operating characteristic (ROC).

RESULTSThe mean values of NLR, the combination of serum CA-125 and NLR (combination) of the groups with stages III and IV endometriosis were significantly higher than the other two groups. Serum CA-125, NLR, and the combined biomarkers could significantly discriminate the stages III and IV endometriosis group from the other two groups (P < 0.05). NLR shows a lower sensitivity of 57.9% and specificity of 65.2% with a cutoff value at 1.82. And the combination of biomarkers has the highest AUC of 0.949 with a sensitivity of 86.8% and specificity of 92.0% at the cutoff value of 44.40. In addition, for patients with negative CA-125, 55.36% and 53.57% of the patients were able to be diagnosed with endometriosis by using NLR alone and the combination of biomarkers.

CONCLUSIONFor diagnosing stages III and IV endometriosis, the neutrophil-to-lymphocyte ratio is a better adjuvant to serum CA-125, and the neutrophil-to-lymphocyte ratio is valuable in diagnosing stages III and IV endometriosis for patients with negative serum CA-125.

Adult ; Biomarkers ; blood ; CA-125 Antigen ; blood ; Endometriosis ; blood ; diagnosis ; Female ; Humans ; Lymphocytes ; Neutrophils ; Retrospective Studies

OBJECTIVETo explore the effect of genistein on proliferation of human endometrial endothelial cells (HEECs) and glandular epithelium.

METHODSIn vitro HEECs and human endometrial cancer-1B cell (HEC-1B) were cultured with 0, 1, 10, 50, 100, and 200 micromol/L of genistein alone or indicated concentrations of genistein combined with 0.2 or 1 nmol/L 17beta-estradiol (17beta-E2). Cell proliferation was determined by [3H]-thymidine incorporation and cell cycle was measured by flow cytometry.

RESULTSAfter 96 hours of treatment, genistein inhibited the proliferation of HEECs in a dose-dependent manner. The stimulation index reduced from 100% (without genistein treatment) to about 1% (200 micromol/L genistein). HEECs were arrested at G1/0 and G2/M phase when treated with genistein for 96 hours. When the concentration of genistein was 200 micromol/L, the percentages of HEECs at G1/0, G2/M, and S phase were 96.0%, 2.1%, and 1.9%, respectively. However, when HEECs were treated without genistein, the percentages of HEECs at G1/0, G2/M, and S phase were 76.7%, 8.5%, and 14.7%, respectively. 17beta-E2 could not influence the effects of genistein on the proliferation of HEECs. Meanwhile, genistein could suppress the proliferation of HEC-1B. If the stimulation index of HEC-1B was defined as 100% when HEC-1B was treated with different doses of 17beta-E2 (without genistein), it was 67%, 19%, as well as 32% when cell was supplemented with 200 micromol/L genistein combined with 0, 0.2, or 1 nmol/L 17beta-E2, respectively.

CONCLUSIONGenistein at the concentration of 200 micromol/L can sufficiently inhibit the proliferation of HEECs and endometrial glandular epithelium simultaneously in vitro.

Cell Cycle ; drug effects ; Cell Proliferation ; drug effects ; Cells, Cultured ; Endometrium ; cytology ; drug effects ; Endothelium ; cytology ; drug effects ; Female ; Flow Cytometry ; Genistein ; pharmacology ; Humans

OBJECTIVETo explore the potential mechanism of breakthrough bleeding associated with progestin with in vitro methods.

METHODSThe isolation and culture of human endometrial endothelial cells (HEECs) was performed with the method established in our laboratory. The content and activity of urokinase-type plasminogen activator (uPA) and the content of plasminogen activator inhibitor-1 (PAI-1) in cell supernatants after incubated with different concentrations of progesterone (0-5 micromol/L) and 17beta-estradiol (0, 0.1, or 1 nmol/L) were measured by method of ELISA. Apoptosis rate of HEECs was measured by flow cytometry. Viable cell count was measured by MTT.

RESULTSThe increased level of progesterone (0.5-5 micromol/L) combined with 17beta-estradiol elevated content and activity of uPA while the production of PAI-1 remained unchanged. The apoptosis of HEECs was inhibited along with the increment of total viable cell counts at higher concentrations of progesterone with 17beta-estradiol.

CONCLUSIONThe inhibition of apoptosis and increased content and activity of uPA may contribute to the occurrence of irregular bleeding associated with progestin use to some extent

Apoptosis ; drug effects ; physiology ; Endometrium ; cytology ; physiology ; Endothelium ; cytology ; physiology ; Estradiol ; pharmacology ; Female ; Humans ; Metrorrhagia ; etiology ; Progestins ; physiology

OBJECTIVESome recent studies revealed that phenthiazine might be able to reverse tumor cell drug-resistance. Chlorderazin belongs to the phenthiazine compounds. The study aimed to investigate the reversing effect and mechanism of chlorderazin on multidrug resistance of leukemic cell line K562/AO2.

