scholarly journals Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection

2020 ◽  
Vol 2020 ◽  
pp. 1-4 ◽  
Author(s):  
Eigo Ikushima ◽  
Manabu Hisahara ◽  
Takuya Nishijima ◽  
Hikaru Uchiyama ◽  
Tatsushi Onzuka ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Type A ◽  
Acute Type ◽  
Microangiopathic Hemolytic Anemia ◽  
Type A Aortic Dissection ◽  
Uremic Syndrome

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)-related disease that manifests as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused by uncontrolled activation of the complement system. We report the case of a 61-year-old woman with acute type A aortic dissection that subsequently developed into aHUS. The hematologic disorders underlying aHUS improved after treatment with the complement inhibitor eculizumab. It is important to consider aHUS when a patient clinically develops a triad of microangiopathic hemolytic anemia, thrombocytopenia, and an increasing creatinine level following cardiovascular surgery.

scholarly journals Life-Threatening Extrarenal Manifestations in an Infant with Atypical Hemolytic Uremic Syndrome Caused by a Complement 3-Gene Mutation

2019 ◽  
Vol 44 (5) ◽  
pp. 1300-1305
Author(s):  
Sa Ra Han ◽  
Myung Hyun Cho ◽  
Jin Soo Moon ◽  
Il Soo Ha ◽  
Hae Il Cheong ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Hemolytic Uremic Syndrome ◽  
Gene Mutation ◽  
Hemolytic Anemia ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Pulmonary Hemorrhage ◽  
Microangiopathic Hemolytic Anemia ◽  
Life Threatening ◽  
Uremic Syndrome ◽  
Extrarenal Manifestations

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment caused by uncontrolled activation of the complement system. About 20% of patients show extrarenal manifestations, with central nervous system involvement being the most frequent. We described the clinical course and management of aHUS in an infant, that was caused by a complement 3 (C3) gene mutation with severe extrarenal manifestations. Case Presentation: A 4-month-old girl visited our hospital for jaundice and petechiae. Laboratory tests revealed microangiopathic hemolytic anemia, thrombocytopenia, and hyperazotemia. She was diagnosed with aHUS with a C3 p.E1160K mutation. Daily fresh-frozen plasma (FFP) therapy was administered; however, she experienced the severe extrarenal manifestations of pulmonary hemorrhage and gastrointestinal bleeding. With aggressive treatment, supportive care, and daily FFP transfusion, the patient recovered and was discharged after 72 days of hospital stay, on a regular FFP transfusion. Four months after diagnosis, she was switched to eculizumab treatment. Twenty months have passed since then and she has been relapse-free until now. Conclusion: aHUS is rare but has a devastating course if not properly treated. Severe extrarenal manifestations, such as pulmonary hemorrhage and gastrointestinal bleeding, can develop in aHUS caused by a C3 mutation. In our case, long-term management with eculizumab resulted in relapse-free survival.

scholarly journals Can Renal Resistive Index Predict Acute Kidney Injury After Acute Type A Aortic Dissection Repair?

2017 ◽  
Vol 104 (5) ◽  
pp. 1583-1589 ◽  
Author(s):  
Hai-Bo Wu ◽  
Huai Qin ◽  
Wei-Guo Ma ◽  
Hong-Lei Zhao ◽  
Jun Zheng ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Aortic Dissection ◽  
Resistive Index ◽  
Kidney Injury ◽  
Type A ◽  
Renal Resistive Index ◽  
Acute Type ◽  
Type A Aortic Dissection

scholarly journals Preoperative renal malperfusion is an independent predictor for acute kidney injury and operative death but not associated with late mortality after surgery for acute type A aortic dissection

2020 ◽  
Vol 58 (2) ◽  
pp. 302-308
Author(s):  
Kosaku Nishigawa ◽  
Toshihiro Fukui ◽  
Kohei Uemura ◽  
Shuichiro Takanashi ◽  
Tomoki Shimokawa
Keyword(s):  
Acute Kidney Injury ◽  
Aortic Dissection ◽  
Odds Ratio ◽  
Independent Predictor ◽  
Kidney Injury ◽  
Type A ◽  
Acute Type ◽  
Operative Death ◽  
Type A Aortic Dissection ◽  
Late Mortality

