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RANKL inhibition reduces lesional cellularity and Gαs variant expression and enables osteogenic maturation in fibrous dysplasia
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作者 Luis F.de Castro Jarred M.Whitlock +11 位作者 Zachary Michel Kristen Pan Jocelyn Taylor Vivian Szymczuk Brendan Boyce Daniel Martin Vardit Kram Rebeca Galisteo Kamran Melikov Leonid V.Chernomordik Michael T.Collins Alison M.Boyce 《Bone Research》 SCIE CAS CSCD 2024年第1期165-179,共15页
Fibrous dysplasia(FD)is a rare,disabling skeletal disease for which there are no established treatments.Growing evidence supports inhibiting the osteoclastogenic factor receptor activator of nuclear kappa-B ligand(RAN... Fibrous dysplasia(FD)is a rare,disabling skeletal disease for which there are no established treatments.Growing evidence supports inhibiting the osteoclastogenic factor receptor activator of nuclear kappa-B ligand(RANKL)as a potential treatment strategy.In this study,we investigated the mechanisms underlying RANKL inhibition in FD tissue and its likely indirect effects on osteoprogenitors by evaluating human FD tissue pre-and post-treatment in a phase 2 clinical trial of denosumab(NCT03571191)and in murine in vivo and ex vivo preclinical models.Histological analysis of human and mouse tissue demonstrated increased osteogenic maturation,reduced cellularity,and reduced expression of the pathogenic Gαs variant in FD lesions after RANKL inhibition.RNA sequencing of human and mouse tissue supported these findings.The interaction between osteoclasts and mutant osteoprogenitors was further assessed in an ex vivo lesion model,which indicated that the proliferation of abnormal FD osteoprogenitors was dependent on osteoclasts.The results from this study demonstrated that,in addition to its expected antiosteoclastic effect,denosumab reduces FD lesion activity by decreasing FD cell proliferation and increasing osteogenic maturation,leading to increased bone formation within lesions.These findings highlight the unappreciated role of cellular crosstalk between osteoclasts and preosteoblasts/osteoblasts as a driver of FD pathology and demonstrate a novel mechanism of action of denosumab in the treatment of bone disease. 展开更多
关键词 dysplasia treatment finding
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Recent Advances in Bronchopulmonary Dysplasia Protection and Therapy
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作者 Pingfan Xia Hongyi Li +1 位作者 Zhe Xu Yongping Lu 《Health》 2024年第5期470-489,共20页
For preterm infants, bronchopulmonary dysplasia (BPD) is usually caused by abnormal lung development due to various factors during prenatal and postnatal process. One of the reasons for death and bad prognosis of pret... For preterm infants, bronchopulmonary dysplasia (BPD) is usually caused by abnormal lung development due to various factors during prenatal and postnatal process. One of the reasons for death and bad prognosis of preterm infants is to have BPD. Up to now, there are no unified strategies or drugs to treat BPD. In clinical, many intervention treatments have been applied to achieve BPD therapy, mainly including preterm protection, protective ventilation strategies, and delivery of corticosteroids, pulmonary vasodilators, and antioxidants. This review summarizes the current advances in BPD protection and treatment, and notes that gut microbiota and mesenchymal stem cells (MSCs) can be the promising strategy for protecting and treating BPD in the future. 展开更多
关键词 Bronchopulmonary dysplasia Preterm Infants Protection and Therapy Mesenchymal Stem Cells Gut Microbiota
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Treatment of bilateral developmental dysplasia of the hip joint with an improved technique:A case report
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作者 Xing-Xing Yu Jian-Ye Chen +3 位作者 Hong-Sheng Zhan Ming-Da Liu Yun-Fei Li Yu-Yan Jia 《World Journal of Clinical Cases》 SCIE 2024年第7期1320-1325,共6页
BACKGROUND Developmental dysplasia of the hip(DDH)is a common osteoarticular deformity in pediatric orthopedics.A patient with bilateral DDH was diagnosed and treated using our improved technique"(powerful overtu... BACKGROUND Developmental dysplasia of the hip(DDH)is a common osteoarticular deformity in pediatric orthopedics.A patient with bilateral DDH was diagnosed and treated using our improved technique"(powerful overturning acetabuloplasty)"combined with femoral rotational shortening osteotomy.CASE SUMMARY A 4-year-old girl who was diagnosed with bilateral DDH could not stand normally,and sought surgical treatment to solve the problem of double hip extension and standing.As this child had high dislocation of the hip joint and the acetabular index was high,we changed the traditional acetabuloplasty to"powerful turnover acetabuloplasty"combined with femoral rotation shortening osteotomy.During the short-term postoperative follow-up(1,3,6,9,12,and 15 months),the child had no discomfort in her lower limbs.After the braces and internal fixation plates were removed,formal rehabilitation training was actively carried out.CONCLUSION Our"powerful overturning acetabuloplasty"combined with femoral rotational shortening osteotomy is feasible in the treatment of DDH in children.This technology may be widely used in the clinic. 展开更多
