Hereditary hemochromatosis

Pathology

Volumn 32, Issue 1, 2000, Pages 24-36

  Powell, Lawrie W ^a,b  

^a UNIVERSITY OF QUEENSLAND   (Australia)

^b QIMR BERGHOFER MEDICAL RESEARCH INSTITUTE   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

BETA 2 MICROGLOBULIN; MAJOR HISTOCOMPATIBILITY ANTIGEN CLASS 1; PROTEIN; HLA ANTIGEN CLASS 1;

CLINICAL FEATURE; CORONARY ARTERY DISEASE; DISEASE CLASSIFICATION; ENVIRONMENTAL FACTOR; FATTY LIVER; GENE LINKAGE DISEQUILIBRIUM; GENE MAPPING; GENE MUTATION; GENETICS; GENOTYPE; HAPLOTYPE; HEMOCHROMATOSIS; HEPATITIS B; HEPATITIS C; HEREDITY; HUMAN; INHERITANCE; IRON OVERLOAD; KNOCKOUT MOUSE; LIVER CELL CARCINOMA; LIVER FIBROSIS; LIVER INJURY; MOLECULAR CLONING; MOUSE; NEWBORN DISEASE; NONHUMAN; PATHOGENESIS; PATHOPHYSIOLOGY; PHENOTYPE; POPULATION RESEARCH; PORPHYRIA CUTANEA TARDA; REVIEW; SCREENING; ADOLESCENT; ADULT; ANIMAL; FEMALE; MALE; MIDDLE AGED; PATHOLOGY;

ADOLESCENT; ADULT; ANIMALS; FEMALE; HEMOCHROMATOSIS; HISTOCOMPATIBILITY ANTIGENS CLASS I; HUMANS; MALE; MICE; MIDDLE AGED;

EID: 0033999750     PISSN: 00313025     EISSN: None     Source Type: Journal    
DOI: 10.1080/003130200104529     Document Type: Article

References (122)

