Objective To investigate the genetic causes of auditory neuropathy with optic atrophy in a family.Methods The proband's medical history and family history were inquired in detail,and relevant clinical examina-tions were performed to confirm the diagnosis of auditory neuropathy with optic atrophy,and the genetic pedigree of the family was drawn.Peripheral blood of proband(Ⅲ-7)was collected for whole exome sequencing,and the patho-genicity of the detected mutations were interpreted.Blood samples of proband's wife(Ⅲ-8),eldest daughter(Ⅳ-7),second daughter(Ⅳ-9)and son(Ⅳ-10)were tested for mutation sites by Sanger sequencing.Combined with clinical manifestations and examination results,the family was studied.Results The genetic pattern of this family was autosomal dominant.The proband showed decreased visual acuity at the age of 19,bilateral sensorineural deaf-ness at the age of 30,and decreased speech recognition rate.Among 20 members of the family of 5 generations,10(2 deceased)showed similar symptoms of hearing and visual impairment.Proband(Ⅲ-7),eldest daughter(Ⅳ-7)and son(Ⅳ-10)underwent relevant examination.Pure tone audiometry showed bilateral sensorineural deafness.ABR showed no response bilaterally.The 40 Hz AERP showed no response in both ears.OAE showed responses in some or all of the frequencies.No stapedial reflex was detected.The eye movement of Ⅲ-7 and Ⅳ-10 were reasona-ble in all directions,and color vision was normal.Ocular papilla atrophy was observed in different degrees in fundus examination.OCT showed thinning of optic disc nerve fibers in both eyes,and visual evoked potential showed pro-longed P100 wave peak.They were diagnosed as hereditary auditory neuropathy with optic atrophy.A mutation of the OPA1 gene c.1334G＞A(p.Arg445His,NM_015560.2)at a pathogenic locus on chromosome 3 was detected by whole exon detection in Ⅲ-7.The results of generation sequencing analysis showed that the OPA1 gene c.1334G＞A(p.Arg445His,NM_015560.2)mutation of chromosome 3 was also found in Ⅳ-7 and Ⅳ-10.Meanwhile,the gen-otypes of Ⅲ-8 and Ⅳ-9 were wild homozygous,that is,no mutation occurred.Conclusion The OPA1 c.1334G＞A(p.Arg445His,NM_015560.2)mutation site might be the pathogenic mutation in this family.

ObjectiveTo mine the medication patterns of Chinese medicines for neurodermatitis based on contemporary medical cases in published articles. MethodThe medical cases of treating neurodermatitis with Chinese medicines were retrieved from the medical case articles published by contemporary famous and old Chinese medicine doctors in the library of Shandong University of Traditional Chinese Medicine， CNKI， VIP， and Wanfang Data. A case library was established， and SPSS Statistics 26.0 and SPSS Modeler 18.0 were employed to analyze the symptoms and syndromes of neurodermatitis and mine the medication patterns. ResultAccording to the inclusion and exclusion criteria， 130 medical case articles were included in this study. Neurodermatitis was prevalent in young adults between 20 and 39 years old （female patients of 30-49 years old and male patients of 20-39 years old）， and male patients were more than female patients. The patients mainly presented the clinical manifestations of itchy rashes， thickened skin， and lichenification. Symptoms included skin injury， emotional abnormalities， and Yin damage caused by prolonged illness. Red tongue， thin white or yellow tongue coating， and wiry pulse were common in the patients. The patients with the syndrome of blood deficiency and wind dryness were often treated with Angelicae Sinensis Radix， Rehmanniae Radix， Glycyrrhizae Radix et Rhizoma， Tribuli Fructus， and Chuanxiong Rhizoma. The commonly used herb pairs included Chuanxiong Rhizoma-Paeoniae Radix Alba， Chuanxiong Rhizoma-Glycyrrhizae Radix et Rhizoma， and Rehmanniae Radix Praeparata-Saposhnikoviae Radix， and the commonly used prescriptions were Siwutang and Dangguiyinzi. The patients with the syndrome of muscle and skin dystrophy were mainly treated with Rehmanniae Radix， Sophorae Flavescentis Radix， Paeoniae Radix Alba， Tribuli Fructus， and Dictamni Cortex. The commonly used herb pairs included Polygoni Multiflori Caulis-Sophorae Flavescentis Radix， Polygoni Multiflori Caulis-Dictamni Cortex， and Salviae Miltiorrhizae Radix et Rhizoma-Paeoniae Radix Alba， and the commonly used prescriptions were Jingjie Siwutang and Baixianpiyin. The patients with the syndrome of liver depression transforming into fire were often treated with Rehmanniae Radix， Gentianae Radix et Rhizoma， Gardeniae Fructus， Bupleuri Radix， and Scutellariae Radix. The commonly used herb pairs included Gentianae Radix et Rhizoma-Polygoni Multiflori Caulis， Polygoni Multiflori Caulis-Gardeniae Fructus， and Gentianae Radix et Rhizoma-Saposhnikoviae Radix， and the commonly used prescriptions were Longdan Xiegantang and Danzhi Xiaoyaosan. ConclusionThis study enriches the knowledge about neurodermatitis， clarifies the treatment principles and methods as well as the medication patterns， and provides a theoretical basis for clinical treatment and medication based on syndrome differentiation.

