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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 | A new laboratory EUV reflectometer for large optics using a laser plasma source显示文摘 | LUDWING V L THOMAS B HERMANN M | 2003 | SPIE2003，5038，： | 2 |
| 3 | Polycystic ovary syndrome as a form of functional ovarian hyperandrogenism due to dysregulation of androgen secretion显示文摘 | Hermann D A Barnes R B Rosenfield R L | 1995 | Endocr Rev1995，15，5： | 1 |
| 4 | Identification of novel mammalian caspases reveals an important role of gene loss in shaping the human caspase repertoire 显示文摘 | Eckhart L Ballaun C Hermann M | 2008 | Mol Biol Evol2008，25，5： | 1 |
| 5 | Distinct populations of cancer stem cells determine tumor growth and metastatic activity in human pancreatic cancer显示文摘 | Hermann P C Huber S L Herrler T | 2007 | Cell Stem Cell2007，1，： | 1 |
| 6 | Analysis of the cellular uptake and nuclear delivery of insulin-like growth factor binding proteim3 in human osteosarcoma cells显示文摘 | Micutkova L Hermann M Offterdinger M | 2012 | Int J Cancer2012，130，7： | 1 |
| 7 | Acute decompensated heart failure: formulating an evidence-based approach to diagnosis and treatment(part I)显示文摘 | CHUNG P HERMANN L | 2006 | Mt Sinai J Med2006，73，2： | 1 |
| 8 | The Oblique-plate Impact Experiment显示文摘 | Abou-Sayed Clifton R J Hermann L | 1976 | Experimental Mechanics1976，，4： | 1 |
| 9 | Muhielement stable isotope ratio analysis of glycerol to determine its origin in wine显示文摘 | ROSSMANN A SCHMIDT H L HERMANN A | 1998 | Zeitschrift fur Lebensmittel- Untersuchung und Forschung A1998，207，3： | 1 |
| 10 | Effects of chain length, chlorination degree, and structure on the octanol-water partition coefficients of polychlorinated n-alkanes显示文摘 | Bettina H Hermann F Wolfgang V L | 2011 | Environmental Science and Technology2011，45，： | 1 |
| 11 | Absorption of estrogens from vaginal creams显示文摘 | Rigg L A Hermann H Yen S S | 1978 | N Engl J Med1978，33，6： | 1 |
| 12 | Multielement stable isotope ratio analysis of glycerol to determine its origin in wine显示文摘 | Roβmann A Schmidt H L Hermann A | 1998 | Zeitschrift fur Lebensmitteluntersuchung und-Forschung A1998，207，3： | 1 |
| 13 | Distinct populations of cancer stem cells determine tumor growth and metastatic activity in human pancreatic cancer显示文摘 | HERMANN P C HUBER S L HERRLER T | 2007 | Cell Stem Cell2007，1，3： | 1 |
| 14 | An Evidence-based practice primer for infu-sion nurses显示文摘 | Bays C L Hermann C P | 2010 | Infus Nuts2010，33，4： | 1 |
| 15 | Tumor inifltration of the bone marrow:comparative study using computed tomography显示文摘 | Hermann G Rose JS Strauss L | | 0，，01： | 1 |
| 16 | Surface plasmon polaritons in generalized slab heterostructures with negative permittivity and permeability 显示文摘 | TSAKMAKIDIS K L HERMANN C KLAEDTKE A et al | 2006 | Phys Rev B2006，73，08： | 1 |
| 17 | Energetic and thermal performance of high-gain diode-side-pumped Nd : YAG rods显示文摘 | Fluck R Hermann M R Haekel L A | 2000 | Appl Phys B2000，70，4： | 1 |
| 18 | Refinement of the 12q14 microdeletion syndrome: primordial dwarfism and developmental delay with or without ostenpoikilosis 显示文摘 | Marl F Hermanns P Maria L Galluzzi F Scott D Lee B | 2009 | EuropJHumanGenet2009，17，10： | 1 |
| 19 | CRP and CD14 polymorphisms correlate with coronary plaque volume in patients with coronary artery disease – IVUS substudy of the ENCORE trials显示文摘 | Matthias Hermann Dieter Fischer Michael M. Hoffmann Theo Gasser Kurt Quitzau Thomas Meinertz Thomas Münzel Thomas F. Lüscher | 2011 | Atherosclerosis2011，，1： | 1 |
| 20 | Pyogenic hepatic abscess: results of currentmanagement 显示文摘 | Hashhnoto L Hermann R Grundrest - Broniaowski S | 1999 | AmSurg1999，61，5： | 1 |
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