L W Powell
Affiliation: Queensland Institute of Medical Research
- The Queensland Institute of Medical Research. Excellence from the interplay of medicine and scienceL W Powell
Queensland Institute of Medical Research, Brisbane
Med J Aust 165:645-8. 1996..Yet the clinical objectives of research still motivate and inform the research effort...
- Role of early case detection by screening relatives of patients with HFE-associated hereditary haemochromatosisLawrie W Powell
The Queensland Institute of Medical Research, Brisbane, Australia
Best Pract Res Clin Haematol 18:221-34. 2005..The most cost-effective methods of early case detection are family (cascade) screening and evaluation of potential cases by primary care physicians with a high index of clinical suspicion...
- Hereditary hemochromatosis and iron overload diseasesLawrie W Powell
The Queensland Institute of Medical Research and the Royal Brisbane Hospital, Australia
J Gastroenterol Hepatol 17:S191-5. 2002
- Bridging the gap between basic science and clinical medicine: mentors and memoriesLawrie W Powell
Teaching and Research Unit, Royal Brisbane and Women s Hospitals, QLD
Med J Aust 177:657-60. 2002
- HemochromatosisL W Powell
Department of Medicine, University of Queensland, Brisbane, Australia
Clin Liver Dis 4:211-28, viii. 2000..In addition, it is now apparent that iron, even in the modest tissue concentrations, can act as a co-factor in potentiating cell injury in other liver diseases...
- Haemochromatosis in the new millenniumL W Powell
The Queensland Institute of Medical Research and the Joint Clinical Sciences Program, The University of Queensland, Brisbane, Australia
J Hepatol 32:48-62. 2000..In the future, population screening by genotype should be feasible once the relevant technical, legal and ethical issues are resolved...
- Diagnosis of hemochromatosisLawrie W Powell
Queensland Institute of Medical Research and the University of Queensland, Brisbane, Australia
Semin Gastrointest Dis 13:80-8. 2002..Because of its familial occurrence all first-degree relatives of patients with hereditary hemochromatosis should be tested for the disease...
- A population-based study of the clinical expression of the hemochromatosis geneJ K Olynyk
Department of Medicine, University of Western Australia, Fremantle, Australia
N Engl J Med 341:718-24. 1999..We recommended liver biopsy for subjects with serum ferritin levels of 300 ng per milliliter or higher. The subjects were followed for up to four years...
- Lipid peroxidation in hepatic steatosis in humans is associated with hepatic fibrosis and occurs predominately in acinar zone 3G A Macdonald
Population and Clinical Sciences Division, The Queensland Institute of Medical Research, Departments of Medicine, The University of Queensland, Australia
J Gastroenterol Hepatol 16:599-606. 2001..The aim of this study was to assess the distribution of lipid peroxidation in association with these factors, and the relationship of this to the fibrogenic cascade...
- HFE gene and hemochromatosisG J Anderson
The Queensland Institute of Medical Research, Brisbane, Queensland, Australia
J Gastroenterol Hepatol 19:712. 2004
- Interrelationships of alcohol and iron in liver disease with particular reference to the iron-binding proteins, ferritin and transferrinL M Fletcher
The Clinical Sciences Unit, The Queensland Institute of Medical Research, Brisbane, Australia
J Gastroenterol Hepatol 14:202-14. 1999....
- Patient-focused outcomes following detection in a hospital-based screening programme for C282Y haemochromatosisM A McCullen
Department of Gastroenterology and Hepatology, Royal Brisbane Hospital, Brisbane, QLD, Australia
Intern Med J 38:651-6. 2008..Haemochromatosis is a common genetic disease in populations of a northern European origin. However, there is uncertainty as to whether it is a condition that should be screened for...
- What's new in hemochromatosisP A Gochee
Population and Clinical Sciences Division, The Queensland Institute of Medical Research, Brisbane, Australia
Curr Opin Hematol 8:98-104. 2001..In addition, mechanisms of iron transport and metabolism are unfolding and are providing clues to the enigma of iron homeostasis and the pathophysiology of iron overload...
- Increased hepatic iron concentration in nonalcoholic steatohepatitis is associated with increased fibrosisD K George
Clinical Sciences Unit, Queensland Institute of Medical Research, Brisbane, Australia
Gastroenterology 114:311-8. 1998..The aim of this study was to investigate the role of the hemochromatosis mutation Cys282Tyr in development of the mild hepatic iron overload found in some patients with NASH and its association with hepatic damage in these patients...
- Hepatic stellate cell activation in genetic haemochromatosis. Lobular distribution, effect of increasing hepatic iron and response to phlebotomyG A Ramm
The University of Queensland Department of Medicine, Australia
J Hepatol 26:584-92. 1997..This study examined the relationship between hepatic iron concentration and hepatic stellate cell activation in genetic haemochromatosis...
