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	题名	作者	年代	出处	被引量
1	Differential diagnosis in patients with suspected bile acid synthesis defects显示文摘AIM: To investigate the clinical presentations associated with bile acid synthesis defects and to describe identification of individual disorders and diagnostic pitfalls. METHODS: We describe semiquantitative determination of 16 urinary bile acid metabolites by electrospray ionization-tandem mass spectrometry. Sample preparation was performed by solid-phase extraction. The total analysis time was 2 min per sample. We determined bile acid metabolites in 363 patients with suspected defects in bile acid metabolism. RESULTS: Abnormal bile acid metabolites were found in 36 patients. Two patients had bile acid synthesis defects but presented with atypical presentations. In 2 other patients who were later shown to be affected by biliary atresia and cystic fibrosis the profile of bile acid metabolites was initially suggestive of a bile acid synthesis defect. Three adult patients suffered from cerebrotendinous xanthomatosis. Nineteen patients had peroxisomal disorders, and 10 patients had cholestatic hepatopathy of other cause. CONCLUSION: Screening for urinary cholanoids should be done in every infant with cholestatic hepatopathy as well as in children with progressive neurological disease to provide specific therapy.	Dorothea Haas Hongying Gan-Schreier Claus-Dieter Langhans Tilman Rohrer Guido Engelmann Maura Heverin David W Russell Peter T Clayton Georg F Hoffmann Jürgen G Okun	2012	World Journal of Gastroenterology2012，18，10：	2
2	Clinical prospective study of biochemical markers and evoked potentials for identifying adverse neurological outcome after thoracic and thoracoabdominal aortic aneurysm surgery显示文摘	Lases E C Schepens M A Haas F J	2005	Br J Anaesth2005，95，5：	1
3	Ⅳ immunoglobulins as add-on treatment to methylprednisolone for acute relapses in MS 显示文摘	Sorensen PS Haas J Sellebjerg F	2004	Neurology2004，63，11：	1
4	Expression of suwivin in rectal cancer is inversely correlated to apoptosis in-vitro and in-vivo and may predict tumor response to neoadjuvant radiochemotherapy显示文摘	F ROdel J hOffman J haas	2002	Radiation Oncology2002，54，2：	1
5	The importance of the histologic processing of pelvic lymph nodes in the treatment on cervical cancer显示文摘	Haas J	1993	Int J Gynecol Cancer1993，3，4：	1
6	Interaction of weak shock waves with cylindrical and spherical gas inhomogeneities 显示文摘	Haas J F Sturtevant B	1987	J Fluid Mech1987，181，：	1
7	Uhrastructure of ma- lignant rhabdoid tumor of the kidney: a distinctive renal tumor of children显示文摘	Haas J E Palmer N F Weinberg A G	1981	Hum Pathol1981，12，7：	1
8	The major genetic determinants of HIV-1control affect HLA class I peptide presentation显示文摘	International HIV Controllers Study Pereyra F Jia X McLaren P J Telenti A de Bakker P I Walker B D Ripke S Brumme C J Pulit S L Carrington M Kadie C M Carlson J M Heckerman D Graham R R Plenge R M Deeks S G Gianniny L Crawford G Sullivan J Gonzalez E Davies L Camargo A Moore JM Beattie N Gupta S Crenshaw A Burtt N P Guiducci C Gupta N Gao X Qi Y Yuki Y Piechocka-Trocha A Cutrell E Rosenberg R Moss K L Lemay P O'Leary J Schaefer T Verma P Toth I Block B Baker B Rothchild A Lian J Proudfoot J Alvino D M Vine S Addo M M Allen T M Altfeld M Henn M R Le Gall S Streeck H Haas D W Kuritzkes D R Robbins G K Shafer R W Gulick R M Shikuma C M Haubrich R Riddler S Sax P E Daar E S Ribaudo H J Agan B Agarwal S Ahern R L Allen B L Altidor S Altschuler E L Ambardar S Anastos K Anderson B Anderson V Andrady U Antoniskis D Bangsberg D Barbaro D Barrie W Bartczak J Barton S Basden P Basgoz N Bazner S Bellos N C Benson A M Berger J Bernard N F Bernard A M Birch C Bodner S J Bolan R K Boudreaux E T Bradley M Braun J F Brndjar J E Brown S J Brown K Brown S T Burack J Bush LM Cafaro V Campbell O Campbell J Carlson R H Carmichael J K Casey K K Cavacuiti C Celestin G Chambers S T Chez N Chirch L M Cimoch P J Cohen D Cohn LE Conway B Cooper D A Cornelson B Cox D T Cristofano M V Cuchural G Jr Czartoski J L Dahman J M Daly J S Davis B T Davis K Davod S M DeJesus E Dietz C A Dunham E Dunn M E Ellerin T B Eron J J Fangman J J Farel C E Ferlazzo H Fidler S Fleenor-Ford