Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease.NOTCH3 missense mutation causes its coded cysteine occurring odd change and then affects the conformation and function of protein of NOTCH3.The abnormal NOTCH3 protein has vascular smooth muscle toxicity and finally deposits in the cerebral small blood vessels and causes the disease.Usually,CADASIL can be suspected by its typical clinical manifestations and neuroimaging findings.Its diagnosis needs genetic testing or skin biopsy to find the outer granular osmiophilic deposits of small vascular smooth muscle cells or immunohistochemical NOTCH3-ECD staining positive.For nearly two decades,the studies on genetics,pathogenesis,clinical manifestations,and diagnostic techniques of CADASIL have made great progress,however,many important questions have not been fully clarified and have new discoveries,such as the NOTCH3 gene mutation pattern and loci,and the relationship between gene phenotype and clinical phenotype,optimization of diagnosis process,depth study of pathogenic mechanism,exploration of new discoveries,new therapeutic targets and concepts.This article reviews the genetic characteristics,pathogenesis,and clinical diagnosis and treatment technology of CADASIL.

Objective:To observe the therapeutic effect of electroacupuncture (EA) at local points plus point injection on House-Brackmann facial nerve function classification grade (H-B) and facial disability index (FDI) in pregnant women with Bell palsy (BP). Methods:A total of 40 eligible BP patients during pregnancy were randomized into a treatment group (n=20) and a control group (n=20). The treatment group was intervened by EA at Sizhukong (TE 23), Tongziliao (GB 1), Sibai (ST 2), Quanliao (SI 18), Yifeng (TE 17), Dicang (ST 4), Jiache (ST 6) and point injection at Zusanli (ST 36) and Yanglingquan (GB 34) on the affected side, 30 min every time, 10 sessions as a course, for 2 courses in total while control group by EA with the same points, manipulation and courses as the treatment group. Results:After the treatment, the H-B classification in the treatment group was better than that in the control group, with a significant inter-group difference (P<0.05); after the treatment, the change of FDI score in the treatment group was significantly different from that in the control group (P<0.05). Conclusion:EA at local points plus point injection can produce a more significant efficacy than EA in the treatment of BP during pregnancy.

Objective To investigate the risk factors and the influence of intravenous thrombolysis of acute cerebral infarction with cerebral microbleeds(CMBs)by SWI.Methods 1 64 patients with acute cerebral infarction were enrolled in this study.All pa-tients were scanned with routine MRI and SWI.According to the presence of CMBs on SWI,the patients were classified into two groups:CMBs group(73 cases)and non-CMBs group(91 cases).Past history was recorded and risk factors of CMBs were explored. 76 cases patients(including 35 cases of CMBs group and 41 cases of non-CMBs group)were treated by intravenous thrombolysis and rescanned with routine MRI and SWI to compare the changes in the number of CMBs and hemorrhage transformation 24 hours after thrombolysis.Results The difference age,hypertension,lacunar infarction and leukoaraiosis between the two groups were significant (P <0.05).The difference of CMBs and hemorrhagic transformation between the two groups treated by thrombolysis were not sig-nificant(P >0.05).Conclusion Acute cerebral infarction with CMBs are influenced by age,hypertension,lacunar infarction and leu-koaraiosis.Thrombolysis in acute cerebral infarction with CMBs can not augment the incidence of hemorrhagic transformation.

OBJECTIVETo evaluate the clinical efficacy and mechanism of Feiyangqin Rectum Condensed Liquid (FRCL) in treating children syncytial viral pneumonia. Methods Seventy-two patients were randomly divided into two groups, the 36 patients in the treated group were treated with FRCL, and the other 36 patients in the control group simply treated with Western medicine. Efficacy of treatment on clinical condition and some immune function (IgA, IgG, CD3, CD4) were observed.

RESULTSIn the treated group, 28 patients were cured (77.8%), treatment was markedly effective in 4 patients (11.1%), effective in 2 (5.5%) and ineffective in 2 (5.6%), with the total effective rate of 94.4%. The corresponding number in the control group was 20 (55.6%), 7 (19.4%), 6 (16.7%), 3 (8.3%) and 91.7%, respectively. The cure rate in the treated group was obviously superior to that in the control group (P < 0.05). FRCL could improve serum IgA, IgG, CD3, CD4, and CD4/CD8, lower serum IgE, these indexes in the treated group were significantly different to those in the control group (P < 0.05).

CONCLUSIONFRCL had the action in treating children syncytial viral pneumonia without any adverse reaction, one of its mechanisms might be related to its regulation on immune function.