METHODS(1) The cytotoxicities of chlorderazin were assayed with the tetrazolium dye, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) method. (2) The reverse effect of chlorderazin on K562/AO2 cells was analyzed with MTT method. The multidrug resistance reversal index (RI) was equal to the ratio of control group IC(50)/test group half inhibition concentration IC(50). (3) The intracellular daunorubicin (DNR) concentrations were measured by the flow cytometry. (4) Mdr1 mRNA expression was detected by reverse transcription-polymerase chain reaction (RT-PCR). The ratio of mdr-1/beta-actin density was calculated.

RESULTS(1) Chlorderazin 3 micro g/ml showed little toxicity to K562/AO2 cells and the suppression rate was less than 5%, so the concentration of 3 micro g/ml chlorderazin was selected as the experiment concentration. (2) The cytotoxicities of DNR to K562/AO2 were enhanced by 3 micro g/ml of chlorderazin (P < 0.05) and RI was 1.901. (3) Chlorderazin of 3 micro g/ml could increase the intracellular DNR accumulation significantly (P < 0.05), and the fluorescence staining by the flow cytometry was higher (250.95 +/- 18.96) than the control group (112.75 +/- 15.78) and shift right in K562/AO2 cells treated with chlorderazin, and the difference was significant (P < 0.05). (4) Chlorderazin has no significant influence to the expression level of mdr-1 mRNA. Both test group and control group showed a clear mdr-1 mRNA band located at the position of 157 kb. The ratios of mdr-1/beta-actin density were 0.414 +/- 0.012 in the test group and 0.447 +/- 0.027 in the control group, respectively, and the difference was not significant (P > 0.05).

CONCLUSIONChlorderazin could reverse the multidrug resistance by increasing the intracellular DNR accumulation in K562/AO2 cells. The effects had no correlation to the mdr-1 gene. Further study is needed.

ATP-Binding Cassette, Sub-Family B, Member 1 ; genetics ; Antiemetics ; pharmacology ; Cell Division ; drug effects ; Chlorpromazine ; pharmacology ; Drug Resistance, Multiple ; drug effects ; genetics ; Drug Resistance, Neoplasm ; drug effects ; Flow Cytometry ; Humans ; K562 Cells ; RNA, Messenger ; genetics ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction

Objective To develop the National Norms of Negligence (NNN) for rural children aged 0 to 35 months.Methods According to multi-stage stratified cluster sampling principle,10 provinces or municipalities (Jilin,Shaanxi,Shanxi,Beijing,Anhui,Jiangsu,Hunan,Hubei,Yunnan,Chongqing) in China were selected.A national research group was formed collaboratively.A questionnaire was designed by ourselves.According to several statistical analysis methods,such as item,factor and reliability analysis etc.we determined the norm.The evaluation criteria of the scale were determined by percentile method.Finally,the reliability and validity of the norm were evaluated.Results In total,2310 children were surveyed,in which the effective sample were 2227,with an effective rate as 96.4％.The scale consisted of 6 neglected dimensions and 65 items in total.The total Cronbach's α coefficient of the scale was 0.903,with the split-half reliability coefficient as 0.829,the parallel reliability as 0.720 and the re-test reliability as 0.678,respectively.The total neglect cut-off score of this scale was 139.Conclusion The scale seemed to have perfect stability and reliability and all the statistical indicators met the psychometric demands.

OBJECTIVETo develop the national neglect norms for urban primary school students in China.

METHODSAccording to multi-stage stratified cluster sampling principle, 24 cities of 13 provinces (municipalities) in China were selected during December 1 to 31, 2008. A total of 1491 students in grade 1 - 3 and 2236 students in grade 4 - 6 were selected. Questionnaire was designed by authors and the final norms were determined through several statistical analysis methods, such as item analysis method, factor analysis method, reliability analysis method. The reliability analysis and validity analysis were used to test the stability and reliability of the norms. The evaluation criteria of the scale was determined by the percentile method, then the initial development of the norm was completed.

RESULTSThe two questionnaires of grade 1 - 3 and grade 4 - 6 students consisted of 55 and 57 items, respectively, whose item loadings were ranged from 0.301 to 0.687 and 0.321 to 0.730, which met the statistical requirements. For grade 1 - 3 students, the scale's total Cronbach α coefficients was 0.914, the total split-half reliability coefficients was 0.896, the Cronbach α coefficients of four level was above 0.737 except medical and social neglect, split-half reliability was ranged from 0.461 to 0.757; for grade 4-6 students, the scale's total Cronbach α coefficients was 0.916, split-half reliability was 0.883, except social neglect, the Cronbach α coefficients of other level was ranged 0.457 to 0.856, split-half reliability was ranged from 0.500 to 0.798. The total neglect cut-off score of the two scales grade 1-3 and 4-6 were 125 and 155, respectively.

CONCLUSIONThe structure of two norms was reasonable. The scales have good stability and reliability.

