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Clinical, cellular, microscopic, and ultrastructural studies of a case of fibrogenesis imperfecta ossium

Bone Research 2017年第2期5卷 123-136页

作者： Melissa L Barron Mark S Rybchyn Sutharshani Ramesh Rebecca S Mason S Fiona Bonar Paul Stalley Sundeep Khosla Bernie Hudson Christopher Arthur Edward Kim roderick j clifton-bligh Phillip B clifton-bligh Department of Physiology School of Medical Sciences Bosch Institute University of Sydney Sydney 2006 New South Wales Australia Douglas HanlyMoir Pathology Macquarie Park 2113 New South Wales Australia Department of Orthopaedics Royal Prince Alfred HospitalCamperdown 2050 New South Wales Australia Department of Endocrinology Mayo Clinic Rochester 55905 MN USA Department of Microbiology Royal North Shore Hospital St Leonards 2065 New South Wales Australia Department ofMicrobiology Royal North Shore Hospital St Leonards 2065 New South Wales Australia Department of Haematology Royal North Shore Hospital St Leonards 2065 New South Wales Australia Department of Endocrinology Royal North Shore Hospital St Leonards 2065 New SouthWales Australia and Faculty of Medicine University of Sydney Sydney 2006 New South Wales Australia

Fibrogenesis imperfecta ossium is a rare disorder of bone usually characterized by marked osteopenia and associated with variable osteoporosis and osteosclerosis, changing over time. Histological examination shows that newly formed collagen is abnormal, lacking birefringence when examined by polarized light. The case presented demonstrates these features and, in addition, a previously undocumented finding of a persistent marked reduction of the serum C3 and C4. Osteoblasts established in culture from a bone biopsy showed abnormal morphology on electron microscopy and increased proliferation when cultured with benzoylbenzoyl-ATP and 1,25-dihydroxyvitamin D, contrasting with findings in normal osteoblasts in culture. A gene microarray study showed marked upregulation of the messenger RNA(mRNA) for G-protein-coupled receptor 128(GPR 128), an orphan receptor of unknown function and also of osteoprotegerin in the patient's osteoblasts in culture. When normal osteoblasts were cultured with the patient's serum, there was marked upregulation of the mRNA for aquaporin 1. A single pathogenetic factor to account for the features of this disorder has not been defined, but the unique findings described here may facilitate more definitive investigation of the abnormal bone cell function.

关键词： ultrastructural cellular Clinical

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Sonic hedgehog signaling in kidney fibrosis: a master communicator

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Science China(Life Sciences) 2016年第9期59卷 920-929页

作者： Dong Zhou roderick j.Tan Youhua Liu Department of Pathology University of Pittsburgh School of Medicine Department of Medicine University of Pittsburgh School of Medicine State Key Laboratory of Organ Failure Research National Clinical Research Center of Kidney Disease Nanfang Hospital Southern Medical University

The hedgehog signaling cascade is an evolutionarily conserved pathway that regulates multiple aspects of embryonic development and plays a decisive role in tissue homeostasis. As the best studied member of three hedgehog ligands, sonic hedgehog(Shh) is known to be associated with kidney development and tissue repair after various insults. Recent studies uncover an intrinsic link between dysregulated Shh signaling and renal fibrogenesis. In various types of chronic kidney disease(CKD), Shh is upregulated specifically in renal tubular epithelium but targets interstitial fibroblasts, thereby mediating a dynamic epithelialmesenchymal communication(EMC). Tubule-derived Shh acts as a growth factor for interstitial fibroblasts and controls a hierarchy of fibrosis-related genes, which lead to the excessive deposition of extracellular matrix in renal interstitium. In this review, we recapitulate the principle of Shh signaling, its activation and regulation in a variety of kidney diseases. We also discuss the potential mechanisms by which Shh promotes renal fibrosis and assess the efficacy of blocking this signaling in preclinical settings. Continuing these lines of investigations will provide novel opportunities for designing effective therapies to improve CKD prognosis in patients.