Abstract OBJECTIVES This study was aimed to investigate the impact of preoperative renal malperfusion on early and late outcomes after surgery for acute type A aortic dissection (AAAD). METHODS Of 915 patients who underwent surgery for AAAD between September 2004 and September 2017, we enrolled 534 patients whose preoperative enhanced computed tomography images were retrospectively available in this study. Exclusion criteria were single kidney (n = 3) and dialysis-dependent preoperatively (n = 12). We compared early and late outcomes between patients who had preoperative renal malperfusion (n = 64) and those who did not have renal malperfusion (n = 470). RESULTS The incidence of postoperative acute kidney injury, defined using the Kidney Disease: Improving Global Outcomes criteria, was higher in the renal malperfusion group than in the no renal malperfusion group (76.6% vs 39.4%; P < 0.001). Similarly, operative death was more frequently seen in the renal malperfusion group (12.5% vs 3.8%; P = 0.003). Multivariate analyses showed that renal malperfusion was the independent predictor for postoperative acute kidney injury [odds ratio 4.32, 95% confidence interval (CI) 2.25–8.67; P < 0.001] and operative death (odds ratio 3.08, 95% CI 1.02–8.86; P = 0.046). The median follow-up period in the hospital survivors was 3.3 years (interquartile range 2.1–6.7 years). The cumulative survival rate at 8 years was similar between the groups (74.6% in the renal malperfusion group and 76.0% in the no renal malperfusion group; P = 0.349). CONCLUSIONS Preoperative renal malperfusion is an independent predictor for postoperative acute kidney injury and operative death but not associated with late mortality after surgery for acute type A aortic dissection.

scholarly journals Atypical Hemolytic Uremic Syndrome After Traumatic Rectal Injury: A Case Report

2020 ◽  
Author(s):  
Yunchul Park ◽  
Ji-Hyoun Kang
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Urine Output ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Traumatic Injury ◽  
Intermittent Hemodialysis ◽  
Microangiopathic Hemolytic Anemia ◽  
Uremic Syndrome

Abstract Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, life-threatening condition of thrombotic microangiopathy (TMA) that is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and renal impairment. The mechanisms of aHUS are still unknown. To our knowledge, there is currently no report establishing aHUS after traumatic injury. We herein present a case of aHUS after traumatic injury.Case presentation: A 55-year-old man with a medical history of percutaneous coronary intervention due to ST elevation myocardial infarction visited the emergency room after a traumatic injury caused by a tree limb. Abdominal computed tomography revealed a rectal wall defect with significant air density in the perirectal space and preperitoneum, implying rectal perforation and, therefore, an emergency operation was performed. As there was an absence of intraperitoneal intestinal perforation, we performed diverting sigmoid loop colostomy. An additional intermittent simple repair was performed due to perianal and anal injuries. One day after the operation, his urine output abruptly decreased and serum creatinine level increased. In addition, his platelet level decreased, and a spiking fever occurred after 2 days. The patient was diagnosed with acute renal failure secondary to aHUS and was treated with fresh frozen plasma replacement. Continuous renal replacement therapy (CRRT) was also started for oliguria and uremic symptoms. The patient received CRRT for 3 days and intermittent hemodialysis thereafter. He received four weekly sessions of hemodialysis for 2 weeks. After hemodialysis and subsequent supportive treatment, his urine output and renal function continuously improved. Similarly, his hemolytic anemia and thrombocytopenia also gradually improved. His dialysis was terminated on day 22 of admission and he was discharged after recovery. His improved state has since been maintained.Conclusions: This case suggests that a traumatic event can be a triggering factor for aHUS, and it should be considered in patients who have thrombocytopenia and acute renal failure with microangiopathic hemolytic anemia after trauma. Early diagnosis and appropriate management can be critical to achieve favorable outcomes in posttraumatic patients with aHUS.

scholarly journals Hemolytic Anemia Caused by a Kinked Graft after Ascending Aortic Replacement for Acute Type A Aortic Dissection

2021 ◽  
Vol 50 (4) ◽  
pp. 287-290
Author(s):  
Hiroki Moriuchi ◽  
Naoki Washiyama ◽  
Yuko Ohashi ◽  
Kazumasa Tsuda ◽  
Daisuke Takahashi ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Hemolytic Anemia ◽  
Type A ◽  
Acute Type ◽  
Type A Aortic Dissection ◽  
Aortic Replacement ◽  
Ascending Aortic Replacement

scholarly journals Hemolytic Uremic Syndrome Caused by Mycoplasma Pneumoniae Infection in Children: A Case Report and Literature Review

