Juvenile retinoschisis (RS or XLRS,MIM#312700)is a rare X-linked inherited disorder,mainly affects bilateral retina,and is characterized by cartwheel-like changes of the macular region of the retina and schisis or splitting within the inner retinal layers,leading to visual deterioration.The electroretinogram is beneficial in the diagnosis of juvenile retinoschisis.The a-wave can be of normal or nearly normal amplitude in this disorder,whereas the amplitude of the b-wave is appreciably reduced,giving a decrease in the proportion of b/a.The responsible gene,XLRSl,maps to Xp22 and was identified by positional cloning.This paper makes a brief review about the latest XLRS research of pathogenesis,animal experiments,clinical therapy,and 25 references are cited.

Hernia, Ventral ; surgery ; Humans ; Laparoscopy ; methods ; Surgical Mesh

[Objective]To investigate the indications,complications and mid-term results of total knee arthroplasties using rotating hinge knee prosthesis. [Methods]Thirty total knee arthroplasties using rotating hinge prosthesis were performed in twenty-eight patients from July 1998 to July 2008.Seven patients were male and twenty-one patients were female.The average age was 62 years(42-75 years).The diagnosis included osteoarthritis in 8 patients,rheumatoid arthritis in 2 patients,traumatic arthritis in 3 patients,Paget's disease patient,serious genu valgum in 7 patients and 10 failure TKAs.All patients were followed up for 12 to 120 months.The patients were evaluated according to knee score system and the complications were observed.[Results]The pain was relieved and function was improved in all knees.The average knee pain score increased from 19.5 to 77.9,and knee function score from 22.5 to 50.5.Range of motion of knees increased from 58.4?to 72.8?.No revision was needed in all patients,The complications included anterior knee pain(8 cases),extension lag(5 cases),prosthesis broken(1 case) and patella tendon rupture(1 case).[Conclusion]The mid-term results of total knee arthroplasty using rotating hinge knee for severe knee deformity are satisfactory.Complications should be prevented carefully.

Objective:To explore the e ect of QingreJiedu herbs in combination with Jiegeng decoction on expression of NF-?B p65 in ALI induced by LPS.Methods:Male Sprague-Dawley rats were randomly divided into normal group,model control group,QingreJiedu group,and the group of QingreJiedu herbs in combination with Jiegeng decoction.The model of ALI was made by injected with a single dose of LPS through vena femorali,the pathologic morphology,MPO activity and the expression of NF-?B p65 was observed.Result:Compared with normal group,infiltration.of PMNs,capillary congestion and swelling were found in model control group,and the content of MPO and expression of NF-?B p65 in the nuclear were significantly increased(P

Child ; Consensus ; Diarrhea ; diagnosis ; therapy ; Humans

Objective To evaluate the efficacy and safety of endoscopic mucosal resection (EMR) for rectal carcinoid tumors. Methods From January 2006 to January 2009, EMR was performed in 28 patients with rectal carcinoids, who were followed up to evaluate the therapeutic effect and safety. Results Tumor diameters varied from 0.4 cm to 1. 2 cm (mean 0.7± 0. 2 cm). Negative resection margin was a-chieved in 26 cases (92. 9% ), tumor margin within 0. 1 cm of resection margin in 1 (7. 1% ) , and two margins coincided in 1 patient (7. 1% ). Hemostasis was performed with metal clips in 14 patients (50% ) and argon plasma coagulation (APC) in 9 (32. 1% ). Except for rectal bleeding in 1 patient (3. 6% ) , no other complications were observed. There was no recurrence in any patients during a follow-up of 6-36 months. Conclusion EMR is a useful and safe method for treatment of small rectal carcinoid tumor which does not cross submucosal layer.

A total of 105 patients with ketosis-onset diabetes were divided into four groups (A+B-,A + B +,A - B -,A - B +) based on the results of islet-associated autoantibodies (A +,A - ) and β-cell function (B +,B - ).Nearly 94％ patients in the A -B + group and 1/10 patients in the A - B - group were non-insulin dependent.While in the A + B - and A + B + groups,the detection rates of subsequent non-insulin dependency were 20％ and 58％,respectively.Among the four groups,patients in the A - B + groups had a lower level of IL-6 and a higher level of TNF-α. Patients in the A + B - and A + B + groups had a higher level of IL-18.Our study indicates that patients with ketosis-onset diabetes may present different clinical characteristics. Evaluate the islet-associated autoantibodies and pancreatic β-cell function may be helpful to the clinical diagnostic and therapeutic strategies in diabetes.