关键词： Sonic hedgehog Gli tubular cells fibroblast renal fibrosis

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The Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

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Congenital Heart Disease 2021年第3期16卷 221-232页

作者： roderick W.j.van Grootel Allard T.van den Hoven Dan Bowen Tijmen Ris jolien W.Roos-Hesselink Annemien E.van den Bosch Department of Cardiology Erasmus Medical CentreRotterdamThe Netherlands

Aims:To assess the prognostic value of left ventricular(LV)global longitudinal strain(GLS)and global longitudinal early diastolic strain rate(GLSre)with regard to cardiovascular events,as congenital aortic stenosis(AoS)is associated with significant mortality and morbidity but predictors for clinical outcome are *** analysis provides a robust and reproducible method for early detection of LV dysfunction,which might be of prognostic ***:This prospective study,included clinically stable patients with congenital AoS between 2011–*** GLS and GLSre was performed in the apical 4,3 and 2-chamber views using Tomtec *** endpoint was a composite of death,heart failure,hospitalization,arrhythmia,thrombo-embolic events and ***:In total 138 patients were included(33[26–43]years,86(62%)male),NYHA class I:134(97%).Mean LV GLS was–15.3±3.2%,GLSre 0.66±0.18 s–*** correlated with NT-proBNP,LV volumes and ejection fraction(strongest LV GLS with LV EF:r–0.539,p<0.001,strongest LV GLSre with age:r–0.376,p<0.001).During median follow-up of 5.9[5.5–6.2]years,the endpoint occurred in 53(38%)patients:4 patients died,9 developed heart failure,22 arrhythmias,8 thrombo-embolic events and 35 *** LV GLS(standardized HR(sHR 0.62(95%CI 0.47–0.81)and GLSre(sHR 0.62(95%CI 0.47–0.83)were associated with the *** multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume,NT-proBNP and prior ***:Left ventricular GLS and GLSre are reduced in adult patients with congenital *** markers are associated with adverse cardiac events and have clear clinical relevance.

关键词： Speckle tracking echocardiography congenital aortic stenosis strain prognosis

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Endometriosis: An “Unexplored”, “Neglected” or “Forgotten” Condition in Africa?

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Open journal of Obstetrics and Gynecology 2019年第1期9卷 29-41页

作者： john j. K. Annan roderick E. Larsen-Reindorf George A. A. Frimpong Osei Owusu-Afriyie Department of Obstetrics and Gynaecology School of Medical Sciences Kwame Nkrumah University of Science and Technology Kumasi Ghana Department of Radiology School of Medical Sciences Kwame Nkrumah University of Science and Technology Kumasi Ghana Department of Pathology Komfo Anokye Teaching Hospital Kumasi Ghana

Endometriosis is a condition that is commonly encountered in gynaecological practice. Endometriosis has varied, non-specific clinical presentations and its diagnosis sometimes poses a challenge to the clinician. We have encountered various women with clinical presentation highly suggestive of endometriosis. Further diagnosis using imaging and biopsy confirmed endometriosis. This paper presents our experience of endometriosis in our care of African women and a review of the literature on endometriosis. We want to highlight the various clinical presentations of endometriosis, appraise the various methods for diagnosis, discuss the treatment modalities available and evaluate the impact of this condition on women. We want to emphasize the fact that African women also suffer from this condition, and impress upon clinicians, the necessity for closer look for the condition, stimulate discussion and research into endometriosis in Africa as it seems this condition is “neglected or forgotten” in Africa.

关键词： Endometriosis Endometrioma Laparoscopy Pelvic Pain

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Systolic and Diastolic Strain Measurements Show Left Ventricular Dysfunction in Women with Turner Syndrome

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Congenital Heart Disease 2021年第4期16卷 357-368页

作者： Allard T.van den Hoven Sultan Yilmazer Lidia R.Bons roderick W.jvan Grootel L.W.Geenen An M.van Berendoncks Anthonie L.Duijnhouwer Hans-Marc j.Siebelink jolien W.Roos-Hesselink Annemien E.van den Bosch Department of Congenital Cardiology Erasmus MCRotterdamNetherlands Department of Cardiology Radboud University Medical CenterNijmegenNetherlands Department of Cardiology Leiden University Medical CenterRotterdamNetherlands

