Neurofibromatosis type 2 (NF2) is a dominantly inherited genetic condition. Bilateral vestibular schwannoma, which are benign tumors, composed of neoplastic Schwann cells that arise from the eighth cranial nerve, are the hallmark of NF2. Standard approaches for treatment of growing vestibular schwannoma include observation, surgical removal and radiation therapy. Molecular targeted therapies also present great prosperity in recent years. In this review, we summarize the latest progresses on the treatment of NF2-associated vestibular schwannoma.
Humans ; Molecular Targeted Therapy ; Neurofibromatosis 2 ; radiotherapy ; surgery ; therapy ; Neuroma, Acoustic ; radiotherapy ; surgery ; therapy

Objective To explore the effects of salmeterol/fluticasone propionate complicated or combined with N-acetylcysteine on the pulmonary function and arterial blood gas analysis of the patients with chronic obstructive pulmonary disease (COPD), and to evaluate its curative effect.Methods 84 cases of COPD patients were randomly divided into combination treatment group (n=44)and simple treatment group (n=40).The patients in combination treatment group were treated with salmeterol/fluticasone propionate combinated with N-acetylcysteine while the patients in simple treatment group were treated only with salmeterol/fluticasone propionate, both of which were followed up for 6 months.The changes of pulmonary function (FEV1%FVC,FEV1%Pred,PEF daily variation rate:ΔPEF%)and the arterial blood gas analysis indexes (PaO2 and PaCO2 )of the patients in two groups were recorded before treatment, 3 months after treatment, and 6 months after treatment. Results The FEV1%FVC,FEV1%Pred and PaO2 of the patients in combination treatment group and simple treatment group were obviously increased 3 and 6 months after treatment compared with before treatment (P<0.01 ), and the PaCO2 were obviously decreased (P<0.01).And there were significant differences of the indexes mentioned above of the patients between two groups (P<0.05 );but the indexes of each group had no significant differences between 3 months and 6 months after treatment (P>0.05).TheΔPEF% of the patients in two groups had no significant differences between inter-group and intra-group before and after treatment (P > 0.05 ). Conclusion Combination of salmeterol/fluticasone propionate and N-acetylcysteine can obviously improve the pulmonary function and arterial blood gas analysis indexes of the COPD patients, which is superior to the simple application of salmeterol/fluticasone propionate and has definite and lasting curative effect on the treatment of COPD.

Objective To evaluate the clinical outcomes of pedicle screw fixation in the treatment of Hangmans fracture. Methods The 15 patients who had been treated for acute Hangmans fracture in our department after 1999 were retrospectively analyzed. 4 of them were type I, and 11 type II. All the patients presented symptoms of nerve system at different degrees. They were treated with AO pure titanium cortex screw through isthmus after skull traction replacement. Results All the patients were followed up for 12 to 60 months. According to Frankle classification, 6 cases of Grade D and 5 cases of Grade C recovered up to Grade E, 2 cases of Grade C recovered to Grade D, 1 case of Grade B recovered to Grade C and 1 case of Grade B recovered to Grade D. X ray showed all the fractures healed up without vertebra artery injury or other complications. Conclusion The outcomes of pedicle screw fixation and reduction in treatment of acute Hangmans fracture are satisfactory, for the clinic technique promotes functional recovery of spinal cord, decreases the rate of infection and scarcely affects the function of upper cervical spine.

Transient receptor potential M2 (TRPM2) ion channel is a non-selective cationic channel that can permeate calcium ions, and plays an important role in neuroinflammation, ischemic reperfusion brain injury, neurodegenerative disease, neuropathic pain, epilepsy and other neurological diseases. In ischemic reperfusion brain injury, TRPM2 mediates neuronal death by modulating the different subunits of glutamate N-methyl-D-aspartic acid receptor in response to calcium/zinc signal. In Alzheimer's disease, TRPM2 is activated by reactive oxygen species generated by β-amyloid peptide to form a malignant positive feedback loop that induces neuronal death and is involved in the pathological process of glial cells by promoting inflammatory response and oxidative stress. In epilepsy, the TRPM2-knockout alleviates epilepsy induced neuronal degeneration by inhibiting autophagy and apoptosis related proteins. The roles of TRPM2 channel in the pathogenesis of various central nervous system diseases and its potential drug development and clinical application prospects are summarized in this review.
Amyloid beta-Peptides/metabolism* ; Humans ; Neurodegenerative Diseases ; Neuroglia ; TRPM Cation Channels/genetics* ; Transient Receptor Potential Channels

