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CLiC Cholestatic Liver Disease Consortium |
Washington University School of Medicine |
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Washington University School of Medicine
Contact Information:
Ross Shepherd, MD, FRACP, FRCP [Search for "Shepherd"]
CLiC Site Principal Investigator
E-mail: shepherd_r@kids.wustl.edu
Frances V. White, MD [Search for "White"]
CLiC Pathologist
Rosemary Nagy, MBA, RD, LD
CLiC Study Coordinator
E-mail: nagy_r@kids.wustl.edu
Current Studies:
6001: Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis
Useful Links:
Saint Louis Children's Hospital
Children's Hospital of Saint Louis: Find a Doctor
Saint Louis Children's Hospital Gastroenterology and Nutrition Department
Saint Louis Children's Hospita Liver Transplant Program
Publications:
1: Pereira TN, Lewindon PJ, Smith JL, Murphy TL, Lincoln DJ, Shepherd RW, Ramm GA.
Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease.
J Hepatol. 2004 Oct;41(4):576-83.
PMID: 15464237 [PubMed - indexed for MEDLINE]
2: Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA.
Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis.
Hepatology. 2004 Jun;39(6):1673-82.
PMID: 15185309 [PubMed - indexed for MEDLINE]
3: Weerasooriya VS, White FV, Shepherd RW.
Hepatic fibrosis and survival in biliary atresia.
J Pediatr. 2004 Jan;144(1):123-5.
PMID: 14722530 [PubMed - indexed for MEDLINE]
4: Greer R, Lehnert M, Lewindon P, Cleghorn GJ, Shepherd RW.
Body composition and components of energy expenditure in children with end-stage liver disease.
J Pediatr Gastroenterol Nutr. 2003 Mar;36(3):358-63.
PMID: 12604974 [PubMed - indexed for MEDLINE]
5: Perlmutter DH, Shepherd RW.
Extrahepatic biliary atresia: a disease or a phenotype?
Hepatology. 2002 Jun;35(6):1297-304. Review. No abstract available.
PMID: 12029613 [PubMed - indexed for MEDLINE]
6: Lewindon PJ, Pereira TN, Hoskins AC, Bridle KR, Williamson RM, Shepherd RW, Ramm GA.
The role of hepatic stellate cells and transforming growth factor-beta(1) in cystic fibrosis liver disease.
Am J Pathol. 2002 May;160(5):1705-15.
PMID: 12000722 [PubMed - indexed for MEDLINE]
7: Lehnert ME, Clarke DD, Gibbons JG, Ward LC, Golding SM, Shepherd RW, Cornish BH, Crawford DH.
Estimation of body water compartments in cirrhosis by multiple-frequency bioelectrical-impedance analysis.
Nutrition. 2001 Jan;17(1):31-4.
PMID: 11165885 [PubMed - indexed for MEDLINE]
8: Trocki O, Wotton MJ, Cleghorn GJ, Shepherd RW.
Value of total body potassium in assessing the nutritional status of children with end-stage liver disease.
Ann N Y Acad Sci. 2000 May;904:400-5.
PMID: 10865778 [PubMed - indexed for MEDLINE]
9: Shepherd RW.
Metabolic liver diseases in childhood.
Indian J Pediatr. 1995 Sep-Oct;62(5):519-24. No abstract available.
PMID: 10829918 [PubMed - indexed for MEDLINE]
10: Thomson MA, Lynch S, Strong R, Shepherd RW, Marsh W.
Orthotopic liver transplantation with poor neurologic outcome in valproate-associated liver failure: a need for critical risk-benefit appraisal in the use of valproate.
Transplant Proc. 2000 Feb;32(1):200-3. No abstract available.
PMID: 10701024 [PubMed - indexed for MEDLINE]
11: Ishikawa M, Lynch SV, Balderson GA, Shepherd RW, Ong TH, Strong RW.
Liver transplantation in Japanese and Australian/New Zealand children with biliary atresia: a 10-year comparative study.
Eur J Surg. 1999 May;165(5):454-9.
PMID: 10391162 [PubMed - indexed for MEDLINE]
12: Ramm GA, Nair VG, Bridle KR, Shepherd RW, Crawford DH.
Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia.
Am J Pathol. 1998 Aug;153(2):527-35.
PMID: 9708812 [PubMed - indexed for MEDLINE]
13: An JK, Blomenkamp K, Lindblad D, Teckman JH.
Quantitative isolation of alphalAT mutant Z protein polymers from human and mouse livers and the effect of heat.
Hepatology. 2005 Jan;41(1):160-7.
PMID: 15619240 [PubMed - indexed for MEDLINE]
14: Teckman JH.
Lack of effect of oral 4-phenylbutyrate on serum alpha-1-antitrypsin in patients with alpha-1-antitrypsin deficiency: a preliminary study.
J Pediatr Gastroenterol Nutr. 2004 Jul;39(1):34-7.
PMID: 15187777 [PubMed - indexed for MEDLINE]
15: Rudnick DA, Liao Y, An JK, Muglia LJ, Perlmutter DH, Teckman JH.
Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency.
Hepatology. 2004 Apr;39(4):1048-55.
PMID: 15057909 [PubMed - indexed for MEDLINE]
16: Teckman JH, An JK, Blomenkamp K, Schmidt B, Perlmutter D.
Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.
Am J Physiol Gastrointest Liver Physiol. 2004 May;286(5):G851-62. Epub 2003 Dec 18.
PMID: 14684378 [PubMed - indexed for MEDLINE]
17: Teckman JH, An JK, Loethen S, Perlmutter DH.
Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.
Am J Physiol Gastrointest Liver Physiol. 2002 Nov;283(5):G1156-65. Erratum in:
Am J Physiol Gastrointest Liver Physiol 2003 Jan;284(1):following table of contents.
PMID: 12381530 [PubMed - indexed for MEDLINE]
18: Teckman JH, Burrows J, Hidvegi T, Schmidt B, Hale PD, Perlmutter DH.
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
J Biol Chem. 2001 Nov 30;276(48):44865-72. Epub 2001 Sep 27.
PMID: 11577074 [PubMed - indexed for MEDLINE]
19: Lin L, Schmidt B, Teckman J, Perlmutter DH.
A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum.
J Biol Chem. 2001 Sep 7;276(36):33893-8. Epub 2001 Jun 26.
PMID: 11427540 [PubMed - indexed for MEDLINE]
20: Teckman JH, Perlmutter DH.
Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response.
Am J Physiol Gastrointest Liver Physiol. 2000 Nov;279(5):G961-74.
PMID: 11052993 [PubMed - indexed for MEDLINE]
21: Teckman JH, Gilmore R, Perlmutter DH.
Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum.
Am J Physiol Gastrointest Liver Physiol. 2000 Jan;278(1):G39-48.
PMID: 10644560 [PubMed - indexed for MEDLINE]
22: Marcus N, Teckman JH, Perlmutter DH.
Alpha1-antitrypsin deficiency: from genotype to childhood disease.
J Pediatr Gastroenterol Nutr. 1998 Jul;27(1):65-74. Review. No abstract available.
PMID: 9669729 [PubMed - indexed for MEDLINE]
23: Shteyer E, Liao Y, Muglia LJ, Hruz PW, Rudnick DA.
Disruption of hepatic adipogenesis is associated with impaired liver regeneration in mice.
Hepatology. 2004 Dec;40(6):1322-32.
PMID: 15565660 [PubMed - indexed for MEDLINE]
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