{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T00:44:10Z","timestamp":1772498650218,"version":"3.50.1"},"reference-count":7,"publisher":"BMJ","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Archives of Disease in Childhood"],"published-print":{"date-parts":[[1978,3,1]]},"DOI":"10.1136\/adc.53.3.255","type":"journal-article","created":{"date-parts":[[2008,12,22]],"date-time":"2008-12-22T21:33:49Z","timestamp":1229981629000},"page":"255-256","source":"Crossref","is-referenced-by-count":20,"title":["Familial haemolytic uraemic syndrome."],"prefix":"10.1136","volume":"53","author":[{"given":"A D","family":"Edelsten","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Tuck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","reference":[{"key":"ref_1","article-title":"The fatal outcome in this family, the study by Farr","volume":"1975","author":"and 4 sibs from Zurich (Blattler","year":"1975","unstructured":"The fatal outcome in this family, the study by Farr et al. (1975), and the reports of 3 infant sibs (Kaplan et al., 1975) and 4 sibs from Zurich (Blattler et al., 1975) all of whom died, together indicate a grave prognosis in cases where more than 2 family members are affected. It seems likely that the stronger the genetic evidence, the worse the prognosis. This family and its case pattern strongly support a genetic predisposition as an aetiological factor in some cases of HUS. Farr et al. (1975), however, were unable to identify red cell or HLA genetic markers in their family study. The inheritance pattern in this family, where first-cousin marriages were frequent, would fit an autosomal recessive rather than dominant mode of transmission. Dialysis and renal transplantation offer survival prospects to some victims, but it is doubtful whether conventional therapeutic measures for HUS can alter the outcome in these cases.","journal-title":"et al; and the reports of 3 infant sibs (Kaplan"},{"key":"ref_2","first-page":"1773","article-title":"Familiares hamolytisch-uramisches Syndrom","volume":"105","author":"Blattler, W.; Wegman, W.; Herold, H.","year":"1975","unstructured":"Blattler, W., Wegman, W., and Herold, H. (1975). Familiares hamolytisch-uramisches Syndrom. Schweizerische Medizinische Wochenschrift, 105, 1773-1774.","journal-title":"Schweizerische Medizinische Wochenschrift"},{"key":"ref_3","first-page":"161","article-title":"The haemolytic uraemic syndrome-a family study","volume":"44","author":"Farr, M.J.; Roberts, S.; Morley, A.R.; Dewar, P.J.; Roberts, D.F.; Uldall, P.R.","year":"1975","unstructured":"Farr, M. J., Roberts, S., Morley, A. R., Dewar, P. J., Roberts, D. F., and Uldall, P. R. (1975). The haemolytic uraemic syndrome-a family study. Quarterly Journal of Medicine, 44, 161-188.","journal-title":"Quarterly Journal of Medicine"},{"key":"ref_4","first-page":"905","article-title":"Hamolytisch-uramische Syndrome: Bilaterale Nierenrindennekrosen bei akuten erworbenen hamolytischen Anamien","volume":"85","author":"Gasser, C.; Gautier, E.; Steck, A.; Siebenman, R.E.; Oechslin, R.","year":"1955","unstructured":"Gasser, C., Gautier, E., Steck, A., Siebenman, R. E., and Oechslin, R. (1955). Hamolytisch-uramische Syndrome: Bilaterale Nierenrindennekrosen bei akuten erworbenen hamolytischen Anamien. Schweizerische Medizinische Wochenschrift, 85, 905-909.","journal-title":"Schweizerische Medizinische Wochenschrift"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1056\/NEJM197505222922102","article-title":"Hemolytic uremic syndrome in families","volume":"292","author":"Kaplan, B.S.; Chesney, R.W.; Drummond, K.N.","year":"1975","unstructured":"Kaplan, B. S., Chesney, R. W., and Drummond, K. N. (1975). Hemolytic uremic syndrome in families. New England Journal of Medicine, 292, 1090-1092.","journal-title":"New England Journal of Medicine"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1136\/adc.49.6.432","article-title":"Haemolytic-uraemic syndrome: epidemiological and clinical study","volume":"49","author":"Van Wieringen, P.M.; Monnens, L.A.H.; Schretlen, E.D.A.M.","year":"1974","unstructured":"Van Wieringen, P. M., Monnens, L. A. H., and Schretlen, E. D. A. M. (1974). Haemolytic-uraemic syndrome: epidemiological and clinical study. Archives of Disease in C'hildhood, 49 432-437.","journal-title":"Archives of Disease in C'hildhood"},{"key":"ref_7","article-title":"Congenital heart block and hypothyroidism Complete heart block has been reported in association","volume-title":"in adults. Autoimmunity (Zoob and Smith, 1963) and myocarditis","author":"with; thyrotoxicosis (Stem","year":"1975","unstructured":"Congenital heart block and hypothyroidism Complete heart block has been reported in association with thyrotoxicosis (Stem et al., 1970; Kemoff et al., 1973; Fairfax and Leatham, 1975), and hypothyroidism (Singh et al., 1973; Fairfax and Leatham, 1975) in adults. Autoimmunity (Zoob and Smith, 1963) and myocarditis (Hudson, 1965) have been implicated. I describe a case here in which hypothyroidism was associated with congenital heart block. I have not been able to discover another such case in the literature."}],"container-title":["Archives of Disease in Childhood"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/adc.53.3.255","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,26]],"date-time":"2020-09-26T09:10:28Z","timestamp":1601111428000},"score":1,"resource":{"primary":{"URL":"https:\/\/adc.bmj.com\/lookup\/doi\/10.1136\/adc.53.3.255"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1978,3,1]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1978,3,1]]}},"URL":"https:\/\/doi.org\/10.1136\/adc.53.3.255","relation":{},"ISSN":["0003-9888","1468-2044"],"issn-type":[{"value":"0003-9888","type":"print"},{"value":"1468-2044","type":"electronic"}],"subject":[],"published":{"date-parts":[[1978,3,1]]}}}