title: Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome: an adult and paediatric comparison.
abstract: Although diarrhoea-associated haemolytic uremic syndrome (HUS) in children is well described, the clinical features of bloody diarrhoea-associated thrombotic thrombocytopenic purpura (TTP)-HUS in adults are not documented. Twenty-one adults, 6.5% of the 322 adults in The Oklahoma TTP-HUS Registry, 1989-2006, have presented with bloody diarrhoea. There were no case clusters. Escherichia coli O157:H7 was identified in five patients, but many patients did not have appropriate studies. The annual incidence was 0.68/10(6), 10-fold less than the incidence of diarrhoea-associated HUS in children in Oklahoma. Two (13%) of 16 patients in whom ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) was measured had <10% activity. Severe neurological abnormalities (67%) and renal failure (62%) were common; seven patients (33%) died; no survivors have relapsed. Compared to the 38 other Oklahoma Registry patients with ADAMTS13 <10%, frequency of severe neurological abnormalities and death was not different; frequency of renal failure was greater; frequency of relapse was less. Compared to 5999 children with sporadic diarrhoea-associated HUS in published reports, frequency of renal failure and relapse was not different; frequency of severe neurological abnormalities and death was greater (P < 0.05 for all differences). Awareness of the continuous occurrence of sporadic bloody diarrhoea-associated TTP-HUS in adults is important for prompt diagnosis and appropriate management.
authors.affiliation: College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK 73190, USA.
authors.forename: Charity A
authors.initials: CA
authors.lastname: Karpac
authors.forename: Xiaoning
authors.initials: X
authors.lastname: Li
authors.forename: Deirdra R
authors.initials: DR
authors.lastname: Terrell
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
authors.forename: Sara K
authors.initials: SK
authors.lastname: Vesely
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D058186:Acute Kidney Injury / Q000209:etiology; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003967:Diarrhea / Q000209:etiology*; D004927:Escherichia coli Infections / Q000150:complications; D019453:Escherichia coli O157 / Q000302:isolation & purification; D005260:Female; D005500:Follow-Up Studies; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis* / Q000382:microbiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis* / Q000382:microbiology / Q000628:therapy; D012008:Recurrence; D012042:Registries
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human