Prader-Willi syndrome is a rare genetic disorder characterized by hypotonia,poor feeding in infancy,hyperphagia with evolving obesity,hypogonadism,decreased adult height,and cognitive and behavioral disabilities.Recombinant human growth hormone (rhGH) has been used in treating Prader-Willi syndrome,and it has achieved good results.Several aspects still need to be concerned,including evaluation before rhGH treatment,age at treatment initiation,dosing,monitoring of potential side effects,tolerability,endpoint,response evaluation,use of adjunct therapies,and issues of consent.

Objective To investigate the role of cell mediated immunity in HBV infection.Methods tive responses of peripheral blood mononuclear cell(PBMC)to hepatitis B virus antigen HBcAg,HBehg and HBsAg were studied in 20 patients with acute hepatitis B and 23 patients with chronic hepatitis B as well as 20 heath normal donrors.Results Patients with acute hepatitis B showed stronger proliferative responses of PBMC to HBV antigen than patients with chronic hepatitis B as wdl as normal donors.The responses to HBcAg and HBeAg were stronger than that to HBsAg in both acute and chronic group.It was also found that cellular pro|ifertive response,which showed no correlation with ALT level,was significantly increased in the patients with HBV DNA+or/and HBeAg+.Conclusion The results indicate that cellular immune responses to HBV antigens exist in peripheral blood of patients with hepatitis B and potentially play an essential role not only in eliminating virus but also in the pathogenesis of hepatitis B.

Objective To study the course of development of aneurysm of abdominal aorta (AAA) with nonsurgical treatment in senile patients. Methods 37 subjects were included in the study, and they were divided into 3 groups according to the size of aneurysm observed at the first visit. The diameter of aneurysm was measured, and the measurement was repeated at periodic follow up. The yearly increase of the diameter was calculated and analyzed. Results For the 37 patients, the AAA mean diameter was 4.68cm at the first visit. The period of follow-up was 0.5 to 11 years, with a mean of 6.1 years. The average yearly increase in tumor diameter was 0.47cm/year. 14 patients with aneurysm diameter of 6.0cm had an average increase of 0.67cm/year. 5 patients died during the follow-up period, 2 of them died of rupture of the aneurysm, the diameter of which was 8.35cm and 8.91cm, respectively. Endoaneurysmorrhaphy with stent was performed in 7 patients. Conclusion The size of AAA in the senile patients increased slowly if the aneurysm diameter is 6.0 cm, the tumor size might increase quickly, and active intervention is recommended.

Patients on Porcelain-fused-to-metal restorations of vital teeth are increasingly year by year, this article mainly discusses the psychological characteristics of adult on porcelain-fused-to-metal restorations of vital teeth on the view of medical ethics.It is helpful to lighten patients’burden and complete treatment successfully by improving the qualities of dentists and giving patients psychological guidance.

OBJECTIVE To know the risk factors about lung infection in patients after tracheal intubation general anesthesia and take control measures.METHODS All the patients with tracheal intubation general anesthesia from Jan to Dec in 2007 were investigated retrospectively.RESULTS Among 2914 operation cases,471 cases were of tracheal intubation general anesthesia and 95 cases were infected(20.17%).The infection rate was 5.38%(24 hours).Sputum culture: G-bacteria accounted for 57.65%,G+ bacteria 28.83%,fungi(13.52%).CONCLUSIONS The infection rate increase with longer intubation time.Mechanical ventilation is a risk factor of lung infection.The measures to control infection include shortening intubation hours,reducing mechanical ventilation and strictly sterilization and isolation.

Prader-Willi syndrome (PWS) is a multisystemic complex genetic disorder caused by the lack of expression of genes associated with the 15q11.2-q13 region of the paternal chromosome.There are three main types of genetic mechanisms, deletion of the paternal critical region, maternal uniparental disomy and imprinting center defect.Genetic counseling can be carried out based on different genetic mechanisms of PWS, both re-fertility assessments and prenatal diagnoses were performed on couples whose children have already had the disease.The pathogeny and mechanism of PWS are complex.The rapid development of molecular genetics and related research have provided a basis for further understanding of this disease.In this paper, the advances in the genetics of PWS were reviewed.