关键词 Developmental dysplasia of the hip Improved technique Case report
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Roles of combined femoral and acetabular anteversion in pathological changes of hip dysplasia and hip reconstructive surgery
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作者 Can Liu Zheng Xu +5 位作者 Jian-Fa Zeng Zhen-Qi Song Yu-Yin Xie Zhong-Wen Tang Jie Wen Sheng Xiao 《World Journal of Orthopedics》 2024年第5期390-399,共10页
Combined femoral and acetabular anteversion is the sum of femoral and acetabular anteversion,representing their morphological relationship in the axial plane.Along with the increasing understanding of hip dysplasia in... Combined femoral and acetabular anteversion is the sum of femoral and acetabular anteversion,representing their morphological relationship in the axial plane.Along with the increasing understanding of hip dysplasia in recent years,numerous scholars have confirmed the role of combined femoral and acetabular anteversion in the pathological changes of hip dysplasia.At present,the reconstructive surgery for hip dysplasia includes total hip replacement and redirectional hip preservation surgery.As an important surgery index,combined femoral and acetabular anteversion have a crucial role in these surgeries.Herein,we discuss the role of combined femoral and acetabular anteversion in pathological changes of hip dysplasia,total hip replacement,and redirectional hip preservation surgery. 展开更多
关键词 Combined anteversion Femoral anteversion Acetabular anteversion Hip dysplasia Hip preservation surgery Total hip replacement
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HIPPO artificial intelligence:Correlating automated radiographic femoroacetabular measurements with patient-reported outcomes in developmental hip dysplasia
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作者 Ahmed Alshaikhsalama Holden Archer +3 位作者 Yin Xi Richard Ljuhar Joel E Wells Avneesh Chhabra 《World Journal of Experimental Medicine》 2024年第4期136-146,共11页
BACKGROUND Hip dysplasia(HD)is characterized by insufficient acetabular coverage of the femoral head,leading to a predisposition for osteoarthritis.While radiographic measurements such as the lateral center edge angle... BACKGROUND Hip dysplasia(HD)is characterized by insufficient acetabular coverage of the femoral head,leading to a predisposition for osteoarthritis.While radiographic measurements such as the lateral center edge angle(LCEA)and Tönnis angle are essential in evaluating HD severity,patient-reported outcome measures(PROMs)offer insights into the subjective health impact on patients.AIM To investigate the correlations between machine-learning automated and manual radiographic measurements of HD and PROMs with the hypothesis that artificial intelligence(AI)-generated HD measurements indicating less severe dysplasia correlate with better PROMs.METHODS Retrospective study evaluating 256 hips from 130 HD patients from a hip preservation clinic database.Manual and AI-derived radiographic measurements were collected and PROMs such as the Harris hip score(HHS),international hip outcome tool(iHOT-12),short form(SF)12(SF-12),and Visual Analogue Scale of the European Quality of Life Group survey were correlated using Spearman's rank-order correlation.RESULTS The median patient age was 28.6 years(range 15.7-62.3 years)with 82.3%of patients being women and 17.7%being men.The median interpretation time for manual readers and AI ranged between 4-12 minutes per patient and 31 seconds,respectively.Manual measurements exhibited weak correlations with HHS,including LCEA(r=0.18)and Tönnis angle(r=-0.24).AI-derived metrics showed similar weak correlations,with the most significant being Caput-Collum-Diaphyseal(CCD)with iHOT-12 at r=-0.25(P=0.042)and CCD with SF-12 at r=0.25(P=0.048).Other measured correlations were not significant(P>0.05).CONCLUSION This study suggests AI can aid in HD assessment,but weak PROM correlations highlight their continued importance in predicting subjective health and outcomes,complementing AI-derived measurements in HD management. 展开更多
关键词 Hip dysplasia Patient reported outcome measures Deep-learning Artificial intelligence RADIOGRAPHS Lateral center edge angle
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Spontaneous Coronary Artery Disease in a Healthy Fibromuscular Dysplasia Patient: A Case Report
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作者 Munir Shunnar Obada Alshaikh +1 位作者 Maha Alzubaidi Hyun-Ryung Choi 《Case Reports in Clinical Medicine》 2024年第9期375-381,共7页
