LEADER 05154nam 2200661 a 450 001 9910437985703321 005 20200520144314.0 010 $a3-642-37334-8 024 7 $a10.1007/978-3-642-37334-3 035 $a(CKB)3280000000020568 035 $a(EBL)1316856 035 $a(SSID)ssj0000904301 035 $a(PQKBManifestationID)11545181 035 $a(PQKBTitleCode)TC0000904301 035 $a(PQKBWorkID)10920510 035 $a(PQKB)11365066 035 $a(DE-He213)978-3-642-37334-3 035 $a(MiAaPQ)EBC1316856 035 $a(PPN)170491447 035 $a(EXLCZ)993280000000020568 100 $a20130603d2013 uy 0 101 0 $aeng 135 $aur|n|---||||| 181 $ctxt 182 $cc 183 $acr 200 00$aJIMD reports$hVolume 10 /$fJohannes Zschocke, K. Michael Gibson, editors-in-chief ; Garry Brown, Eva Morava, editors ; Verena Peters, managing editor 205 $a1st ed. 2013. 210 $aHeidelberg ;$aNew York $cSpringer $cSSIEM$dc2013 215 $a1 online resource (124 p.) 225 1 $aJIMD reports,$x2192-8304 ;$vv. 10 300 $aDescription based upon print version of record. 311 $a3-642-37333-X 320 $aIncludes bibliographical references. 327 $aContents; Prevalence and Novel Mutations of Lysosomal Storage Disorders in United Arab Emirates; Abstract; Introduction; Materials and Methods; Patients; Birth Prevalence; Mutation Analysis; Results; Discussion; Disclosure Statement; References; Chitotriosidase Deficiency: A Mutation Update in an African Population; Abstract; Introduction; Materials and Methods; Subjects; Methods; Plasma Chitotriosidase Assay; CHIT1 Mutation Detection; Population Screening for CHIT1 Mutations; Evaluation of Missense Mutations; Results; Plasma chito activities and CHIT1 genotyping; Discussion and Conclusion 327 $aReferencesNDUFS8-related Complex I Deficiency Extends Phenotype from ``PEO Plus ? ? to Leigh Syndrome; Abstract; Introduction; Case Reports; Patient 1; Patient 2; Patient 3; Results; Discussion; Synopsis; Author Contribution; Funding; Ethical Issues; Competing Interest Statement; References; Accuracy of Six Anthropometric Skinfold Formulas Versus Air Displacement Plethysmography for Estimating Percent Body Fat in Female Adolescents with Phenylketonuria; Abstract; Introduction; Subjects and Methods; Subjects; Data Collection; Anthropometric Calculations; Statistical Analysis; Results 327 $aDemographics, BMI, and ADP Body CompositionMean Values and Correlations; Bland-Altman Agreement; Variation in Percent Error; Discussion; Agreement of Tested Equations with ADP Results; Adiposity in PKU and Clinical Relevance; Conclusion; Synopsis; Contributions of Individual Authors; Author Serving as Guarantor; Competing Interest Statement; Details of Funding; Details of Ethics Approval; Patient Consent Statement; References; Noncompaction of the Ventricular Myocardium and Hydrops Fetalis in Cobalamin C Disease; Abstract; Introduction; Case Report; Discussion; Synopsis; References 327 $aPrimary Carnitine (OCTN2) Deficiency Without Neonatal Carnitine DeficiencyAbstract; Introduction; References; MNGIE Syndrome: Liver Cirrhosis Should Be Ruled Out Prior to Bone Marrow Transplantation; Abstract; Introduction; Financial Disclosure; References; Differential Phonological Awareness Skills in Children with Classic Galactosemia: A Descriptive Study of Four Cases; Abstract; Introduction; Method; Participants; Child 1; Child 2; Child 3; Child 4; Measures; Procedure; Descriptive Analyses; Results; Discussion; Clinical Implications and Conclusion; References 327 $aDihydropteridine Reductase Deficiency and Treatment with Tetrahydrobiopterin: A Case ReportAbstract; Introduction; Case Report; Biochemical Results; Discussion; Synopsis; References to Electronic Databases; References; Stem Cell Transplantation for Adult-Onset Krabbe Disease: Report of a Case; Abstract; Introduction; Report of a Case; Disclosures and Funding; References; Detection by Urinary GAG Testing of Mucopolysaccharidosis Type II in an At-Risk Spanish Population; Abstract; Introduction; Objective; Patients and Methods; Study Population; Methods; Statistical Analysis; Results; Discussion 327 $aSynopsis 330 $aJIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder. 410 0$aJIMD reports ;$vv. 10. 