1. 1. Trousseau A. Anonymous Clinique Medicale de l'Hotel-Dieu de Paris, 2nd: Glycosurie, diabete sucre. Paris: Balliere, 1865; 663.
2. 2. von Recklinghausen FD. Taggeblet der (62) Versainumlung Deutsch Naturforscher und Arzte in Heidelberg. 1889; 324-5.
3. 3. Sheldon JH. Haemochromatosis. London: Oxford University Press, 1935; 382
4. 4. Bassett ML, Halliday JW, Powell LW. Value of hepatic iron measurements in early hemochromatosis and determination of the critical iron level associated with fibrosis. Hepatology 1986; 6(1): 24-9.
5. 5. Scheuer PJ, Williams R, Muir AR. Hepatic pathology in relatives of patients with haemochromatosis. J Pathol Bacteriol 1962; 84: 53-64.
6. 6. Feder JN, Gnirke A, Thomas W, et al. A novel MHC class -I like gene is mutated in patients with hereditary haemochromatosis. Nat Genet 1996; 13: 399-408.
7. 7. Powell LW, Leggett BA, Crawford DHG, et al., editors. Hemochromatosis and other iron storage disorders. In, Schiff's Diseases of the Liver, Eighth Edition. Philadelphia: Lippincott-Raven, 1998; in press.
8. 8. Milman N. Hereditary haemochromatosis in Denmark 1950-1985: clinical, biochemical and histological features in 179 patients and 13 preclinical cases. Dan Med Bull 1991; 38: 385-93.
9. 9. Adams PC, Deugnier Y, Moirand R, Brissot P. The relationship between iron overload, clinical symptoms, and age in 410 patients with genetic hemochromatosis. Hepatology 1997; 25: 162-6.
10. 10. Lamon JM, Marynick SP, Roseblatt R, Donnelly S. Idiopathic hemochroamtosis in a young female. A case study and review of the syndrome in young people. Gastroenterology 1979; 76: 178-83.
11. 11. Felts JH, Nelson JR, Herndon CN, Spurr CL. Hemochromatosis in two young sisters. Case studies and a family survey. Ann Intern Med 1967; 67: 117-23.
12. 12. Charlton RW, Abrahams C, Bothwell TH. Idiopathic hemochromatosis in young subjects. Clinical, pathological, and chemical findings in four patients. Arch Pathol 1967; 83: 132-40.
13. 13. Cazzola M, Ascari E, Claudiani G, et al. Juvenile idiopathic haemochromatosis: a life-threatening disorder presenting as hypogonadotropic hypogonadism. Hum Genet 1983; 65: 149-54.
14. 14. Camaschella C, Cicilano M, Bosio S, et al. Juvenile and adult hemochromatosis are distinct genetic disorders. Eur J Hum Genet 1997; 5: 371-5.
15. 15. Knisely AS, Magid MS, Dische MR, Cutz E. Neonatal hemochromatosis. Birth Defects 1987; 23: 75-102.
16. 16. Witzelbin CL, Urí A. Perinatal hemochromatosis: entity or end result? Hum Pathol 1989; 20: 335-40.
17. 17. Silver MM, Beverley DW, Valberg LS, et al. Perinatal hemochromatosis: clinical morphologic and quantitative iron studies. Am J Pathol 1987; 128: 538-54.
18. 18. Verloes A, Temple IK, Hubert A, et al. Recurrence of neonatal haemochromatosis in half sibs born of unaffected mothers. J Med Genet 1996; 33: 444-9.
19. 19. Rand EB, McClenathan DT, Whitington PF. Neonatal hemochromatosis: report of successful orthotopic liver transplantation. J Pediatr Gastroenterol Nutr 1992; 15: 325-9.
20. 20. Knisely AS. Neonatal hemochromatosis. Adv Pediatr 1992; 39: 383-403.
21. 21. MacDonald RA. Hemochromatosis and hemosideroais. (Springfield, IL: Charles C. Thomas, 1964.
22. 22. Simon M, Bourel M, Fauchet R, Genetet B. Association of HLA A3 and HLA B14 antigens with idiopathic hemochromatosis. Gut 1976; 17: 332-4.
23. 23. Walters JM, Watt DW, Stevens FM, McCarthy CF. HLA antigens in haemochromatosis. Br Med J 1975; 4: 520.
24. 24. Simon M, Alexandre JL, Bourel M, et al. Heredity of idiopathic hemochromatosis: a study of 106 families. Clin Genet 1977; 11: 327-41.
25. 25. Doran T, Bashir HV, Trejaul J, et al. Idiopathic hemochromatosis in the Australian population: HLA linkage and recessivity. Hum Immunol 1981; 2: 191-200.
26. 26. Ritter B, Safwenberg J, Olsson KS. HLA as a marker of the hemochromatosis gene in Sweden. Hum Genet 1984; 68: 62-6.
27. 27. Summers KM, Tam KS, Halliday JW, Powell LW. HLA determinants in an Australian population of hemochromatosis patients and their families. Am J Hum Genet 1989; 45: 41-8.
