Chondrodysplasia Punctata ; congenital ; Humans ; Infant, Newborn ; Male

To explore and discuss the application of case teaching method for pharmaceutical administration science according to the actual teaching situation and the teaching experience of the authors. The teaching effects can be improved by the method, which is worthy of promotion and popularization.

Objective To discuss the changes of correlative ultrasonic parameter index of normal hips and abnormal hips with developmental dislocation of the hip (DDH) in infants of different months in order to provide objective information for the diagnosis. Methods Three-hundred and seventy-eight normal hips and 244 hips with DDH among 622 hips of 311 infants were detected by ultrasonography(US). The morphology and structure information of hips were observed, and the values of ultrasonic parameter index,including angle α,angle 3, acetabular index( AI), femoral head percentage of cover(FHC) of normal hips and abnormal hips were measured. The values of each parameter index were collected by being divided into different groups (3 months a group) ,then the correlation was analyzed. Results Morphology and structure,position relation between femoral head and acetabulum of the hips were demonstrated by US. Normal or abnormal hips,the degrees of abnormal hips and the types of hips could be judged according to the findingsof US. Analysis of values of parameter index of normal hips:①There was significantly statistical significance in the values of ultrasonic parameter index, such as angle α, angle β, AI, FHC of normal hip between the groups of different age (P＜0.01). ②There was correlation between the age and the values of each parameter index, among which angle α, FHC had positive correlations with age ( r = 0. 537, 0. 554,respectively ) while angle β and Al negative correlations ( r = -0. 465, -0.424, respectively ). ③There was correlation between the values of different parameter index. Both angle β and AI had negative correlation with angle α,among which the latter correlation was closely ( r = - 0. 794). No statistical significance was found between the ultrasonic values of each group under different ages of different type abnormal hips( P ＞0.05) ,but closely negative correlations still existed between angle α and AI. ConclusionsUS can be viewed as an early definite and a screening method of diagnosing DDH.For older infants (above 6 months) it will be more accurate to analyze the ultrasonic parameter index together with the age of infants.

There are some shortcomings of animal experiments applied in chemical toxicity testing, such as long period, large cost, species differences and dose differences, which limit the use of animal experiments' results in predicting human toxicity.Accordingly, we established a toxicity testing alternative screening system in line with the toxicity endpoints which required in chemical safety evaluation and risk assessment (genotoxicity, carcinogenicity, reproductive toxicity, acute toxicity and general toxicity) based on 3R principles (replacement, reduction, refinement) for animal experiments.This system covers most of the endpoints of toxicity assessment, and molecular biology technology was also applied to integrate the toxicity test, as well as some operation was optimized in order to shorten the experimental period, reduce experimental costs, improve animal welfare.Furthermore, the results from the screening system have higher clinical relevance because it is based on the toxicity mechanisms.

Purpose To investigate the clinicopathologic features, diagnosis and differential diagnosis of ovarian juvenile granulosa cell tumor (JGCT).Methods The history records, pathologic features and immunophenotype of 8 cases of JGCT were retrospectively evaluated and their prognosis was achieved by follow-up.Results The age of patients ranged from 6～21 years old,with an average age of 15.1 years.The main clinical manifestations included an abdominal mass, ascites and isosexual pseudoprecocity. Cut surface of the tumor was typically solid with cysts formed. The histopathological changes displayed solid nests, diffuse sheet, multiple round or ovoid follicles in variable size.Macrofollicles could be seen in some cases.The follicular pattern consisted of small cystic cavities containing eosinophilic secretions. The tumor cells were round or polygonal, medium in size. The tumor cells had abundant pale or slightly eosinophilic cytoplasm, round nuclei with fine chromatin. Nuclear grooves were inconspicuous.Mitosis figures could be found. Immunohistochemical results showed that the tumor cells expressed inhibin-α,CD99,vimentin; while Melan-A,calretinin and S-100 were positive staining in part of the cases.CKpan,EMA,PLAP,Syn and CgA were negative in all the cases.Conclusions Ovarian juvenile granulosa cell tumor is a rather rare, low malignant tumor with good prognosis. Its diagnosis depends on the histologic and immunohistochemical findings and clinical features. Its differential diagnosis includes adult granulose cell tumor, hypercalcaemic type small cell carcinoma, carcinoid and dysgerminoma.