Child ; Child Abuse ; prevention & control ; statistics & numerical data ; China ; epidemiology ; Female ; Humans ; Male ; Reproducibility of Results ; Schools ; Students ; Surveys and Questionnaires

There are two types of decoction of Smilax glabra due to its reddish brown or off-white colored cross section. These two kinds of decoction were found that they have large difference in anti-inflammatory effects and chemical constituents in the preliminary experiments. Comparing and analyzing the content of total tannin in these two kinds of decoction of S. glabra from 28 areas by UV-Vis spectrophotometry were first used to provide some experimental and theoretical development and utilization of this medicinal resource and quality control. Also, the sample recovery test required in Chinese Pharmacopoeia was improved by adding tannic acid instead of gallic acid to samples.
Geography ; Pigmentation ; Reproducibility of Results ; Smilax ; chemistry ; Tannins ; analysis ; isolation & purification

OBJECTIVEWe aimed to establish a sensitive quantified method for the simultaneous determination of melamine and cyanuric acid residues in water and urine by hydrophilic interaction liquid chromatography coupled with electrospray tandem mass spectrometry (HILIC-ESI-MS/MS) with the pretreatment of hydrophilic functional silica gel and cation exchange resin mixed solid phase extraction column(MCT), and to investigate the melamine and cyanuric acid residues in 501 water and 216 urine from several province and city.

METHODSAbout 100 ml water (or 10 ml urine) was adjusted to pH 3.0 with concentrated hydrochloric acid, and then mixed with the internal standard solution((15)N3-melamine and (15)N3-(13)C3 -cyanuric acid) and 100 ml acetonitrile (10 ml for urine). The solution was cleaned with MCT solid-phase extraction column, and eluted once by 3 ml methanol and twice by 2.5 ml methanol (containing 5% ammonia water). The effluent was collected and dried by N2 flow at 40 °C, and then diluted to 2 mmol/L ammonium acetate containing 90% volume fraction acetonitrile. The completely dissolved solution was then filtered with 0.22 µm organic membrane; and the filtrate was detected by high performance liquid chromatography-tandem mass spectrometry and quantified with internal standards. The repeatability and sensitivity of the assay were evaluated. Then we detected the melamine and cyanuric acid residues in 501 water and 216 urine samples collected from several province and city.

RESULTSBy the quantification of internal standard (15)N3-melamine and (15)N3-(13)C3-cyanuric acid, the melamine and cyanuric acid were linear in the range of 2.0-1000.0 µg/L with correlation coefficient of 0.9998 and 0.9997. The detection limits of the method were separately 0.4 ng/L (melamine) and 0.3 ng/L (cyanuric acid) for water, and 4.0 ng/L (melamine) and 3.0 ng/L (cyanuric acid) for urine. The average recovery rate was around 95.3%-100.1% with the relative standard deviation (RSD) was <4.02%. Out of the 501 water samples, melamine was detected out in 19.9% (100/501) and cyanuric acid was detected out in 5.2% (26/501). The content was around 0.03-5.00 g/L. Melamine or cyanuric acid was detected out in 24.5% of the urine samples (53/216), with the content around 0.01-1.00 g/L.

CONCLUSIONThe established method of solid phase extraction-hydrophilic interaction liquid chromatography-electrospray tandem mass spectrometry can satisfy the requirement for detection of melamine and cyanuric acid residues in all sorts of water and urine. Meanwhile, the two substances widely existed in water and Chinese population.

Chromatography, Liquid ; methods ; Environmental Monitoring ; methods ; Humans ; Mass Spectrometry ; Solid Phase Extraction ; methods ; Triazines ; analysis ; urine ; Urinalysis ; methods

OBJECTIVETo evaluate the three-year efficacy and safety with standardized dust mite subcutaneous immunotherapy in patients with allergic rhinitis.

METHODSNinety patients who were diagnosed as allergic to mite by skin prick test and serum IgE were include in the standardized allergen-specific dose-escalation regimen. Nasal symptom score (0-3) were collected before treatment and three years after treatment; VAS (visual analogue scale, 0-10) of all nasal symptoms and drug use score were collected every four months; frequency of local and systemic reactions were recorded in the duration of dose escalation and maintenance.

RESULTSNasal blocking, sneeze, rhinorrhea and nasal itch were significantly improved after 3 years treatment (before treatment: 2[2;3], 2[2;3], 2[2;3], 2[1;2] ; after treatment: all were 0[0;0]; Z value were -8.310, -8.408, -8.377, -8.287, all P were 0.000). VAS of all nasal symptoms and drug use score decreased dramatically after escalation period (before treatment: 8.00[7.00;8.85], 2.00[1.50;2.00]; after treatment: 1.00[1.00;1.50], 0 [0;0]; Z value were -8.287, -8.248, P value 0.086, 0.744), and maintained afterwards (F value were 2.483, 0.296; P value were 0.086, 0.744). Ninety-eight case times (64.47%) local reactions mainly happened in maintenance period; the frequency of systemic reactions was 2.54%.

CONCLUSIONThe standardized specific allergen immunotherapy for allergic rhinitis is safe and effective.

Adolescent ; Adult ; Aged ; Allergens ; immunology ; therapeutic use ; Animals ; Child ; Child, Preschool ; Desensitization, Immunologic ; standards ; Female ; Humans ; Male ; Middle Aged ; Pyroglyphidae ; immunology ; Rhinitis, Allergic, Perennial ; therapy ; Treatment Outcome ; Young Adult