{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T11:02:08Z","timestamp":1760266928680},"reference-count":17,"publisher":"Oxford University Press (OUP)","issue":"820","license":[{"start":{"date-parts":[[1994,2,1]],"date-time":"1994-02-01T00:00:00Z","timestamp":760060800000},"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":[[1994,2,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Type I polyglandular autoimmune syndrome is characterized by the triad of hypoparathyroidism, Addison's disease and chronic mucocutaneous candidiasis. Chronic active hepatitis has been associated with this syndrome but its incidence and severity have not been well documented.<\/jats:p>\n               <jats:p>We describe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. The first patient presented to us with advanced disease and died despite an emergent liver transplant, while the second patient responded to steroids.<\/jats:p>\n               <jats:p>Autoimmune chronic active hepatitis, a major cause of mortality in this syndrome, can present without symptoms or physical signs of liver disease. We suggest periodic screening of liver enzymes in subjects with this syndrome.<\/jats:p>","DOI":"10.1136\/pgmj.70.820.128","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T21:25:21Z","timestamp":1230585921000},"page":"128-131","source":"Crossref","is-referenced-by-count":27,"title":["Chronic active hepatitis in the type I polyglandular autoimmune syndrome"],"prefix":"10.1093","volume":"70","author":[{"given":"Theresa M","family":"Michele","sequence":"first","affiliation":[{"name":"Division of Gastroenterology, The Johns Hopkins University Hospital , Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacqueline","family":"Fleckenstein","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, The Johns Hopkins University Hospital , Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anita R","family":"Sgrignoli","sequence":"additional","affiliation":[{"name":"Division of Pathology, The Johns Hopkins University Hospital , Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul J","family":"Thuluvath","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, The Johns Hopkins University Hospital , Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1994,2,1]]},"reference":[{"issue":"16","key":"ref_1","first-page":"1374","article-title":"The syndrome of familial juvenile hypoadrenocorticism, hypoparathyroidism and superficial moniliasis","volume":"1956","author":"Whitaker, J.; Landing, B.H.; Esselborn, V.M.; Williams, R.R.","unstructured":"Whitaker, J., Landing, B.H., Esselborn, V.M. & Williams, R.R. The syndrome of familial juvenile hypoadrenocorticism, hypoparathyroidism and superficial moniliasis. J Clin Endocrinol Metab 1956, 16: 1374-1387.","journal-title":"J Clin Endocrinol Metab"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/0002-9343(84)90444-3","article-title":"Polyglandular autoimmune syndromes","volume":"77","author":"Trence, D.L.; Morley, J.E.; Handwerger, B.S.","year":"1984","unstructured":"Trence, D.L., Morley, J.E. & Handwerger, B.S. Polyglandular autoimmune syndromes. Am J Med 1984, 77: 107-116.","journal-title":"Am J Med"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1159\/000179519","article-title":"Juvenile auto-immune polyendocrinolopathy","volume":"16","author":"Brun, J.M.","year":"1982","unstructured":"Brun, J.M. Juvenile auto-immune polyendocrinolopathy. Hormone Res 1982, 16: 308-316.","journal-title":"Hormone Res"},{"key":"ref_4","first-page":"27","article-title":"Autoimmune polyendocrinopathy-candidiasisectodermal dystrophy (APECED): autosomal recessive inheritance","volume":"1985","author":"Ahonen, P.","unstructured":"Ahonen, P. Autoimmune polyendocrinopathy-candidiasisectodermal dystrophy (APECED): autosomal recessive inheritance. Clin Genetics 1985, 27: 535-542.","journal-title":"Clin Genetics"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1056\/NEJM199006283222601","article-title":"Clinical variation of autoimmune polyendocrinopathycandidiasis-ectodermal dystrophy (APECED) in a series of 68 patients","volume":"322","author":"Ahonen, P.; Myllamiemi, S.; Sipila, I.; Perheentupa, J.","year":"1990","unstructured":"Ahonen, P., Myllamiemi, S., Sipila, I. & Perheentupa, J. Clinical variation of autoimmune polyendocrinopathycandidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med 1990, 322: 1829-1836.","journal-title":"N Engl J Med"},{"key":"ref_6","first-page":"357","article-title":"Polyglandular autoinunune disease","volume":"33","author":"Neufield, M.; Blizzard, R.M.","year":"1980","unstructured":"Neufield, M. & Blizzard, R.M. Polyglandular autoinunune disease. In: Pincherra, A., Doniach, D., Fenzi, G.F. & Baschieri, L. (eds) Autoimmune Aspects of Endocrine Disorders. Proceedings of the Serona Symposia, Vol. 33. Academic Press, New York, 1980, pp. 357-365.","journal-title":"Proceedings of the Serona Symposia; Autoimmune Aspects of Endocrine Disorders"},{"issue":"60","key":"ref_7","first-page":"355","article-title":"Two types of auto-immune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes","volume":"1981","author":"Neufield, M.; Maclaren, N.K.; Blizzard, R.M.","unstructured":"Neufield, M., Maclaren, N.K. & Blizzard, R.M. Two