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| 1 | Imaging of gaucher disease显示文摘Gaucher disease is the prototypical lysosomal storage disease.It results from the accumulation of undegrad-ed glucosylceramide in the reticuloendothelial system of the bone marrow,spleen and liver due to deficiency of the enzyme glucocerebrosidase.This leads to he-matologic,visceral and skeletal maifestions.Build up of glucosylceramide in the liver and spleen results in hepatosplenomegaly.The normal bone marrow is re-placed by the accumulating substrate leading to many of the hematologic signs including anemia.The visceral and skeletal manifestations can be visualized with vari-ous imaging modalities including radiography,com-puted tomography,magnetic resonance imaging(MRI)and radionuclide scanning.Prior to the development of enzyme replacement therapy,treatment was only sup-portive.However,once intravenous enzyme replace-ment therapy became available in the 1990s it quickly became the standard of care.Enzyme replacement therapy leads to improvement in all manifestations.Thevisceral and hematologic manifestations respond more quickly usually within a few months or years.The skel-etal manifestations take much longer,usually several years,to show improvement.In recent years newer treatment strategies,such as substrate reduction thera-py,have been under investigation.Imaging plays a key role in both initial diagnosis and routine monitoring of patient on treatment particularly volumetric MRI of the liver and spleen and MRI of the femora for evaluating bone marrow disease burden. | William L Simpson George Hermann Manisha Balwani | 2014 | World Journal of Radiology2014，6，9： | 3 |
| 2 | Probability of random sire exclusion using microsatellite markers for parentage verification显示文摘 | Usha A P Simpson S P Williams J L | 1995 | Anita Genet1995，26，3： | 1 |
| 3 | Hysterosalpingography:A Reemerging Study显示文摘 | William L Simpson Laura G B | 2006 | Education Exhibit2006，26，2： | 1 |
| 4 | Probability of random sire exclusion using microsatellite markers for parentage verification显示文摘 | Usha A P Simpson S P Williams J L | 1995 | Animal Genetics1995，26，3： | 1 |
| 5 | A novel glucose-sensing mechanism contributing to glu- cagon-like peptide-1 secretion from the GLUTag cell line 显示文摘 | GRIBBLE F WILLIAMS L SIMPSON A | 2003 | Diabetes2003，52，5： | 1 |
| 6 | The impact of tidal straining on the cycle of turbulence in a partially strat- ified estuary显示文摘 | SIMPSON J H WILLIAMS E BRASSEUR L H | 2005 | Continental Shelf Research2005，25，： | 1 |
| 7 | The impact of tidal straining on the cycle of turbulence in a partially strati-fied estuary显示文摘 | SIMPSON J H WILLIAMS E BRASSEUR L H | 2005 | Continental Shelf Research2005，25，： | 1 |
| 8 | Heat sterilization times of red pine boards显示文摘 | William T Simpson Barbara L lllman | 2004 | Forest Products Laboratory2004，52，12： | 1 |
| 9 | Atmospheric composition change: Ecosystems-Atmosphere interactions显示文摘 | Fowler D Pilegaard K Sutton MA Ambus P Raivonen M Duyzer J Simpson D Fagerli H Fuzzi S Schjoerring JK Granier C Neftel A Isaksen ISA Laj P Maione M Monks PS Burkhardt J Daemmgen U Neirynck J Personne E Wichink-Kruit R Butterbach-Bahl K Flechard C Tuovinen JP Coyle M Gerosa G Loubet B Altimir N Gruenhage L Ammann C Cieslik S Paoletti E Mikkelsen TN Ro-Poulsen H Cellier P Cape JN Horwith L Loreto F Niinemets Palmer PI Rinne J Misztal P Nemitz E Nilsson D Pryor S Gallagher MW Vesala T Skiba U Briiggemann N Zechmeister-Boltenstern S Williams J O'dowd C Facchini MC De LeeuwG Flossman A Chaumerliac N Erisman JW | 2009 | Atmospheric Environment2009，43，33： | 1 |
| 10 | A novel glucose-sensing mechanism contributing to glucagon-like peptide-1 secretion from the GLUTag cell line显示文摘 | Gribble FM Williams L Simpson AK Reimann F | 2003 | Diabetes2003，52，5： | 1 |
| 11 | Isolation of Edwardsiella tarda form aquatic animal species and surface waters in Florida显示文摘 | WHITE F H SIMPSON C F WILLIAMS L E | 1973 | J Wildlife Dis1973，，9： | 1 |
| 12 | Cloning of a cDNA for s stylar glycoprotein associated with expression of self-incompatibility in Nicotiana alata显示文摘 | Anderson M A Cornish E C Mau S L Williams E G Hoggart R Atkison A Bonig I Grego B Simpson R Roche P J Haley J D Penschow J D Niall H D Tregear G W Coghlan J P Crawford R J Clark A E | 1986 | Nature1986，321，： | 1 |
| 13 | probability of random sire exclusion using microsatellite markers for parentage verification显示文摘 | Usha A P Simpson S P Williams J L | 1995 | Animal Genetics1995，26，3： | 1 |
| 14 | Probability of random sire exclusion using microsatellite markers for parentage of vertificaiong显示文摘 | Usga A P Simpson S P Williams J L | 1995 | Animal Genetics1995，26，3： | 1 |
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