{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T15:01:23Z","timestamp":1761058883374},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"808","license":[{"start":{"date-parts":[[1993,2,1]],"date-time":"1993-02-01T00:00:00Z","timestamp":728524800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1993,2,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Hereditary angioedema is an autosomal dominant disorder of the complement system in which there is a deficiency of the inhibitor of the activated first component of complement. We have previously reported on three generations of a family with classic hereditary angioedema. Three members of this family have now developed IgA nephropathy. The association of hereditary angioedema with various immunoregulatory disorders has been previously reported but this is the first report of IgA nephropathy in association with this condition.<\/jats:p>","DOI":"10.1136\/pgmj.69.808.95","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T21:25:21Z","timestamp":1230585921000},"page":"95-99","source":"Crossref","is-referenced-by-count":9,"title":["IgA nephropathy in hereditary angioedema"],"prefix":"10.1093","volume":"69","author":[{"given":"Jayashri","family":"Srinivasan","sequence":"first","affiliation":[{"name":"Department of Medicine, Llandough Hospital , Penarth, South Glanorgan CF6 1XX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Beck","sequence":"additional","affiliation":[{"name":"Department of Medicine, Llandough Hospital , Penarth, South Glanorgan CF6 1XX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1993,2,1]]},"reference":[{"issue":"77","key":"ref_1","first-page":"749","article-title":"Immunoregulatory disorders associated with hereditary angioedema. 1. Clinical manifestations of autoimmune disease","volume":"1986","author":"Brickman, C.M.; Tsokos, G.C.; Balow, J.E.","unstructured":"Brickman, C.M., Tsokos, G.C., Balow, J.E. et al. Immunoregulatory disorders associated with hereditary angioedema. 1. Clinical manifestations of autoimmune disease. J Allergy Clin Immunol 1986, 77: 749-757.","journal-title":"J Allergy Clin Immunol"},{"issue":"91","key":"ref_2","first-page":"417","article-title":"Hereditary angioedema: association with IgA deficiency and otolaryngologic disorders","volume":"1981","author":"Bond, W.R.; Herrod, H.G.; Duberstein, L.E.","unstructured":"Bond, W.R., Herrod, H.G. & Duberstein, L.E. Hereditary angioedema: association with IgA deficiency and otolaryngologic disorders. Laryngoscope 1981, 91: 417-421.","journal-title":"Laryngoscope"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1136\/pgmj.53.624.627","article-title":"Hereditary angioedema with mesangiocapillary glomerulonephritis","volume":"53","author":"Plaza, J.; Malasit, P.; Kerr, D.N.S.","year":"1977","unstructured":"Plaza, J., Malasit, P. & Kerr, D.N.S. Hereditary angioedema with mesangiocapillary glomerulonephritis. Postgrad Med J 1977, 53: 627-630.","journal-title":"Postgrad Med J"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1097\/00000441-198401000-00014","article-title":"Hereditary angioedema and coronary arteritis","volume":"287","author":"Harrington, T.M.; Torretti, D.; Pytko, V.F.; Plotkin, G.R.","year":"1984","unstructured":"Harrington, T.M., Torretti, D., Pytko, V.F. & Plotkin, G.R. Hereditary angioedema and coronary arteritis. Am J Med Sci 1984, 287: 50-52.","journal-title":"Am J Med Sci"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1056\/NEJM197612232952602","article-title":"Treatment of hereditary angioedema with danazol: reversal of clinical and biochemical abnormalities","volume":"295","author":"Gelfand, J.A.; Sherins, R.J.; Alling, D.W.; Frank, M.M.","year":"1976","unstructured":"Gelfand, J.A., Sherins, R.J., Alling, D.W. & Frank, M.M. Treatment of hereditary angioedema with danazol: reversal of clinical and biochemical abnormalities. N Engl J Med 1976, 295: 1444-1448.","journal-title":"N Engl J Med"},{"key":"ref_6","first-page":"317","article-title":"A family study of hereditary angioedema","volume":"166","author":"Beck, P.; Wills, D.; Davies, G.T.; Lachmann, P.J.; Sussman, M.","year":"1973","unstructured":"Beck, P., Wills, D., Davies, G.T., Lachmann, P.J. & Sussman, M. A family study of hereditary angioedema. Q J Med 1973, 166: 317-339.","journal-title":"Q J Med"},{"key":"ref_7","first-page":"455","article-title":"HLA and Disease Associations","author":"Tiwari, J.L.; Terasaki, P.I.","year":"1985","unstructured":"Tiwari, J.L. & Terasaki, P.I. HLA and Disease Associations. Springer Verlag, New York, 1985, p. 455."