- Identification of a novel Krueppel-related zinc finger gene (ZNF184) mapping to 6p21.3S Goldwurm
The Queensland Institute of Medical Research, University of Queensland, Brisbane, Queensland, 4029, Australia
Genomics 40:486-9. 1997..This zinc finger gene maps to a region approximately 200 kb distal to the microsatellite marker D6S105 and approximately 300 kb proximal to D6S1260...
- A novel mutation in ferroportin1 is associated with haemochromatosis in a Solomon Islands patientK E Arden
Membrane Transport Laboratory, The Queensland Institute of Medical Research, Brisbane, Australia
Gut 52:1215-7. 2003..The genetic basis of the disorder has not been identified. The disorder has similarities to type 4 haemochromatosis, which is caused by mutations in ferroportin1...
- Population screening for HFE-associated haemochromatosis: should we have to pay for our genes?T R Yapp
Queensland Institute of Medical Research, University of Queensland, Brisbane, Australia
Intern Med J 31:48-52. 2001..The present paper summarizes the evidence in favour of population screening and analyses the relative advantages of genotypic (DNA test) versus phenotypic (transferrin saturation) testing...
- Novel mutation in ferroportin1 is associated with autosomal dominant hemochromatosisDaniel F Wallace
Queensland Institute of Medical Research, the Digestive Health Clinics, and the Department of Pathology, Royal Brisbane Hospital, Brisbane, Australia
Blood 100:692-4. 2002..We propose that this mutation disrupts the function of the ferroportin1 protein, leading to impaired iron homeostasis and iron overload...
- Hemojuvelin (HJV)-associated hemochromatosis: analysis of HJV and HFE mutations and iron overload in three familiesDaniel F Wallace
Haematologica 90:254-5. 2005..In this study we identified the molecular basis of juvenile hemochromatosis in three Australian families and assessed the role of potential modifying genes in individuals carrying HFE mutations...
- Excess alcohol greatly increases the prevalence of cirrhosis in hereditary hemochromatosisLinda M Fletcher
Department of Gastroenterology and Hepatology, Princess Alexandra Hospital, Brisbane, Queensland, Australia
Gastroenterology 122:281-9. 2002....
- Effects of HFE gene mutations and alcohol on iron status, liver biochemistry and morbidityJohn K Olynyk
School of Medicine and Pharmacology, University of Western Australia, Crawley, Australia
J Gastroenterol Hepatol 20:1435-41. 2005....
- Steatosis is a cofactor in liver injury in hemochromatosisElizabeth E Powell
School of Medicine, The University of Queensland, Brisbane, Australia
Gastroenterology 129:1937-43. 2005....
- Psychological adjustment and knowledge about hereditary hemochromatosis in a clinic-based sample: a prospective studyBettina Meiser
Hereditary Cancer Clinic, Department of Medical Oncology, Prince of Wales Hospital, Sydney, Australia
J Genet Couns 14:453-63. 2005..Results also show that patients may benefit from strategies aimed at improving recall of genetic testing results...
- Screening for hemochromatosis in asymptomatic subjects with or without a family historyLawrie W Powell
Centre for the Advancement of Clinical Research, Royal Brisbane and Women s Hospital, Brisbane, Queensland 4029, Australia
Arch Intern Med 166:294-301. 2006..This study compared screening for hemochromatosis in subjects with or without a family history...
- The clinical relevance of compound heterozygosity for the C282Y and H63D substitutions in hemochromatosisAlissa Walsh
The Department of Gastroenterology and Hepatology, Royal Brisbane and Women s Hospital, Brisbane, Australia
Clin Gastroenterol Hepatol 4:1403-10. 2006..This study assessed the phenotypic expression in C282Y/H63D subjects as compared with C282Y homozygotes...
- Iron-overload-related disease in HFE hereditary hemochromatosisKatrina J Allen
Murdoch Children s Research Institute, University of Melbourne, Melbourne, Australia
N Engl J Med 358:221-30. 2008..Diseases related to iron overload develop in some C282Y homozygotes, but the extent of the risk is controversial...
- Hereditary hemochromatosis genetic testing of at-risk children: what is the appropriate age?Martin B Delatycki
Bruce Lefroy Centre for Genetic Health Research, Murdoch Children s Research Institute, Department of Paediatrics, University of Melbourne, Victoria, Australia
Genet Test 8:98-103. 2004..In the cascade setting, genetic testing for hemochromatosis need not be offered until late teenage years...
- Patient and graft survival after liver transplantation for hereditary hemochromatosis: Implications for pathogenesisDarrell H G Crawford
Queensland Liver Transplant Service and The University of Queensland, Princess Alexandra Hospital, Brisbane, Australia
Hepatology 39:1655-62. 2004..Our data suggest that iron excess in HH does not wholly depend on intestinal iron absorption but is also influenced by liver factors that moderate iron metabolism...
