Atypical Presentation of Pregnancy-Related Hemolytic Uremic Syndrome

Baghli, Salim; Abendroth, Catherine; Farooq, Umar; Schaub, Jennifer A.

doi:10.1053/j.ajkd.2017.11.006

Cited by 8 publications

(5 citation statements)

References 22 publications

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“…After delivery, inflammation, the release of foetal cells in the maternal circulation, infections, and haemorrhage can lead to activation of the alternative complement pathway, which, in the absence of effective regulatory mechanisms, may induce postpartum atypical haemolytic-uremic syndrome (aHUS); complement dysregulation was also found to be associated with the HELLP syndrome, which shares several features with pregnancy-associated TMA [ 148 , 149 , 150 , 151 , 152 ]. The new frontiers of treatment with eculizumab make diagnosis of pivotal importance for allowing timely treatment [ 149 , 150 , 151 , 153 ].…”

Section: The Role Of Immunologic Diseases In the Pathogenesis Of Tmentioning

confidence: 99%

Acute Kidney Injury in Pregnancy: The Need for Higher Awareness. A Pragmatic Review Focused on What Could Be Improved in the Prevention and Care of Pregnancy-Related AKI, in the Year Dedicated to Women and Kidney Diseases

Piccoli

Zakharova

Attini

et al. 2018

JCM

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Pregnancy-related acute kidney injury (pAKI), preeclampsia (PE), and the hypertensive disorders of pregnancy are closely related conditions, which are, in turn, frequently linked to pre-existing and often non-diagnosed chronic kidney disease (CKD). The current literature and research mainly underline the effects of pregnancy complications on the offspring; this review strongly emphasizes the maternal health as well. These conditions not only negatively affect pregnancy outcomes, but have a relevant effect on the future health of affected mothers and their children. Therefore, dedicated diagnostic and follow-up programs are needed, for optimizing materno-foetal health and reducing the impact of pregnancy-related problems in the mothers and in the new generations. This narrative review, performed on the occasion of the 2018 World Kidney Day dedicated to women’s health, focuses on three aspects of the problem. Firstly, the risk of AKI in the hypertensive disorders of pregnancy (the risk is the highest in developing countries; however PE is the main cause of pregnancy related AKI worldwide). Secondly, the effect of AKI and the hypertensive disorders of pregnancy on the development of CKD in the mother and offspring: long-term risks are increased; the entity and the trajectories are still unknown. Thirdly, the role of CKD in the pathogenesis of AKI and the hypertensive disorders of pregnancy: CKD is a major risk factor and the most important element in the differential diagnosis; pregnancy is a precious occasion for early diagnosis of CKD. Higher awareness on the importance of AKI in pregnancy is needed to improve short and long term outcomes in mothers and children.

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Section: The Role Of Immunologic Diseases In the Pathogenesis Of Tmentioning

confidence: 99%

Acute Kidney Injury in Pregnancy: The Need for Higher Awareness. A Pragmatic Review Focused on What Could Be Improved in the Prevention and Care of Pregnancy-Related AKI, in the Year Dedicated to Women and Kidney Diseases

Piccoli

Zakharova

Attini

et al. 2018

JCM

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“…Initially, development of HUS can be detected through a decrease in platelet counts and AKI. Differentiation from other causes of AKI that may occur during pregnancy, including preeclampsia, eclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet) syndrome, and thrombotic thrombocytopenic purpura (TTP), is important (6). Other common causes of AKI, such as volume depletion or acute tubular necrosis, should also be considered.…”

