AIM:To evaluate the diagnostic value of panoramic immersion B-scan ultrasonography(Pano-immersion B-scan,PIB)in complex retinal detachment(RD),persistent hyperplastic primary vitreous(PHPV)and intraocular tumors.METHO...AIM:To evaluate the diagnostic value of panoramic immersion B-scan ultrasonography(Pano-immersion B-scan,PIB)in complex retinal detachment(RD),persistent hyperplastic primary vitreous(PHPV)and intraocular tumors.METHODS:The clinical data of 44 patients collected from May 2012 to December 2019 in Chinese PLA General Hospital was retrospectively studied.All of these patients underwent PIB of the eye,because it was difficult to diagnose by routine ocular fundus examination,conventional ultrasound or/and ultrasonic biomicroscope(UBM)due to opacity of refractive media,pupillary occlusion,large involvement or special location of the lesion.The imaging features of difficult cases in PIB were analyzed.The diagnosis accuracy rating of PIB were evaluated and contrasted with conventional ultrasound or UBM by the standard of intraoperative diagnosis or/and pathological results.RESULTS:According to intraoperative diagnosis or pathological results as gold standard,among the 44 cases,there were 19 cases missed diagnosis,misdiagnosed or difficult-to-diagnose by conventional ultrasound or UBM,including 4 cases of long-standing RD difficult to diagnose,4 cases misdiagnosed,and 11 cases incompletely observed or miss diagnosed.The diagnostic accuracy rate of PIB and conventional ultrasound or UBM were 100%(44/44)and 56.82%(25/44),and the sensitivity of them were 100%and 56.82%.All the patients underwent PIB and were diagnosed as RD(15 cases),retinal and choroidal detachment(4 cases),subchoroidal hematocele(1 case),vitreous opacity and/or organic membrane formation(4 cases),PHPV(12 cases),iris and/or ciliary body tumors(3 cases),and choroidal tumors(6 cases).According to the intraoperative diagnosis or pathological results,the diagnostic coincidence rate of PIB was 100%,which was significantly higher than conventional ultrasound and UBM.CONCLUSION:PIB can help to accurately diagnose complex RD,PHPV,and intraocular masses with special location or/and excessive size.It has important diagnostic value for patients with equivocal findings at conventional ultrasound examination.展开更多
AIM:To evaluate the accuracy of axial length(AL)measurements obtained from immersion B-scan ultrasonography(immersion B-scan)for intraocular lens(IOL)power calculation in patients with high myopia and cataracts.METHOD...AIM:To evaluate the accuracy of axial length(AL)measurements obtained from immersion B-scan ultrasonography(immersion B-scan)for intraocular lens(IOL)power calculation in patients with high myopia and cataracts.METHODS:Immersion B-scan,contact A-scan ultrasonography(contact A-scan),and the IOLMaster were used to preoperatively measure the AL in 102 eyes from 102 patients who underwent phacoemulsification and IOL implantation.Patients were divided into two groups according to the AL:one containing patients with22 mm≤AL【26 mm(group A)and the other containing patients with AL≥26 mm(group B).The mean error(ME)was calculated from the difference between the AL measurement methods predicted refractive error and the actual postoperative refractive error.RESULTS:Ingroup A,ALs measured byimmersion Bscan(23.48±1.15)didn’t differ significantly from those measured by the IOLMaster(23.52±1.17)or from those by contact A-scan(23.38±1.20).In the same group,the standard deviation(SD)of the mean error(ME)of immersion B-scan(-0.090±0.397 D)didn’t differ significantly from those of IOLMaster(-0.095±0.411 D)and contact A-scan(-0.099±0.425 D).In group B,ALs measured by immersion B-scan(27.97±2.21 mm)didn’t differ significantly from those of the IOLMaster(27.86±2.18 mm),but longer than those measured by Contact A-scan(27.75±2.23 mm,P=0.009).In the same group,the standard deviation(SD)of the mean error(ME)of immersion B-scan(-0.635±0.157 D)didn’t differ significantly from those of the IOLMaster(-0.679±0.359 D),but differed significantly from those of contact A-scan(-0.953±1.713 D,P=0.028).CONCLUSION:ImmersionB-scanexhibitsmeasurement accuracy comparable to that of the IOLMaster,and is thus a good alternative in measuring AL in eyes with high myopia when the IOLMaster can’t be used,and it is more accurate than the contact A-scan.展开更多
Steroid 5β-reductase [aldo-keto reductase family 1 member D1(AKR1D1)] is essential for bile acid biosynthesis. Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and...Steroid 5β-reductase [aldo-keto reductase family 1 member D1(AKR1D1)] is essential for bile acid biosynthesis. Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and cholestasis. There is still limited experience regarding the treatment of this disease. We describe an infant who presented with hyperbilirubinemia and coagulopathy but normal bile acid and γ-glutamyltransferase. Gene analysis was performed using genomic DNA from peripheral lymphocytes from the patient, his parents, and his elder brother. The patient was compound heterozygous for c.919C>T in exon 8 and exhibited a loss of heterozygosity of the AKR1D1 gene, which led to an amino acid substitution of arginine by cysteine at amino acid position 307(p.R307C). Based on these mutations, the patient was confirmed to have primary 5β-reductase deficiency. Ursodeoxycholic acid(UDCA) treatment did not have any effect on the patient. However, when we changed to chenodeoxycholic acid(CDCA) treatment, his symptoms and laboratory tests gradually improved. It is therefore crucial to supplement with an adequate dose of CDCA early to improve clinical symptoms and to normalize laboratory tests.展开更多