Our case is a 72-year-old female with Fibromuscular Dysplasia (FMD) presented to the Emergency Department (ED) with acute coronary syndrome (ACS), more specifically NSTEMI. In ED, troponin levels were elevated and non... Our case is a 72-year-old female with Fibromuscular Dysplasia (FMD) presented to the Emergency Department (ED) with acute coronary syndrome (ACS), more specifically NSTEMI. In ED, troponin levels were elevated and non-ST segment elevation myocardial infarction (NSTEMI) was detected on electrocardiogram (ECG). Computed tomography (CT) scan showed Internal Carotid artery (ICA) pseudoaneurysm. Cardiac catheterization (CATH) was performed and revealed occlusion of the Left anterior descending (LAD) artery. Spontaneous coronary artery dissection (SCAD) was suspected due to the history of FMD without a history of hypertension, smoking or substance use. 展开更多
关键词 SCAD NSTEMI STEMI ACS Eliquis PSEUDOANEURYSM Fibromuscular dysplasia ANTICOAGULANT ANTIPLATELET Coronary Dissection Neuroendovascular Cardiology WARFARIN Cath Lab APIXABAN Clopidogrel
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Influence of Gut and Lung Microbiota and the Gut-Lung Axis on Bronchopulmonary Dysplasia
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作者 Binxiang Xu Yumei Liang 《Journal of Clinical and Nursing Research》 2024年第9期30-35,共6页
Bronchopulmonary dysplasia(BPD),also known as neonatal chronic lung disease,is a common respiratory disease in preterm infants.Preterm infants with BPD often exhibit changes in gut and lung microbiota.In recent years,... Bronchopulmonary dysplasia(BPD),also known as neonatal chronic lung disease,is a common respiratory disease in preterm infants.Preterm infants with BPD often exhibit changes in gut and lung microbiota.In recent years,with the development of high-throughput sequencing technology,more and more mechanisms of the gut-lung axis have been confirmed,helping to explore new directions for the treatment of BPD using microecological agents.This paper reviews the roles of gut microbiota,lung microbiota,and the gut-lung axis in the pathogenesis of BPD in preterm infants,providing new research avenues for the prevention and treatment of BPD. 展开更多
关键词 Bronchopulmonary dysplasia Gut-lung axis Gut microbiota Lung microbiota
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Surgical treatment of atlantoaxial dysplasia and scoliosis in spondyloepiphyseal dysplasia congenita:A case report 被引量:1
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作者 Yang Jiao Jun-Duo Zhao +2 位作者 Xu-An Huang Hao-Yu Cai Jian-Xiong Shen 《World Journal of Orthopedics》 2023年第11期827-835,共9页
BACKGROUND Spondyloepiphyseal dysplasia congenita(SEDC)is a rare autosomal dominant hereditary disease caused by COL2A1 mutations.SEDC primarily involves the skeletal system,with typical clinical manifestations,includ... BACKGROUND Spondyloepiphyseal dysplasia congenita(SEDC)is a rare autosomal dominant hereditary disease caused by COL2A1 mutations.SEDC primarily involves the skeletal system,with typical clinical manifestations,including short stature,hip dysplasia,and spinal deformity.Due to the low incidence of SEDC,there are only a few case reports regarding the surgical treatment of SEDC complicated with spinal deformities.CASE SUMMARY We report a case of a 16-year-old male patient with SEDC.He presented with typical short stature,atlantoaxial dysplasia,scoliosis,and hip dysplasia.Cervical magnetic resonance imaging showed spinal canal stenosis at the atlas level and cervical spinal cord compression with myelopathy.The scoliosis was a right thoracic curve with a Cobb angle of 65°.He underwent atlantoaxial reduction,decompression,and internal fixation from C1–C2 to relieve cervical myelopathy.Three months after cervical surgery,posterior correction surgery for scoliosis was performed from T3 to L4.Scoliosis was corrected from 66°to 8°and remained stable at 2-year follow-up.CONCLUSION This is the first case report of a patient with SEDC who successfully underwent surgery for atlantoaxial dysplasia and scoliosis.The study provides an important reference for the surgical treatment of SEDC complicated with spinal deformities. 展开更多
关键词 Spondyloepiphyseal dysplasia congenita Surgical treatment Atlantoaxial dysplasia SCOLIOSIS Hip dysplasia Case report
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Significance of dysplasia in bile duct resection margin in patients with extrahepatic cholangiocarcinoma:A retrospective analysis
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作者 Jung Wan Choe Hyo Jung Kim Jae Seon Kim 《World Journal of Clinical Cases》 SCIE 2022年第10期3078-3087,共10页
BACKGROUND Radical resection is the only indicator associated with survival in extrahepatic cholangiocarcinoma(EHCC).However,limited data are available regarding the implications of dysplasia at the resection margin f... BACKGROUND Radical resection is the only indicator associated with survival in extrahepatic cholangiocarcinoma(EHCC).However,limited data are available regarding the implications of dysplasia at the resection margin following surgery.AIM To evaluate the prognostic significance of dysplasia-positive margins in patients diagnosed with EHCC.METHODS We reviewed the records of patients who had undergone surgery for EHCC with curative intent between January 2013 and July 2017.We retrospectively analyzed the clinicopathological data of 116 patients followed for longer than 3 