Objective:To examine the effect of pancreaticojejunostomy with pancreatic duct binding external drainage in laparoscopic pancreatoduodenectomy.Methods:The data of 21 patients who underwent laparoscopic pancreaticoduodenectomy in the same treatment group from January 2017 to October 2019 in Department of Hepatobiliary Surgery of the Second Hospital of Hebei Medical University were analyzed retrospectively.All patients underwent pancreaticojejunostomy with external drainage of pancreatic ducts.There were 12 males and 9 females, aged (63.1±8.1)years old (range: 46 to 77 years old), body mass index (24.8±3.2)kg/m 2(range: 18.8 to 29.1 kg/m 2).There were 3 cases of hypertension, 5 cases of diabetes, 3 cases of hypertension and diabetes, 3 cases of liver cirrhosis. Results:Laparoscopic pancreatoduodenectomy was successfully performed in all 21 patients.The operation time was (359.3±71.0)minutes, the pancreaticojejunostomy time was (23.8±7.4)minutes, the diameter of pancreatic duct was(3.3±0.6)mm, the intraoperative blood loss was (247.6±90.1)ml, the postoperative hospital stay was(13.7±4.9)days, the leakage of B-level fistula occurred in 1 case(4.8%), and there was no C-level pancreatic fistula.There were 3 cases of bile leakage, 1 case of incision infection, 2 cases of gastroparesis, 1 case of hydrops abdominis, no death and secondary operation.Conclusion:It is a simple and easy method of pancreatoenterostomy with pancreatic duct binding external drainage, which can reduce the incidence of pancreatic fistula and related complications after laparoscopic pancreatoduodenectomy for patients with high risk pancreatic fistula.

Objective:To examine the effect of pancreaticojejunostomy with pancreatic duct binding external drainage in laparoscopic pancreatoduodenectomy.Methods:The data of 21 patients who underwent laparoscopic pancreaticoduodenectomy in the same treatment group from January 2017 to October 2019 in Department of Hepatobiliary Surgery of the Second Hospital of Hebei Medical University were analyzed retrospectively.All patients underwent pancreaticojejunostomy with external drainage of pancreatic ducts.There were 12 males and 9 females, aged (63.1±8.1)years old (range: 46 to 77 years old), body mass index (24.8±3.2)kg/m 2(range: 18.8 to 29.1 kg/m 2).There were 3 cases of hypertension, 5 cases of diabetes, 3 cases of hypertension and diabetes, 3 cases of liver cirrhosis. Results:Laparoscopic pancreatoduodenectomy was successfully performed in all 21 patients.The operation time was (359.3±71.0)minutes, the pancreaticojejunostomy time was (23.8±7.4)minutes, the diameter of pancreatic duct was(3.3±0.6)mm, the intraoperative blood loss was (247.6±90.1)ml, the postoperative hospital stay was(13.7±4.9)days, the leakage of B-level fistula occurred in 1 case(4.8%), and there was no C-level pancreatic fistula.There were 3 cases of bile leakage, 1 case of incision infection, 2 cases of gastroparesis, 1 case of hydrops abdominis, no death and secondary operation.Conclusion:It is a simple and easy method of pancreatoenterostomy with pancreatic duct binding external drainage, which can reduce the incidence of pancreatic fistula and related complications after laparoscopic pancreatoduodenectomy for patients with high risk pancreatic fistula.