BACKGROUND Endoscopic balloon dilatation (EBD) has become the first line of therapy for benign esophageal strictures (ESs);however,there are few publications about the predictive factors for the outcomes of this treat...BACKGROUND Endoscopic balloon dilatation (EBD) has become the first line of therapy for benign esophageal strictures (ESs);however,there are few publications about the predictive factors for the outcomes of this treatment.AIM To assess the predictive factors for the outcomes of EBD treatment for strictures after esophageal atresia (EA) repair.METHODS Children with anastomotic ES after thoracoscopic esophageal atresia repair treated by EBD from January 2012 to December 2016 were included.All procedures were performed under tracheal intubation and intravenous anesthesia using a three-grade controlled radial expansion balloon with gastroscopy.Outcomes were recorded and predictors of the outcomes were analyzed.RESULTS A total of 64 patients were included in this analysis.The rates of response,complications,and recurrence were 96.77%,8.06%,and 2.33%,respectively.The number of dilatation sessions and complications were significantly higher in patients with a smaller stricture diameter (P=0.013 and 0.023,respectively) and with more than one stricture (P=0.014 and 0.004,respectively).The length of the stricture was significantly associated with complications of EBD (P=0.001).A longer interval between surgery and the first dilatation was related to more sessions and a poorer response (P=0.017 and 0.024,respectively).CONCLUSION The diameter,length,and number of strictures are the most important predictive factors for the clinical outcomes of endoscopic balloon dilatation in pediatric ES.The interval between surgery and the first EBD is another factor affectingresponse and the number of sessions of dilatation.展开更多
BACKGROUND Gastrointestinal cytomegalovirus(CMV)disease occurs commonly in immunocompromised/immunodeficient patients with advanced human immunodeficiency virus infection,neoplasm,solid organ transplantation,hematopoi...BACKGROUND Gastrointestinal cytomegalovirus(CMV)disease occurs commonly in immunocompromised/immunodeficient patients with advanced human immunodeficiency virus infection,neoplasm,solid organ transplantation,hematopoietic stem cell transplantation,or treatment with immunosuppressants,but is rarely reported in association with measles infection.CASE SUMMARY We describe a case of extensive gastrointestinal CMV disease secondary to measles infection in a 9-mo-old boy who presented with persistent fever and bloody diarrhea.His condition was improved after ganciclovir treatment.Serological analysis of CMV showed negative immunoglobulin(Ig)M and positive IgG.Blood CMV-DNA was 9.26×103 copies/mL.The diagnosis of gastrointestinal CMV disease was confirmed by histopathological findings of intranuclear and intracytoplasmic inclusions and Owl’s eye inclusion.This case highlights the differential diagnosis and histopathological characteristics of gastrointestinal CMV infection and laboratory tests.CONCLUSION Extensive gastrointestinal CMV lesions can be induced by the immune suppression secondary to measles infection.Rational,fast,and effective laboratory examinations are essential for suspected patients.展开更多
Based on the 16 scenes GF-1 satellite multi-spectral remote sensing images,through the adoption of data processing methods including orthorectification,geometric rectification,data fusion and image mosaic,integrated w...Based on the 16 scenes GF-1 satellite multi-spectral remote sensing images,through the adoption of data processing methods including orthorectification,geometric rectification,data fusion and image mosaic,integrated with field surveys,the remote sensing interpretation signs for the inland wetland types have been built,and the remote sensing survey of inland wetlands in Yadong region has been initiated,with six types of inland wetlands recognized in Yadong region,namely permanent rivers,seasonal rivers,lakes,salt lakes,alpine meadows,and inundated land.The spatial distribution characteristics and the spreading rules of these wetlands have also been revealed.Based on full understanding of the overall characteristics of the inland wetlands in the Yadong region,using the three phases of TM images acquired in 1989,2003 and 2008 as well as the PMS2 data gathered by GF-1 in 2014,and the wide-range data(WFV3)gathered by GF-1 in 2020.As to the typical salt lakes,a long-time salt lakes transition study was carried out.The results show that the typical salt lakes in Yadong have been shrinking in the past three decades.The average annual shrinkage of Duoqing Co(Co means lake in Tibetan)was stronger than that of Gala Co,which are respective 87.30 hectares(usually short as ha;1 ha equals to 0.01 km^(2))/a and 24.20 ha/a;the shrinkage degree of Gala Co was higher than that of Duoqing Co,shrank by 59.27% and 35.73% respectively.Based on the remote sensing survey results and an integrated analysis of the predecessors’researchers,the reason for the shrinkage of the salt lakes is more inclined to geological factors.Geological process is manifested by a series of extensional faults at the bottom of the lake basin generated from tectonic activities,providing fluid infiltration channels,and inducing the eventual leakage of lake water to the lower strata.The result provides an important instance for understanding the evolution characteristics of wetlands and salt lakes in specific environment of the Tibetan Plateau.展开更多