28. 28. Santos M, Schilham MW, Rademakers LPHM, et al. Defective iron homeostasis in β2-microglobulin knockout mice recapitulates hereditary hemochromatosis in man. J Exp Med 1996; 184: 1975-85.
29. 29. Rothenberg BE, Voland JR. Beta-2-microglobulin knockout mice develop parenchymal iron overload: a putative role for class I genes of the major histocompatibility complex in iron metabolism. Proc Natl Acad Sci USA 1996; 93: 1529-34.
30. 30. Carella M, D'Ambrosio L, Totaro A, et al. Mutation analysis of the HLA-H gene in Italian hemochromatosis patients. Am J Hum Genet 1997; 60: 828-82.
31. 31. Jazwinska EC, Cullen LM, Busfield F, et al. Haemochromatosis and HLA-H [letter]. Nat Genet 1996; 14: 249-51.
32. 32. Jouanolle AM, Gandon G, Jezequel P, et al. Haemochromatosis and HLA-H. Nat Genet 1996; 14: 251-2.
33. 33. Worwood M, Shearman JD, Wallace DF, et al. A simple genetic test identifies 90% of UK patients with haemochromatosis. Gut 1997; 41: 841-4.
34. 34. Bacon BR, Powell LW, Adams PC, et al. Molecular medicine and hemochromatosis: at the crossroads. Gastroenterology 1999; 116: 193-207.
35. 35. Edwards CQ, Griffin LM, Goldgar D, et al. Prevalence of hemocmomatosis among 11,065 presumably healthy blood donors. New Engl J Med 1988; 318(1): 1355-62.
36. 36. Leggett BA, Halliday JW, Brown NN, et al. Prevalence of haemochromatosis amongst asymptomatic Australians. Br J Haematol 1990; 74: 525-30.
37. 37. Merryweather-Clarke AT, Pointon JJ, Shearman JD, Robson KJH. Global prevalence of putative haemochromatosis mutations. J Med Genet 1997; 34: 275-8.
38. 38. Cullen LM, Summerville L, Glassick TV, et al. Neonatal screening for the haemochromatosis defect. Blood 1997; 90: 423-7.
39. 39. Roberts AG, Whatley SD, Morgan RR, et al. Increased frequency of the haemochromatosis Cys282Tyr mutation in sporadic porphyria cutanea tarda. Lancet 1997; 349: 321-3.
40. 40. Sampietro M, Piperno A, Lupica L, et al. High prevalence of the His63Asp HFE mutation in Italian patients with porphyria curtanea tarda. Hepatology 1998; 27: 181-4.
41. 41. Stuart KA, Busfield F, Jazwinska EC, et al. The C282Y mutation in haemochromatosis (HFE) gene and hepatitis C virus infection are independent cofactors for porphyria cutanea tarda in Australian patients. J Hepatol 1998; 28: 404-9.
42. 42. George DK, Goldwurm S, Macdonald GA, et al. Increased hepatic iron stores on non alcoholic steatohepatitis are associated with the hemochromatosis mutation and increased liver damage. Gastroenterology 1998; 114: 311-8.
43. 43. Turlin B, Juguet F, Moirand R, et al. Increased liver iron stores in patients with hepatocellular carcinoma developed on a noncirrhotic liver. Hepatology 1995; 22: 446-50.
44. 44. Deugnier YM, Charalambous P, Le Quilleuc D, et al. Preneoplastic significance of hepatic iron-free foci in genetic hemochromatosis: a study of 185 patients. Hepatology 1993; 18: 1363-9.
45. 45. Sullivan JL. Iron and the sex difference in heart disease risk. Lancet 1981; 1293-4.
46. 46. Lauffer RB. Iron stores and the international variation in mortality from coronary artery disease. Med Hypotheses 1990; 35: 96-102.
47. 47. Miller M, Hutchins GM. Hemochromatosis, multiorgan hemosiderosis, and coronary artery disease. J Am Med Assoc 1994; 272: 231-3.
48. 48. Smith BC, Grove J, Guzail MA, et al. Heterozygosity for hereditary hemochromatosis is associated with more fibrosis in chronic hepatitis C. Hepatology 1998; 27: 1695-9.
49. 49. Barton JC, Harmon L, Rivers C, Acton RT. Hemochromatosis: association of severity of iron overload with genetic markers. Blood Cells Molecules Dis 1996; 22: 195-204.
50. 50. Crawford DH, Halliday JW, Summers KM, et al. Concordance of iron storage in siblings with genetic hemochromatosis: evidence for a predominantly genetic effect on iron storage. Hepatology 1993; 17: 833-7.
51. 51. Muir WA, McLaren GD, Braun W, Askari A. Evidence for heterogeneity in hereditary hemochromatosis. Evaluation of 174 persons in nine families. Am J Med 1984; 76: 806-14.