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1159\/000182849","article-title":"Strong association of HLA DR4 with benign IgA nephropathy","volume":"32","author":"Hiki, Y.; Kobayashi, Y.; Tateno, S.","year":"1982","unstructured":"Hiki, Y., Kobayashi, Y., Tateno, S. et al. Strong association of HLA DR4 with benign IgA nephropathy. Nephron 1982, 32: 222-226.","journal-title":"Nephron"},{"key":"ref_9","first-page":"283","article-title":"Immunogenetique des glomerulonephrites a depts d'IgA","volume":"4","author":"Berthoux, F.C.; Genin, C.; LePetit, J.C.","year":"1983","unstructured":"Berthoux, F.C., Genin, C., LePetit, J.C. et al. Immunogenetique des glomerulonephrites a depts d'IgA. Nephrologie 1983, 4: 283-287.","journal-title":"Nephrologie"},{"issue":"95","key":"ref_10","first-page":"362","article-title":"Hereditary angio-neurotic oedema","volume":"1988","author":"Osler, W.","unstructured":"Osler, W. Hereditary angio-neurotic oedema. Am J Med Sci 1988, 95: 362-372.","journal-title":"Am J Med Sci"},{"issue":"35","key":"ref_11","first-page":"37","article-title":"A biochemical abnormality in hereditary angioedema: absence of serum inhibitor of Cl esterase","volume":"1963","author":"Donaldson, V.H.; Evans, R.R.","unstructured":"Donaldson, V.H. & Evans, R.R. A biochemical abnormality in hereditary angioedema: absence of serum inhibitor of Cl esterase. Am J Med 1963, 35: 37-44.","journal-title":"Am J Med"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"809","DOI":"10.7326\/0003-4819-93-6-809","article-title":"Long term therapy of hereditary angioedema with danazol","volume":"93","author":"Hosea, S.W.; Santaella, M.L.; Brown, E.J.","year":"1980","unstructured":"Hosea, S.W., Santaella, M.L., Brown, E.J. et al. Long term therapy of hereditary angioedema with danazol. Ann Intern Med 1980, 93: 809-812.","journal-title":"Ann Intern Med"},{"key":"ref_13","first-page":"381","article-title":"C I inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema","volume":"65","author":"Oltvai, Z.N.; Wong, E.C.C.; Atkinson, J.P.; Tung, K.S.K.","year":"1991","unstructured":"Oltvai, Z.N., Wong, E.C.C., Atkinson, J.P. & Tung, K.S.K. C I inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema. Lab Invest 1991, 65: 381-388.","journal-title":"Lab Invest"},{"issue":"148","key":"ref_14","first-page":"957","article-title":"Hereditary angioedema: two genetic variants","volume":"1965","author":"Rosen, F.S.; Charache, P.; Pensky, J.; Donaldson, V.","unstructured":"Rosen, F.S., Charache, P., Pensky, J. & Donaldson, V. Hereditary angioedema: two genetic variants. Science 1965, 148: 957-958.","journal-title":"Science"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/0090-1229(72)90006-2","article-title":"Acquired C1 inhibitor deficiency in lymphosarcoma","volume":"1","author":"Caldwell, J.R.; Ruddy, S.; Schur, P.H.; Austen, K.F.","year":"1972","unstructured":"Caldwell, J.R., Ruddy, S., Schur, P.H. & Austen, K.F. Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopath 1972, 1: 39-42.","journal-title":"Clin Immunol Immunopath"},{"issue":"48","key":"ref_16","first-page":"642","article-title":"Permeability increasing activity in hereditary angioneurotic oedema plasma. II. Mechanism of formation of and partial characterisation","volume":"1969","author":"Donaldson, V.H.; Ratnoff, O.D.; Da Silva, W.D.; Rosen, F.S.","unstructured":"Donaldson, V.H., Ratnoff, O.D., Da Silva, W.D. & Rosen, F.S. Permeability increasing activity in hereditary angioneurotic oedema plasma. II. Mechanism of formation of and partial characterisation. J Clin Invest 1969, 48: 642-653.","journal-title":"J Clin Invest"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1050","DOI":"10.1056\/NEJM198305053081802","article-title":"Prekallikrein activation and high molecular weight kininogen consumption in