·AIM: To evaluate the accuracy of 20 MHz immersion Bscan ultrasonography in observing lens and to investigate the value of this noninvasive preoperative diagnosis method in alkali burn eyes.·METHODS: It was ...·AIM: To evaluate the accuracy of 20 MHz immersion Bscan ultrasonography in observing lens and to investigate the value of this noninvasive preoperative diagnosis method in alkali burn eyes.·METHODS: It was a comparative study. Fifty-six cases(56 eyes) of alkali burn eyes were examined by ultrasound biomicroscopy(UBM) and immersion 20 MHz B-scan ultrasonography from June 2011 to April 2013,the images were analyzed, and the ultrasonographic diagnosis compared with the operation results.·RESULTS: In 56 alkali burn eyes examined by UBM, the lens were not detected in 16 eyes; the IOL could be detected in 2 eyes; the anterior lens capsule surface or/and the front lens could be detected in 18 eyes, and lens opacification in 3 eyes of them; suspected abnormal lens were detected in the other 20 eyes. In all the same eyes examined by immersion 20 MHz B-scan ultrasonography,the lens were not detected in 16 eyes; the IOL could be detected in 2 eyes; 24 abnormal lens(opacity, lens expansion, shrinkage) and 14 normal lens were found.Compared with the intraoperative findings, the diagnostic accordance rate of the immersion 20 MHz B-scan appearance of lens was 100%(56/56), which was significantly higher than examined by UBM 57.14%(32/56)(χ2=30.55, P =0.0000).·CONCLUSION: Immersion 20 MHz B-scan ultrasonography can observe the lens accurately in alkali burn eyes. It has important clinical value to combine with UBM in eyes of alkali burn.展开更多
AIM:To evaluate the accuracy of segmented measurement of axial length(AL)in high myopia filled with silicone oil by immersion B-scan ultrasonography(immersion B-scan).METHODS:From June 2016 to June 2020,a total of 67 ...AIM:To evaluate the accuracy of segmented measurement of axial length(AL)in high myopia filled with silicone oil by immersion B-scan ultrasonography(immersion B-scan).METHODS:From June 2016 to June 2020,a total of 67 ultra-high myopia inpatients(67 eyes)who underwent silicone oil removal combined with cataract extraction and intraocular lens(IOL)implantation were retrospectively enrolled.The preoperative axial length(AL)of 31 patients with severe cataract were segmented measured using immersion B-scan(B-scan group)and another 36 patients with mild or moderate cataract were measured using IOLMaster 500(IOLMaster group).The post-operative ALs in two groups were both measured using IOLMaster 500.The IOL power was calculated with Haigis formula.The differences in ALs between pre-and post-surgery,as well as the postoperative refractive spherical equivalent,absolute refractive error,the prediction deviation of postoperative refraction and best corrected visual acuity(BCVA)were compared.RESULTS:The pre-and post-operative ALs were 30.46±1.63 mm(range 28.09-33.51 mm)and 30.42±1.70 mm(range 28.03-33.90 mm)in B-scan group(t=0.644,P=0.542)and 30.51±1.21 mm(range 28.03-33.90 mm)and 30.43±1.27 mm(range 28.54-33.50 mm)in IOLMaster group(t=1.843,P=0.074),respectively.Three months after surgery,BCVA were 0.45±0.13(range 0.3-0.9)and 0.44±0.20(range 0.2-1.0)in B-scan and IOLMaster group respectively(t=0.086,P=0.932).There was no significant difference of the postoperative spherical equivalent(-3.11±0.65 D vs-3.21±0.51 D,t=0.671,P=0.505)and the absolute refractive error(0.589±0.340 vs 0.470±0.245 D,t=1.615,P=0.112)between two groups.In B-scan group,absolute refractive error within±0.50 D was found in 18 eyes(58.1%),within±1.00 D in 26 eyes(83.9%),and within±1.50 D in 31 eyes(100%).In IOLMaster group,absolute refractive error within±0.50 D was found in 23 eyes(63.9%),within±1.00 D in 34 eyes(94.4%),and within±1.50 D in 36 eyes(Z=0.757,P=0.449).CONCLUSION:The segmented measurement of ALs by immersion B-scan shows comparable measurement accuracy with that of IOLMaster 500 in ultra-high myopia patients with severe cataract secondary to silicone oil filling and can obtain an ideal postoperative refractive state.展开更多
Objective To investigate the effects of the flexible surgical approaches on therapeutic and cosmetic outcomes of facial nevi.Methods From August 2002 to January 2008,16 cases with facial nevi had been treated in our w...Objective To investigate the effects of the flexible surgical approaches on therapeutic and cosmetic outcomes of facial nevi.Methods From August 2002 to January 2008,16 cases with facial nevi had been treated in our wards with the selected approaches.Surgical approaches including serial excision or one-time radical excision,free skin graft,and expanded flap were adopted in accordance with the individual size and location of the facial nevi.Results All cases experienced complete excision and had satisfactory cosmetic appearance in the end.Conclusion The flexible surgical measures help to minimize the risk of malignant transformation and achieve good cosmetic results.展开更多