Aim:This study aimed to describe the systolic left ventricular global longitudinal strain(LvGLS)and left ventricular peak early diastolic strain rate(Sre)in adult women with Turner syndrome(TS)and to determine its relationship with exercise capacity and clinical ***:In this cross-sectional cohort study,consecutively included adult TS women underwent an electrocardiogram,transthoracic echocardiogram(TTE)and cardiopulmonary exercise test(CPET)on the same *** and Sre were measured using 2D speckle tracking analysis(STE)and compared with age-matched healthy female ***:Ninety-four adult women(age 36±13 years)with TS and 32 healthy age-matched female controls were *** with TS had a significantly impaired/reduced systolic LvGLS(–17.82±2.98%vs.–21.80±1.85%,p0.001),compared to healthy female ***,TS women had reduced diastolic function as measured by conventional echocardiographic parameters:A higher A-wave(p<0.001),lower E/A-ratio(p=0.001),longer deceleration time(p=0.006),and a higher E/E’-ratio(p<0.001).Women with TS also had a significantly reduced maximal workload(p=0.033),reduced oxygen uptake(p<0.001)and a reduced maximal heart rate(p<0.001)during *** linear regression analysis revealed that Age,karyotype and QT-duration were significantly associated with Sre,but not with LvGLS,in the TS ***:Systolic and diastolic strain and exercise capacity were significantly reduced in TS women compared to healthy *** correlation between strain itself and exercise capacity could be demonstrated,but correlations with conventional TTE parameters and baseline characteristics were found.

关键词： Turnersyndrome speckletracking echocardiography strain exercise capacity

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TEPCO 2000MW联合循环发电站的设计、建造和运行

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东方电气评论 1989年第2期3卷 54-62页

作者： L.O.Tomlinson j.M.roderick 李平

东京电力公司于1988年完成了世界上最大的,2000MW联合发电站的建造任务,14套165MW单轴联合循环系统中的发电设备已相继投运。本文介绍2000MW联合循环电站的设计特点、建造、起动及初期运行。

关键词：联合循环发电联合循环电站发电设备运行发电站东京电力公司投运最大世界系统

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Functional outcome in conservatively treated non-displaced scaphoid fractures

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Open journal of Clinical Diagnostics 2012年第4期2卷 66-69页

作者： Tessa Drijkoningen Frank j. P. Beeres roderick H. van Leerdam Daan Ootes Diana C. Grootendorst Marleen Otoide-Vree Steven j. Rhemrev Department of Trauma Surgery Medisch Centrum Haaglanden Hague Netherlands Landsteiner Instituut Medisch Centrum Haaglanden Hague Netherlands Marleen Otoide-Vree

Purpose: This study evaluated the functional outcome after conservative treatment of non-displaced scaphoid fractures using an international validated outcome scale (DASH). Methods and materials: Between 2005 and 2010, 60 patients with a non-displaced scaphoid fracture were included. When a patient visited the emergency department and was clinically suspected of a scaphoid fracture radiography was performed. If no fracture was diagnosed with this modality a CT or MRI-scan both in combination with bonescintigraphy was performed. Patients with scaphoid fractures diagnosed with CT/MRI and bonescintigraphy were treated with a six-week scaphoid forearm cast. Within 1 year after cast removal patients filled in the DASH questionnaire. Results: Sixty (80%) patients returned the DASH questionnaire at 12 months after treatment. Thirty-eight (63.3%) were male and the mean age was 35 (range 11 - 83). Forty-four (73.3%) patients had a mid-waist fracture of the scaphoid, 13 (21.7%) had a fracture of the distal pole and three (0.05%) had a proximal fracture. Median DASH score at one year after the trauma was 6 (range 3 - 15) for patients with a distal pole fracture and 5 (range 0 - 21.5) for mid-waist fractures (p = 0.7, table 2). For the three patients with a proximal scaphoid fracture the DASH scores appeared higher and were 83, 82 and 30 respectively. Conclusion: Conservative treatment for six weeks with a below the elbow cast is sufficient for the majority of patients with an occult distal or mid-waist scaphoid fracture and results in a good functional outcome according to the DASH questionnaire.