hereditary angioedema","volume":"308","author":"Schapira, M.; Silver, L.D.; Scott, C.F.","year":"1983","unstructured":"Schapira, M., Silver, L.D., Scott, C.F. et al. Prekallikrein activation and high molecular weight kininogen consumption in hereditary angioedema. N Engl J Med 1983, 308: 1050-1053.","journal-title":"N Engl J Med"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/0091-6749(83)90052-0","article-title":"Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of spontaneous formation of bradykinin","volume":"72","author":"Fields, T.; Ghebrehiwet, B.; Kaplan, A.P.","year":"1983","unstructured":"Fields, T., Ghebrehiwet, B. & Kaplan, A.P. Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of spontaneous formation of bradykinin. J Allergy Clin Immunol 1983, 72: 54-60.","journal-title":"J Allergy Clin Immunol"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"640","DOI":"10.4049\/jimmunol.123.2.640","article-title":"Effects of Cl on the size of soluble immune aggregates and their processing by macrophages","volume":"123","author":"Kijlstra, A.; Van Es, L.A.; Daha, M.R.","year":"1979","unstructured":"Kijlstra, A., Van Es, L.A. & Daha, M.R. Effects of Cl on the size of soluble immune aggregates and their processing by macrophages. J Immunol 1979, 123: 640-645.","journal-title":"J Immunol"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/S0272-6386(84)80061-X","article-title":"Effect of danazol on solubilisation of immune deposits in patients with IgA nephropathy","author":"Tomino, Y.; Sakai, H.; Miura, M.","year":"1984","unstructured":"Tomino, Y., Sakai, H., Miura, M. et al. Effect of danazol on solubilisation of immune deposits in patients with IgA nephropathy. Am J Kidney Dis 1984, IV (2): 135-140.","journal-title":"Am J Kidney Dis"},{"issue":"74","key":"ref_21","first-page":"694","article-title":"Les depots intracapillaries d'IgAIgG","volume":"1968","author":"Berger, J.; Hinglais, N.","unstructured":"Berger, J. & Hinglais, N. Les depots intracapillaries d'IgAIgG. J Urol Nephrol 1968, 74: 694-695.","journal-title":"J Urol Nephrol"},{"key":"ref_22","first-page":"709","article-title":"The commonest glomerulonephritis in the world: IgA nephropathy","volume":"245","author":"D'Amico, G.","year":"1987","unstructured":"D'Amico, G. The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med 1987, 245: 709-727.","journal-title":"Q J Med"},{"issue":"5","key":"ref_23","first-page":"437","article-title":"Activation of complement in IgA nephropathy","volume":"1988","author":"Wyatt, R.J.; Julian, B.A.","unstructured":"Wyatt, R.J. & Julian, B.A. Activation of complement in IgA nephropathy. Am J Kidney Dis 1988, 5: 437-442.","journal-title":"Am J Kidney Dis"},{"key":"ref_24","first-page":"312","volume":"1985","author":"Julian, B.J.; Quiggins, P.A.; Thompson, J.S.","unstructured":"Julian, B.J., Quiggins, P.A., Thompson, J.S. et al. Familial IgA nephropathy. N Engl J Med 1985, 312: 202-208.","journal-title":"et al. Familial IgA nephropathy. N Engl J Med"},{"issue":"4","key":"ref_25","first-page":"192","article-title":"IgA nephropathy: presentation, clinical course and prognosis in children and adults","volume":"1984","author":"Wyatt, R.J.; Julian, B.A.; Bhathena, D.","unstructured":"Wyatt, R.J., Julian, B.A., Bhathena, D. et al. IgA nephropathy: presentation, clinical course and prognosis in children and adults. Am J Kidney Dis 1984, 4: 192-200.","journal-title":"Am J Kidney Dis"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/69\/808\/95\/50072794\/postgradmedj-69-95.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/69\/808\/95\/50072794\/postgradmedj-69-95.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,23]],"date-time":"2023-05-23T07:03:51Z","timestamp":1684825431000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/69\/808\/95\/7062768"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,2,1]]},"references-count":25,"journal-issue":{"issue":"808","published-online":{"date-parts":[[1993,2,1]]},"published-print":{"date-parts":[[1993,2,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pgmj.69.808.95","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[1993,2]]},"published":{"date-parts":[[1993,2,1]]}}}