AIM:To present the clinical manifestations of 5 autosomal dominant cone-rod dystrophy(ad CORD)patients from two Chinese families with cone-rod homeobox(CRX)mutation(p.R41W),and to explore the clinical heterogeneity of...AIM:To present the clinical manifestations of 5 autosomal dominant cone-rod dystrophy(ad CORD)patients from two Chinese families with cone-rod homeobox(CRX)mutation(p.R41W),and to explore the clinical heterogeneity of ad CORD with CRX mutation(p.R41W).METHODS:Interrogation and ophthalmological examinations were undertaken in all patients and unaffected members.Analysis of clinical features was performed by visual acuity,slit lamp examination,visual field examination,fundoscopy,autofluorescence and spectral domain optical coherence tomography.Targeted next-generation sequencing was applied as a useful tool to identify the causative mutation of CORD genes.RESULTS:A CRX missense mutation c.121C>T was identified in all patients,resulting in an amino acid change from arginine acid to tryptophan(p.R41W).The patients presented with early onset,progressive and different severities with CORD.CONCLUSION:This is the first report of the clinical phenotype of CRX mutation(p.R41W)in Chinese families,and the mutation can lead to a wide range of various retinal phenotypes.展开更多
Objective:To explore the correlation of different hypertension grades with carotid atherosclerotic plaque in middle-aged patients with hypertension.Methods:A total of 300 patients with primary hypertension who were ad...Objective:To explore the correlation of different hypertension grades with carotid atherosclerotic plaque in middle-aged patients with hypertension.Methods:A total of 300 patients with primary hypertension who were admitted in the Cardiology Department and Neurology Department of our hospital from January, 2015 to September, 2016 were included in the study and divided into 3 groups with 100 cases in each group according to the hypertension grade criteria. Moreover, 100 normal individuals who came for physical examinations were served as the control group. TCD was used to detect MCA, ACA, PCA, VA1, and BA. CDFI was used to detect CCA, ICA, ECA, and VA2. EDV, PSV, PI, and RI were detected, respectively. The nitrate reductase colorimetric method was used to detect NO, MDA, and SOD.Results:PSV and EDV in the internal carotid artery system in patients with hypertension were significantly reduced (P<0.05), while PI and RI were significantly increased (P<0.05);moreover, with the increasing of hypertension grading, PSV and EDV were gradually reduced, while PI and RI were increased. PSV and EDV in the vertebral artery system in patients with hypertension were significantly reduced (P<0.05), while PI and RI were significantly increased (P<0.05);moreover, with the increasing of hypertension grading, PSV and EDV were gradually reduced, while PI and RI were increased. SOD and NO in patients with hypertension were significantly lower than those in the control group (P<0.05), while MDA was significantly higher than that in the control group (P<0.05);moreover, with the increasing of hypertension grading, SOD and NO were gradually reduced, while MDA was gradually increased.Conclusions: TCD in combined with CDFI can make a comprehensive evaluation of hemodynamic indicators of intracranial and extracranial vessels in patients with hypertension, and is of great significance in the early detection of intracranial and extracranial arteriosclerosis.展开更多
Objective:To explore the correlation of carotid IMT (cIMT) with Hcy, Cyst-C, UA, and Lp(a) in middle-aged patients with H-type hypertension.Methods:A total of 120 middle-aged patients with H-type hypertension who were...Objective:To explore the correlation of carotid IMT (cIMT) with Hcy, Cyst-C, UA, and Lp(a) in middle-aged patients with H-type hypertension.Methods:A total of 120 middle-aged patients with H-type hypertension who were admitted in our hospital from July, 2015 to July, 2016 were included in the study. The color Doppler ultrasound diagnostic apparatus was used for the two-dimensional ultrasound examination. The patients were divided into the normal group, thickening group, and plaque group with 40 cases in each group according to the atherosclerosis (AS). The full automatic biochemical analyzer was used to detect the plasma Hcy, Cyst-C, UA, Scr, Bun, TC, TG, LDL-C, and HDL-C. Emulsion turbidity method was used to detect Lp(a).Results:With the thickening of cIMT, Hcy, Cyst-C, Lp(a), and UA levels were elevated, while HDL-C level was reduced.Conclusions:The serum Hcy, Cyst-C, UA, and Lp(a) levels in middle-aged patients with H-type hypertension are positively correlated with cIMT, are the independent risk factors for developing AS, and can be served as the early indicators to estimate the occurrence and development of AS.展开更多
