Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection

Ikushima, Eigo; Hisahara, Manabu; Nishijima, T.; Uchiyama, Hikaru; Onzuka, Tatsushi; Ochiai, Yoshie; Muta, Tsuyoshi; Tokunaga, Shigehiko

doi:10.1155/2020/2467953

Cited by 4 publications

(3 citation statements)

References 11 publications

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“…Device-related TMA as a secondary TMA could be difficult to diagnose if device implementation was performed years ago. Previous reports have demonstrated that cardiac valve-related TMA can appear soon after cardiac valve surgery [4,5], where it was diagnosed by excluding other TMA possibilities [6]. Here, we report the case of an older woman with a chief complaint of dyspnea and the pathophysiology of secondary TMA.…”

Section: Introductionmentioning

confidence: 85%

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Device-Related Thrombotic Microangiopathy in an Elderly Patient With a History of Aortic Surgery

Tanaka¹,

Naito²,

Suehiro³

et al. 2022

Cureus

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Thrombotic microangiopathy (TMA) is caused by several diseases, including infections, congenital and autoimmune diseases, and malignancies, usually requiring admission to intensive care. The primary pathophysiology of TMA is microvascular thrombosis, and its diagnosis is based on the presence of hemolysis, thrombocytopenia, schistocytes in a blood smear, and organ damage. Among secondary TMAs, device-related TMA could be difficult to diagnose if device implementation was performed years ago. We report the case of an 87-year-old woman with a chief complaint of dyspnea diagnosed with device-related TMA. In device-related TMA, thrombogenesis/thrombocytopenia is triggered by hemolysis/fragmented red blood cells. However, in other TMAs, thrombogenesis or thrombocytopenia is preceded by hemolysis and the presence of fragmented red blood cells. Thus, rapid plasma exchange is necessary to address TMA pathogenesis. TMA can be managed in a community hospital if the facility has access to plasma exchange. It is possible to treat complex TMAs even in community hospitals by carefully considering their pathophysiology. Additionally, improving the quality of general practice in community hospitals will allow for more effective diagnosis and treatment of TMAs.

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Section: Introductionmentioning

confidence: 85%

“…Although it is relatively easy to differentiate between STEC-HUS and TTP, the differential diagnosis of other TMAs is often difficult; all of these diseases are diagnosed as aHUS [5,9]. It is now widely recognized that aHUS diseases are complement-associated TMA [9].…”

Section: Discussionmentioning

confidence: 99%

Device-Related Thrombotic Microangiopathy in an Elderly Patient With a History of Aortic Surgery

Tanaka¹,

Naito²,

Suehiro³

et al. 2022

Cureus

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“…Various studies concerning surgical interventions not related to transplantation have reported the development of TMA in the postoperative setting procedures [ 9 ]. Three case reports describe the new onset of aHUS as a differential diagnosis to AKI following cardiovascular procedures [ 10 , 11 , 12 , 13 ]. In a short period of time, several cases of TMA after a cardiovascular surgery were diagnosed at our institution.…”

Section: Introductionmentioning

confidence: 99%

Severe Acute Kidney Injury in Cardiovascular Surgery: Thrombotic Microangiopathy as a Differential Diagnosis to Ischemia Reperfusion Injury. A Retrospective Study

Grigorescu

Kamla

Wassilowsky

et al. 2020

JCM

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Background: Acute kidney injury (AKI) after cardiovascular surgery (CVS) infers high morbidity and mortality and may be caused by thrombotic microangiopathy (TMA). This study aimed to assess incidence, risk factors, kidney function, and mortality of patients with a postoperative TMA as possible cause of severe AKI following cardiovascular surgery. Methods: We analyzed retrospectively all patients admitted to the ICU after a cardiovascular procedure between 01/2018 and 03/2019 with severe AKI and need for renal replacement therapy (RRT). TMA was defined as post-surgery-AKI including need for RRT, hemolytic anemia, and thrombocytopenia. TMA patients were compared to patients with AKI requiring RRT without TMA. Results: Out of 893 patients, 69 (7.7%) needed RRT within one week after surgery due to severe AKI. Among those, 15 (21.7%) fulfilled TMA criteria. Aortic surgery suggested an increased risk for TMA (9/15 (60.0%) vs. 7/54 (31.5%), OR 3.26, CI 1.0013-10.64). Ten TMA patients required plasmapheresis and/or eculizumab, and five recovered spontaneously. Preoperative kidney function was significantly better in TMA patients than in controls (eGFR 92 vs. 60.5 mL/min, p = 0.004). However, postoperative TMA resulted in a more pronounced GFR loss (ΔeGFR −54 vs. −17 mL/min, p = 0.062). There were no deaths in the TMA group. Conclusion: Our findings suggest TMA as an important differential diagnosis of severe AKI following cardiovascular surgery, which may be triggered by aortic surgery. Therefore, early diagnosis and timely treatment of TMA could reduce kidney damage and improve mortality of AKI following cardiovascular surgery, which should be further investigated.

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Thrombotic microangiopathy following aortic surgery with hypothermic circulatory arrest: a single-centre experience of an underestimated cause of acute renal failure

Kamla

Grigorescu-Vlass

Wassilowsky

et al. 2021

Interactive CardioVascular and Thoracic Surgery

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OBJECTIVES Acute kidney injury (AKI) following surgery involving the heart-lung-machine is associated with high mortality and morbidity. In addition to the known mechanisms, thrombotic microangiopathy (TMA) triggered by the dysregulation of complement activation was recently described as another pathophysiological pathway for AKI following aortic surgery. The aim of this retrospective study was to analyse incidence, predictors and outcome in these patients. METHODS Between January 2018 and September 2019, consecutive patients undergoing aortic surgery requiring hypothermic circulatory arrest were retrospectively reviewed. If suspected, diagnostic algorithm was initiated to identify a TMA and its risk factors, and postoperative outcome parameters were comparably investigated. RESULTS The incidence of TMA in the analysed cohort (n = 247) was 4.5%. Multivariable logistic regression indicated female gender {odds ratio (OR) 4.905 [95% confidence interval (CI) 1.234–19.495], P = 0.024} and aortic valve replacement [OR 8.886 (95% CI 1.030–76.660), P = 0.047] as independent predictors of TMA, while cardiopulmonary bypass, X-clamp and hypothermic circulatory arrest times showed no statistically significance. TMA resulted in postoperative AKI (82%), neurological disorders (73%) and thrombocytopaenia [31 (interquartile range 25–42) G/l], corresponding to the diagnostic criteria. Operative mortality and morbidity were equal to patients without postoperative TMA, despite a higher incidence of re-exploration for bleeding (27 vs 6%; P = 0.027). After 6 months, survival, laboratory parameters and need for dialysis were comparable between the groups. CONCLUSIONS TMA is a potential differential diagnosis for the cause of AKI following aortic surgery regardless of the hypothermic circulatory arrest time. Timely diagnosis and appropriate treatment resulted in a comparable outcome concerning mortality and renal function.

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Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection

Cited by 4 publications

References 11 publications

Device-Related Thrombotic Microangiopathy in an Elderly Patient With a History of Aortic Surgery

Device-Related Thrombotic Microangiopathy in an Elderly Patient With a History of Aortic Surgery

Severe Acute Kidney Injury in Cardiovascular Surgery: Thrombotic Microangiopathy as a Differential Diagnosis to Ischemia Reperfusion Injury. A Retrospective Study

Thrombotic microangiopathy following aortic surgery with hypothermic circulatory arrest: a single-centre experience of an underestimated cause of acute renal failure

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