606 $aMetabolism, Inborn errors of 615 0$aMetabolism, Inborn errors of. 676 $a616.3 676 $a616.39042 701 $aZschocke$b Johannes$0291660 701 $aGibson$b K. Michael$01758394 701 $aBrown$b Garry$01758395 701 $aMorava$b Eva$01758396 701 $aPeters$b Verena$01758397 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910437985703321 996 $aJIMD reports$94196592 997 $aUNINA LEADER 03775nam 22006855 450 001 9910299432703321 005 20200704023931.0 010 $a3-319-09849-7 024 7 $a10.1007/978-3-319-09849-4 035 $a(CKB)3710000000332326 035 $a(EBL)1965412 035 $a(OCoLC)899495744 035 $a(SSID)ssj0001424303 035 $a(PQKBManifestationID)11891200 035 $a(PQKBTitleCode)TC0001424303 035 $a(PQKBWorkID)11367566 035 $a(PQKB)11705248 035 $a(DE-He213)978-3-319-09849-4 035 $a(MiAaPQ)EBC1965412 035 $a(PPN)18351680X 035 $a(EXLCZ)993710000000332326 100 $a20150105d2015 u| 0 101 0 $aeng 135 $aur|n|---||||| 181 $ctxt 182 $cc 183 $acr 200 10$aEmergent Spatio-temporal Dimensions of the City $eHabitus and Urban Rhythms /$fby Fabian Neuhaus 205 $a1st ed. 2015. 210 1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2015. 215 $a1 online resource (335 p.) 300 $aDescription based upon print version of record. 311 $a3-319-09848-9 320 $aIncludes bibliographical references and index. 327 $aUrban Rhythms -- Urban Machine and Time Space -- Body Space and Spatial Narrative -- Urban Diary -- New City Landscape -- Structuring Time -- Structuring Space -- Temporality - The Rhythmic City. 330 $aThis book focuses on the creation of space as an activity. The argument draws not only on aspects of movement in time, but also on a cultural and specifically social context influencing the creation of the spatial habitus. The book reconsiders existing theories of time and space in the field of urban planning and develops an updated account of spatial activity, experience and space-making. Recent developments in spatial practice, specifically related to new technologies, make this an important and timely task. Integrating spatial-temporal dynamics into the way we think about cities aids the implementation of sustainable forms of urban planning. The study is composed of two different case studies. One case is based on fieldwork tracking individual movement using GPS, the other case utilises data mined from Twitter. One of the key elements in the conclusion to this book is the definition of temporality as a status rather than a transition. It is argued that through repetitive practices as habitus, time has presence and agency in our everyday lives. This book is based on the work undertaken for a PhD at the Centre for Advanced Spatial Analysis and was and accepted as thesis by University College London in 2013. 606 $aRegional planning 606 $aCity planning 606 $aCity planning 606 $aSociophysics 606 $aEconophysics 606 $aLandscape/Regional and Urban Planning$3https://scigraph.springernature.com/ontologies/product-market-codes/J15000 606 $aUrbanism$3https://scigraph.springernature.com/ontologies/product-market-codes/K18006 606 $aData-driven Science, Modeling and Theory Building$3https://scigraph.springernature.com/ontologies/product-market-codes/P33030 615 0$aRegional planning. 615 0$aCity planning. 615 0$aCity planning. 615 0$aSociophysics. 615 0$aEconophysics. 615 14$aLandscape/Regional and Urban Planning. 615 24$aUrbanism. 615 24$aData-driven Science, Modeling and Theory Building. 676 $a710 676 $a711.4 676 $a910 700 $aNeuhaus$b Fabian$4aut$4http://id.loc.gov/vocabulary/relators/aut$01063532 906 $aBOOK 912 $a9910299432703321 996 $aEmergent Spatio-temporal Dimensions of the City$92532909 997 $aUNINA