{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T03:47:55Z","timestamp":1788925675824,"version":"build-2803163510"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2000,5]]},"DOI":"10.1093\/qjmed\/93.5.269","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T22:32:47Z","timestamp":1027722767000},"page":"269-275","source":"Crossref","is-referenced-by-count":74,"title":["Curative hepatorenal transplantation in systemic amyloidosis caused by the Glu526Val fibrinogen \u03b1\u2010chain variant in an English family"],"prefix":"10.1093","volume":"93","author":[{"given":"J.D.","family":"Gillmore","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.R.","family":"Booth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Rela","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.D.","family":"Heaton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Rahman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.J.","family":"Stangou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.B.","family":"Pepys","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.N.","family":"Hawkins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2000,5]]},"reference":[{"key":"key\n\t\t\t\t20170413030558_R1","unstructured":"Pepys MB. Amyloidosis. In: Weatherall DJ, Ledingham JGG, Warrell DA, eds. Oxford Textbook of Medicine, 3rd edn. Oxford, Oxford University Press, 1995:1512\u201324."},{"key":"key\n\t\t\t\t20170413030558_R2","doi-asserted-by":"crossref","unstructured":"Jones LA, Harding JA, Cohen AS, Skinner M. New USA family has apolipoprotein AI (Arg26) variant. In: Natvig JB, F\u00f8rre \u00d8, Husby G, Husebekk A, Skogen B, Sletten K, Westermark P, eds. Amyloid and Amyloidosis 1990. Dordrecht, Kluwer Academic Publishers, 1991:385\u20138.","DOI":"10.1007\/978-94-011-3284-8_97"},{"key":"key\n\t\t\t\t20170413030558_R3","doi-asserted-by":"crossref","unstructured":"Soutar AK, Hawkins PN, Vigushin DM, Tennent GA, Booth SE, Hutton T, Nguyen O, Totty NF, Feest TG, Hsuan JJ, Pepys MB. Apolipoprotein AI mutation Arg\u201060 causes autosomal dominant amyloidosis. Proc Natl Acad Sci USA1992; 89:7389\u201393.","DOI":"10.1073\/pnas.89.16.7389"},{"key":"key\n\t\t\t\t20170413030558_R4","unstructured":"Booth DR, Tan SY, Booth SE, Hsuan JJ, Totty NF, Nguyen O, Tennent GA, Hutchinson WL, Vigushin DM, Thomson N, Soutar AK, Hawkins PN, Pepys MB. Hereditary renal amyloidosis caused by a new apolipoprotein AI variant, Trp50Arg. Nephrol Dial Transplant1995; 10:943."},{"key":"key\n\t\t\t\t20170413030558_R5","doi-asserted-by":"crossref","unstructured":"Booth DR, Tan SY, Booth SE, Tennent GA, Hutchinson WL, Hsuan JJ, Totty NF, Nguyen O, Soutar AK, Hawkins PN, Bruguera M, Caballer\u00eda J, Sol\u00e9 M, Campistol JM, Pepys MB. Hereditary hepatic and systemic amyloidosis caused by a new deletion\/insertion mutation in the apolipoprotein AI gene. J Clin Invest1996; 97:2714\u201321.","DOI":"10.1172\/JCI118725"},{"key":"key\n\t\t\t\t20170413030558_R6","doi-asserted-by":"crossref","unstructured":"Hamidi Asl K, Liepnieks JJ, Nakamura M, Parker F, Benson MD. A novel apolipoprotein A\u20101 variant, Arg173Pro, associated with cardiac and cutaneous amyloidosis. Biochem Biophys Res Commun1999; 257:584\u20138.","DOI":"10.1006\/bbrc.1999.0518"},{"key":"key\n\t\t\t\t20170413030558_R7","doi-asserted-by":"crossref","unstructured":"Hamidi