52. 52. Adams PC. Prevalence of abnormal iron studies in heterozygotes for hereditary hemochromatosis: an analysis of 255 heterozygotes. Am J Hematol 1994; 45: 146-9.
53. 53. Bulaj ZJ, Griffen BA, Jorde LB, et al. Clinical and biochemical abnormalities in people heterozygous for hemochromatosis. New Engl J Med 1996; 335: 1799-805.
54. 54. Borot N, Roth M, Malfroy L, et al. Mutations in the MHC class I-like candidate gene for hemochromatosis in French patients. Immunogenetics 1997; 45: 320-4.
55. 55. Sham RL, Ou CY, Cappuccio J, et al. Correlation between genotype and phenotype in hereditary hemochromatosis: analysis of 61 cases. Blood Cells Molecules Dis 1997; 23: 314-20.
56. 56. Crawford DH, Powell LW, Leggett BA, et al. Evidence that the ancestral haplotype in Australian hemochromatosis patients may be associated with a common mutation in the gene. Am J Hum Genet 1995; 57: 362-7.
57. 57. Crawford DHG, Jazwinska EC, Cullen LM, Powell L.W. Expression of HLA-linked hemochromatosis in subjects homozygous or heterozygous subjects for the C282Y mutation. Gastroenterology 1998; 114: 1003-8.
58. 58. Cox TM, Peters T. Uptake of iron by duodenal biopsy specimens from patients with iron deficiency anaemia and primary haemochromatosis. Lancet 1978; 1: 123-4.
59. 59. Raja KB, Pountney D, Bomford A, et al. A duodenal mucosal abnormality in the reduction of Fe(III) in patients with genetic haemochromatosis. Gut 1996; 38: 765-9.
60. 60. Powell LW, Campbell CB, Wilson E. Intestinal mucosal uptake of iron and iron retention in idiopathic hemochromatosis as evidence of a mucosal abnormality. Gut 1970; 11: 727-31.
61. 61. Feder JN, Penny DM, Irrinki A, et al. The hemochromatosis gene product complexes with the transferrin receptor and lowers its affinity for ligand binding. Proc Nat Acad Sci USA 1998; 1477.
62. 62. Fleming M, Trenor C, Su M, et al. Microcylic anaemia mice have a mutation in Nramp2, a candidate iron transporter gene. Nat Genet 1997; 16: 383-6.
63. 63. Gunshin H, Mackenzie B, Berger U, et al. Cloning and characterization of a mammalian proton-coupled metal-ion transporter. Nature 1997; 388: 482-8.
64. 64. Hentze MW, Kuhn LC. Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric-oxide, and oxidative stress. Proc Natl Acad Sci USA 1996; 93: 8175-82.
65. 65. Sciot R, Paterson AC, Van Den Oord JJ, Desmet V.J. Lack of hepatic transferrin receptor expression in hemochromatosis. Hepatology 1987; 7: 831-7.
66. 66. Lombard M, Bomford A, Hynes M, et al. Regulation of the hepatic transferrin receptor in hereditary hemochromatosis. Hepatology 1989; 9: 1-5.
67. 67. Pietrangelo A, Rocchi E, Rigo G, et al. Regulation of transferrin, transferrin receptor and ferritin gene expression in the duodenum of normal anemic and siderotic subjects. Gastroenterology 1992; 102: 802-9.
68. 68. Flanagan PR, Hajdu A, Adams PC. Iron-responsive element-binding protein in hemochromatosis liver and intestine. Hepatology 1995; 22: 828-32.
69. 69. Britton RS, Bacon BR, Recknagel RO. Lipid peroxidation and associated hepatic organelle dysfunction in iron overload. Chem Phys Lipids 1987; 45: 207-39.
70. 70. Tavill AS, Sharma BK, Bacon BR. Iron and the liver: genetic hemochromatosis and other hepatic iron overload disorders. Prog Liver Dis 1990; 9: 281-305.
71. 71. Young IS, Trouton TG, Torney JJ, et al. Genetic hemochromatosis and Wilson's Disease: role for oxidant stress? Free Radic Biol Med 1994; 16: 393-7.
72. 72. Stocker R. Lipoprotein oxidation: mechanistic aspects, methodological approaches and clinical relevance. Curr Opin Lipidol 1994; 5: 422-33.
73. 73. Ramm GA, Halliday JW, Fletcher LM, et al. Hepatic stellate cell activation in genetic hemochromatosis: lobular distribution, effect of increasing hepatic iron and response to phlebotomy. J Hepatol 1997; 26: 584-92.
74. 74. Houglum K, Bedossa P, Chojkier M. TGF-B and collagen-al (I) gene expression are increased in hepatic acinar zone I of rats with iron overload. Am J Physiol 1994; 5: G908-13.
