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      Hemolytic Uremic syndrome associated with pregnancy: Outcome from acute Kidney Injury

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          Abstract

          Objective:

          To report here, case series of women developing acute kidney injury (AKI) in association with hemolytic uremic syndrome (HUS) during pregnancy or within days postpartum.

          Methods:

          Subjects for the study reported here comprised of a cohort of 49 women referred from OBGYN and diagnosed having AKI and HUS. AKI was defined according to RIFLE criteria and HUS on basis of hematological, biochemical and histological features. All patients had normal size kidneys on ultrasonography and no previous co- morbidity.

          Results:

          From January 2000 – January 2020, 49 such women were admitted/ referred to this facility. The mean age of these patients was 29.02±5.258 years. Two had HUS during pregnancy while rest during postpartum. Majority of women had more than one insults these include hemorrhages, intrauterine deaths, operative measure (lower segment caesarean section). Renal replacement was required in all women. Complete renal recovery was observed in 14 patients, while one died during acute phase of illness. CKD-V developed in 17 patients, 16 patients lost long term follow up, but were dialysis free till last follow up and one left against medical advice during acute phase of illness. Treatment with plasmapheresis revealed significantly better renal recovery (p value 0.03) in this group of patients.

          Conclusion:

          AKI with background of Hemolytic Uremic Syndrome (HUS) may remain irreversible in many of these young women. Plasmapheresis should be offered to patients with established diagnosis of HUS.

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          Most cited references15

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          Atypical hemolytic-uremic syndrome.

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            Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations.

            In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) remain ill-defined. We conducted a retrospective study to assess the presentation and outcomes of patients presenting with P-aHUS and the prevalence of alternative C3 convertase dysregulation. P-aHUS occurred in 21 of the 100 adult female patients with atypical HUS, with 79% presenting postpartum. We detected complement abnormalities in 18 of the 21 patients. The outcomes were poor: 62% reached ESRD by 1 month and 76% by last follow-up. The risk for P-aHUS was highest during a second pregnancy. Thirty-five women, 26 (74%) of whom had complement abnormalities, had at least one pregnancy before the onset of a non-pregnancy-related aHUS. Outcomes did not differ between patients with pregnancy-related and non-pregnancy-related aHUS. Mutations in the SCR19-20 domains of factor H were less frequent in P-aHUS patients compared with non-pregnancy-related aHUS. Pregnancies in female patients with complement abnormalities (n = 44) were complicated by fetal loss and preeclampsia in 4.8% and 7.7%, respectively. Better understanding of complement dysregulation in pregnancy complications is essential, especially to guide development of pharmacologic agents to modulate this system.
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              Hemolytic Uremic Syndrome in Pregnancy and Postpartum.

              Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation.
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                Author and article information

                Journal
                Pak J Med Sci
                Pak J Med Sci
                Pakistan Journal of Medical Sciences
                Professional Medical Publications (Pakistan )
                1682-024X
                1681-715X
                Sep-Oct 2020
                : 36
                : 6
                : 1153-1157
                Affiliations
                [1 ]Prof. Dr. Rubina Naqvi Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan
                Author notes
                Correspondence: Prof. Dr. Rubina Naqvi Department of Nephrology, Sindh Institute of Urology and Transplantation (SIUT), Karachi. 74200. Pakistan. E-mail: naqvirubina@ 123456yahoo.com rubinanaqvi@ 123456gmail.com
                Article
                PJMS-36-1153
                10.12669/pjms.36.6.2931
                7500999
                17e95a8e-ed4e-4089-a75a-1277104600ef
                Copyright: © Pakistan Journal of Medical Sciences

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 May 2020
                : 29 July 2020
                : 05 August 2020
                Categories
                Original Article

                aki,rifle criteria,hemolytic uremic syndrome,pregnancy,plasmapheresis

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