Objective To investigate the effect of local mild hypothermia on the cerebral hemodynamic parameters,plasma Endothelin-1 (ET-1s) and calcitonin gene-related peptide (CGRPs) of the subarachnoid hemorrhage patients (SAH).Methods Sixty patients were divided randomly into local mild hypothermia group and control group (n =30 patients each group).The middle cerebral artery average blood flow rates (VMCAs) and pulse index (PIs) were detected with transcranial Doppler (TCD),plasma ET-1 s and CGRPs were tested on the D1,D7,D10,and D14,respectively.Results The VMCAs in the mild hypothermia group were lower on the D7,D10,and D14 [7 d:(95.46 ±22.48)cm/s vs (110.35 ±32.38) cm/s,t =2.07,P ＜ 0.05 ; 10 d:(85.57 ± 17.47) cm/s vs (97.64 ± 20.55) cm/s,t =2.45,P＜0.05 ;14 d:(57.16 ± 14.36)cm/s vs (70.56 ± 19.42) cm/s,t =3.04,P ＜ 0.01],PIs and plasma ET-1s were lower on the D10 and D14 compared with the control group [PIs:10 d:0.76 ±0.21 vs 0.88±0.25,t =2.01,P ＜0.05;14 d:0.72±0.18 vs 0.84 ±0.19,t =2.51,P ＜0.05] [ET-1s:10 d:(71.37 ± 16.63) pg/ml vs (81.46 ±21.38)pg/ml,t =2.04,P ＜0.05 ;14 d:(55.73 ± 15.18) pg/ml vs (68.28 ± 20.57) pg/ml,t =2.69,P ＜ 0.01].Plasma CGRPs were higher compared with the control group on the D7,D10,and D14 [7 d:(26.55 ±6.45)pg/ml vs (23.64 ±4.56)pg/ml,t =2.02,P ＜0.05;10 d:(24.15 ±7.35)pg/ml vs (20.52 ±6.18) pg/ml,t =2.07,P ＜0.05;14 d:(30.37 ±6.28)pg/ml vs (26.88 ± 4.39) pg/ml,t =2.49,P ＜ 0.05].Conclusions The mild hypothermia treatment could reduce the plasma ET-1s,improve plasma CGRPs,and improve the prognosis of the patients.

Objective To study the cause,prevention and treatment of postpancreaticoduodenectomy hemorrhage.Method The clinical data of 422 patients who underwent PD in our hospital between January 2000 and January 2012 were retrospective analyzed.Results The incidence of postoperative hemorrhage was 8.1％ (34/422),and the mortality was 20.6％ (7/34).Early and delayed hemorrhage occurred in 19 and 15 patients,respectively.Intra-abdominal and gastrointestinal hemorrhage occurred in 20 and 14 patients,respectively.For the 19 patients who underwent reoperation,the mortality was 20.6％ (7/34).When compared with the delayed hemorrhage group,the mortality of the early hemorrhage group was significantly lower (P＜0.05).Conclusions Meticulous operation and reliable hemostasis during operation and prevention of pancreatic fistula,biliary fistula and peritoneal fluid collection after operation are the key points in reducing postoperative hemorrhage.A timely and decisive reoperation is important to manage postoperative hemorrhage.