Administration, Rectal ; Anti-Infective Agents ; therapeutic use ; Child, Preschool ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; administration & dosage ; Female ; Humans ; Infant ; Male ; Phytotherapy ; Pneumonia, Viral ; drug therapy ; immunology ; Respiratory Syncytial Virus Infections ; drug therapy ; immunology

Objective To summarize our experience in robotic-assisted laparoscopic surgery for adrenal diseases Methods The clinical data of 243 patients with adrenal tumor treated by robotic-assisted laparoscopic surgery from March 2010 to February 2017 were retrospectively reviewed.There were 99 men and 144 women.The mean age was 51.6 years (range, 19-84).Tumors located at left adrenal in 140 cases, right in 97 cases,and both sides in 6 cases.The average diameter was 3.32 cm (range, 0.8-12 cm).However, there were 41 cases whose tumor diameter were greater than 5 cm.Results There were 2 cases of conversion during operation, 1 case converted to open surgery and the other to the traditional laparoscope surgery.The mean operative time was 35 min (range, 20-130 min).Estimated blood loss was 80 ml (range,20-1 200 ml).Blood transfusion was needed in 6 cases.The mean postoperative hospital stay was 5d (range, 3-20 d).The pathological diagnosis included 37 cases of pheochromocytoma, 149 cases of cortical adenoma, 3 cases of cortical carcinoma, 5 cases of metastatic tumor, 9 cases of adrenal myelolipoma, 3 cases of adrenal cyst, 2 cases of bronchogenic cyst, 25 cases of adrenal nodular hyperplasia,2 cases of angiomyolipomas, 1 case of mature teratoma, 1 case of diffuse large B-Cell lymphoma, 1 case of angioma, and 4 cases of neurofibromatosis.Conclusions Robotic-assisted laparoscopic adrenalectomy was safe and effective.Robotic-assisted laparoscopic surgery has the advantages over laparoscopic surgery in treatment of complicated adrenal tumor, such as large adrenal tumors, pheochromocytoma, bilateral adrenal diseases, overweight and obese patients with adrenal diseases.

Objective: This study aimed to evaluate the zygomaticotemporal suture (ZTS) maturation, analyze the age distribution patterns of ZTS maturation stages, and investigate the relationship between ZTS and cervical vertebral maturation (CVM). Methods: A total of 261 patients who underwent cone-beam computed tomography (112 males, mean age, 13.1 ± 3.3 years; 149 females, mean age, 13.7 ± 3.1 years) were examined to evaluate the ZTS stages. The ZTS stages were defined based on a modified method from previous studies on zygomaticomaxillary sutures. Differences between groups and correlations between indicators were analyzed using the Spearman correlation test, intraclass coefficient of correlation (ICC), one-way analysis of variance and rank sum test.Statistical significance was set at p < 0.05. The diagnostic value of CVM stages in identifying ZTS maturation stages was evaluated using positive likelihood ratios (LRs). Results: A positive relationship was found between the ZTS and CVM stage (r = 0.747, ICC = 0.621, p < 0.01) and between the ZTS stage and chronological age (r = 0.727, ICC = 0.330, p < 0.01). Positive LRs > 10 were found for several cervical stages (CSs), including CS1 and CS2 for the diagnosis of stage B, CS1 to CS3 for the diagnosis of stages B and C, and CS6 for the diagnosis of stages D and E. Conclusions The ZTS maturation stage may be more relevant to the CVM stage than to the chronological age. The CVM stages can be good indicators for clinical decisions regarding maxillary protraction, except for CS4 and CS5.

Background: Implant-supported dentures have become an essential means of restoring occlusal function after jaw reconstruction. Bone mineral density (BMD) may influence the success rate of implant denture restorations. This study aimed to explore whether the Hounsfield unit (HU) value can be used to monitor the changing trend of fibular BMD after jaw reconstruction. Results: A total of 54 patients who underwent maxillar/mandibular reconstruction with a fibula flap were included in this study. There was a significant correlation between the HU value and BMD at 1 week, 3 months, and 6 months after surgery, and both were significantly correlated with follow-up time. The difference between each pair of absorption rates (DAR) was less than 10% in 66.7% and 75.9% of patients at 3 and 6 months; however, the DAR was more than 20% in 12% and 13.8% of patients at 3 and 6 months, respectively. Conclusions There is a significant correlation between HU value and BMD. The HU value can be used to roughly reflect the fibular BMD changing trend in a group of patients as opposed to an individual, and the HU value is not equivalent to BMD.