关键词： DASH Scaphoid Fracture Os scaphoideum Hand Trauma Conservative Treatment

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Spatially and spectrally resolved orbital angular momentum interactions in plasmonic vortex generators

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Light(Science & Applications) 2019年第1期8卷 907-915页

作者： jordan A.Hachtel Sang-Yeon Cho roderick B.Davidson II Matthew A.Feldman Matthew F.Chisholm Richard F.Haglund juan Carlos Idrobo Sokrates T.Pantelides Benjamin j.Lawrie Center for Nanophase Materials Science Oak Ridge National LaboratoryOak RidgeTN 37831USA Klipsch School of Electrical and Computer Engineering New Mexico State UniversityLas CrucesNM 88003USA Department of Physics and Astronomy Vanderbilt UniversityNashvilleTN 37235USA Quantum Information Science Group Oak Ridge National LaboratoryOak RidgeTN 37831USA Materials Science and Technology Division Oak Ridge National LaboratoryOak RidgeTN 37831USA Department of Electrical Engineering and Computer Science Vanderbilt University NashvilleNashvilleTN 37235USA Chemistry Division U.S.Naval Research LaboratoryWashingtonD.C.20375USA

Understanding the near-field electromagnetic interactions that produce optical orbital angular momentum(OAM)is crucial for integrating twisted light into ***,we examine the cathodoluminescence(CL)of plasmonic vortices carrying OAM generated in spiral *** nanospiral geometry defines a photonic local density of states that is sampled by the electron probe in a scanning transmission electron microscope(STEM),thus accessing the optical response of the plasmonic vortex with high spatial and spectral *** map the full spectral dispersion of the plasmonic vortex in spiral structures designed to yield increasing topological ***,we fabricate nested nanospirals and demonstrate that OAM from one nanospiral can be coupled to the nested nanospiral,resulting in enhanced luminescence in concentric spirals of like handedness with respect to concentric spirals of opposite *** results illustrate the potential for generating and coupling plasmonic vortices in chiral nanostructures for sensitive detection and manipulation of optical OAM.

关键词： momentum spectral angular

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终末期心力衰竭患者的肌成纤维细胞表型和纤维化的可逆性

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中华高血压杂志(中英文) 2019年第10期 999-999页

作者： Nagaraju CK Robinson EL Abdesselem M Trenson S Dries E Gilbert G janssens S Van Cleemput j Rega F Meyns B roderick HL Driesen RB Sipido KR 林立超余惠珍朱鹏立

心肌间质纤维化通过激活成纤维细胞分化为肌成纤维细胞(myofibroblasts, MyoFb)导致舒张及收缩功能不全,为心力衰竭的重要病理基础。近期临床证据表明,在终末期心力衰竭患者中,心肌间质纤维化是不可逆转的病理过程。本文研究目的为探讨... 详细信息

心肌间质纤维化通过激活成纤维细胞分化为肌成纤维细胞(myofibroblasts, MyoFb)导致舒张及收缩功能不全,为心力衰竭的重要病理基础。近期临床证据表明,在终末期心力衰竭患者中,心肌间质纤维化是不可逆转的病理过程。本文研究目的为探讨终末期心力衰竭患者的心肌组织中MyoFb的分化程度和细胞表型逆转的潜在性。方法:两种来源获取成纤维细胞:①从因缺血性或扩张性心肌病需心脏移植患者的左心室分离;②从捐献者的心脏中分离。成纤维细胞经体

关键词：终末期心力衰竭肌成纤维细胞纤维化可逆性

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高电压电动汽车竞速赛事给实现成熟技术纯电动汽车的终极目标展现了曙光

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工业设计 2009年第11期 27-30页

作者： Charles j.Murray

通过装备数百磅的电池并且给驱动马达"猛灌"电流,在短程加速赛车中小型电动赛车可打败汽油赛车。roderick Wilde的汽车性能如此强大,以至于它们都可以抬前轮(只后轮着地),也发生过折断车轴的情况。并且,在短程加速赛中,他们打败了450马... 详细信息

通过装备数百磅的电池并且给驱动马达"猛灌"电流,在短程加速赛车中小型电动赛车可打败汽油赛车。roderick Wilde的汽车性能如此强大,以至于它们都可以抬前轮(只后轮着地),也发生过折断车轴的情况。并且,在短程加速赛中,他们打败了450马力的Dodge Viper。"要是我们开得更快一些,会让那些Viper车看起来就像没动一样,"Wilde吹嘘,他是一名资深赛车爱好者。"那些车手可能自己也会气得甩车门而去。"

关键词：电动车电力动车马达控制器电动马达扭矩计算电池制造商并联调节器汽车曙光

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