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| 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 | The relationships among root and leaf traits of 76 grassland species and relative abundance along fertility and disturbance gradients 显示文摘 | Craine J M Froehle J Tilman D G | 2001 | Oikos2001，93，2： | 1 |
| 3 | RF MEMS for ubiquitous wireless connectivity:Part 1-Fabrication显示文摘 | De Los Santos H J Fischer G Tilmans H A C | 2004 | IEEE Microwave Mag2004，5，4： | 1 |
| 4 | Agricultural sustainability and intensive production practices 显示文摘 | Tilman D Cassman K G Matson P A | 2002 | Nature2002，418，： | 1 |
| 5 | The relationships amono groot and leaftraits of 76 grassland species and relativive abundance along fertility and disturbance gradients显示文摘 | Crine J M Frochle J Tilman D G | 2001 | Oikos2001，93，： | 1 |
| 6 | Eutrophication: impacts of excess nutrient inputs on freshwater, marine, and terrestrial ecosystems显示文摘 | Smith V H Tilman G D Nekola J C | 1999 | Environ Poll1999，100，： | 1 |
| 7 | Agricultural sustainability and intensive production practices显示文摘 | Tilman D Cassman K G Matson P A | 2002 | Nature2002，418，6898： | 1 |
| 8 | Agent-based simulation of consumer behavior in grocery shopping on a regional level显示文摘 | Tilman A. Schenk Günter L?ffler Jürgen Rauh | 2007 | Journal of Business Research2007，，8： | 1 |
| 9 | Eutrophication:impacts of excess nutrient inputs on freshwater,marine and terrestrial ecosystems显示文摘 | Smith V H Tilman G D Nekola J C | 1999 | Environ Poll1999，100，： | 1 |
| 10 | Drueke parathyroid hormone,Vitamin D and Cardiovascular disease in CRF显示文摘 | Stephen G Rostand Tilman B | 1999 | Kidney Int1999，56，： | 1 |
| 11 | Agricultural sustain- ability and intensive production practices 显示文摘 | Tilman D Cassman K G Matsan P A | 2002 | Nature2002，418，6898： | 1 |
| 12 | Eutrophication : impacts of excessnutrient inputs on freshwater, marine and terrestrial ecosystems 显示文摘 | Smith V H Tilman G D Nekola J C | 1999 | Environmental Pollution1999，100，1： | 1 |
| 13 | Eutrophication: impacts of excess nutrient inputs on freshwater, Marine and terrestrial ecosystems显示文摘 | SMITH V H TILMAN G D NEKOLA J C | 1999 | Environmental Pollution1999，100，： | 1 |
| 14 | Robust vehicle steering control design based on the disturbance observer 显示文摘 | i TILMAN B DIRK O BILIN A G | 2002 | Annual Reviews in Control2002，26，： | 1 |
| 15 | Agricultural sustainability and intensive production practices 显示文摘 | David Tilman Kenneth G Cassman Pamela A Matson | 2002 | Nature2002，418，： | 1 |
| 16 | Human periostin gene expression in normal tissues,tumors and melanoma:evidences for periostin production by both stromal and melanoma cells显示文摘 | Tilman G Mattiussi M Brasseur F | 2007 | Mol Cancer2007，17，6： | 1 |
| 17 | Drueke parathyoid homone, vitamin D, and cardiovascular disease in CRF 显示文摘 | STEPHEN G ROSTAND TILMAN B | 1999 | Kindney Int1999，56，2： | 1 |
| 18 | The rela- tionships among root and leaf traits of 76 grassland spe- cies and relative abundance along fertility and disturb- ance gradients显示文摘 | Craine J M Froehle J Tilman D G | 2001 | Oikos2001，93，27： | 1 |
| 19 | Drueke Parathyroid hormone , Vitamin D, and cardiovascular disease in CRF显示文摘 | Stephen G Rostend Tilman B | 1999 | Kidney Int1999，56，： | 1 |
| 20 | Eutrophication: im- pacts of excess nutrient inputs on freshwater, marine, and terrestrial ecosystem显示文摘 | Smith V H Tilman G D Nekola J C | 1999 | Environment Pollution1999，100，13： | 1 |
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