2021 ◽  
Author(s):  
Sirinthip Kittivisuit ◽  
Prayong Vachvanichsanong ◽  
Thirachit Chotsampancharoen
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Mycoplasma Pneumoniae ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Dialysis ◽  
Microangiopathic Hemolytic Anemia ◽  
Uremic Syndrome ◽  
Red Cell Transfusion ◽  
Stage Renal Disease ◽  
End Stage

We describe the case of a 6-year-old boy with a Mycoplasma pneumoniae (M. pneumoniae) respiratory tract infection associated with thrombotic microangiopathic hemolytic anemia and thrombocytopenia with renal failure which was diagnosed as atypical hemolytic uremic syndrome. Renal biopsy showed features of thrombotic microangiopathy. The patient was treated with azithromycin for the M. pneumoniae infection, and supportive care with red cell transfusion and renal dialysis in the acute period. The microangiopathic hemolytic anemia and thrombocytopenia resolved within 2 months after diagnosis but the renal function damage was irreversible. The patient developed end-stage renal disease and required long term renal replacement therapy.

scholarly journals Atypical hemolytic uremic syndrome: a brief review

2017 ◽  
Vol 9 (2) ◽  
Author(s):  
Kuixing Zhang ◽  
Yuxin Lu ◽  
Kevin T. Harley ◽  
Minh-Ha Tran
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Regulatory Gene ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Regulatory Mechanisms ◽  
Complement Inhibitor ◽  
Loss Of Function ◽  
Microangiopathic Hemolytic Anemia ◽  
Uremic Syndrome ◽  
Glomerular Capillaries

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (<em>CFH, CFI, CD46</em> or <em>THBD</em>) or gain-of-function in an effector gene (<em>CFB</em> or <em>C3</em>). Treatment with the late complement inhibitor, eculizumab – a monoclonal antibody directed against C5 – is effective.

scholarly journals Continued Eculizumab Therapy for Persistent Atypical Hemolytic Uremic Syndrome

2013 ◽  
Vol 6 (1) ◽  
pp. 46-48 ◽  
Author(s):  
Arif Asif ◽  
Syed S. Haqqie ◽  
Ketan Ghate ◽  
Roy O Mathew ◽  
Tushar Vachharajani ◽  
...  
Keyword(s):  
Renal Function ◽  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Marked Improvement ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Microangiopathic Hemolytic Anemia ◽  
Atypical Hus ◽  
Neutralizing Monoclonal Antibody ◽  
Uremic Syndrome

Atypical hemolytic uremic syndrome (atypical HUS) is characterized by endothelial injury and microvascular thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia, and ischemic injury to organs, especially the kidney. Activation of complements is involved in the pathogenesis of atypical HUS. Eculizumab, a neutralizing monoclonal antibody directed against complement C5, has shown salutary effects in patients with atypical HUS. In this report, we present a 23-year-old man with atypical HUSwho was treated with eculizumab. During the first four weeks of treatment, eculizumab failed to achieve a remission. Microangiopathic hemolytic anemia and thrombocytopenia persisted, while renal function deteriorated necessitating initiation of hemodialysis. Continuation of eculizumab therapy, however, led to marked improvement in hemolytic anemia, thrombocytopenia, and renal function. After 10 weeks of eculizumab therapy, hemodialysis was discontinued. At 5-month follow-up, serum creatinine was 1.1 mg/dL with continued eculizumab therapy every other week. In addition, platelet count was normal, while there was no evidence of hemolysis. We conclude that in patients with persistent atypical HUS continued treatment with eculizumab can be helpful in achieving remission.

scholarly journals Significance of preoperative acute kidney injury in patients with acute type A aortic dissection

2019 ◽  
Vol 118 (4) ◽  
pp. 815-820 ◽  
Author(s):  
Tsu-Ming Chien ◽  
Hao Wen ◽  
Jiann-Woei Huang ◽  
Chong-Chao Hsieh ◽  
Huai-Min Chen ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Aortic Dissection ◽  
Kidney Injury ◽  
Type A ◽  
Acute Type ◽  
Type A Aortic Dissection
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