Objective To analyze the causes of misdiagnosis and mistreatment of Dravet syndrome. Methods Patients with Dravet syndrome diagnosed according to clinical features and SCN1A gene mutation detection were recruited within recent 3 years. The patients were grouped into correct diagnosis-treatment group and misdiagnosis-mistreatment group according to whether the patients had ever been misdiagnosed and mistreated by sodium channel blockers. The clinical features were compared between two groups. Results Thirty-five cases with Dravet syndrome were collected and the rate of misdiagnosis reached 40%, Nine cases were misdiagnosed as symptomatic focal epilepsy, 4 as Lennox-Gastaut syndrome and 1 as Doose syndrome. The average age of onset in misdiagnosis-mistreatment group was (5.50 ± 3.56) months,and the age of confirmed diagnosis was (83.57 ± 105.62) months. The percentage of abnormal EEG, onset seizure with partial seizure, the seizure frequency within the first year from onset, onset with afebrile seizure, patients with status epilepticus or cluster seizures was higher in misdiagnosis-mistreatment group but it showed no significant statistical significance when compared with that of correct diagnosis-treatment group. The percentage of patients with mental retardation and focal neurological signs was significantly higher in misdiagnosis-mistreatment group (P=0.005 and 0.002, respectively). Conclusions Dravet syndrome is frequently misdiagnosed as symptomatic focal epilepsy. The appearance of focal neurological signs and mental retardation before confirmed diagnosis are important factors for misdiagnosis. Gene mutation screening will be helpful for differential diagnosis of Dravet syndrome.

OBJECTIVETo give some theory support of Cistanche tubulosa cultivation by searching dry matter accumulation and echinacoside content of C. tubulosa.

METHODDry matter accumulation content of C. tubulosa culturing in Huabei plain was analysed in different growth season of C. tubulosa. Echinacoside content was determined by HPLC.

RESULTDry matter accumulation of C. tubulosa showed "S" variation. Dry matter accumulation increased fastest in September among growing seasons. Dry matter amount was 138.58 g after C. tubulosa grew a year. Dry matter amount decreased significantly along with inoculation time retarded. Echinacoside content was 30.59% when C. tubulosa grew in 5 months, decreased guadully after that, and 9.76% in annual.

CONCLUSIONVariation rule of dry matter accumulation and echinacoside content was found in C. tubulosa that grew one year in Huabei plain.

Biomass ; China ; Cistanche ; chemistry ; growth & development ; Glycosides ; metabolism ; Plants, Medicinal ; chemistry ; growth & development ; Seasons

OBJECTIVETo observe the antiulcer effects of pomegranate tannins in animal models.

METHODGastric ulcer models were established by pylorus ligation, intragastric absolute ethanol, and water-immersion stress, respectively. The ulcer index, the contents of nitric oxide (NO) and malondialdehyde (MDA), the activities of glutathione peroxidase (GSH-PX) and superoxide dismutase (SOD) from gastric mucosa of rats, the gastric juice volume, free acidity, total acidity,total acid output, the pepsin activity, the amount of adherent mucus and free mucus were measured, respectively.

RESULTPomegranate tannins (500, 150, 50 mg x kg(-1)) significantly inhibited ulcerative formation induced by both water immersion stress and pylorus ligation, obviously decreased the gastric mucosa damages induced by intragastric absolute ethanol, in dose-dependent manner. Pomegranate tannins significantly inhibited absolute alcohol-induced elevation of MDA as well as decreasing of NO level, and activities of both SOD and GHS-PX from gastric mucosa. Pomegranate tannins significantly increased the secretion of adherent mucus and free mucus, but did not affect elevation of the free acidity, total acidity, and total acid output, gastric juice volume, gastric pepsin activity induced by pylorus ligation.

CONCLUSIONPomegranate tannins play a protective role against gastric ulcer. Its antiulcer effect is related to increasing secretion of adherent mucus and free mucus from the stomach wall, which may inhibit generation of oxygen-derived free radicals, and decrease the consumption of GSH-PX and SOD, and maintain content of NO at normal level.

Animals ; Anti-Ulcer Agents ; therapeutic use ; Disease Models, Animal ; Ethanol ; adverse effects ; Female ; Gastric Juice ; drug effects ; Male ; Mice ; Nitric Oxide ; adverse effects ; Plant Extracts ; therapeutic use ; Punicaceae ; chemistry ; Pylorus ; Rats ; Rats, Sprague-Dawley ; Stomach Ulcer ; chemically induced ; drug therapy ; Tannins ; therapeutic use