years.The status of resection margin was used to classify patients into negative low-grade dysplasia(LGD)and high-grade dysplasia(HGD)/carcinoma in situ(CIS)categories.RESULTS Based on postoperative status,72 patients underwent resection with negative margins,19 had LGD-positive margins,and 25 showed HGD/CIS-positive margins.The mean survival rates of the patients with negative margins,LGD margins,and HGD/CIS margins were 49.1±4.5,47.3±6.0,and 20.8±4.4 mo,respectively(P<0.001).No difference in survival was found between groups with LGD margins and negative margins(P=0.56).In the multivariate analysis,age>70 years and HGD/CIS-positive margins were significant independent factors for survival(hazard ratio=1.90 and 2.47,respectively).CONCLUSION HGD/CIS margin in resected EHCC is associated with a poor survival.However,the LGDpositive resection margin is not a significant indicator of survival in patients with EHCC. 展开更多
关键词 dysplasia CHOLANGIOCARCINOMA SURVIVAL Extrahepatic cholangiocarcinoma Low-grade dysplasia High-grade dysplasia
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Survival of dental implants in patients with bone dysplasia:A systematic review
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作者 Alexandre Perez Sarah Dib +2 位作者 Andreij Terzic Delphine Courvoisier Paolo Scolozzi 《Discussion of Clinical Cases》 2021年第4期8-13,共6页
Objective:This study used published studies to assess the survival rate of dental implants placed in patients with bone dysplasia of the maxillofacial region.Material and methods:An electronic search without a specifi... Objective:This study used published studies to assess the survival rate of dental implants placed in patients with bone dysplasia of the maxillofacial region.Material and methods:An electronic search without a specified date range was performed using the MEDLINE,PubMed,EMBASE,Web of Science,and Cochrane databases.No gender or age restrictions were applied.Results:Eighteen publications were found that met the study’s criteria,reporting data on 18 patients with bone dysplasia including cleidocranial dysplasia(CDD),fibrous dysplasia(FD),florid cemento-osseous dysplasia(FCOD),and odonto-maxillary segmental dysplasia(SOMD),who received a total of 130 implants,an average of 7.2 implants/patient(range 1 to 16).The mean age of the patients was 36.7 years(range 15 to 70 years).For implants placed in bone dysplasia,the survival rates were 100% for patients with CDD(n=8),FD(n=5),SOMD(n=2),FCOD with implants inserted far from the lesions(n=2)and 0% for dental implants inserted within FCOD(n=1).The mean follow-up was 38.2 months(min 6,max 60).Conclusions:Dental implants placed in patients with dysplastic bone lesions show high survival rates,similar to those in the general population for CDD,FD,and SOMD.For FCOD,the failure rate was 100%. 展开更多
关键词 Dental implants BONE Osseous dysplasia Fibrous dysplasia Florid cemento-osseous dysplasia Bone diseases Treatment outcome OSTEOMYELITIS
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Differences in biological features of gastric dysplasia, indefinite dysplasia, reactive hyperplasia and discriminant analysis of these lesions 被引量:7
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作者 BinDong Yu-QuanXie +4 位作者 KeChen TaoWang WeiTang Wei-ChengYou Ji-YouLi 《World Journal of Gastroenterology》 SCIE CAS CSCD 2005年第23期3595-3600,共6页
AIM: To investigate the differences in biological features of gastric dysplasia (Dys), indefinite dysplasia (IDys) and reactive hyperplasia (RH) by studying the biomarker alterations in cell proliferation, cell differ... AIM: To investigate the differences in biological features of gastric dysplasia (Dys), indefinite dysplasia (IDys) and reactive hyperplasia (RH) by studying the biomarker alterations in cell proliferation, cell differentiation, cell cycle control and the expression of house-keeping genes, and further to search for markers which could be used in guiding the pathological diagnosis of three lesions. METHODS: Expressions of MUC5AC, MUC6, adenomatous polyposis coli (APC), p53, Ki-67, proliferation cell nuclear antigen (PCNA) and EGFR were studied by immunohistochemistry with a standard Envision technique in formalinfixed and paraffin-embedded specimens from 43 RH, 35 IDys, 35 Dys and 36 intestinal type gastric carcinomas (IGC). In addition, Bayes discriminant analysis was used to investigate the value of markers studied in differential diagnosis of RH, IDys, Dys and IGC. RESULTS: The MUC5AC and MUC6 antigen expressions in RH, IDys, Dys and IGC decreased gradually (MUC5AC:86.04%, 77.14%, 28.57%, 6.67%; MUC6: 65.15%, 54.29%, 20.00%, 25.00%, respectively). The expressions of the two markers had no significant difference between RH and IDys, but were all significantly higher than those ofthe other two lesions (MUC5AC: x2 = 27.607, 38.027 and 17.33, 26.092; MUC6: x2= 16.54, 12.665 and 9.282, 6.737, P<0.01). There was no significant differencebetween RH and IDys, Dys and IGC in MUC6 expression. The APC gene expression in the four lesions had a similar decreasing tendency (RH 69.76%, IDys 68.57%, Dys39.39%, IGC 