types of auto-immune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes. Medicine (Baltimore) 1981, 60: 355-362.","journal-title":"Medicine (Baltimore)"},{"key":"ref_8","first-page":"312","article-title":"Isolated gonadotrope failure in the polyglandular autoimmune syndrome","volume":"1985","author":"Barkan, A.L.; Kelch, R.P.; Marshall, J.C.","unstructured":"Barkan, A.L., Kelch, R.P. & Marshall, J.C. Isolated gonadotrope failure in the polyglandular autoimmune syndrome. N Engl J Med 1985, 312: 1535-1540.","journal-title":"N Engl J Med"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1056\/NEJM197901253000403","article-title":"Evidence for defective immunoregulation in the syndrome of familial candidiasis endocrinopathy","volume":"300","author":"Arulanantham, K.; Dwyer, J.M.; Genel, M.","year":"1979","unstructured":"Arulanantham, K., Dwyer, J.M. & Genel, M. Evidence for defective immunoregulation in the syndrome of familial candidiasis endocrinopathy. N Engl J Med 1979, 300: 164-168.","journal-title":"N Engl J Med"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1016\/0002-9343(91)90215-J","article-title":"Frequent occurrence of asplenism and cholelithiasis in patients with autoimmune polyglandular disease Type 1","volume":"91","author":"Friedman, T.C.; Thomas, P.M.; Fleisher, T.A.","year":"1991","unstructured":"Friedman, T.C., Thomas, P.M., Fleisher, T.A. et al. Frequent occurrence of asplenism and cholelithiasis in patients with autoimmune polyglandular disease Type 1. Am J Med 1991, 91: 625-630.","journal-title":"Am J Med"},{"key":"ref_11","first-page":"485","article-title":"Autoimmune chronic active hepatitis; three decades of progress","volume-title":"Progress in Liver Disease","volume":"8","author":"Schaffner, F.","year":"1986","unstructured":"Schaffner, F. Autoimmune chronic active hepatitis; three decades of progress. In: Popper, H. & Schaffner, F. (eds) Progress in Liver Disease, Vol. 8. Orlando, Grune & Stratton, 1986, p. 485."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/S0022-3476(05)82892-6","article-title":"Autoimmune hepatitis with initial presentation as acute hepatic failure in young children","volume":"116","author":"true","year":"1990","unstructured":"Maggiore, G., Porta, G., Bernard, 0. et al. Autoimmune hepatitis with initial presentation as acute hepatic failure in young children. J Pediatr 1990, 116: 280-282.","journal-title":"J Pediatr"},{"issue":"89","key":"ref_13","first-page":"669","article-title":"Chronic moniliasis associated with Addison's disease","volume":"1955","author":"Craig, J.M.; Schiff, L.H.; Boone, J.E.","unstructured":"Craig, J.M., Schiff, L.H. & Boone, J.E. Chronic moniliasis associated with Addison's disease. Am J Dis Child 1955, 89: 669-683.","journal-title":"Am J Dis Child"},{"issue":"21","key":"ref_14","first-page":"634","article-title":"Idiopathic hypoparathyroidism and Addison's disease","volume":"1956","author":"Perlmutter, M.; Ellson, R.R.; Norsa, L.; Kantrowitz, A.R.","unstructured":"Perlmutter, M., Ellson, R.R., Norsa, L. & Kantrowitz, A.R. Idiopathic hypoparathyroidism and Addison's disease. Am J Med 1956, 21: 634-643.","journal-title":"Am J Med"},{"issue":"34","key":"ref_15","first-page":"302","article-title":"The syndrome of hypoparathyroidism and steatorrhoea","volume":"1959","author":"Williams, E.; Wood, C.","unstructured":"Williams, E. & Wood, C. The syndrome of hypoparathyroidism and steatorrhoea. Arch Dis Childhood 1959, 34: 302-306.","journal-title":"Arch Dis Childhood"},{"issue":"34","key":"ref_16","first-page":"856","article-title":"The syndrome of hypoparathyroidism and adrenocortical insufficiency, a possible sequel of hepatitis","volume":"1963","author":"Kunin, A.S.; MacKay, B.R.; Bums, S.L.; Halberstam, M.J.","unstructured":"Kunin, A.S., MacKay, B.R., Bums, S.L. & Halberstam, M.J. The syndrome of hypoparathyroidism and adrenocortical insufficiency, a possible sequel of hepatitis. Am J Med 1963, 34: 856-866.","journal-title":"Am J Med"},{"key":"ref_17","first-page":"397","article-title":"Variable cellmediated immune defects in a family with 'candida endocrinopathy syndrome.'","volume":"20","author":"Kaffe, S.; Petigrow, C.S.; Cahill, L.T.","year":"1975","unstructured":"Kaffe, S., Petigrow, C.S., Cahill, L.T. et al. Variable cellmediated immune defects in a family with 'candida endocrinopathy syndrome.' Clin Exp Immunol 1975, 20: 397-408.","journal-title":"Clin Exp Immunol"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/70\/820\/128\/50217652\/postgradmedj-70-128.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/70\/820\/128\/50217652\/postgradmedj-70-128.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,15]],"date-time":"2023-05-15T10:59:46Z","timestamp":1684148386000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/70\/820\/128\/7042374"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1994,2,1]]},"references-count":17,"journal-issue":{"issue":"820","published-online":{"date-parts":[[1994,2,1]]},"published-print":{"date-parts":[[1994,2,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pgmj.70.820.128","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[1994,2]]},"published":{"date-parts":[[1994,2,1]]}}}