An experimental setup for cold extrusion process with electric-hydraulic chattering was developed and its working principle was introduced. The finite element (FE) model for a kind of cup part (material: 20Cr) wa...An experimental setup for cold extrusion process with electric-hydraulic chattering was developed and its working principle was introduced. The finite element (FE) model for a kind of cup part (material: 20Cr) was built by using the software Deform-3D. FE simulation experiments with and without electric-hydraulic chattering were carried out to analyze the velocity fields and the metal grid flow lines. The extrusion ex- periments of the cup part were also performed under different conditions. The difference of metal flow lines with and without electric-hydraulic chattering was discussed via a scanning electron microscope (SEM) and the Keyence super-depth three-dimensional microscopic system. The results showed that with the electric-hydraulic chattering, the velocity of material flow increases, whereas deformation resistance decreases. Electric hydraulic chattering results in easy metal flow, small bending degree of metal flow lines, slender and dense metal grains, and thereby an improved quality of the deformed parts.展开更多
文摘AIM:To evaluate the diagnostic value of panoramic immersion B-scan ultrasonography(Pano-immersion B-scan,PIB)in complex retinal detachment(RD),persistent hyperplastic primary vitreous(PHPV)and intraocular tumors.METHODS:The clinical data of 44 patients collected from May 2012 to December 2019 in Chinese PLA General Hospital was retrospectively studied.All of these patients underwent PIB of the eye,because it was difficult to diagnose by routine ocular fundus examination,conventional ultrasound or/and ultrasonic biomicroscope(UBM)due to opacity of refractive media,pupillary occlusion,large involvement or special location of the lesion.The imaging features of difficult cases in PIB were analyzed.The diagnosis accuracy rating of PIB were evaluated and contrasted with conventional ultrasound or UBM by the standard of intraoperative diagnosis or/and pathological results.RESULTS:According to intraoperative diagnosis or pathological results as gold standard,among the 44 cases,there were 19 cases missed diagnosis,misdiagnosed or difficult-to-diagnose by conventional ultrasound or UBM,including 4 cases of long-standing RD difficult to diagnose,4 cases misdiagnosed,and 11 cases incompletely observed or miss diagnosed.The diagnostic accuracy rate of PIB and conventional ultrasound or UBM were 100%(44/44)and 56.82%(25/44),and the sensitivity of them were 100%and 56.82%.All the patients underwent PIB and were diagnosed as RD(15 cases),retinal and choroidal detachment(4 cases),subchoroidal hematocele(1 case),vitreous opacity and/or organic membrane formation(4 cases),PHPV(12 cases),iris and/or ciliary body tumors(3 cases),and choroidal tumors(6 cases).According to the intraoperative diagnosis or pathological results,the diagnostic coincidence rate of PIB was 100%,which was significantly higher than conventional ultrasound and UBM.CONCLUSION:PIB can help to accurately diagnose complex RD,PHPV,and intraocular masses with special location or/and excessive size.It has important diagnostic value for patients with equivocal findings at conventional ultrasound examination.
基金Supported by National Key Basic Research Program of China(973 Program,No.2013CB967000)National Natural Science Foundation of China(No.81271052)
文摘AIM:To evaluate the accuracy of axial length(AL)measurements obtained from immersion B-scan ultrasonography(immersion B-scan)for intraocular lens(IOL)power calculation in patients with high myopia and cataracts.METHODS:Immersion B-scan,contact A-scan ultrasonography(contact A-scan),and the IOLMaster were used to preoperatively measure the AL in 102 eyes from 102 patients who underwent phacoemulsification and IOL implantation.Patients were divided into two groups according to the AL:one containing patients with22 mm≤AL【26 mm(group A)and the other containing patients with AL≥26 mm(group B).The mean error(ME)was calculated from the difference between the AL measurement methods predicted refractive error and the actual postoperative refractive error.RESULTS:Ingroup A,ALs measured byimmersion Bscan(23.48±1.15)didn’t differ significantly from those measured by the IOLMaster(23.52±1.17)or from those by contact A-scan(23.38±1.20).In the same group,the standard deviation(SD)of the mean error(ME)of immersion B-scan(-0.090±0.397 D)didn’t differ significantly from those of IOLMaster(-0.095±0.411 D)and contact A-scan(-0.099±0.425 D).In group B,ALs measured by immersion B-scan(27.97±2.21 mm)didn’t differ significantly from those of the IOLMaster(27.86±2.18 mm),but longer than those measured by Contact A-scan(27.75±2.23 mm,P=0.009).In the same group,the standard deviation(SD)of the mean error(ME)of immersion B-scan(-0.635±0.157 D)didn’t differ significantly from those of the IOLMaster(-0.679±0.359 D),but differed significantly from those of contact A-scan(-0.953±1.713 D,P=0.028).CONCLUSION:ImmersionB-scanexhibitsmeasurement accuracy comparable to that of the IOLMaster,and is thus a good alternative in measuring AL in eyes with high myopia when the IOLMaster can’t be used,and it is more accurate than the contact A-scan.