22.86%), and it was significantly higher in the first two lesions than in the last two (x2 = 7.011,16.995 and 14.737, 19.817, P<0.05). The p53 expressionin RH, IDys, Dys and IGC was 6.98%, 20%, 57.14% and 50%, respectively. There was no significant differencebetween RH and IDys or Dys and IGC, but the p53 expression in RH and IDys was significantly lower than that in Dys and IGC (x2 = 7.011, 16.995 and 14.737, 19.817, P<0.01).The Ki-67 label index was significantly different among four lesions (RH: 0.298±8.92%, IDys: 0.358±9.25%,Dys: 0.498±9.03%, IGC: 0.620±10.8%, P<0.001). Positive immunostaining of PCNA was though observed in all specimens, significant differences were detected among four lesions (F= 95.318, P<0.01). In addition, we used Bayes discriminant analysis to investigate molecular pathological classification of the lesions, and obtained the best result with the combination of MUC5AC, Ki-67 and PCNA. The overall rate of correct classification was67.4% (RH), 68.6% (IDys), 70.6% (Dys) and 84.8% (IGC), respectively.CONCLUSION: Dys has neoplastic biological characteristics, while RH and IDys display hyperplastic characteristics. MUC5AC and proliferation-related biomarkers (Ki-67, PCNA) are more specific in distinguishing Dys from RH and IDys. 展开更多
关键词 Gastric dysplasia Indefinite dysplasia Reactive hyperplasia
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Geleophysic dysplasia caused by a mutation in FBN1:A case report 被引量:3
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作者 Ying Tao Qing Wei +1 位作者 Xun Chen Guang-Min Nong 《World Journal of Clinical Cases》 SCIE 2021年第24期7175-7180,共6页
BACKGROUND Geleophysic dysplasia(GD)presents the characterized clinical manifestations of acromelic dysplasia,including extremely short stature,short limbs,small hands and feet,stubby fingers and toes,joint stiffness ... BACKGROUND Geleophysic dysplasia(GD)presents the characterized clinical manifestations of acromelic dysplasia,including extremely short stature,short limbs,small hands and feet,stubby fingers and toes,joint stiffness and others.It is clinically distinct from the other acromelic dysplasia in terms of symptoms such as cardiac valvular abnormalities,progressive hepatomegaly and tracheal stenosis.CASE SUMMARY We report on a Chinese 9-year-old girl with GD with the c.5243G>T(p.C1748F)mutation in FBN1(fibrillin 1,OMIM 134797).She was born in Guangxi Zhuang Autonomous Region of China.The patient presented with typical clinical features of GD and recurrent respiratory tract infections over 6 years.Laboratory studies and chest computed tomography(CT)scan indicated bronchopneumonia.Her echocardiography revealed mild mitral valve thickening with regurgitation.Laryngopharyngeal CT and electronic bronchoscopy revealed severe glottic stenosis.Echocardiography examination displayed mild mitral valve thickening and regurgitation.Ophthalmic examination did not reveal myopia or lens dislocation.Treated with ceftriaxone sodium and methylprednisolone sodium succinate for injection as well as methylprednisolone orally,patient’s symptoms had improved.CONCLUSION GD is a rare genetic condition that can cause life-threatening cardiovascular and respiratory problems.This study also found that the identified genotype of GD could be related to different clinical phenotypes. 展开更多
关键词 Fibrillin 1 Geleophysic dysplasia Acromelic dysplasia Short stature Tracheal stenosis Case report
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Confusion and prospects for carcinogenesis of gastric adenoma and dysplasia: What is the correct answer currently? 被引量:1
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作者 Shinichi Kinami Sohsuke Yamada Hiroyuki Takamura 《World Journal of Gastroenterology》 SCIE CAS 2022年第48期6900-6908,共9页
There are differences in the diagnoses of superficial gastric lesions between Japan and other countries.In Japan,superficial gastric lesions are classified as adenoma or cancer.Conversely,outside Japan,the same lesion... There are differences in the diagnoses of superficial gastric lesions between Japan and other countries.In Japan,superficial gastric lesions are classified as adenoma or cancer.Conversely,outside Japan,the same lesion is classified as low-grade dysplasia(LGD),high-grade dysplasia,or invasive neoplasia.Gastric carcinogenesis occurs mostly de novo,and the adenoma-carcinoma sequence does not appear to be the main pathway of carcinogenesis.Superficial gastric tumors can be roughly divided into the APC mutation type and the TP53 mutation type,which are mutually exclusive.APC-type tumors have low malignancy and develop into LGD,whereas TP53-type tumors have high malignancy and are considered cancerous even if small.For lesions diagnosed as category 3 or 4 in the Vienna classification,it is desirable to perform complete en bloc resection by endoscopic submucosal dissection followed by staging.If there is lymphovascular or submucosal invasion after mucosal resection,additional surgical treatment of gastrectomy with lymph node dissection is required.In such cases,functionpreserving curative gastrectomy guided by sentinel lymph node biopsy may be a good alternative. 展开更多