Section: Discussionmentioning

confidence: 99%

Atypical hemolytic uremic syndrome after childbirth: a case report

Choi¹,

Yun²,

Kim³

et al. 2021

Ann Transl Med

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We report a case of atypical hemolytic uremic syndrome (HUS) that occurred after childbirth. A 33-year-old female was admitted to the emergency room, complaining of abdominal pain six days after giving birth to twins. The patient was diagnosed with hemoperitoneum due to hepatic hemangioma rupture and a left lateral hepatectomy was performed. Angioembolization was performed for the accompanying uterine artery bleeding. After that, her kidney function worsened after the 12th day postpartum. Microangiopathic anemia, thrombocytopenia and renal dysfunction were observed. Shiga toxin-producing Escherichia coli was negative in the stool. Plasma ADMATS 13 activity was normal. After transfer to the nephrology department with suspected atypical HUS, the patient underwent fresh frozen plasma (FFP) transfusion with three hemodialysis sessions. The patient improved without additional dialysis, but a renal biopsy was performed because of persistent proteinuria. Renal pathologic findings were compatible with thrombotic microangiopathy. A genetic test for atypical HUS revealed variants of uncertain significance in the complement factor H related (CFHR) 4 gene and the presence of CFHR3-CFHR1 copy number gain. The CFHR3-CFHR1 copy number gain found in this case is a rare causative mutation of atypical HUS. This case suggests that genetic testing of atypical HUS should include analysis of CFH-CFHR rearrangements as well as general screening for complement-associated genes.

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“…23 The advent of the humanized monoclonal anti-C5 antibody (eculizumab) has radically improved the prognosis of aHUS. 61 Its use appears to be safe for both mother and fetus in pregnancy, as documented in women with paroxysmal nocturnal hemoglobinuria 62 and aHUS (supplemental Table 2 23,24,55, ), even though the drug was detected in one-third of cord blood samples. 62 No controlled clinical trials have been performed with anti-C5 treatment in pregnancy-associated HUS; however, .35 patients who received anti-C5 treatment of HUS during pregnancy or postpartum are reported in the literature (supplemental Table 2).…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…Results of complement workup are not required for aHUS diagnosis and treatment (normal tests do not exclude the diagnosis of complement-mediated aHUS). ) the detection of a pathogenic complement variant retrospectively confirms the diagnosis of complement-mediated HUS, although, negative genetic tests do not rule out pregnancy-associated HUS,1,38,39 (c) genetics tests are helpful in the long-term for the decision of whether to discontinue anti-C5 treatment 55.…”

mentioning

confidence: 99%

Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group

Fakhouri

Scully

François

et al. 2020

Blood

104

149

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Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill-defined. This report by an international multidisciplinary (obstetricians, nephrologists, hematologists, intensivists, neonatologists and complement biologists) working group summarizes the current knowledge of these potentially severe disorders and proposes a practical clinical approach to diagnose and manage an episode of pregnancy-associated TMA. This approach takes into account the timing of TMA in pregnancy or postpartum, co-existing symptoms, first-line laboratory work-up and probability-based assessment of possible causes of pregnancy-associated TMA. Its aims are to urgently rule in or out thrombotic thrombocytopenic purpura (TTP) with ADAMTS13 activity testing, to consider alternative disorders with features of TMA (preeclampsia/eclampsia, Hemolysis Elevated Liver enzymes Low Platelets (HELLP) syndrome, antiphospholipid syndrome (APS)) or ultimately to diagnose complement-mediated atypical hemolytic uremic syndrome (aHUS) as a diagnosis of exclusion. Even though rare, it is paramount to diagnose TTP and aHUS associated with pregnancy and postpartum, as both require urgent specific treatment.

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Atypical Presentation of Pregnancy-Related Hemolytic Uremic Syndrome

Cited by 8 publications

References 22 publications

Acute Kidney Injury in Pregnancy: The Need for Higher Awareness. A Pragmatic Review Focused on What Could Be Improved in the Prevention and Care of Pregnancy-Related AKI, in the Year Dedicated to Women and Kidney Diseases

Acute Kidney Injury in Pregnancy: The Need for Higher Awareness. A Pragmatic Review Focused on What Could Be Improved in the Prevention and Care of Pregnancy-Related AKI, in the Year Dedicated to Women and Kidney Diseases

Atypical hemolytic uremic syndrome after childbirth: a case report

Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group

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