- A population-based study of the biochemical and clinical expression of the H63D hemochromatosis mutationPeter A Gochee
Queensland Institute of Medical Research and the University of Queensland, Brisbane, Western Australia
Gastroenterology 122:646-51. 2002..We accessed a well-defined population in Busselton, Australia, and determined the frequency of the H63D mutation and its influence on total body iron stores...
- Concordance of iron indices in homozygote and heterozygote sibling pairs in hemochromatosis families: implications for family screeningPeter W Whiting
Department of Gastroenterology and Hepatology, Princess Alexandra Hospital, Ipswich Road, Woolloongabba, Brisbane, Australia
J Hepatol 37:309-14. 2002....
- Effects of body iron stores and haemochromatosis genotypes on coronary heart disease outcomes in the Busselton health studyChristopher J Fox
Department of Gastroenterology, Fremantle Hospital, Fremantle, Western Australia, Australia
J Cardiovasc Risk 9:287-93. 2002..The aims of this study were to determine in a stable community population whether increased iron stores or haemochromatosis gene mutations were risk factors for coronary heart disease...
- Reciprocal relationship between methylation status and loss of heterozygosity at the p14(ARF) locus in Australian and South African hepatocellular carcinomasNirmitha I Herath
Conjoint Gastroenterology Laboratory, Clinical Research Center, Royal Brisbane Hospital Research Foundation, The Queensland Institute of Medical Research, Queesland, Australia
J Gastroenterol Hepatol 17:301-7. 2002..05). No significant association between p14 and p53 was seen in this study. The reciprocal relationship identified indicates different pathways of tumorigenesis and likely reflects different etiologies of HCC in the two countries...
- Frequency of the S65C mutation of HFE and iron overload in 309 subjects heterozygous for C282YDaniel F Wallace
Centre for Hepatology, Department of Medicine, Royal Free and University College Medical School, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
J Hepatol 36:474-9. 2002..Some are compound heterozygotes for C282Y/H63D. A small proportion have neither of these genotypes. We have investigated the phenotype of compound heterozygotes for C282Y and another missense mutation S65C...
- Disrupted hepcidin regulation in HFE-associated haemochromatosis and the liver as a regulator of body iron homoeostasisKim R Bridle
Hepatic Fibrosis Group, Queensland Institute of Medical Research, Royal Brisbane Hospital, Queensland, Herston, Australia
Lancet 361:669-73. 2003..To investigate this possible association, we studied the hepatic expression of the gene for hepcidin (HAMP) and a gene important in iron transport (IREG1) in patients with haemochromatosis, in normal controls, and in Hfe-knockout mice...
- Relative importance of female-specific and non-female-specific effects on variation in iron stores between womenJohn B Whitfield
Department of Clinical Biochemistry, Royal Prince Alfred Hospital, Sydney, Australia
Br J Haematol 120:860-6. 2003....
- Evidence for a sub-morphological inflammatory process in the liver in haemochromatosisKim R Bridle
Hepatic Fibrosis Group, Population Health and Clinical Sciences Division, The Queensland Institute of Medical Research, Post Office, Royal Brisbane Hospital, Herston, QLD 4029, Australia
J Hepatol 38:426-33. 2003..The aim of the current study was to examine the hepatic gene expression of potential proinflammatory and profibrogenic cytokines in haemochromatosis...
- HFE and non-HFE hemochromatosisGregory J Anderson
The Queensland Institute of Medical Research, PO Royal Brisbane Hospital, Australia
Int J Hematol 76:203-7. 2002..Recently, other forms of HH have been described that are not related to HFE but are due to mutations in genes coding iron transport proteins...
- Hemochromatosis and alcoholic liver diseaseLinda M Fletcher
Department of Gastroenterology and Hepatology, Princess Alexandra Hospital, Brisbane, QLD 4102, Australia
Alcohol 30:131-6. 2003..In addition, the cumulative effects of other forms of liver injury may result when iron and alcohol are present concurrently. Clearly, the addition of dietary iron in subjects homozygous for hemochromatosis would be unwise...
- A novel mutation in ferroportin implicated in iron overloadDaniel F Wallace
Membrane Transport Laboratory, The Queensland Institute of Medical Research, Brisbane, QLD, Australia
J Hepatol 46:921-6. 2007..In this study we examined the molecular basis of iron overload in an individual from New Zealand and characterised the molecular and cellular defect...
- Environmental and genetic modifiers of the progression to fibrosis and cirrhosis in hemochromatosisMarnie J Wood
Hepatic Fibrosis Group, The Queensland Institute of Medical Research, Brisbane, Australia
Blood 111:4456-62. 2008..This review explores recent developments in knowledge of environmental and genetic modifiers of this process...