75. 75. Houglum K, Filip M, Witztum JL, Chojkier M. Malondialdehyde and 4-hydroxynoneal protein adducts in plasma and liver of rats with iron overload. J Clin Invest 1990; 86: 1991-8.
76. 76. Houglum K, Lee KS, Chojkier M. Proliferation of hepatic stellate cells is inhibited by phosphorylation of CREB on serine 133. J Clin Invest 1997; 99: 1322-8.
77. 77. Houglum K, Ramm GA, Crawford DHG, et al. Excess iron induces hepatic transforming growth factor Beta1 in genetic hemochromatosis: role of oxidative stress. Hepatology 1997; 27: 605-10.
78. 78. Houglum K. Alcohol and iron: a radical combination? Hepatology 1996; 23: 1700-3.
79. 79. Jazwinska EC, Lee SC, Webb SI, et al. Localization of the hemochromatosis gene close to D6S105. Am J Hum Genet 1993; 53: 347-52.
80. 80. Stone C, Pointon JJ, Jazwinska EC, et al. Isolation of CA dinucleotide repeats close to D6S105; linkage disequilibrium with haemochromatosis. Hum Mol Genet 1994; 3: 2043-6.
81. 81. Worwood M, Raha-Chowdhury R, Dorak M, Darke C, Bowen D, Burnett A. Alleles at D6S265 and D6S105 define a haemochromatosis-specific genotype. Br J of Haematol 1994; 86: 863-6.
82. 82. Jazwinska EC, Pyper WR, Burt MJ, et al. Haplotype analysis in Australian hemochromatosis patients: evidence for a predominant ancestral haplotype exclusively associated with hemochromatosis. Am J Hum Genet 1995; 56: 428-33.
83. 83. Raha-Chowdhury R, Bowen DJ, Stone C, et al. New polymorphic microsatellite markers place the haemochromatosis gene telomeric to D6S105. Hum Mol Genet 1995; 4: 1869-74.
84. 84. Raha-Chowdhury R, Bowen D, Burnett A, Worwood M. Allelic associations and homozygosity in loci from HLA-B to D6S299 in genetic haemochromatosis. J Med Genet 1995; 32: 446-52.
85. 85. Malfroy L, Roth MP, Carrington M et al. Heterogeneity in rates of recombination in the 6-Mb region telomeric to the human major histocompatibility complex. Genomics 1997; 43: 226-31.
86. 86. Worwood M, Dorak MT, Nicklin S. The frequency of the haemochromatosis-associated genotype D6S265-1:D6S105-8 in blood donors. Br J Haematol 1996; 93: 838-40.
87. 87. Campbell RD, Trowsdale J. Map of the human MHC. Immunol Today 1993; 14: 349-52.
88. 88. El Kalhoun A, Chauvel B, Mauvieux N, et al. Localization of seven new genes around the HLA-A locus. Hum Mol Genet 1993; 2: 55-60.
89. 89. Abderrahim H, Sambucy JL, Iris F, et al. Cloning the human major histocompatibility complex in YACs. Genomics 1994; 23: 520-7.
90. 90. Burt MJ, Smit DJ, Pyper WR, et al. A 4.5 megabase YAC contig and physical map over the hemochromatosis gene region. Genomics 1996; 33: 205-9.
91. 91. Totaro A, Rommens JM, Grifa A. Hereditary hemochromatosis: generation of a transcription map within a refined and extended map of the HLA class I region. Genomics 1996; 31: 319-26.
92. 92. Malaspina P, Roetto A, Trettel F, et al. Construction of a YAC contig covering human chromosome 6p22. Genomics 1996; 36: 399-407.
93. 93. Beutler E, Gelbart T, West C, et al. Mutation analysis in hereditary hemochromatosis. Blood Cells Molecules Dis 1996; 22: 187-94.
94. 94. Bodmer JG, Parham P, Ekkehard DA, Marsh SGE. Putting a hold on "HLA-H". Nat Genet 1997; 15: 234-5.
95. 95. Mercier B, Mura C, Ferec C. Putting a hold on "HLA-H". Nat Genet 1997; 15: 234-5.
96. 96. Beutler E. The significance of the 187G (H63D) mutation in hemochromatosis. [letter; comment]. Am J Hum Genet 1997; 61: 762-4.
97. 97. Beutler E. Genetic irony beyond haemochromatosis: clinical effect of HLA-H mutations. Lancet 1997; 349: 296-7.
98. 98. Martinez PA, Biron C, Blanc F, et al. Compound heterozygotes for hemochromatosis gene mutations: may they help to understand the pathophysiology of the disease? Blood Cells Molecules Dis 1997; 23: 269-76.
99. 99. Risch R. Haemochromatosis, HFE and genetic complexity. Nat Genet 1997; 17: 375-6.
100. 100. Lebron JA, Bennett MJ, Vaughn DE, et al. Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor. Cell 1998; 93: 111-23.