Asl L, Liepnieks JJ, Hamidi Asl K, Uemichi T, Moulin G, Desjoyaux E, Loire R, Delpech M, Grateau G, Benson MD. Hereditary amyloid cardiomyopathy caused by a variant apolipoprotein A1. Am J Pathol1999; 154:221\u20137.","DOI":"10.1016\/S0002-9440(10)65268-6"},{"key":"key\n\t\t\t\t20170413030558_R8","doi-asserted-by":"crossref","unstructured":"Obici L, Bellotti V, Mangione P, Stoppini M, Arbustini E, Verga L, Zorzoli I, Anesi E, Zanotti G, Campana C, Vigan\u00f2 M, Merlini G. The new apolipoprotein A\u2010I variant Leu174\u2192Ser causes hereditary cardiac amyloidosis, and the amyloid fibrils are constituted by the 93\u2010residue N\u2010terminal polypeptide. Am J Pathol1999; 155:695\u2013702.","DOI":"10.1016\/S0002-9440(10)65167-X"},{"key":"key\n\t\t\t\t20170413030558_R9","doi-asserted-by":"crossref","unstructured":"Pepys MB, Hawkins PN, Booth DR, Vigushin DM, Tennent GA, Soutar AK, Totty N, Nguyen O, Blake CCF, Terry CJ, Feest TG, Zalin AM, Hsuan JJ. Human lysozyme gene mutations cause hereditary systemic amyloidosis. Nature1993; 362:553\u20137.","DOI":"10.1038\/362553a0"},{"key":"key\n\t\t\t\t20170413030558_R10","doi-asserted-by":"crossref","unstructured":"Benson MD, Liepnieks J, Uemichi T, Wheeler G, Correa R. Hereditary renal amyloidosis associated with a mutant fibrinogen a\u2010chain. Nature Gen1993; 3:252\u20135.","DOI":"10.1038\/ng0393-252"},{"key":"key\n\t\t\t\t20170413030558_R11","doi-asserted-by":"crossref","unstructured":"Uemichi T, Liepnieks JJ, Benson MD. Hereditary renal amyloidosis with a novel variant fibrinogen. J Clin Invest1994; 93:731\u20136.","DOI":"10.1172\/JCI117027"},{"key":"key\n\t\t\t\t20170413030558_R12","doi-asserted-by":"crossref","unstructured":"Uemichi T, Liepnieks JJ, Yamada T, Gertz MA, Bang N, Benson MD. A frame shift mutation in the fibrinogen A a\u2010chain gene in a kindred with renal amyloidosis. Blood1996; 87:4197\u2013203.","DOI":"10.1182\/blood.V87.10.4197.bloodjournal87104197"},{"key":"key\n\t\t\t\t20170413030558_R13","doi-asserted-by":"crossref","unstructured":"Uemichi T, Liepnieks JJ, Alexander F, Benson MD. The molecular basis of renal amyloidosis in Irish\u2010American and Polish\u2010Canadian kindreds. Q J Med1996; 89:745\u201350.","DOI":"10.1093\/qjmed\/89.10.745"},{"key":"key\n\t\t\t\t20170413030558_R14","unstructured":"Hamidi Asl L, Liepnieks JJ, Uemichi T, Rebibou JM, Justrabo E, Droz D, Mousson C, Chalopin JM, Benson MD, Delpech M, Grateau G. Renal amyloidosis with a frame shift mutation in fibrinogen \u03b1\u2010chain gene producing a novel amyloid protein. Blood1997; 90:4799\u2013805."},{"key":"key\n\t\t\t\t20170413030558_R15","doi-asserted-by":"crossref","unstructured":"Tan SY, Pepys MB, Hawkins PN. Treatment of amyloidosis. Am J Kidney Dis1995; 26:267\u201385.","DOI":"10.1016\/0272-6386(95)90647-9"},{"key":"key\n\t\t\t\t20170413030558_R16","doi-asserted-by":"crossref","unstructured":"Hawkins PN, Pepys MB. A primed state exists in vivo following histological regression of amyloidosis. Clin Exp Immunol1990; 81:325\u20138.","DOI":"10.1111\/j.1365-2249.1990.tb03339.x"},{"key":"key\n\t\t\t\t20170413030558_R17","doi-asserted-by":"crossref","unstructured":"Hawkins PN. Studies with radiolabelled serum amyloid P component provide evidence for turnover and regression of amyloid deposits in vivo. Clin Sci1994; 