Objective To examine the changing levels of serum iron and hemoglobin in Streptococcus pneumoniae-induced rat pneumonia model and the relationship among infection severity,serum iron and hemoglobin.Methods Sixty Sprague Dawley (SD)rats were randomly assigned to one of the four groups,1 5 SD rats in each group,including three treatment groups and one control group. Low, medium and high doses of bacteria were administered to the animals in the treatment groups respectively through tracheal cannula.The severity of pneumonia was indicated by the level of bacterial load.The animals in the control group did not receive bacterial challenge.The mean serum iron and hemoglobin levels were calculated on day 3,5, and 7 to analyze the relationship between bacterial dose and serum iron or hemoglobin levels.Results The serum iron level in the animals receiving medium or high doses of bacteria was significantly lower compared with that in control group on day 3 (P=0.009,P=0.005).The serum iron level in the animals receiving low dose of bacteria showed significant difference compared with that in the control group on day 5 (P=0.007).The hemoglobin level in the animals receiving medium or high doses of bacteria was significantly different from that in the control group on day 5 (P=0.031,P=0.046).The hemoglobin level in the animals receiving low dose of bacteria did not show significant difference compared with that in the control group on day 3,5 or 7.The bacterial dose level was negatively correlated with the mean level of serum iron (correlation coefficient r=-0.65,r=-0.53,r=-0.61,respectively).There was no definite correlation between the bacterial dose and the mean hemoglobin level.Conclusions Streptococcus pneumoniae infection may be associated with lower serum iron and hemoglobin levels in rats.The severity of infection is negatively correlated with serum iron level,but not hemoglobin level.

Objective To explore the clinical effects of Gonadotropin releasing hormone agonist (GnRH-a) combined with levonorgestrel-releasing intrauterine system (LNG-IUS) in the treatment of refractory adenomyosis by magnetic resonance-guided high intensity focused ultrasound (MRgHIFU).Methods 80 patients with refractory adenomyosis admitted to our hospital from April 2015 to June 2017 were randomly divided into observation group and control group.They were treated with MRgHIFU as observation group.LNS-IUS was placed after the third injection of GnRH-a as control group.The uterine volume,pain score,menstrual volume and adverse reactions were compared between the two groups at 3 and 6 months after treatment.Results After 6 months of treatment,the uterine volume of the observation group [(100.23 ±4.65) cm3] was significantly lower than that of the control group [(145.46 ± 10.23) cm3],with statistically significant difference (t =6.01,P ＜ 0.05).After 6 months,the degree of dysmenorrhea in the observation group was significantly lower than that in the control group [(2.12 ± 0.24) point vs (0.27 ± 0.09) point],with statistically significant difference (t =5.21,P ＜ 0.05).At 3 and 6 months after treatment,the menstrual volume of the observation group was significantly lower than that of the control group [(64.67 ± 10.12) point vs (96.45 ± 15.12) point,(42.46 ± 11.18) point vs (66.26 ± 12.48) point],with statistically significant difference (t =4.51,P ＜ 0.05;t =3.58,P ＜ 0.05).The adverse reactions such as vaginal bleeding,hot flashes and night sweats were significantly lower than those in the control group (x2 =3.967,P ＜ 0.05).Conclusions As a new non-invasive and refractory treatment of adenomyosis,high intensity focused ultrasound guided by magnetic resonance is safe and effective with less adverse reactions,which is worthy of clinical application.

Objective:To investigate the risk factors of low anterior resection syndrome (LARS)after low anterior resection of rectal cancer (Dixon).Methods:This retrospective study was conducted in Peking University First Hospital and Traditional Chinese Medicine Hospital of Shanxi Provice from Jan 2012 to Jun 2019. A cohort of 504 patients with rectal cancer was enrolled in the study. All the patients underwent anterior resection. The relationship between clinical-pathological data were analyzed retrospectively. Univariate analysis using χ 2 test. Logistic regression analysis was used to screen the influencing factors of LARS, and the Nomogram method was used to score each factors. Results:Univariate analysis showed that BMI≥28 kg/m 2(χ 2=9.450, P=0.002), the distance from the lower edge of the tumors to the anus <6 cm (χ 2=12.070, P=0.001), high ligation of the inferior mesenteric artery (IMA) (χ 2=8.279, P=0.004), preoperative neoadjuvant therapy (χ 2=11.230, P=0.001), postoperative anastomotic leakage (χ 2=11.840, P=0.001) were associated with severe LARS.Multivariate analysis showed that the distance from the lower edge of the tumors to the anus <6 cm ( OR=1.861, 95% CI: 1.289-2.688, P=0.001), BMI≥28 kg/m 2 ( OR=1.747, 95% CI: 1.022-2.987, P=0.041), high IMA ligation ( OR=1.688, 95% CI: 1.157-2.463, P=0.007), preoperative neoadjuvant therapy ( OR=2.719, 95% CI: 1.343-5.505, P=0.005) were independent risk factors for LARS. Nomogram model showed that the total factor ranged from 2 to 212, and the corresponding risk rate ranged from 30% to 80%. The patients with higher score have greater risk for severe LARS. The area under the predictive power curve of Nomogram model (AUC) was 0.749 (95% CI: 0.705-0.793, P<0.001). Conclusion:Lower tumor location, obesity, preoperative neoadjuvant therapy, high IMA ligation and postoperative anastomotic leakage increase the risk of severe LARS.