Objective To compare the appearances between chronic mastitis and breast cancer on MRI and investigate the differ-ential diagnostic value.Methods MRI data of 20 patients with chronic mastitis pathologically proved by biopsy or operation were ret-rospectively analyzed.30 cases of breast cancer were contemporaneously chosen as the control group.Morphological feature and dy-namic contrast-enhanced(DCE)manifestation of the lesions were evaluated and statistical difference was compared between mastitis and breast cancer.Morphological feature included configuration,spiculated sign,ring-like enhancement,peri-focal edema,skin thick-ening,nipple involvement and axillary lymph nodes enlargement.DCE manifestation contained calculating early enhancement ratio and drawing time-intensity curve (TIC).Results There were significant statistical differences among configuration,ring-like en-hancement and peri-focal edema between mastitis and breast cancer,respectively.No statistical differences could be found among spiculated sign,skin thickening,nipple involvement and axillary lymph nodes enlargement.Early enhancement ratio in the group of mastitis was 1.1 56±0.635 while 1.253±0.499 in the group of breast cancer and there was no statistical difference between them. There were 1 1 cases with type Ⅰ TIC,6 with type Ⅱ,3 with type Ⅲ in the lesions of mastitis,while 4 with type Ⅰ,1 1 with typeⅡ,1 5 with type Ⅲ in the lesions of breast cancer and significant statistical differences could be found between two groups.Conclu-sion Mastitis usually manifests as non-mass-like lesions on MRI.Ring-like enhancement,peri-focal edema and benign type TIC can be applied to discriminate mastitis from breast cancer.

Objective To observe the clinical features of acute macular neuroretinopathy (AMN).Methods Six patients (11 eyes) with AMN were included in this study,with every 2-week follow-ups till six months.Among them,five had preceding dengue fever (83.3％),one had history of head trauma (16.7％).All patients received routine examination,fundus photography,infrared reflectance (IR) imaging,spectral-domain optical coherence tomography (SD-OCT) scanning and fluorescein fundus angiography (FFA) initially,and fundus photography,IR,SD-OCT during follow-up.Results Sudden onset of central/paracentral scotoma in one eye or both eyes was the main visual symptom.There were 1 eye with normal fundus,2 eyes with wedge-shape lesions,8 eyes with yellow-white or brown sheet lesion.IR imaging demonstrated localized areas of hypo-reflection in the macula.SD-OCT scanning through these areas revealed hyper-reflection in the photoreceptor layer and disruption of its normal reflective structures.Subsequent SD-OCT demonstrated that the hyper-reflection of the photoreceptor layer regressed gradually,followed by thinning of the outer nuclear layer.The external limiting membrane and ellipsoid zone became continuous;however,the interdigitation zone was not restored.There was no remarkable findings of the AMN lesions on FFA.The scotomas persisted in all 6 patients (11 eyes) by the last visit.Conclusions IR imaging demonstrated localized areas of hypo-reflection in the macula.SD-OCT revealed hyper-reflection in the photoreceptor layer in acute stage and the interdigitation zone was not restored in late stage.AMN has a relative poor prognosis with persistent scotomas through at least 6 months.

Objective To explore the efficiency and safety of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosis and treatment of children with pancreaticobiliary diseases.Methods A retrospective study was conducted by data collection from 74 children with biliary and pancreatic diseases undergoing ERCP from January 2012 to December 2016 for observation of therapeutic efficacy and postoperative complications.Results A total of 96 ERCPs were performed on 74 patients.The success rate was 94.8％ (91/96) using 29 operating methods.The complication rate of pancreatitis was 10.8％ (8/74),as well as hyperamylasemia was 13.5％ (10/74) and stress hyperglycemia was 10.8％ (8/74).Conclusion The application of ERCP in children with biliary tract and pancreatic diseases is effective and safe.