关键词 Gastric adenoma Low-grade dysplasia High-grade dysplasia Intramucosal carcinoma Submucosal carcinoma Endoscopic submucosal dissection
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Progression from low-grade dysplasia to malignancy in patients with Barrett's esophagus diagnosed by two or more pathologists
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作者 Harsha Moole Jaymon Patel +10 位作者 Zohair Ahmed Abhiram Duvvuri Sreekar Vennelaganti Vishnu Moole Sowmya Dharmapuri Raghuveer Boddireddy Pratyusha Yedama Naveen Bondalapati Achuta Uppu Prashanth Vennelaganti Srinivas Puli 《World Journal of Gastroenterology》 SCIE CAS 2016年第39期8831-8843,共13页
AIM To evaluate annual incidence of low grade dysplasia(LGD) progression to high grade dysplasia(HGD) and/or esophageal adenocarcinoma(EAC) when diagnosis was made by two or more expert pathologists.METHODS Studies ev... AIM To evaluate annual incidence of low grade dysplasia(LGD) progression to high grade dysplasia(HGD) and/or esophageal adenocarcinoma(EAC) when diagnosis was made by two or more expert pathologists.METHODS Studies evaluating the progression of LGD to HGD or EAC were included. The diagnosis of LGD must be made by consensus of two or more expert gastrointestinal pathologists. Articles were searched in Medline, Pubmed, and Embase. Pooled proportions were calculated using fixed and random effects model. Heterogeneity among studies was assessed using the I2 statistic. RESULTS Initial search identified 721 reference articles, of which 53 were selected and reviewed. Twelve studies(n = 971) that met the inclusion criteria were included in this analysis. Among the total original LGD diagnoses in the included studies, only 37.49% reached the consensus LGD diagnosis after review by two or more expert pathologists. Total follow up period was 1532 patient-years. In the pooled consensus LGD patients, the annual incidence rate(AIR) of progression to HGD and or EAC was 10.35%(95%CI: 7.56-13.13) and progression to EAC was 5.18%(95%CI: 3.43-6.92). Among the patients down staged from original LGD diagnosis to No-dysplasia Barrett's esophagus, the AIR of progression to HGD and EAC was 0.65%(95%CI: 0.49-0.80). Among the patients down staged to Indefinite for dysplasia, the AIR of progression to HGD and EAC was 1.42%(95%CI: 1.19-1.65). In patients with consensus HGD diagnosis, the AIR of progression to EAC was 28.63%(95%CI: 13.98-43.27). CONCLUSION When LGD is diagnosed by consensus agreement of two or more expert pathologists, its progression towards malignancy seems to be at least three times the current estimates, however it could be up to 20 times the current estimates. Biopsies of all Barrett's esophagus patients with LGD should be reviewed by two expert gastroenterology pathologists. Follow-up strict surveillance programs should be in place for these patients. 展开更多
关键词 Barrett’s esophagus Low grade dysplasia High grade dysplasia Esophageal adenocarcinoma Annual incidence of progression Systematic review Meta-analysis
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Atelosteogenesis Type 2/Diastrophic Dysplasia Phenotypic Spectrum: From Prenatal to Preimplantation Genetic Diagnosis
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作者 Eduardo P.Mattos José Antonio A.Magalhaes +9 位作者 Lauréane Mittaz-Crettol Ricardo Azambuja Lilian Okada Denise P.Cavalcanti Juliana Cuzzi Mariangela Badalotti Rafaella Petracco Alvaro Petracco Lavinia Schüler-Faccini Maria Teresa V.Sanseverino 《Open Journal of Obstetrics and Gynecology》 2014年第7期399-404,共6页
Atelosteogenesis type II (AO2) and diastrophic dysplasia (DTD) are two recessively inherited, severe skeletal dysplasias caused by mutations in the SLC26A2 gene. AO2 is an invariably lethal condition, while DTD patien... Atelosteogenesis type II (AO2) and diastrophic dysplasia (DTD) are two recessively inherited, severe skeletal dysplasias caused by mutations in the SLC26A2 gene. AO2 is an invariably lethal condition, while DTD patients may reach adult life, although both diseases have overlapping diagnostic features. Here we report a patient with an intermediate phenotype between AO2 and DTD and present the successful application of preimplantation genetic diagnosis (PGD) in this situation. Sequencing of SLC26A2 alleles in the infant identified two compound heterozygous mutations, p.Arg178Ter and p.Arg279Trp, of paternal and maternal origin, respectively. At request from the parents, PGD was developed by haplotype mapping of parental SLC26A2 alleles in eleven five-day embryos. Transference to the mother was attempted twice, finally resulting in pregnancy and delivery of a healthy baby. This exemplifies the utility of PGD for inherited lethal conditions with a significant risk of recurrence, and highlights the importance of accurate diagnosis of skeletal dysplasias with prenatal manifestation. 展开更多
关键词 Atelosteogenesis Type 2 Diastrophic dysplasia Preimplantation Genetic Diagnosis Prenatal Diagnosis Skeletal dysplasia
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Implant Placement in a Cemento-Osseous Dysplasia:A case report