87:289\u201395.","DOI":"10.1042\/cs0870289"},{"key":"key\n\t\t\t\t20170413030558_R18","doi-asserted-by":"crossref","unstructured":"Hawkins PN, Lavender JP, Pepys MB. Evaluation of systemic amyloidosis by scintigraphy with 123I\u2010labeled serum amyloid P component. N Engl J Med1990; 323:508\u201313.","DOI":"10.1056\/NEJM199008233230803"},{"key":"key\n\t\t\t\t20170413030558_R19","doi-asserted-by":"crossref","unstructured":"Booth DR, Tan SY, Hawkins PN, Pepys MB, Frustaci A. A novel variant of transthyretin, 59Thr\u2010Lys, associated with autosomal dominant cardiac amyloidosis in an Italian family. Circulation1995; 91:962\u20137.","DOI":"10.1161\/01.CIR.91.4.962"},{"key":"key\n\t\t\t\t20170413030558_R20","doi-asserted-by":"crossref","unstructured":"Mills KA, Even D, Murray JC. Tetranucleotide repeat polymorphism at the human alpha fibrinogen locus (FGA). Hum Mol Genet1992; 1:779.","DOI":"10.1093\/hmg\/1.9.779"},{"key":"key\n\t\t\t\t20170413030558_R21","doi-asserted-by":"crossref","unstructured":"Tan SY, Pepys MB. Amyloidosis. Histopathology1994; 25:403\u201314.","DOI":"10.1111\/j.1365-2559.1994.tb00001.x"},{"key":"key\n\t\t\t\t20170413030558_R22","unstructured":"Persey MR, Lovat LB, Apperley JF, Madhoo S, Pepys MB, Hawkins PN. Intensive chemotherapy for AL amyloidosis. Br J Rheumatol1996; 35(Suppl. 1):12."},{"key":"key\n\t\t\t\t20170413030558_R23","doi-asserted-by":"crossref","unstructured":"Moreau P, Leblond V, Bourquelot P, Facon T, Huynh A, Caillot D, Hermine O, Attal M, Hamidou M, Nedellec G, Ferrant A, Audhuy B, Bataille R, Milpied N, Harousseau JL. Prognostic factors for survival and response after high\u2010dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a report on 21 patients. Br J Haematol1998; 101:766\u20139.","DOI":"10.1046\/j.1365-2141.1998.00772.x"},{"key":"key\n\t\t\t\t20170413030558_R24","doi-asserted-by":"crossref","unstructured":"Comenzo RL, Vosburgh E, Falk RH, Sanchorawala V, Reisinger J, Dubrey S, Dember LM, Berk JL, Akpek G, LaValley M, O'hara C, Arkin CF, Wright DG, Skinner M. Dose\u2010intensive melphalan with blood stem\u2010cell support for the treatment of AL (amyloid light\u2010chain) amyloidosis: survival and responses in 25 patients. Blood1998; 91:3662\u201370.","DOI":"10.1182\/blood.V91.10.3662.3662_3662_3670"},{"key":"key\n\t\t\t\t20170413030558_R25","doi-asserted-by":"crossref","unstructured":"Persey MR, Booth DR, Booth SE, van Zyl\u2010Smit R, Adams BK, Fattaar AB, Tennent GA, Hawkins PN, Pepys MB. Hereditary nephropathic systemic amyloidosis caused by a novel variant apolipoprotein A\u2010I. Kidney Int1998; 53:276\u201381.","DOI":"10.1046\/j.1523-1755.1998.00770.x"},{"key":"key\n\t\t\t\t20170413030558_R26","doi-asserted-by":"crossref","unstructured":"Gillmore JD, Booth DR, Madhoo S, Pepys MB, Hawkins PN. Hereditary renal amyloidosis associated with variant lysozyme in a large English family. Nephrol Dial Transplant1999; 14:2639\u201344.","DOI":"10.1093\/ndt\/14.11.2639"},{"key":"key\n\t\t\t\t20170413030558_R27","unstructured":"Herbert J, Younger D, Latov N, Martone RL. Clinical spectrum of familial amyloidotic polyneuropathy. In: Kisilevsky R, Benson MD, Frangione B, Gauldie J, Muckle TJ, Young ID, eds. Amyloid and Amyloidosis 1993. Pearl River NY, Parthenon Publishing,1994:486\u20138."