ObjectiveTo investigate the clinical and pathological features of Hirschprung disease (HD), intestinal neuro-nal dysplasia (IND) and hypoganglionosis (IH) in children.MethodsThe clinical data and pathologic slices from 238 children with intestinal dysganglionosis were retrospectively analyzed. The age, sex, involved intestinal length of children and prognosis were compared.ResultsIn 238 patients, 138 (58.0%) were diagnosed by rectal mucosal biopsies. There were 122 HD patients whose median age at diagnosis was 9 months and the ratio of male to female was 4.3:1, without involvement of whole colon. There were 45 IND patients whose median age at diagnosis was 14 months and the ratio of male to female was 1.05:1, and the whole colon of 33.3% patients was involved. There were two male IH patients whose ages at diagnosis were 12 years and 18 years respectively, and their whole colon was involved. There were 59 patients with HD complicated by IND whose median age at diagnosis was 13 months and the ratio of male to female was 5.56:1 and the whole colon of 16.9% patients was involved. There were 10 male patients with HD complicated by IH whose median age at diagnosis was 11.5 months and the whole colon of 80.0% patients was involved. The ages at diagnosis, the sex ratio, the rates of whole colon involved, and the cure rates among 5 groups were signiifcantly different (allP<0.01).ConclusionsThe rectal mucosal biopsy was the main method in diagnosis of intestinal dysganglionsis in children. Patients with HD had higher incidence and mild condition and favorable prognosis. Patients with IH or patients with HD complicated by IH had lower incidence rates and severe condition and poor prognosis, followed by patients with IND or patients with HD complicated by IND.

Objective To investigate the changes in the expression of matrix metalloproteinase-1 (MMP-1),matrix metalloproteinase-3 (MMP-3) and tissue inhibitor of metalloproteinase-3 (TIMP-3) in the articular cartilage of patients with Kashin-Beck disease (KBD) and the role of these proteins in pathogenesis of KBD.Methods The postoperative cartilage samples were collected from patients with KBD,osteoarthritis and patients with non-bone disease (control).The expression of MMP-1,MMP-3 and TIMP-3 in the cartilage was detected by immuohistochemistry,and the positive chondrocytes were counted in different layers of the articular cartilage under microscope.Results The positive rates of MMP-1 in the upper [(67.00 ± 1.69)％] and deeper [(22.07 ± 29.66)％] layers of articular cartilage from patients with KBD,and in the deeper layer of articular cartilage from patients with osteoarthritis [(70.52 ± 37.84)％] were significantly higher than those in the control group [(51.73 ± 36.74)％,(3.75 ± 6.85)％,all P ＜ 0.05].The positive rates of MMP-3 in the deeper layer of articular cartilage from patients with KBD [(28.84 ± 31.13)％] and in the middle and deeper layers of articular cartilage from patients with osteoarthritis [(55.69 ± 35.00)％,(45.96 ± 35.38％)] were significantly higher than those in normal cartilage [(34.09 ± 28.54)％,(14.46 ± 18.32)％,all P ＜ 0.05].The positive rates of TIMP-3 in the middle layer of articular cartilage from patients with KBD [(21.25 ± 25.23)％] and in the middle and deeper layers of articular cartilage from patients with osteoarthritis [(20.40 ± 22.19)％,(18.10 ± 22.58)％] were significantly lower than those in normal cartilage [(36.74 ± 26.61)％,(7.81 ± 20.58)％,all P ＜ 0.05].Conclusion MMP-1,MMP-3 and TIMP-3 play important roles in the articular cartilage damage of KBD.

Objective: To observe the influence of moxibustion sertma of mice on the expression of Fas, bcl-2 mRNA and protein of EL-4 lymphoma cells in vitro. Method: The EL-4 lymphoma cells were cultivated for 24 h in the moxibustion serum of mice. The expression of Fas and bcl-2 mRNA of EL-4 lymphoma cells were detected by in-situ hybridization method, and the protein expression of Fas and bcl-2 were observed by the immuocytochemistry method. Results: The expression of bcl-2 mRNA and protein decreased, and the expression of Fas rnRNA and protein increased significantly in EL-4 cells, which were cultivated in the moxibustion serum compared those cultivated in normal mice serum (P<0.05). Conclusion: Moxibustion serum could down-regulate the bcl-2 mRNA and protein and up-regulate the Fas mRNA and protein of EL-4 cells.