101. 101. Parkkila S, Waheed A, Britton RS, et al. Immunohistochemistry of HLA-H, the protein defective in patients with hereditary hemochromatosis, reveals unique pattern of expression in gastroentestinal tract. Proc Natl Acad Sci USA 1997; 94: 2534-9.
102. 102. Bjorkman PJ, Parham P. Structure, function and diversity of class I major histocompatibility complex molecules. Annu Rev Biochem 1990; 59: 253-88.
103. 103. Feder JN, Tsuchibashi Z, Irrinki A, et al. The hemochromatosis founder mutation in HLA-H disrupts beta-2-microglobulin interaction and cell surface expression. J Biol Chem 1997; 272: 14025-8.
104. 104. Waheed A, Parkkila S, Zhou XY, et al. Hereditary hemochromatosis: Effects of C282Y and H63D mutations on association with beta2-microglobulin, intracellular processing and cell surface expression of the HFE protein in COS-7 cells. Proc Natl Acad Sci USA 1997; 94: 12384-9.
105. 105. Parkkila S, Waheed A, Britton RS, et al. Association of the transferrin receptor in human placenta with HFE, the protein defective in hereditary hemochromatosis. Proc Natl Acad Sci USA 1997; 94: 13198-202.
106. 106. Anderson GJ, Powell LW, Halliday JW. Transferrin receptor distribution and regulation in the rat small intestine: effect of iron stores and erythropoiesis. Gastroenterology 1990; 98: 576-85.
107. 107. Banerjee D, Flanagan PR, Cluett J, Valberg LS. Transferrin receptors in the human gastrointestinal tract. Relationship to body iron stores. Gastroenterology 1986; 91: 861-9.
108. 108. De Sousa M, Reimao R, Lacerda P, et al. Iron overload in beta2-microglobulin deficient mice. Immunol Lett 1994; 39: 105-11.
109. 109. Hashimoto K, Hirai M, Kurosawa Y. Identification of a mouse homolog for the human hereditary haemochromatosis candidate gene. Biochem Biophys Res Commun 1997; 230: 35-9.
110. 110. Zhou XY, Tomatsu S, Fleming RE, et al. HFE gene knockout produces mouse model of hereditary hemochromatosis. Proc Natl Acad Sci USA 1998; 95: 2492-7.
111. 111. Lebouef RC, Tolson D, Heinecke JW. Dissociation between tissue iron concentrations and transferrin saturation among inbred mouse strains. J Lab Clin Med 1995; 126: 128-36.
112. 112. Yang B, Kirby S, Lewis J, et al. A mouse model for beta O-thalassemia. Proc Natl Acad Sci USA 1995; 92: 11608-12.
113. 113. Poss KD, Tonegawa S. Heme oxygenase 1 is required for mammalian iron reutilization. Proc Natl Acad Sci USA 1997; 94: 10919-24.
114. 114. Craven CM, Alexander J, Eldridge M, et al. Tissue distribution and clearance kinetics of non-transferrin-bound iron in the hypotransferrinemic mouse; a rodent model for hemochromatosis. Proc Natl Acad Sci USA 1987; 84: 3457-61.
115. 115. Guyader D, Jacquelinet C, Moirand R, et al. Non-invasive prediction of fibrosis in C282Y homozygous hemochromatosis. Gastoenterology 1998; 115: 929-36.
116. 116. Adams PC. Implications of genotyping of spouses to limit investigation of children in genetic hemochromatosis. Clin Genet 1998; 53: 176-8.
117. 117. McLaren C, McLachlan G, Halliday J, et al. The distribution of transferrin saturation in an asymptomatic Australian population: relevance to the early diagnosis of hemochromatosis. Gastroenterology 1998; 114: 543-9.
118. 118. Burke W, Thomson E, Khoury MJ, et al. Hereditary hemochromatosis: gene discovery and its implications for population-based screening. J Am Med Assoc 1998; 280: 172-8.
119. 119. Hickman P, Hourigan L, Powell LW, Crawford DHG. Automated measurement of unsaturated iron binding capacity allows a cost effective, ongoing strategy for screening for haemochromatosis. Hepatology 1998; 28: (4) 422A.
120. 120. Niederau C, Fisher R, Sonnenberg A, et al. Survival and causes of death in cirrhotic and in noncirrhotic patients with primary hemochromatosis. N Engl J Med 1985; 313: 125-62.
121. 121. Niederau C, Fischer R, Purschel A, et al. Long-term survival in patients with hereditary hemochromatosis. Gastroenterology 1996; 110: 1107-19.
122. 122. Baker ER, Monk CD, Do K, et al. The changing role of liver biopsy in hereditary haemochromatosis after the cloning of the HFE gene. Hepatology 1998; 28: 421A