Objective To explore the value of endoscopic retrograde cholangiopancreatography (ERCP) in patients with choledocholithiasis after Billroth Ⅱ gastrointestinal anastomosis.Methods A retrospective cohort study was conducted to retrospective review the data of 189 patients with choledocholithiasis treated by ERCP from December 2015 to November 2017 in Department of Hepatobiliary Surgery,Second Hospital of Hebei Medical University.According to the history of Billroth Ⅱ gastrointestinal anastomosis,the patients who have not undergone digestive surgery were divided into the normal group(n =167) and patients who have undergone digestive surgery were divided into reconstruction group (n =22).The operation time,the success rate of stone extraction,complications,the hospitalization time,total hospitalization expenses were compared between the two groups.Measurement data with normal distribution were represented as (Mean ± SD) and comparison between groups was analyzed using the t test.Measurement data with skewed distribution were described as M (range).Comparisons of count data were analyzed using the x2 test or Fisher exact probability.Results The operation time of the normal group was (40.18 ± 11.80) min,and the success rate of ERCP was 97.60％ (163/167),the operation time of reconstruction group was(61.81 ± 13.21) min,and the success rate of ERCP was 81.82％ (18/22),There were significant differences between the two groups (t =0.105,x2 =10.400,P ＜ 0.05).The complications,the hospitalization time and the total hospitalization expense of the normal group were 16.17％ (27/167),(3.47 ± 1.55) d,(20 620.69 ± 3 117.88) yuan,the reconstruction group were 18.18％ (4/22),(4.18 ± 2.08) d,(22 426.41 ±5 916.30) yuan,with no statistically significant difference (x2 =0.000,t =4.204),t =10.828,P ＞ 0.05).Conclusions ERCP is safe and feasible for patients with choledocholithiasis after Billroth Ⅱ gastrointestinal anastomosis.It also has a high success rate of stone removal and small trauma,which is worthy of promotion.

Objective: To investigate the clinicopathologic characteristics of intractable epilepsy. Methods: Based on the classification criteria proposed by the International League Against Epilepsy (ILAE), a retrospective analysis of the pathological characteristics was done in 822 patients who underwent epilepsy surgery in Sanbo Brain Hospital, Capital Medical University, from June 2008 to December 2012. Results: The mean age of epilepsy onset was 9.9 years, mean duration of epilepsy was 11.9 years. Complex partial seizures were the main presenting features. Histopathological study showed 33 cases (4.01%) with mild forms of cortical malformations, 690 cases (83.94%) with focal cortical dysplasia (FCD) and 99 cases with others (including 39 pure hippocampal sclerosis, 20 cystosclerosis, 19 Sturge-Weber syndrome, 8 tuberous sclerosis complex, 6 without significant pathological changes, 5 gyral malformations and 2 hamartoma). Among the 690 FCD cases, 106 were FCD typeⅠ, 91 were FCD typeⅡ and 493 were FCDⅢ(Ⅲa: 160, Ⅲb: 106, Ⅲc: 26 and Ⅲd: 201). Conclusions FCDⅢd is the most common histopathological subtype causing intractable epilepsy, mainly due to focal hypoxia/ischemia in the perinatal period, which results in scarring of local brain tissue; this is followed by other isolated forms of FCD (FCDⅠand FCDⅡ), and then FCD Ⅲa and FCD Ⅲb. The reason to distinguish isolated forms of FCD (types Ⅰ and Ⅱ) from FCD Ⅲ and to subclassify FCD Ⅲ is to allow better definition of cortical dyslamination. Therefore, the pathogenic factors of intractable epilepsy can be grouped in greater details, and facilitate the diagnosis and potential curative treatment of intractable epilepsy.