},{"key":"key\n\t\t\t\t20170413030558_R28","doi-asserted-by":"crossref","unstructured":"Hall R, Hawkins PN. Cardiac transplantation for AL amyloidosis: a personal account. Br Med J1994; 309:1135\u20137.","DOI":"10.1136\/bmj.309.6962.1135"},{"key":"key\n\t\t\t\t20170413030558_R29","doi-asserted-by":"crossref","unstructured":"Lovat LB, Persey MR, Madhoo S, Pepys MB, Hawkins PN. The liver in systemic amyloidosis: insights from 123I serum amyloid P component scintigraphy in 484 patients. Gut1998; 42:727\u201334.","DOI":"10.1136\/gut.42.5.727"},{"key":"key\n\t\t\t\t20170413030558_R30","doi-asserted-by":"crossref","unstructured":"Holmgren G, Ericzon B\u2010G, Groth C\u2010G, Steen L, Suhr O, Andersen O, Wallin BG, Seymour A, Richardson S, Hawkins PN, Pepys MB. Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet1993; 341:1113\u201316.","DOI":"10.1016\/0140-6736(93)93127-M"},{"key":"key\n\t\t\t\t20170413030558_R31","doi-asserted-by":"crossref","unstructured":"Steen L, Holmgren G, Suhr O, Wikstr\u00f6m L, Groth C\u2010G, Ericzon B\u2010G. World\u2010wide survey of liver transplantation in patients with familial amyloidotic polyneuropathy. Amyloid: Int J Exp Clin Invest1994; 1:138\u201342.","DOI":"10.3109\/13506129409148636"},{"key":"key\n\t\t\t\t20170413030558_R32","doi-asserted-by":"crossref","unstructured":"Stangou AJ, Heaton ND, Rela M, Pepys MB, Hawkins PN, Williams R. Domino hepatic transplantation using the liver from a patient with familial amyloid polyneuropathy. Transplantation1998; 65:1496\u20138.","DOI":"10.1097\/00007890-199806150-00016"},{"key":"key\n\t\t\t\t20170413030558_R33","doi-asserted-by":"crossref","unstructured":"Rydh A, Suhr O, Hietala S\u2010O,\u2009\u00c5hlstr\u00f6m KR, Pepys MB, Hawkins PN. Serum amyloid P component scintigraphy in familial amyloid polyneuropathy: regression of visceral amyloid following liver transplantation. Eur J Nucl Med1998; 25:709\u201313.","DOI":"10.1007\/s002590050273"},{"key":"key\n\t\t\t\t20170413030558_R34","doi-asserted-by":"crossref","unstructured":"Gillmore JD, Davies J, Iqbal A, Madhoo S, Russell NH, Hawkins PN. Allogeneic bone marrow transplantation for systemic AL amyloidosis. Br J Haematol1998; 100:226\u20138.","DOI":"10.1046\/j.1365-2141.1998.00527.x"},{"key":"key\n\t\t\t\t20170413030558_R35","doi-asserted-by":"crossref","unstructured":"Rasmussen A, Davies HF, Jamieson NV, Evans DB, Calne RY. Combined transplantation of liver and kidney from the same donor protects the kidney from rejection and improves kidney graft survival. Transplantation1995; 59:919\u201321.","DOI":"10.1097\/00007890-199503270-00025"}],"container-title":["QJM: An International Journal of Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/qjmed\/article-pdf\/93\/5\/269\/12884064\/930269.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,3,4]],"date-time":"2020-03-04T08:41:56Z","timestamp":1583311316000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/qjmed\/article-lookup\/doi\/10.1093\/qjmed\/93.5.269"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,5]]},"references-count":35,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2000,5]]},"published-print":{"date-parts":[[2000,5]]}},"URL":"https:\/\/doi.org\/10.1093\/qjmed\/93.5.269","relation":{},"ISSN":["1460-2393","1460-2725"],"issn-type":[{"value":"1460-2393","type":"electronic"},{"value":"1460-2725","type":"print"}],"subject":[],"published":{"date-parts":[[2000,5]]}}}