In this study, to investigate the effect on expression of IL-2 in lymphocytes from bone marrow and peripheral blood of normal donors after they were mobilized by G-CSF in allo-BMT, 7 normal donors bone marrow and peripheral blood were harvested before and after G-CSF administration. The separated lymphocytes were measured by FCM after they were stained intracellularly by anti-IL-2, and their expressions of IL-2 were compared. The degree of aGVHD in patients after bone marrow transplantation was evaluated clinically, and it was compared with the status of aGVHD of 15 patients whose donors didn't receive G-CSF administration in our department, and 2 groups of patients are comparable in age, types of diseases and status of donors. The results showed that the expression of IL-2 in lymphocytes in 7 G-CSF mobilized donors decreased significantly after G-CSF administration and more severe aGVHD than grade II didn't develop in these recipient patients, and comparing with 15 patients received the bone marrow from donors who didn't receive G-CSF, the incidence of aGVHD decreased. It is suggested that the expression of IL-2 in lymphocytes was influenced by donors' G-CSF administration, and it is likely that thereby reduces the incidence of aGVHD in patients after BMT.
Adult ; Blood Donors ; Bone Marrow Cells ; drug effects ; metabolism ; Bone Marrow Transplantation ; adverse effects ; Female ; Flow Cytometry ; Graft vs Host Disease ; etiology ; Granulocyte Colony-Stimulating Factor ; pharmacology ; Hematopoietic Stem Cell Mobilization ; Humans ; Interleukin-2 ; biosynthesis ; Lymphocytes ; drug effects ; metabolism ; Male

OBJECTIVETo study the relationship between spiral CT findings and histological differentiation and expressions of p53 and Ki67 in gastric carcinoma.

METHODSTriphasic spiral CT was performed in 158 patients. CT findings included maximal diameter and thickness of tumor in three dimensional CT images, degree of enhancement, mucous situation, lymph nodes, and visceral metastasis were recorded. The expressions of p53 and Ki67 were detected with immunohistochemistry and reverse transcription polymerase chain reaction.

RESULTSThe thickness of tumor (chi2 = 5.554, P = 0.018), degree of enhancement (chi2 = 4.978, P = 0.026), and lymph nodes metastasis (chi2 = 6.061, P = 0.014) in the three dimensional CT images were significantly correlated with tumor histological differentiation. Lymph nodes metastasis was significantly correlated with the expression of p53 (chi2 = 5.028, P = 0.025). The expression of Ki67 was significantly correlated with the thickness of tumor (chi2 = 5.674, P = 0.017) and lymph nodes metastasis (chi2 = 5.028, P = 0.025).

CONCLUSIONMulti-slice CT is a simple and noninvasive technique, and can be used for assessing the histological differentiation of gastric cancer as well as the expressions of p53 and Ki67 before the operation.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Ki-67 Antigen ; metabolism ; Male ; Middle Aged ; Stomach Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Tomography, Spiral Computed ; Tumor Suppressor Protein p53 ; metabolism ; Young Adult

OBJECTIVETo study the clinical characteristics and outcomes of the hereditary medullary thyroid carcinoma (HMTC) and the sporadic medullary thyroid carcinoma (SMTC).

METHODSThe clinical data of 78 patients with medullary thyroid carcinoma who underwent surgery in our hospital between July 1980 and May 2011 were retrospectively analyzed.

RESULTSOf these 78 patients, there were 23 HMTC cases and 55 SMTC cases. The HMTC group was significantly younger age of onset [(36.4±13.5) years vs. (46.6±11.2) years, P<0.01] and a lower pre/post-operative serum calcitonin levels [(850.4±110.20) ng/L vs. (1450.4±118.3) ng/L, P<0.01 and (410.8±133.2) ng/L vs. (1585.4±129.5) ng/L, P<0.01] than the SMTC group. In addition, the mean tumor diameter was also significantly smaller in the HMTC group (14.3 mm vs. 21.0 mm in SMTC group, P<0.05). Tumor multifocality was seen in a significantly higher proportion of HMTC cases compared with the SMTC cases (56.6% vs. 29.1%, P<0.05). The overall 10-year survival was 100% in HMTC group and 80.2% in SMTC group (P<0.05).

CONCLUSIONHMTC has a better prognosis than SMTC.

Adolescent ; Adult ; Age of Onset ; Aged ; Carcinoma, Medullary ; classification ; genetics ; pathology ; Carcinoma, Neuroendocrine ; Female ; Genetic Diseases, Inborn ; pathology ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Survival Rate ; Thyroid Neoplasms ; classification ; genetics ; pathology ; Young Adult

The androgen receptor (AR) plays an important role in the development and progression of prostate cancer (PCa). Androgen deprivation therapy is initially effective in blocking tumor growth, but it eventually leads to the hormone-refractory state. The detailed mechanisms of the conversion from androgen dependence to androgen independence remain unclear. Several PCa cell lines were established to study the role of AR in PCa, but the results were often inconsistent or contrasting in different cell lines, or in the same cell line grown under different conditions. The cellular and molecular alteration of epithelial cells and their microenvironments are complicated, and it is difficult to use a single cell line to address this important issue and also to study the pathophysiological effects of AR. In this paper, we summarize the different effects of AR on multiple cell lines and show the disadvantages of using a single human PCa cell line to study AR effects on PCa. We also discuss the advantages of widely used epithelium-stroma co-culture systems, xenograft mouse models, and genetically engineered PCa mouse models. The combination of in vitro cell line studies and in vivo mouse models might lead to more credible results and better strategies for the study of AR roles in PCa.
Animals ; Cell Line, Tumor ; Disease Models, Animal ; Epithelial Cells ; pathology ; Humans ; Male ; Mice ; Prostatic Neoplasms ; pathology ; physiopathology ; Receptors, Androgen ; physiology ; Stromal Cells ; pathology

OBJECTIVETo investigate the subcellular expression of mammary serine proteinase inhibitor (Maspin) in oral squamous cell carcinoma and its relationship to the clinicopathological features.