基金the Guangdong Medical Research Foundation,No.A2018550
文摘Steroid 5β-reductase [aldo-keto reductase family 1 member D1(AKR1D1)] is essential for bile acid biosynthesis. Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and cholestasis. There is still limited experience regarding the treatment of this disease. We describe an infant who presented with hyperbilirubinemia and coagulopathy but normal bile acid and γ-glutamyltransferase. Gene analysis was performed using genomic DNA from peripheral lymphocytes from the patient, his parents, and his elder brother. The patient was compound heterozygous for c.919C>T in exon 8 and exhibited a loss of heterozygosity of the AKR1D1 gene, which led to an amino acid substitution of arginine by cysteine at amino acid position 307(p.R307C). Based on these mutations, the patient was confirmed to have primary 5β-reductase deficiency. Ursodeoxycholic acid(UDCA) treatment did not have any effect on the patient. However, when we changed to chenodeoxycholic acid(CDCA) treatment, his symptoms and laboratory tests gradually improved. It is therefore crucial to supplement with an adequate dose of CDCA early to improve clinical symptoms and to normalize laboratory tests.
基金Supported by the Shenzhen Innovation and Technology Committee,No.JCYJ20180228175150018the Guangdong Medical Research Foundation(CN),No.A2018550
文摘BACKGROUND Endoscopic balloon dilatation (EBD) has become the first line of therapy for benign esophageal strictures (ESs);however,there are few publications about the predictive factors for the outcomes of this treatment.AIM To assess the predictive factors for the outcomes of EBD treatment for strictures after esophageal atresia (EA) repair.METHODS Children with anastomotic ES after thoracoscopic esophageal atresia repair treated by EBD from January 2012 to December 2016 were included.All procedures were performed under tracheal intubation and intravenous anesthesia using a three-grade controlled radial expansion balloon with gastroscopy.Outcomes were recorded and predictors of the outcomes were analyzed.RESULTS A total of 64 patients were included in this analysis.The rates of response,complications,and recurrence were 96.77%,8.06%,and 2.33%,respectively.The number of dilatation sessions and complications were significantly higher in patients with a smaller stricture diameter (P=0.013 and 0.023,respectively) and with more than one stricture (P=0.014 and 0.004,respectively).The length of the stricture was significantly associated with complications of EBD (P=0.001).A longer interval between surgery and the first dilatation was related to more sessions and a poorer response (P=0.017 and 0.024,respectively).CONCLUSION The diameter,length,and number of strictures are the most important predictive factors for the clinical outcomes of endoscopic balloon dilatation in pediatric ES.The interval between surgery and the first EBD is another factor affectingresponse and the number of sessions of dilatation.
基金Shenzhen Innovation and Technology Committee,No.JCYJ20180228175150018.
文摘BACKGROUND Gastrointestinal cytomegalovirus(CMV)disease occurs commonly in immunocompromised/immunodeficient patients with advanced human immunodeficiency virus infection,neoplasm,solid organ transplantation,hematopoietic stem cell transplantation,or treatment with immunosuppressants,but is rarely reported in association with measles infection.CASE SUMMARY We describe a case of extensive gastrointestinal CMV disease secondary to measles infection in a 9-mo-old boy who presented with persistent fever and bloody diarrhea.His condition was improved after ganciclovir treatment.Serological analysis of CMV showed negative immunoglobulin(Ig)M and positive IgG.Blood CMV-DNA was 9.26×103 copies/mL.The diagnosis of gastrointestinal CMV disease was confirmed by histopathological findings of intranuclear and intracytoplasmic inclusions and Owl’s eye inclusion.This case highlights the differential diagnosis and histopathological characteristics of gastrointestinal CMV infection and laboratory tests.CONCLUSION Extensive gastrointestinal CMV lesions can be induced by the immune suppression secondary to measles infection.Rational,fast,and effective laboratory examinations are essential for suspected patients.
基金funded by the China Geological Survey Project(DD20190545 and DD20221824).
文摘Based on the 16 scenes GF-1 satellite multi-spectral remote sensing images,through the adoption of data processing methods including orthorectification,geometric rectification,data fusion and image mosaic,integrated with field surveys,the remote sensing interpretation signs for the inland wetland types have been built,and the remote sensing survey of inland wetlands in Yadong region has been initiated,with six types of inland wetlands recognized in Yadong region,namely permanent rivers,seasonal rivers,lakes,salt lakes,alpine meadows,and inundated land.The spatial distribution characteristics and the spreading rules of these wetlands have also been revealed.Based on full understanding of the overall characteristics of the inland wetlands in the Yadong region,using the three phases of TM images acquired in 1989,2003 and 2008 as well as the PMS2 data gathered by GF-1 in 2014,and the wide-range data(WFV3)gathered by GF-1 in 2020.As to the typical salt lakes,a long-time salt lakes transition study was carried out.The results show that the typical salt lakes in Yadong have been shrinking in the past three decades.The average annual shrinkage of Duoqing Co(Co means lake in Tibetan)was stronger than that of Gala Co,which are respective 87.30 hectares(usually short as ha;1 ha equals to 0.01 km^(2))/a and 24.20 ha/a;the shrinkage degree of Gala Co was higher than that of Duoqing Co,shrank by 59.27% and 35.73% respectively.Based on the remote sensing survey results and an integrated analysis of the predecessors’researchers,the reason for the shrinkage of the salt lakes is more inclined to geological factors.Geological process is manifested by a series of extensional faults at the bottom of the lake basin generated from tectonic activities,providing fluid infiltration channels,and inducing the eventual leakage of lake water to the lower strata.The result provides an important instance for understanding the evolution characteristics of wetlands and salt lakes in specific environment of the Tibetan Plateau.