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作者 Alexandre Perez Avigail Maman +1 位作者 Edouard Di Donna Tommaso Lombardi 《Discussion of Clinical Cases》 2021年第2期6-10,共5页
We present a 45-year-old black woman diagnosed with a florid cemento-osseous dysplasia(FCOD)affecting the right lower quadrant and the anterior mandibular region.The patient requested dental implants to rehabilitate t... We present a 45-year-old black woman diagnosed with a florid cemento-osseous dysplasia(FCOD)affecting the right lower quadrant and the anterior mandibular region.The patient requested dental implants to rehabilitate the edentulous central lower incisors area(teeth#31 and#41)corresponding to a periapical cemento-osseous dysplasia(PCOD).Successful osseointegration of the two implants was obtained using a two-step procedure in order to limit the risk of complications associated with implant placement.Follow-up at one year showed no recurrence and good implant stability.Due to the abnormal quality of the bone in cemento-osseous dysplasia(COD),implant placement is generally avoided,and no other case reports have been reported in the literature in patients affected by PCOD.The present case suggests that in an appropriate clinical setting,implant placement may be a successful procedure. 展开更多
关键词 Cemento-Osseous dysplasia Periapical Cemento-Osseous dysplasia Dental implant
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Developmental dysplasia of the hip: What has changed in the last 20 years? 被引量:56
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作者 Pavel Kotlarsky Reuben Haber +1 位作者 Victor Bialik Mark Eidelman 《World Journal of Orthopedics》 2015年第11期886-901,共16页
Developmental dysplasia of the hip(DDH) describes the spectrum of structural abnormalities that involve the growing hip. Early diagnosis and treatment is critical to provide the best possible functional outcome. Persi... Developmental dysplasia of the hip(DDH) describes the spectrum of structural abnormalities that involve the growing hip. Early diagnosis and treatment is critical to provide the best possible functional outcome. Persistence of hip dysplasia into adolescence and adulthood may result in abnormal gait, decreased strength and increased rate of degenerative hip and knee joint disease. Despite efforts to recognize and treat all cases of DDH soon after birth, diagnosis is delayed in some children, and outcomes deteriorate with increasing delay of presentation. Different screening programs for DDH were implicated. The suspicion is raised based on a physical examination soon after birth. Radiography and ultrasonography are used to confirm the diagnosis. The role of other imaging modalities, such as magnetic resonance imaging, is still undetermined; however, extensive research is underway on this subject. Treatment depends on the age of the patient and the reducibility of the hip joint. At an early age and up to 6 mo, the main treatment is an abduction brace like the Pavlik harness. If this fails, closed reduction and spica casting is usually done. After the age of 18 mo, treatment usually consists of open reduction and hip reconstruction surgery. Various treatment protocols have been proposed. We summarize the current practice for detection and treatment of DDH, emphasizing updates in screening and treatment during the last two decades. 展开更多
关键词 DEVELOPMENTAL dysplasia NEWBORN INFANT Children HIP DEVELOPMENTAL dysplasia of the HIP
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Developmental dysplasia of the hip in the newborn: A systematic review 被引量:29
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作者 Vivek Gulati Kelechi Eseonu +6 位作者 Junaid Sayani Nizar Ismail Chika Uzoigwe Muhammed Zaki Choudhury Pooja Gulati Adeel Aqil Saket Tibrewal 《World Journal of Orthopedics》 2013年第2期32-41,共10页
Developmental dysplasia of the hip(DDH) denotes a wide spectrum of conditions ranging from subtle acetabular dysplasia to irreducible hip dislocations. Clinical diagnostic tests complement ultrasound imaging in allowi... Developmental dysplasia of the hip(DDH) denotes a wide spectrum of conditions ranging from subtle acetabular dysplasia to irreducible hip dislocations. Clinical diagnostic tests complement ultrasound imaging in allowing diagnosis, classification and monitoring of this condition. Classification systems relate to the alpha and beta angles in addition to the dynamic coverage index(DCI). Screening programmes for DDH show considerable geographic variation; certain risk factors have been identified which necessitate ultrasound assessment of the newborn. The treatment of DDH has undergone significant evolution, but the current gold standard is still the Pavlik harness. Duration of Pavlik harness treatment has been reported to range from 3 to 9.3 mo. The beta angle, DCI and the superior/lateral femoral head displacement can be assessed via ultrasound to estimate the likelihood of success. Success rates of between 7% and 99% have been reported when using the harness to treat DDH. Avascular necrosis remains the most devastating complication of harness usage with a reported rate of between 0% and 28%. Alternative non-surgical treatment methods used for DDH include devices proposed by LeD amany, Frejka, Lorenz and Ortolani. The Rosen splint and Wagner stocking have also been used for DDH treatment. Surgical treatment for DDH comprises open reduction alongside a combination of femoral or pelvic osteotomies. Femoral osteotomies are carried out in cases of excessive anteversion or valgus deformity of the femoral neck. The two principal pelvic osteotomies most commonly performed are the Salter osteotomy and Pemberton acetabuloplasty. Serious surgical complications include epiphyseal damage, sciatic nerve damage and femoral neck fracture. 展开更多
