Objective To study the clinical characteristics and treatment of neurofibromatosis type Ⅱ (neuro-fibromatosis type 2 ,NF2 ,bilateral acoustic neuroma) ,and the effects of auditory brainstem implant for treating to-tal deafness after bilateral acoustic neuroma resection .Methods One case of bilateral acoustic neuroma and all clini-cal data in terms of diagnosis ,treatment and hearing -speech rehabilitation after surgery were retrospectively stud-ied .Results The patient was a thirteen years old boy .His clinical symptoms were hearing loss on the right ear ,tin-nitus ,hoarseness and gait instability three years .MRI showed space occupying lesion in the cerebellopontine angle . The postoperative pathological diagnosis was bilateral acoustic neuroma .The initial switch -on was peformed six weeks after the surgery ,and confirmed that all electrodes generated listening responses .As the extension of recov-ery time ,the correct recognition rate of patients on the natural environment sound ,vowel ,monosyllabic were on the rise and the pure tone hearing threshold gradually decreased .The vowel correct recognition rate of postoperative 6 , 9 ,12 ,24 ,and 36 months were 14% ,18% ,20% ,24% ,and 20% ,respectively .The recognition rate of monosyl-labic and open words at each postoperative rehabilitation stage were 0 .Conclusion The clinical characteristics and treatment of bilateral acoustic neuroma were different from the unilateral acoustic neuroma .The individualized treat-ment should be followed .Auditory brainstem implant could be performed in patients with post - bilateral acoustic neuroma resection .The accurate location of the cochlear complex during the surgery was the crucial point for the success of ABI .

Objective Macrophage activation syndrome (MAS) is a severe, potentially life-threatening clinical condition. The clinical features including precipitating events, clinical presentations, treatment strategies, outcome in systemic onset juvenile idiopathic arthritis (So-JIA) children with MAS were reviewed. Perforin A91V gene analysis was also performed. Methods Retrospective review of fourteen MAS cases with So-JIA from 2003 to 2008 from a collected database. Gene-specific polymerase chain reaction ( PCR) primers were used to analyze the perforin A91V gene polymorphism. Results Fourteen patients with age from 4 months to 12 years were considered to have evidence of MAS. Nine of them were boys. The primary diagnosis was systemic onset juvenile idiopathic arthritis. No medication was identified as trigger. Eleven of them had infections prior to MAS. Among them specific infectious agents were identified in four patients. High fever, new onset of hepatosplenomegaly, lymphadenopathy, liver function abnormality, abnormal lipid metabolism and hemophagocytosis were common clinical features. Two cases presented with acute respiratory distress syndrome (ARDS). Multiple organ failure (MOF) occurred in three cases. Three patients died. The variant form (NCBI: SNP rs35947132) of perforin A91V gene was detected in seven systemic onset juvenile idiopathic arthritis compolicated with MAS cases. However no mutation was detected. Clucocorticoid, intravenous immunoglobulin, immunoimpressive therapy were effective treatment of this condition. Plasmapheresis (HP) was successfully used in one case with severe MAS. Conclusions MAS is a rare and potentially fatal complication of childhood rheumatoid diseases such as systemic onset juvenile idiopathic arthritis. In this series, majority of them were male and most of them were preceded by infection. Bone marrow studies support the diagnosis. MOF may be a poor prognostic sign of So-JIA. Aggressive and early therapy is essential. There is no relationship between the variant form (NCBI: SNP rs35947132) of perforin A91V gene and So-JIA with MAS in this small sample's study. More research need to be done by increasing sample's numbers.

To clone and express 27 kDa cysteine protease (CP) gene of Spirometra erinacei plerocercoid,and analyze the biology characteristics,a total of RNA was extracted from the plerocercoids and reversely transcribed into cDNA.The 27kDaCP gene was amplified by PCR and cloned into pM-19T vector for sequencing.The accurate sequence was subcloned into the expression vector pET-28a (+).The recombinant plasmid was transformed into Transetta (DE3) and the expression protein induced by IPTG.The recombinant protein was purified by Ni2 + affinity chromatography,and analyzed by SDS-PAGE and Western blotting.The 27 kDa CP gene and its expression protein were predicted and analyzed by bioinformatics analysis tools such as NCBI and ExPASy.Results showed that the ORF length of 27 kDa CP gene sequence was 1 011 bp,and the removed signal peptide sequence was 954 bp with the submission number of ANA52569 in GenBank.The whole sequence of 27 kDa CP (Mr 35 669.9,pI 5.92) was 317 amino acids conferred from cDNA,which belongs to the Peptidase_C39_like superfamily.The pET-28a (+)-27kDa-CP was expressed under the induction of IPTG.Western blotting analysis showed that the purified protein reach expectancy,and had better response with positive serum of Spirometra erinacei plerocercoid infection.In conclusion,the 27 kDa CP gene of Spirometra erinacei plerocercoid is successfully cloned and expressed and knowing coded sequences and bioinformatic.

