| Thrombotic thrombocytopenic purpura in childhood: an uncommon but life-threatening cause of thrombocytopenia. | |
| | |
MedLine Citation:
|
PMID: 17688649 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterised by microangiopathic haemolytic anaemia, thrombocytopenia and signs of ischaemic organ dysfunction such as neurological or renal impairment and fever. The diagnosis of TTP should be considered in any child presenting with thrombocytopenia, in particular those children with microangiopathic haemolysis, atypical immune thrombocytopenia purpura or Evan's syndrome. Distinguishing TTP from haemolytic uraemic syndrome is difficult, but where there is doubt about the diagnosis, a presumptive diagnosis of TTP should be made to allow potentially life-saving therapy with therapeutic plasma exchange. Recent advances in the molecular basis of the disease have resulted in assays for ADAMTS-13 activity, inhibitor levels and ADAMTS-13 mutation analysis. These assays help to distinguish TTP from haemolytic uraemic syndrome. However, the performance characteristics of these assays in the diagnosis and management of TTP are yet to be defined. |
| | |
Authors:
|
Amanda Davis; Chris Barnes |
Related Documents
:
|
22274869 - Cushing syndrome treated by radiofrequency ablation of adrenal gland adenoma. 3069089 - Acute renal failure due to postpartum haemolytic uraemic syndrome. 10222439 - A review of the therapy of paraneoplastic neurologic syndromes. 2803069 - Chronic manganese intoxication. 21630079 - Plasma citrulline as marker of bowel adaptation in children with short bowel syndrome. 2088459 - Recurrent haemolytic uraemic syndrome and acquired hypomorphic variant of the third com... 11310989 - Mandibulofacial dysostosis, microcephaly and thorax deformities in two brothers: a new ... 20713929 - Surgical decompression for abdominal compartment syndrome in severe acute pancreatitis. 18260119 - Mycoplasma pneumoniae and atypical stevens-johnson syndrome in a hematopoietic stem cel... |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: Journal of paediatrics and child health Volume: 43 ISSN: 1034-4810 ISO Abbreviation: J Paediatr Child Health Publication Date: 2007 Sep |
Date Detail:
|
Created Date: 2007-08-10 Completed Date: 2008-03-25 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9005421 Medline TA: J Paediatr Child Health Country: Australia |
Other Details:
|
Languages: eng Pagination: 640-2 Citation Subset: IM |
Affiliation:
|
Department of Haematology, The Royal Children's and Women's Hospital, Melbourne, Victoria, Australia. akdavis@partners.org |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
ADAM Proteins
/
analysis Anemia, Hemolytic / diagnosis Child, Preschool Diagnosis, Differential Female Humans Purpura, Thrombotic Thrombocytopenic / diagnosis*, physiopathology, therapy Thrombocytopenia Treatment Outcome |
| Chemical | |
Reg. No./Substance:
|
EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: alpha1-Antitrypsin deficiency is not an important cause of childhood liver diseases in a multi-ethni...
Next Document: Early intervention for the ocular and neurodevelopmental sequelae of Fetal Valproate Syndrome.