A Frankel R Freedberg K A French N K Fuchs JD Fuller J D Gaberman J Gallant J E Gandhi R T Garcia E Garmon D Gathe J C Jr Gaultier C R Gebre W Gilman F D Gilson I Goepfert P A Gottlieb M S Goulston C Groger R K Gurley T D Haber S Hardwicke R Hardy W D Harrigan P R Hawkins T N Heath S Hecht F M Henry W K Hladek M Hoffman R P Horton J M Hsu R K Huhn G D Hunt P Hupert M J Illeman M L Jaeger H Jellinger R M John M Johnson J A Johnson K L Johnson H Johnson K Joly J Jordan W C Kauffman C A Khanlou H Killian R K Kim A Y Kim D D Kinder C A Kirchner J T Kogelman L Kojic E M Korthuis P T Kurisu W Kwon D S LaMar M Lampiris H Lanzafame M Lederman M M Lee D M Lee J M Lee M J Lee E T Lemoine J Levy J A Llibre J M Liguori M A Little S J Liu A Y Lopez A J Loutfy M R Loy D Mohammed D Y Man A Mansour M K Marconi V C Markowitz M Marques R Martin J N Martin H L Jr Mayer K H McElrath M J McGhee T A McGovern B H McGowan K McIntyre D Mcleod GX Menezes P Mesa G Metroka CE Meyer-Olson D Miller A O Montgomery K Mounzer K C Nagami E H Nagin I Nahass R G Nelson M O Nielsen C Norene D L O'Connor D H Ojikutu B O Okulicz J Oladehin O O Oldfield E C Olender S A Ostrowski M Owen WF Jr Pae E Parsonnet J Pavlatos A M Perlmutter A M Pierce M N Pincus J M Pisani L Price L J Proia L Prokesch R C Pujet H C Ramgopal M Rathod A Rausch M Ravishankar J Rhame F S Richards C S Richman D D Rodes B Rodriguez M Rose R C 3rd Rosenberg E S Rosenthal D Ross P E Rubin D S Rumbaugh E Saenz L Salvaggio M R Sanchez WC Sanjana V M Santiago S Schmidt W Schuitemaker H Sestak P M Shalit P Shay W Shirvani V N Silebi V I Sizemore J M Jr Skolnik P R Sokol-Anderson M Sosman J M Stabile P Stapleton J T Starrett S Stein F Stellbrink H J Sterman FL Stone V E Stone D R Tambussi G Taplitz R A Tedaldi E M Telenti A Theisen W Torres R Tosiello L Tremblay C Tribble M A Trinh P D Tsao A Ueda P Vaccaro A Valadas E Vanig T J Vecino I Vega V M Veikley W Wade B H Walworth C Wanidworanun C Ward D J Warner D A Weber R D Webster D Weis S Wheeler D A White D J Wilkins E Winston A Wlodaver C G van't Wout A Wright D P Yang O O Yurdin D L Zabukovic B W Zachary K C Zeeman B Zhao M	2010	Science2010，330，6010：	1
9	Fine-mapping classical HLA variation associated with durable host control of HIV-1infection in African Americans显示文摘	McLaren P J Ripke S Pelak K Weintrob A C Patsopoulos N A Jia X Erlich R L Lennon N J Kadie C M Heckerman D Gupta N Haas D W Deeks S G Pereyra F Walker B D de Bakker P I International HIV Controllers Study	2012	Hum Mol Genet2012，21，19：	1
10	Interaction of weak shock waves with cylindrical and spherical gas inhomogeneities 显示文摘	Haas J F Sturtevan B	1987	J Fluid Mech1987，181，：	1
11	Modified Zakharov equations for plasmas with a quantum correction显示文摘	Garcia L G Haas F de Oliveira L P L Goedert J	2005	Phys Plasmas2005，12，1：	1
12	查看详情显示文摘	Haas F A A1-Kuzee J Braithwaite N S J		0，，：	1
13	Sequendng Medicagotruncatula expressed sequenced tag using 454 life sciences technology显示文摘	Cheung F Haas B J Goldberg S M	2006	BMC Genomics2006，7，：	1
14	IV immunoglobulins as add-on treatment to methylr rednisolone for acute relapses in MS显示文摘	Sorensen PS Haas J Sellebjerg F	2004	Neurology2004，63，11：	1
15	The predictive value of D-dimer measurement for cancer in patients with deep vein thrombo- sis 显示文摘	Schutgens RE Beckers NN Haas F J	2005	Haematologica2005，90，2：	1
16	Quantum ion-acoustic waves显示文摘	HAAS F GARCIA L G GOEDERT J	2003	Physics of Plasmas2003，10，：	1
17	Bioabsorbable interbody cages in a sheep cervical spine fusion model显示文摘	Kandziora F Pflugmacher R Scholz M Eindorf T Schnake K J Haas N P	2004	Spine (Phila Pa 1976)2004，29，：	1
18	Overexpression of c-myc in hepato-cytes promotes activation of hepatic stellate cells and fa-cilitates the onset of liver fibrosis显示文摘	Nevzorova Y A Hu W Cubero F J Haas U FreimuthJ Tacke F	2013	Biochim BiophysActa2013，1832，：	1
19	Interaction of weak shock waves with cylindrical and spherical gas inhomogeneities显示文摘	HAAS J F STURTEVANT B	1987	Journal of Fluid Mechanics1987，181，：	1
20	Triacylglycerol synthesis enzymes mediate lipid droplet growth by relocalizing from the ER to lipid droplets 显示文摘	Wilfling F Wang H Haas J T	2013	Dev Cell2013，24，4：	1

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