Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome
Joint Authors
Agarwal, Indira
Arumugam, Venkatesh
Bhowmick, Rohit
Arumadi, Manjusha
Source
Saudi Journal of Kidney Diseases and Transplantation
Issue
Vol. 30, Issue 3 (30 Jun. 2019), pp.701-705, 5 p.
Publisher
Saudi Center for Organ Transplantation
Publication Date
2019-06-30
Country of Publication
Saudi Arabia
No. of Pages
5
Main Subjects
Topics
- Patients
- Genetic aspects
- Complications(Medicine)
- Diagnosis
- Hemolytic-uremic syndrome
- Thrombotic microangiopathy
Abstract EN
In evaluating a patient with thrombotic microangiopathy (TMA), it is necessary to rule out thrombotic thrombocytopenic purpura before a diagnosis of atypical hemolytic uremic syndrome (aHUS) is made.
There have been reports that mutations of complement factors can coexist with partial A Disintegrin and Metalloproteinase with a ThromboSpondin type 1 motif, member 13 deficiency.
Here, we report the case of a 6-year-old girl who was initially diagnosed as nephrotic syndrome and developed TMA after five years of onset of illness.
She had poor response to treatment and had multiple relapses due to associated complement factor mutation.
Hence, genetic evaluation has to be considered in all children presenting with aHUS.
American Psychological Association (APA)
Arumugam, Venkatesh& Bhowmick, Rohit& Agarwal, Indira& Arumadi, Manjusha. 2019. Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome. Saudi Journal of Kidney Diseases and Transplantation،Vol. 30, no. 3, pp.701-705.
https://search.emarefa.net/detail/BIM-894157
Modern Language Association (MLA)
Arumugam, Venkatesh…[et al.]. Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome. Saudi Journal of Kidney Diseases and Transplantation Vol. 30, no. 3 (May. / Jun. 2019), pp.701-705.
https://search.emarefa.net/detail/BIM-894157
American Medical Association (AMA)
Arumugam, Venkatesh& Bhowmick, Rohit& Agarwal, Indira& Arumadi, Manjusha. Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome. Saudi Journal of Kidney Diseases and Transplantation. 2019. Vol. 30, no. 3, pp.701-705.
https://search.emarefa.net/detail/BIM-894157
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 704-705
Record ID
BIM-894157