Objective:To explore the effect of inherent depression on chronic visceral hypersensitivity. The differences of visceral sensitivity, colitis, and brain activation between Fawn-Hooded ( FH/Wjd) and Sprague-Dawley( SD) rats were identified after neonatal colon acetate stimulation.Methods:The specific pathogen free Fawn-Hooded (FH/Wjd) and Sprague-Dawley(SD) rats were used to establish irritable bowel syndrome (IBS) model.The visceral sensitivity was measured by colorectal distension (CRD). The expression of 5-hydroxytryptamine (5-HT), mast cell (MC), indoleamine 2,3-dioxygenase (IDO) in colon and IDO in specific cerebral regions were detected through immunohistochemistry.Results:Ab-dominal withdrawal reflex ( AWR) scores showed that visceral sensitivity of acetate-enema groups was sig-nificantly higher than that of saline-enema groups ( FH/Wjd:2.44 ±0.04 vs.1.96 ±0.07, P<0.05;SD:1.75 ±0.13 vs.1.32 ±0.05, P<0.05).Furthermore, FH/Wjd rats of IBS group scored signifi-cantly higher than SD rats of IBS group (2.44 ±0.04 vs.1.75 ±0.13, P<0.05).The MC amounts of both SD and FH/Wjd IBS group rats were significantly more than those of their control groups ( FH/Wjd:43.24 ±1.72 vs.24.92 ±1.38, P <0.01.SD:23.80 ±1.28 vs.14.24 ±0.92, P <0.01).
Besides, the MC amounts of control and IBS group of FH/Wjd rats were significantly more than that of SD IBS group rats ( P<0 .01 ) .The IDO and 5-HT positive cells in colonic mucosa of IBS group of both SD and FH/Wjd rats were significantly more than those of their control groups, respectively(P <0.01). The IDO, 5-HT positive cells in colonic mucosa of both control and IBS group of FH/Wjd rats were significantly more than those of both control and IBS group of SD rats ( control:IDO,24.64 ±2.22 vs. 15.52 ±1.39;5-HT,21.32 ±1.26 vs.12.72 ±1.12.IBS: IDO,44.92 ±2.31 vs.20.85 ±1.72;5-HT, 31.84 ±1.57 vs.19.65 ±1.09.P <0.01).The expression of IDO in prelimbic cortex (PrL) areas of FH/Wjd IBS rats was significantly higher than that of IBS group of SD rats (49.60 ±4.31 vs. 35.60 ±2.42, P <0.01) , and the expression of IDO in rostral anterior cingulate cortex ( rACC) areas of FH/Wjd IBS rats was significantly more than that of FH/Wjd control rats (45.44 ±1.16 vs.34.08 ± 2.76, P <0.01) .Conclusion:Inherent depressive FH/Wjd rats were more sensitive to neonatal colon acetate stimulation, presenting as visceral hypersensitivity which maybe associated with increased MC amounts and over-expression of 5-HT and IDO in colon, suggesting that depression disorder may aggra-vate functional disturbance of gastrointestinal tract by regulating the response to inflammatory stimulation.

Objective To study the values of glycosylated hemoglobin in screening for patients with prediabetic state in Guang-zhou region .Methods 525 Guangzhou people who had accepted health examination were enrolled and were subjected to oral glucose tolerance test(OGTT) .BIO-RAD D-10 automatic glycosylated hemoglobin analyzer was employed to detect their glycosylated he-moglobin A1c(GHbA1c) .OGTT results were served as diagnostic criteria ,Receiver operator characteristic (ROC) curve analysis was performed to obtain the optimal threshold of GHbA1c in diagnosing impaired glucose regulation (IGR) .Results The optimal threshold of GHbA1c in diagnosing IGR was 5 .95% .The sensitivities of GHbA1c≥5 .95% and GHbA1c≥5 .7% in diagnosing IGR were 53 .3% and 84 .8% ,respectively ,while their specificities were 72 .8% and 31 .0% ,respectively .The difference of sensitivity between GHbA1c≥5 .95% combined with FPG≥5 .6 mmol/L and GHbA1c≥5 .7% alone in diagnosing IGR showed no statistical significance(P= 0 .406) ,while the specificity increased obviously (P= 0 .000) .Conclusion The criteria of GHbA1c≥5 .7% can be used for prediabetic state screening but not for diagnosis .GHbA1c≥5 .95% combined with FPG≥5 .6 mmol/L can be used effectively for prediabetic state screening in Guangzhou people .