METHODSThe Maspin protein subcellular expression was detected in 45 patients with oral squamous cell carcinoma by immunohistochemical staining. The relationship between the Maspin protein subcellular expression and the clinicopathological parameters was analyzed.

RESULTSThe negative rate of nuclear maspin expression was 64% (29/45), and the weakly positive rate was 11% (5/45), and the strong positive rate was 24% (11/45). Nuclear maspin expression was negatively correlated with T stage (P = 0.019), lymph node metastasis (P = 0.038) and postoperative metastasis (P = 0.004), but positively correlated with the patients' survival rate (P = 0.014). The negative rate of cytoplasmatic maspin expression was 38% (17/45), and the weakly positive rate was 31% (14/45), and the strong positive rate was 31% (14/45). Cytoplasmatic maspin expression was negatively correlated with lymph node metastasis (P = 0.038) and postoperative metastasis (P = 0.004), but positively correlated with the patients' survival rate (P = 0.014).

CONCLUSIONSMaspin expression may be a significant marker in predicting prognosis of oral squamous cell carcinoma.

Adult ; Aged ; Aged, 80 and over ; Biomarkers, Tumor ; metabolism ; Brain Neoplasms ; secondary ; Carcinoma, Squamous Cell ; metabolism ; pathology ; secondary ; Cytoplasm ; metabolism ; Female ; Humans ; Lung Neoplasms ; secondary ; Lymphatic Metastasis ; Male ; Middle Aged ; Mouth Neoplasms ; metabolism ; pathology ; Neoplasm Staging ; Serine Proteinase Inhibitors ; metabolism ; Serpins ; metabolism ; Survival Rate

OBJECTIVETo study the value of multi-slice spiral computed tomography (CT) in the preoperative evaluation of lymph node metastasis.

METHODSTotally 45 patients with gastric cancer detected by 64-slice spiral CT were enrolled in this study. The potential lymph node metastasis was evaluated by measuring or calculating the long diameter, extent of enhancement, and short-to-long diameter ratio of the lymph nodes. The results were compared with postoperative pathological findings.

RESULTA long diameter ≥ 8mm,enhanced density ≥ 80Hu, and short-to-long diameter ratio ≥ 0.7 had the best consistency with postoperative pathological findings.

CONCLUSIONAs a simple and noninvasive technique, multi-slice spiral CT is helpful to identify potential lymph node metastasis of gastric cancer based on long diameter, extent of enhancement, and short-to-long diameter ratio of the lymph nodes, and thus provide important information for surgery selection, prognosis, and development of new procedures.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Lymph Nodes ; diagnostic imaging ; pathology ; Lymphatic Metastasis ; diagnostic imaging ; Male ; Middle Aged ; Stomach Neoplasms ; diagnostic imaging ; pathology ; Tomography, Spiral Computed

OBJECTIVEThis article reports a population genetic study on six short tandem repeat(STR) loci, D7S820, D19S253, D12S391, D5S818, D16S539 and D8S1179, in a sample of unrelated Chinese Han individuals(n=122-173) living in Hebei province.

METHODSDNA extraction from blood samples (200 in number) and multiplex amplification of the above six loci were carried out. Using denaturing polyacrylamide gel electrophoresis and silver stain, the authors investigated the distribution of allele frequencies of the six loci in Han population in Hebei province.

RESULTSThe STR polymorphisms at all of the six loci were observed in Chinese Han population in Hebei province. The observed heterozygosities of D7S820, D19S253, D12S391, D5S818, D16S539 and D8S1179 were 0.828, 0.757, 0.769, 0.837, 0.785 and 0.852, respectively. The measured values of the power of discrimination (PD) were 0.914, 0.919, 0.940, 0.909, 0.917, 0.944; of the mean exclusion chance(MEC) 0.618, 0.740, 0.801, 0.557, 0.655, 0.696 and of the polymorphism information content (PIC) in Chinese 0.771, 0.760, 0.762, 0.708, 0.776 and 0.794, respectively.

CONCLUSIONThe genotype distributions of the six STR were in accordance with Hardy-Weinberg equilibrium. The numerical values of the PD and MEC are relatively high in Hebei province, and thus can be of significant application in population genetics and forensic medicine.

Asian Continental Ancestry Group ; genetics ; China ; ethnology ; DNA ; analysis ; Female ; Gene Frequency ; Genetics, Population ; Humans ; Male ; Microsatellite Repeats ; genetics ; Polymorphism, Genetic ; Population Groups ; Tandem Repeat Sequences ; genetics ; physiology