Objective To explore the clinical effects of magnetic resonance guided high intensity focused ultrasound ( MRgHIFU ) and gonadotropin releasing hormone agonist ( GnRH-a ) combined with levonorgestrel-releasing intrauterine system (LNG-IUS) in the treatment of recurrent uterine fibroids. Methods Eighty patients of recurrent uterine fibroids from April 2015 to June2017 in the First Hospital of Harbin were collected and randomly divided into the observation group and control group. MRgHIFU was used in the observation group were employed,and patients in the control group were treated with ING-IUS after first injecting GnRH-a. The tumor volume,hemoglobin level,menstrual quantity and adverse reactions were compared before and at 3 months,6 months after treatment. Results After 6 months,the size of the uterine myoma in the observation group was ((80. 23 ± 4. 35) cm3 ),significantly lower than that of the control group (( 135. 46 ±9. 25) cm3 ),and the difference was statistically significant ( t = 6. 01,P < 0. 05) . At 3 and 6 months after treatment,the amount of menstruation in the observation group was significantly lower than that of the control group( (66. 37 ± 12. 12) points vs. (40. 46 ± 10. 15) points; (98. 42 ± 14. 12) points vs. ( 68. 26 ± 11. 38) points). The difference was statistically significant (t at 3 months after treatment= 4. 52,P<0. 05,t at 6 months after treatment= 3. 53,P<0. 05). After 6 months of treatment,the hemoglobin value of the observation group was significantly higher than that of the control group (( 142. 12 ± 4. 24) g/ L vs. ( 108. 27 ± 3. 09) g/ L), the difference was statistically significant (t= 5. 02,P<0. 05),while the adverse reactions such as vaginal bleeding, hot flashes and night sweats were lower than those of the control group (2. 5% (1/ 40) vs. 7. 5% (3/ 40)) (χ2= 3. 99,P<0. 05). Conclusion Magnetic resonance guided high intensity focused ultrasound was considered as safe and effective in treating uterine fibroids,which is a new and noninvasive therapeutic methed,

OBJECTIVETo study the clinicopathologic characteristics and prognosis of rectal neuroendocrine neoplasms (NENs).

METHODSFrom January 2000 to May 2017, 84 patients were diagnosed as NENs by pathology and underwent surgical treatment in Peking University First Hospital. Their clinicopathological characteristics, surgial options and prognosis were analyzed retrospectively.

RESULTAmong these 84 cases, 67 cases were NET G1, 6 cases were NET G2, 10 cases were NEC G3 and 1 case was MANEC G3. The median size was 0.8 (0.2 to 18.0) cm. There were 60 cases of stage I(, 2 cases of stage II(, 12 cases of stage III(, 10 cases of stage IIII(. Forty-nine patients accepted examinations because of non-specific symptoms, including altered bowel habits(22/49), bloody stool (19/49) and abdominal pain(10/49); the other 35 cases including 2 patients with liver metastasis were diagnosed by endoscopy or CT during routine physical examination. Forty-four patients received endoscopic ultrasonography(EUS) with 100% of sensitivity and 90.9% of accuracy. Among 20 cases (23.8%) with lymph node metastasis (all ≥T2 stage), 12 cases were NET G1 and G2 (1 case of multiple NET G1) and 8 cases were NEC G3 and MANEC G3. The lymph node metastasis rate of stage T1 NET G1 and G2 was lower than that of stage T2 to T4 NET G1 and G2, also lower than that of NEC G3 and MANEC G3 (all P=0.000), however, stage T2 to T4 NET G1 and G2 showed the similar rate of lymph node metastasis with NEC G3 and MANCE G3(P>0.05). Synchronously distant metastasis was found in 10 (11.9%) patients at the first diagnosis, and ovarian metastasis was found in 1 case 9 years after curative resection of rectal NEN. Among 81 patients receiving operation, 57 patients underwent endoscopic mucosal resection (56 patients of stage T1 NET G1 and G2); 3 patients local excision without lymph node dissection; 13 patients curative resection; 1 patient curative resection with liver metastasis resection; 6 patients palliative surgery and 1 patient metastatic lesion resection only. Overall follw-up time was 1 month to 169 months, and the 3- and 5-year survival rates were 87.7% and 79.7% respectively. No recurrence or metastasis was observed in all the 62 patients with T1 G1 and G2, including 56 cases of ESD, 3 cases of local excision, 3 cases of curative resection, whose 3-year and 5-year survival rates were both 96%. The prognosis was closely associated with grade and stage of NENs (all P=0.000).

CONCLUSIONSThe early symptoms of rectal NENs are insidious and atypical, therefore some patients are diagnosed as stage II( or higher at their first consultation. ESD is safe and effective for NET G1 and G2. The prognosis depends on grade and stage of NENs.