Atypical hemolytic uremic syndrome : a monocentric adult Tunisian study and review of literature
Joint Authors
al-Faqih, R. Khidr
Haji, M.
Bin Fatimah, L.
Jabali, H.
Mami, I.
Krid, M.
Sellami-Kallel, M.
Beji, S.
Rais, L.
Smaoui, W.
Zouaghi, K.
Source
Saudi Journal of Kidney Diseases and Transplantation
Issue
Vol. 29, Issue 2 (30 Apr. 2018), pp.297-302, 6 p.
Publisher
Saudi Center for Organ Transplantation
Publication Date
2018-04-30
Country of Publication
Saudi Arabia
No. of Pages
6
Main Subjects
Abstract EN
Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction.
It is associated with genetic or acquired disorders of regulatory components of the complement system.
For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014.
The mean age was 33.6 years.
The female-to-male ratio was 3.
The median follow-up duration was 27 ± 3.5 months.
The most common clinical presentation was hypertension.
Renal involvement was noted in all cases.
Ten patients had extrarenal manifestations.
Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases.
A genetic study was not available for our patients.
During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge.
One patient underwent renal transplantation.
None of our patients received eculizumab perfusion.
The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02).
Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01).
Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes
American Psychological Association (APA)
al-Faqih, R. Khidr& Haji, M.& Bin Fatimah, L.& Jabali, H.& Mami, I.& Smaoui, W.…[et al.]. 2018. Atypical hemolytic uremic syndrome : a monocentric adult Tunisian study and review of literature. Saudi Journal of Kidney Diseases and Transplantation،Vol. 29, no. 2, pp.297-302.
https://search.emarefa.net/detail/BIM-838513
Modern Language Association (MLA)
al-Faqih, R. Khidr…[et al.]. Atypical hemolytic uremic syndrome : a monocentric adult Tunisian study and review of literature. Saudi Journal of Kidney Diseases and Transplantation Vol. 29, no. 2 (Mar. / Apr. 2018), pp.297-302.
https://search.emarefa.net/detail/BIM-838513
American Medical Association (AMA)
al-Faqih, R. Khidr& Haji, M.& Bin Fatimah, L.& Jabali, H.& Mami, I.& Smaoui, W.…[et al.]. Atypical hemolytic uremic syndrome : a monocentric adult Tunisian study and review of literature. Saudi Journal of Kidney Diseases and Transplantation. 2018. Vol. 29, no. 2, pp.297-302.
https://search.emarefa.net/detail/BIM-838513
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 301-302
Record ID
BIM-838513