关键词 DEVELOPMENTAL dysplasia of the hip Congenital Pavlik HARNESS Ultrasound screening PELVIC OSTEOTOMY
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Endoscopic diagnosis of sessile serrated adenoma/polyp with and without dysplasia/carcinoma 被引量:25
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作者 Takashi Murakami Naoto Sakamoto Akihito Nagahara 《World Journal of Gastroenterology》 SCIE CAS 2018年第29期3250-3259,共10页
Sessile serrated adenoma/polyps(SSA/Ps) are early precursor lesions in the serrated neoplasia pathway, which results in colorectal carcinomas with BRAF mutations, methylation for DNA repair genes, a Cp G island methyl... Sessile serrated adenoma/polyps(SSA/Ps) are early precursor lesions in the serrated neoplasia pathway, which results in colorectal carcinomas with BRAF mutations, methylation for DNA repair genes, a Cp G island methylator phenotype, and high levels of microsatellite instability. Some of these lesions can rapidly become dysplastic or invasive carcinomas that exhibit high lymphatic invasion and lymph node metastasis potentials. Detecting serrated lesions, including SSA/Ps with and without dysplasia/carcinoma, is critical, but SSA/Ps can be difficult to detect, are inconsistently identified by endoscopists and pathologists, and are often incompletely resected. Therefore, SSA/Ps are considered to be major contributors to "interval cancers". If colonoscopists can identify the specific endoscopic characteristics of SSA/Ps, their detection and the effectiveness of colonoscopy may improve. Here, the endoscopic features of SSA/Ps with and without dysplasia/carcinoma, including the characteristics determined using magnifying endoscopy, are reviewed in the context of previous reports. Endoscopically, these subtle polyps are like hyperplastic polyps, because they are slightly elevated and pale. Unlike hyperplastic polyps, SSA/Ps are usually larger than 5 mm, frequently covered by a thin layer called the ‘‘mucus cap'', and are more commonly located in the proximal colon. Magnifying narrow-band imaging findings, which include dark spots inside the crypts and varicose microvascular vessels, in addition to the type II-open pit patterns detected using magnifying chromoendoscopy, effectively differentiate SSA/Ps from hyperplastic polyps. The lesions' endoscopic characteristics, which include their(semi)pedunculated morphologies, double elevations, central depressions, and reddishness, and the use of magnifying endoscopy, might help to detect dysplasia/carcinoma within SSA/Ps. Greater awareness may promote further research into improving the detection, identification, and complete resection rates of SSA/Ps with and without dysplasia/carcinoma and reduce the interval cancer rates. 展开更多
关键词 Sessile serrated adenoma/polyp INVASIVE CARCINOMA arising from sessile serrated adenoma/polyp Serrated NEOPLASIA pathway Endoscopic diagnosis Sessile serrated adenoma/polyp with cytological dysplasia
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Total hip arthroplasty in developmental dysplasia of the hip: Review of anatomy, techniques and outcomes 被引量:20
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作者 Scott Yang Quanjun Cui 《World Journal of Orthopedics》 2012年第5期42-48,共7页
Total hip arthroplasty(THA) in developmental dysplasia of the hip(DDH) presents many challenges to the reconstructive surgeon. The complex femoral and acetabular anatomy makes standard reconstruction technically chall... Total hip arthroplasty(THA) in developmental dysplasia of the hip(DDH) presents many challenges to the reconstructive surgeon. The complex femoral and acetabular anatomy makes standard reconstruction technically challenging. Acetabular coverage can be improved by medialization of the component or augmentation of the deficient areas with bone graft. Femoral shortening osteotomies are considered in cases of severe dysplasia and frankly dislocated hips. Each patient's unique anatomy dictates what options of reconstruction are available. The functional outcomes of THA in DDH are generally excellent, though higher rates of mechanical failure have been reported in this group. This article reviews the anatomy, classification, technical considerations, and outcomes of THA in patients with DDH. 展开更多
关键词 DEVELOPMENTAL dysplasia of the HIP Total HIP ARTHROPLASTY HIP ARTHRITIS HIP replacement
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