Objective To investigate the role of mismatch repair gene hMLH1 in pancreatic carcinoma and its clinicopathological significance.Methods hMLH1 was extracted from 60 cases of pancreatic carcinoma tissues and 60 cases of normol pancreatic tissues.hMLH1 expression in pancreatic carcinoma and normal tissues was detected by SP immunohistochemical staining.Results The strong,weak and loss expression of hMLH1 in pancreatic carcinoma tissues and normal pancreatic tissues was 0 vs 83.33％ (50/60),31.7％ (19/60)vs 16.67％ (10/60),and 68.3％ (41/60) vs 0 respectively.The protein expression of hMLH1 was not related to patient's age,tumor location,or pathological types (P ＞ 0.05),but it was related to lymph node metastasis (x2 =8.579,P =0.004),clinical stage (x2 =9.586,P =0.002) and pathological differentiation (x2 =20.372,P =0.001).Conclusion The loss expression of hMLH1 has a correlation with pancreatic carcinogenesis,differentiation degree,and disease progression.

Objective To explore the immune characteristic of a patient with hypogammaglobulinaemia and low natural killer(NK)cell numbers. Methods Case histories were analyzed repeatedly to grasp important characteristics of the disease. A routine blood examination and peripheral blood immunological detection was done. Complete neutrophil function test,lymphocyte proliferation ability and cytokine profile were evaluated. Results A 3-year-old boy was referred to the hospital because of fever and abdominal pain as well as partial intestinal obstruction. Laboratory examination revealed low immunoglobulin,reduced absolute numbers of B and NK cells and deficient B cell function of lower IFN-γ and IL-12 production. The patient also had mesenteric lymphadenopathy and a caecal mass with histology suggestive of ileocecal junctional tuberculosis. The patient was infected with cytomegalovirus(CMV)and fungus,which are not common in simple antibody deficiencies. The patient was followed for four years and during this period the patient had recurrent periodontitis resulting in the loss of one tooth. Conclusions Although the precise mechanism of the defect is unclear,we speculate that the case may cast new light on primary immunodeficiencies predominantly with antibody deficiency.(J Clin Pediatr,2011,29(7):601-608)

Objective To explore the related risk factors of severe mycoplasma pneumonia in children.Methods 86 children with mycoplasma pneumonia were selected as the observation group.At the same time,30 normal children were selected as the control group.The observation group included 33 cases of mild mycoplasma pneumo nia(A group) and 53 cases of severe mycoplasma pneumonia(B group).The clinical data of the three groups were ret rospectively analyzed,and the related risk factors of severe mycoplasma pneumonia in children were analyzed.Results The risk factors of severe mycoplasma pneumonia in children were age ＞ 5 years (x2 =28.776,P ＜ 0.05),immunoglobulin IgG(x2 =3.004,P ＜ 0.05),immunoglobulin IgM (x2 =2.147,P ＜ 0.05),immunoglobulin IgA (x2 =2.036,P ＜ 0.05),WBC (x2 =6.119,P ＜ 0.05),neutrophil percentage (x2 =8.374,P ＜ 0.05),the positive rate of CD8(x2 =11.665,P＜0.05),the positive rate of CD4(x2 =12.901,P＜0.05).Conclusion For children with risk factors of severe mycoplasma pneumonia should be early diagnosed,prevented and treated,thereby reducing the burden on patients.

Objective To explore the expression of KAI1/CD82,E-cadherin and β-catenin in endometrial carcinoma,and to investigate their correlations to clinicopathological parameters of endometrial carcinoma. Methods The expressions of KAI1/CD82,E-cadherin and β-catenin in 76 specimens of endometrial carcinoma,15 specimens of atypical endometrial hyperplasia and 20 specimens of proliferative endometrium were examined by immunohistochemical envision technique.Their correlations to clinicopathological parameters of endometrial carcinoma were statistically analyzed. Results Compare to normal proliferative phase endometrium and atypical endometrial hyperplasia,the expression of KAI1/CD82 in endometrial carcinoma was significantly decreased(P ＜0.01),the abnormal expression of E-cadherin and β-catenin in endometrial carcinoma were significantly higher(all P <0.01).In endometrial carcinoma,the expression of KAI1/CD82 was negative correlated with histological grade and depth of myometrial invasion(P <0.01,P <0.05); The abnormal expression of the E-cadherin is related to histological grade and type(P <0.01,P<0.05); The abnormal expression of β-catenin was positively correlated with histological grade and FIGO stage(P ＜0.01 ,P <0.05).The down-regulation expression of KAI1/CD82 was closely associated with the abnormal expression of E-cadherin and beta-catenin in endometrial carcinoma(P <0.01,P <0.05). Conclusion The down-regulation of KAI1/CD82 and the aberrant expression of E-cadherin and β-catenin could be involved in the development of endometrial carcinoma.The loss or reduced expression of KAI1/CD82 was closely associated with the abnormal expression of E-cadherin and β-catenin in endometrial carcinoma.