Objective To explore the clinical feasibility of robot-assisted laparoscopic radical cystectomy (RARC) with total intracorporeal othotopic ileal neobladder (TIOIN).Methods A consecutive series of 4 patients (2 male,2 female),who underwent RARC with TIOIN by a single surgeon,were included in the retrospective study,between March 2017 and June 2017.Their age ranged from 59 to 71 years,which the mean age was (65.7 ± 4.9) years.Preoperative urinary CT scan,cystoscopic examination and transurethral resection of bladder tumor were performed for diagnosis.Among these,2 patients underwent side-to-side bowel anastomosis using a linear stapler,while hand-sewn anastomosis was performed in the other 2 patients.The detubularized bowel segment was arranged in a U shape,and then the two medial borders were closed to create the posterior wall of the neobladder,which completed a partial U shape and anastomosed with the end of urethra.After placing the single J stents into the ureter,the uretero-neobladder was anastomosed.To close the urine reservoir,each border of the U-shaped segment was folded again and sutured to form a sealed pouch.Results All operations were performed successfully.The average operation time for RARC was 93.2 min (ranging 79-117 min).The average operation time for urinary diversion was 214.2 min (ranging 163-251 min).The mean estimated blood loss was 304.5 ml (ranging 200-400 ml).The mean hospital stay was 20.5 d (ranging 13-32 day).The number of dissected lymph node ranged from 11 to 16 (mean 3.7 ± 2.6).All the surgical margins were negative.The time for postoperative out-of-bed activity and bowel function recovery was 2-3 days and 3-4 days,respectively.The single-J stents were removed 1 months after operation,generally.No urine leakage was noticed after removing the drainage tube and catheter.The lymph leakage was observed in one case,which was resolved 15 days post-operatively after given nutrient therapy.The performance of urinary continence was satisfactory,except one patient complained about the nocturnal incontinence.After the regular pelvic exercise,the symptom improved two months after the operation.Hydronephrosis and intestinal leakage were not observed.Conclusions Our initial experience showed that RARC with TIOIN is feasible and alterative for experienced surgeon.

Objective: To investigate the prevalence of calf muscle cramps and possible influencing factors for pregnant women in China. Methods: Using a multi-stage stratified probability proportional to size cluster randomization sampling method during 2010-2012. A total of 3 582 pregnant women were investigated at 150 counties from 31 provinces in China mainland. Information on calf muscle cramps, demographic socio-economic status, pregnancy information, and the physical activities was collected through questionnaires. The semi-quantitative food frequency questionnaire (FFQ) was used to collect food intake of pregnant women. Dynamic cluster analysis was used to assess dietary pattern. Multiple logistic regression was used to investigate the possible influencing factors for calf muscle cramps. Results: The prevalence of calf muscle cramps was 32.9% (1 180/3 582) in Chinese pregnant women, which was 11.6% (87/748), 28.2% (420/1 492), and 50.2% (673/1 342), respectively, during the first, second and third trimester. There were significant differences between them (χ²=349.16, P<0.001). Dietary patterns of the pregnant women were classified into three groups, which called relatively balanced pattern, high vegetables and fruits pattern, and high dairy pattern. Among the three groups, the prevalence of calf muscle cramps was 32.0% (952/2 971), 37.2% (186/500), and 37.8% (42/111), with significant differences (χ²=6.39, P=0.041). The OR (95%CI) values of calf muscle cramps in the second and third trimester was 2.96 (2.28-3.83), and 8.02 (6.16-10.44), respectively, comparing with the first trimester. The OR (95%CI) values of calf muscle cramps in the women taking calcium before pregnant was 1.45 (1.19-1.76), comparing with the one who was not taken. The OR (95%CI) values of calf muscle cramps in the women who had been diagnosed by pregnancy-induced hypertension was 5.76 (2.06-16.12), comparing with the one who had not been diagnosed. The OR (95%CI) values of calf muscle cramps in the high vegetables and fruits pattern and high dairy pattern was 1.13 (0.91-1.41), and 1.18 (0.76-1.81), respectively, comparing with the relatively balanced pattern. Conclusion The prevalence of calf muscle cramps was relatively high in Chinese pregnant women, which was significantly different among three trimesters. The residential areas, occupation, and pregnancy-induced hypertension might be related to the prevalence of calf muscle cramps. However, there was no significant difference among different dietary patterns.

Objective: To investigate the prevalence of anemia and related risk factors for pregnant women in China. Method: Based on Chinese National Nutrition and Health Surveillance 2010-2012, a total of 3 501 pregnant women were investigated from 150 counties of 31 provinces, municipalities and autonomous regions in China, using a multi-stage stratified cluster randomization sampling method. General information of pregnant women, health status, and food intake during the gestation, was collected through a questionnaire investigation. 6 ml fasting venous blood was collected for the determination of hemoglobin concentration. Data were analyzed using multiple logistic regression to investigate the prevalence of anemia and the related influencing factors. Results: The 605 of 3 501 pregnant women had anemia. The prevalence of anemia was 17.2%; and mild anemia accounted for about 61.0% (369/605). Compared with the pregnant women living in the large cities, the OR (95%CI) of those living in the poor rural areas was 1.46 (1.08-1.98). Compared with the pregnant women living in the south area of China, the OR (95%CI) of those living in the north area of China was 1.39 (1.15-1.68); Compared with the pregnant women in the first trimester, the OR (95%CI) of those in the second trimester and the third trimester were 1.79 (1.33-2.43) and 2.11 (1.56-2.85), respectively. The OR (95%CI) of pregnant women who had used folic acid supplementation within the 6 months prior to gestation was 0.76 (0.63-0.93) compared with those who had not used. Conclusion From 2010 to 2012, the epidemic characteristics of anemia was mild for the pregnant women in China, and pregnant women residential areas, periods of pregnancy and whether to take folic acid were related to anemia.