基金Supported by National Key Basic Research Program of China(973 Program,No.2013CB967001)National Natural Science Foundation of China(No.81271052,No.31271059)
文摘·AIM: To evaluate the accuracy of 20 MHz immersion Bscan ultrasonography in observing lens and to investigate the value of this noninvasive preoperative diagnosis method in alkali burn eyes.·METHODS: It was a comparative study. Fifty-six cases(56 eyes) of alkali burn eyes were examined by ultrasound biomicroscopy(UBM) and immersion 20 MHz B-scan ultrasonography from June 2011 to April 2013,the images were analyzed, and the ultrasonographic diagnosis compared with the operation results.·RESULTS: In 56 alkali burn eyes examined by UBM, the lens were not detected in 16 eyes; the IOL could be detected in 2 eyes; the anterior lens capsule surface or/and the front lens could be detected in 18 eyes, and lens opacification in 3 eyes of them; suspected abnormal lens were detected in the other 20 eyes. In all the same eyes examined by immersion 20 MHz B-scan ultrasonography,the lens were not detected in 16 eyes; the IOL could be detected in 2 eyes; 24 abnormal lens(opacity, lens expansion, shrinkage) and 14 normal lens were found.Compared with the intraoperative findings, the diagnostic accordance rate of the immersion 20 MHz B-scan appearance of lens was 100%(56/56), which was significantly higher than examined by UBM 57.14%(32/56)(χ2=30.55, P =0.0000).·CONCLUSION: Immersion 20 MHz B-scan ultrasonography can observe the lens accurately in alkali burn eyes. It has important clinical value to combine with UBM in eyes of alkali burn.
基金National Natural Science Foundation of China(No.82070921)。
文摘AIM:To evaluate the accuracy of segmented measurement of axial length(AL)in high myopia filled with silicone oil by immersion B-scan ultrasonography(immersion B-scan).METHODS:From June 2016 to June 2020,a total of 67 ultra-high myopia inpatients(67 eyes)who underwent silicone oil removal combined with cataract extraction and intraocular lens(IOL)implantation were retrospectively enrolled.The preoperative axial length(AL)of 31 patients with severe cataract were segmented measured using immersion B-scan(B-scan group)and another 36 patients with mild or moderate cataract were measured using IOLMaster 500(IOLMaster group).The post-operative ALs in two groups were both measured using IOLMaster 500.The IOL power was calculated with Haigis formula.The differences in ALs between pre-and post-surgery,as well as the postoperative refractive spherical equivalent,absolute refractive error,the prediction deviation of postoperative refraction and best corrected visual acuity(BCVA)were compared.RESULTS:The pre-and post-operative ALs were 30.46±1.63 mm(range 28.09-33.51 mm)and 30.42±1.70 mm(range 28.03-33.90 mm)in B-scan group(t=0.644,P=0.542)and 30.51±1.21 mm(range 28.03-33.90 mm)and 30.43±1.27 mm(range 28.54-33.50 mm)in IOLMaster group(t=1.843,P=0.074),respectively.Three months after surgery,BCVA were 0.45±0.13(range 0.3-0.9)and 0.44±0.20(range 0.2-1.0)in B-scan and IOLMaster group respectively(t=0.086,P=0.932).There was no significant difference of the postoperative spherical equivalent(-3.11±0.65 D vs-3.21±0.51 D,t=0.671,P=0.505)and the absolute refractive error(0.589±0.340 vs 0.470±0.245 D,t=1.615,P=0.112)between two groups.In B-scan group,absolute refractive error within±0.50 D was found in 18 eyes(58.1%),within±1.00 D in 26 eyes(83.9%),and within±1.50 D in 31 eyes(100%).In IOLMaster group,absolute refractive error within±0.50 D was found in 23 eyes(63.9%),within±1.00 D in 34 eyes(94.4%),and within±1.50 D in 36 eyes(Z=0.757,P=0.449).CONCLUSION:The segmented measurement of ALs by immersion B-scan shows comparable measurement accuracy with that of IOLMaster 500 in ultra-high myopia patients with severe cataract secondary to silicone oil filling and can obtain an ideal postoperative refractive state.