Objective To investigate the clinical value of serum insulin-like growth factor-1 (IGF-1),insulin-like growth factor-binding protein-3 (IGFBP-3) and mean height standard deviation score(HtSDS) in recombinant human growth hormone (rhGH)-treated small for gestational age (SGA) children.Methods 32 cases of SGA failed of catch-up growth were subcutaneously injected with rhGH every night before bed at a dose of 0.15-0.20u· kg-1 · d-1.And the variation of serum IGF-1,IGFBP-3,HtSDS,body height and growth velocity (GV) were monitored at 3,6,9 and 12 months of the treatment and compared.Results The GV (cm/years) increased to (12.4 ± 3.2),(11.0 ± 2.3),(10.1 ± 3.5),(9.4 ± 1.8) versus (4.1 ± 0.5) before treatment,suggesting a significant catch-up growth after treatment (F =51.35,P ＜ 0.01).Accordingly,HtSDS increased to (-2.55 ± 0.73),(-2.39 ± 0.65),(-2.21 ± 0.58),(-2.09 ± 0.94) versus (-2.81 ± 0.64),suggesting that height gaps were reduced compared to the normal children of the same age and sex after rhGH treatment (F =4.99,P ＜0.01).The serum levels of IGF-1,IGFBP-3 were significantly increased compared to pre-treatment (F =34.52,14.04,all P ＜ 0.01),especially for serum IGF-1,which maintained at a high level after 3 months of treatment and up to peak after 6 months.However,the ratio of IGF-1/IGFBP-3 showed no increase after treatment (F =1.82,P ＞ 0.05).There was a significant positive correlation between the serum level of IGF-1 and △HtSDS after the rhGH treatment within six months (r3 =0.72,r6 =0.91),while there was no correlation between them after 9 months (r9 =0.26,r12 =0.33).There were no serious adverse events during the rhGH treatment.Conelusion The serum levels of IGF-1,IGFBP-3 were significantly increased after the rhGH treatment,especially for serum IGF-1.There was a significant positive correlation between the serum level of IGF-1 and △HtSDS within six months,but not after 9 months.The rhGH treatment could be a safe and effective strategy for SGA children.

Objective To investigate the immunization effect of influenza A/H1N1 vaccine in health care workers (HCW) in Inner Mongolia Greater Khingan Mountains area. Methods Five hundred and five HCW who received A/H1N1 influenza vaccination (immunized group) and 129 staffs who didn't receive the vaccination (unimmunized group) were randomly sampled for semiquantitative testing of serum H1N1 antibody (IgG) levels by enzyme-linked immunosorbent assay (ELISA).Results were analyzed and stratified by age, sex, occupation and the time interval between the time of vaccination and serum sample collection. The antibody positive rates of the two groups were compared by x2test. Results There were 401 (79. 4%) HCW whose H1N1 antibody were positive and 50 (9.9%) whose antibody were weak positive among 505 immunized HCW. While among 129 unimmunized HCW, there were 59 (45.7%) whose antibody were positive and 15 (11.6%) whose antibody were weak positive. The seroconversion rates of specific antibody were not significantly different among the different age groups after receiving A/H1N1 influenza vaccine (P＞ 0.05).However, there were statistical differences of the seroconversion rates among different sex groups (men 95.7% vs women 87.4% in immunized group, x2=6.40, P＜0.05; and men 73.3% vs women 52.5% in unimmunized group, x2 =4.07, P＜0.05) and different occupation groups (doctor 86.0% vs nurse 94.5% in immunized group, x2 = 9. 16, P＜0.01; and doctor 43. 8% vs nurse 75.0% in unimmunized group, x2=12.61, P＜0.01 ). The seroconversion rate was 81.5% after 80 to 89 days of vaccination, which was significantly lower than those after 30 to 39, 50 to 59 days and 60 to 69 days of vaccination, which was 100.0%, 94.7% and 93.6%, respectively (x2 =3.96, P ＜0.05; x2=7.15, P ＜0. 01; x2 = 9. 98, P＜0. 01). Conclusions A/H1N1 influenza vaccination can induce effective immune response in HCW in Greater Khingan Mountains area of Inner Mongolia. However,the level of specific antibody significantly reduces after 80 to 89 days of vaccination.

Objective To explore the application value of magnetic resonance molecular functional imaging diffusion weighted imaging(DWI) in the identification of pancreatic carcinoma and mass-type pancreatitis of animal model.Methods Each 8 cases of laboratory pancreatic head transplantation tumor model,chronic mass-type pancreatitis model and normal rabbits were selected and performed the MR DWI molecular functional imaging,the b values were 333,667,1 000 s/mm2 respectively.The apparent diffusion coefficients(ADC) of pancreatic carcinoma model,mass-type pancreatitis model and normal pancreas under different b values were observed.Then the change situation of ADC values of pancreatic carcinoma model,mass-type pancreatitis model and normal pancreas under different b values and difference of ADC(DADC) was analyzed.Moreover the differences in molecular diffusion,tissue perfusion among various groups were observed.Results Throughout the study period,the mortality rate of pancreatic head transplantation tumor model was 50%;the mass-type pancreatitis model and 8 normal rabbits were normally survival.The ADC value of pancreatic carcinoma under the same b value was significantly lower than that of chronic inflammation and normal pancreas area.The ADC value in each group was decreased with the increase of b value,and there was significant difference in ADC value when the b value was 333 s/mm2(F=6.662,P=0.014),in the pairwise comparison among groups,the difference between pancreatic cancer and pancreatitis (t=6.773,P=0.003) and between pancreatic cancer and normal pancreas(t=5.883,P=0.016) had statistical significance (P<0.05).The b value was increased,DADC was smaller,the difference change of DADC between pancreatic cancer area and chronic pancreatitis mass area,between pancreatic cancer area and normal pancreatic head area had statistical significance (P<0.05).Conclusion Rationally selecting the molecular functional imaging DWI technology of b value can better distinguish pancreatic cancer from mass-type pancreatitis,which may be promoted and applied in the evaluation of animal pancreatic head cancer model.