To explore the current status of children receiving primary health services at a community health service center in Shenzhen so as to provide references for the formulation of children health care policies.According to the patient electronic data in 2011,the clinical profiles were analyzed for children aged under 7 years seeking the advice of general practitioners (GPs) at Niuhu Community Health Service Center.Children aged under 7 years accounted for 12.4％ of all subjects.The causes of seeing the GPs were as follows:acute upper respiratory.infection(58.2％),acute bronchitis(14.9％),gastroenteritis(5.6％),trauma(3.3％)and dermatitis(3.2％).And no difference existed in the workload between pediatrists and surgeons,internists or herbalists.The common and frequent community diseases may be diagnosed and managed at a community health service center.

Objective To explore the feasibility of inherent depressive Fawn-Hooded (FH/Wjd)rats as a comorbidity model of mental disorder and irritable bowel syndrome (IBS) by comparing visceral sensitivity of FH/Wjd rats and Sprague-Dawley (SD) rats. Methods Depression trait of FH/Wjd rats was validated through forced swimming test (FST) and sucrose preference test (SPT).Visceral sensitivity to colorectal distention (CRD) under various pressures was assessed by abdominal withdrawal reflex (AWR). The expression of 5-HT in rats' colon,c-fos expression in colon and brain areas of infralimbic cortex (IL),prelimbic cortex (PrL) and rostral anterior cingulated cortex (rACC)was tested with immunohistochemistry. Results FST indicated that the immobility time of FH/Wjd rats was significantly longer than that in SD rats (t=-8. 931,P＜0. 01). SPT showed that the ratio of sucrose water in total liquid consumed was significantly lower in FH/Wjd rats than that of SD rats(t=4. 155,P=0. 01). At each CRD pressure gradient (20,40,60 and 80 mm Hg,1 mm Hg=0. 133kPa),AWR score was all significantly higher in FH/Wjd rats than that of SD rats (t=-2. 697,-3.464,-6.822 and -3. 976,P=0. 022,0.006,0.000 and 0.003). The expression level of 5-HT in FH/Wjd rats' control group was significantly higher than that of SD rats' control group (t=-11.371,P=0. 000). Compared with their own control group,5-HT expression increased both in FH/Wjd rats dilated groups and SD rats dilated groups,and the expression level in FH/Wjd rats was higher than that of SD rats (t= -3. 364,P= 0. 007). There was no significant difference of c-fos expression in colon and prefrontal brain areas between FH/Wjd rats' control group and SD rats'control group (colon:t=-0. 129,P= 0. 900;IL:t=-1. 316,P= 0. 218;PrL:t=1. 241,P = 0. 243;rACC:t=2. 151,P = 0. 057). Compared with their own control group,the expression of c-fos in colon and prefrontal brain areas significantly increased both in FH/Wjd rats dilated groups and SD rats dilated groups,and the expression level in FH/Wjd rats was higher than that of SD rats (colon: t=- 5.864,P= 0.000;IL:t=-2. 530,P = 0. 030;PrL:t=-7. 039,P= 0. 000;rACC:t=-6. 489,P=0. 000). Conclusions Inherent depressive FH/Wjd rats present visceral hypersensitivity. Meanwhile,the expression of 5-HT in colon is high,and with hyperreactivity to visceral noxious stimuli in colon and brain IL,PrL,and rACC areas. FH/Wjd rats might be a comorbidity animal model of mental disorder and IBS.