文摘Objective To investigate the effects of the flexible surgical approaches on therapeutic and cosmetic outcomes of facial nevi.Methods From August 2002 to January 2008,16 cases with facial nevi had been treated in our wards with the selected approaches.Surgical approaches including serial excision or one-time radical excision,free skin graft,and expanded flap were adopted in accordance with the individual size and location of the facial nevi.Results All cases experienced complete excision and had satisfactory cosmetic appearance in the end.Conclusion The flexible surgical measures help to minimize the risk of malignant transformation and achieve good cosmetic results.
文摘AIM:To present the clinical manifestations of 5 autosomal dominant cone-rod dystrophy(ad CORD)patients from two Chinese families with cone-rod homeobox(CRX)mutation(p.R41W),and to explore the clinical heterogeneity of ad CORD with CRX mutation(p.R41W).METHODS:Interrogation and ophthalmological examinations were undertaken in all patients and unaffected members.Analysis of clinical features was performed by visual acuity,slit lamp examination,visual field examination,fundoscopy,autofluorescence and spectral domain optical coherence tomography.Targeted next-generation sequencing was applied as a useful tool to identify the causative mutation of CORD genes.RESULTS:A CRX missense mutation c.121C>T was identified in all patients,resulting in an amino acid change from arginine acid to tryptophan(p.R41W).The patients presented with early onset,progressive and different severities with CORD.CONCLUSION:This is the first report of the clinical phenotype of CRX mutation(p.R41W)in Chinese families,and the mutation can lead to a wide range of various retinal phenotypes.
文摘Objective:To explore the correlation of different hypertension grades with carotid atherosclerotic plaque in middle-aged patients with hypertension.Methods:A total of 300 patients with primary hypertension who were admitted in the Cardiology Department and Neurology Department of our hospital from January, 2015 to September, 2016 were included in the study and divided into 3 groups with 100 cases in each group according to the hypertension grade criteria. Moreover, 100 normal individuals who came for physical examinations were served as the control group. TCD was used to detect MCA, ACA, PCA, VA1, and BA. CDFI was used to detect CCA, ICA, ECA, and VA2. EDV, PSV, PI, and RI were detected, respectively. The nitrate reductase colorimetric method was used to detect NO, MDA, and SOD.Results:PSV and EDV in the internal carotid artery system in patients with hypertension were significantly reduced (P<0.05), while PI and RI were significantly increased (P<0.05);moreover, with the increasing of hypertension grading, PSV and EDV were gradually reduced, while PI and RI were increased. PSV and EDV in the vertebral artery system in patients with hypertension were significantly reduced (P<0.05), while PI and RI were significantly increased (P<0.05);moreover, with the increasing of hypertension grading, PSV and EDV were gradually reduced, while PI and RI were increased. SOD and NO in patients with hypertension were significantly lower than those in the control group (P<0.05), while MDA was significantly higher than that in the control group (P<0.05);moreover, with the increasing of hypertension grading, SOD and NO were gradually reduced, while MDA was gradually increased.Conclusions: TCD in combined with CDFI can make a comprehensive evaluation of hemodynamic indicators of intracranial and extracranial vessels in patients with hypertension, and is of great significance in the early detection of intracranial and extracranial arteriosclerosis.
文摘Objective:To explore the correlation of carotid IMT (cIMT) with Hcy, Cyst-C, UA, and Lp(a) in middle-aged patients with H-type hypertension.Methods:A total of 120 middle-aged patients with H-type hypertension who were admitted in our hospital from July, 2015 to July, 2016 were included in the study. The color Doppler ultrasound diagnostic apparatus was used for the two-dimensional ultrasound examination. The patients were divided into the normal group, thickening group, and plaque group with 40 cases in each group according to the atherosclerosis (AS). The full automatic biochemical analyzer was used to detect the plasma Hcy, Cyst-C, UA, Scr, Bun, TC, TG, LDL-C, and HDL-C. Emulsion turbidity method was used to detect Lp(a).Results:With the thickening of cIMT, Hcy, Cyst-C, Lp(a), and UA levels were elevated, while HDL-C level was reduced.Conclusions:The serum Hcy, Cyst-C, UA, and Lp(a) levels in middle-aged patients with H-type hypertension are positively correlated with cIMT, are the independent risk factors for developing AS, and can be served as the early indicators to estimate the occurrence and development of AS.
文摘An experimental setup for cold extrusion process with electric-hydraulic chattering was developed and its working principle was introduced. The finite element (FE) model for a kind of cup part (material: 20Cr) was built by using the software Deform-3D. FE simulation experiments with and without electric-hydraulic chattering were carried out to analyze the velocity fields and the metal grid flow lines. The extrusion ex- periments of the cup part were also performed under different conditions. The difference of metal flow lines with and without electric-hydraulic chattering was discussed via a scanning electron microscope (SEM) and the Keyence super-depth three-dimensional microscopic system. The results showed that with the electric-hydraulic chattering, the velocity of material flow increases, whereas deformation resistance decreases. Electric hydraulic chattering results in easy metal flow, small bending degree of metal flow lines, slender and dense metal grains, and thereby an improved quality of the deformed parts.