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 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 Acute Progression of Adult-Onset Atypical Hemolytic-Uremic Syndrome due to CFH Mutation: A Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Bartlomiej Posnik ◽  
Dorota Sikorska ◽  
Krzysztof Hoppe ◽  
Krzysztof Schwermer ◽  
Krzysztof Pawlaczyk ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Late Onset ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Genetic Mutation ◽  
Current Data ◽  
Factor H ◽  
Rapid Progression ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Atypical hemolytic-uremic syndrome (aHUS), unlike typical HUS, is not due to bacteria but rather to an idiopathic or genetic cause that promotes dysregulation of the alternative complement pathway. It leads to hemolytic anemia, thrombocytopenia, and renal impairment. Although aHUS secondary to a genetic mutation is relatively rare, when occurring due to a mutation in Factor H (CFH), it usually presents with younger onset and has a more severe course, which in the majority ends with end-stage renal failure. Paradoxically to most available data, our case features acute aHUS due to a CFH mutation with late onset (38-year-old) and rapid progression to end-stage renal disease. Due to current data indicating a high risk of graft failure in such patients, the diagnosis of aHUS secondary to a genetic cause has disqualified our patient from a living (family) donor renal transplantation and left her with no other option but to begin permanent renal replacement therapy.

scholarly journals Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab

2020 ◽  
Vol 95 (2) ◽  
pp. 124-128
Author(s):  
Jung Hyun Kim ◽  
Won Kyung Han ◽  
Yu Bum Choi ◽  
Hyung Jong Kim ◽  
Jisu Oh ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
End Stage Renal Disease ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Delayed Diagnosis ◽  
Membrane Attack Complex ◽  
Acute Renal Injury ◽  
Humanized Monoclonal Antibody ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury, which results from uncontrolled complement activation. Delayed diagnosis and treatment of aHUS may result in end-stage renal disease (ESRD) and an associated dependence on dialysis. In extreme cases, it may cause death due to multi-organ failure. Eculizumab, a humanized monoclonal antibody against C5, inhibits the formation of the terminal membrane attack complex and is used to treat aHUS. Here, we report a 46-year-old male patient who suffered from aHUS relapse, despite prior treatment with repeated plasma exchange and hemodialysis. Eculizumab therapy improved his hematologic findings without use of hemodialysis.

scholarly journals Pregnancy-triggered atypical hemolytic uremic syndrome (aHUS): a Global aHUS Registry analysis

2021 ◽  
Author(s):  
Fadi Fakhouri ◽  
Marie Scully ◽  
Gianluigi Ardissino ◽  
Imad Al-Dakkak ◽  
Benjamin Miller ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Clinical Characteristics ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Complement Factor ◽  
Childbearing Age ◽  
History Of ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background Atypical hemolytic uremic syndrome (aHUS) is a rare disease in which uncontrolled terminal complement activation leads to systemic thrombotic microangiopathy (TMA). Pregnancy can trigger aHUS and, without complement inhibition, many women with pregnancy-triggered aHUS (p-aHUS) progress to end-stage renal disease (ESRD) with a high risk of morbidity. Owing to relatively small patient numbers, published characterizations of p-aHUS have been limited, thus the Global aHUS Registry (NCT01522183, April 2012) provides a unique opportunity to analyze data from a large single cohort of women with p-aHUS. Methods The demographics and clinical characteristics of women with p-aHUS (n = 51) were compared with those of women of childbearing age with aHUS and no identified trigger (non-p-aHUS, n = 397). Outcome evaluations, including renal survival according to time to ESRD, were compared for patients with and without eculizumab treatment (a complement C5 inhibitor) in both aHUS groups. Results Baseline demographics and clinical characteristics were broadly similar in both groups. The proportion of women with p-aHUS and non-p-aHUS with pathogenic variant(s) in complement genes and/or anti-complement factor H antibodies was similar (45% and 43%, respectively), as was the proportion with a family history of aHUS (12% and 13%, respectively). Eculizumab treatment led to significantly improved renal outcomes in women with aHUS, regardless of whether aHUS was triggered by pregnancy or not: adjusted hazard ratio for time to ESRD was 0.06 (p = 0.006) in the p-aHUS group and 0.20 (p < 0.0001) in the non-p-aHUS group. Conclusion Findings from this study support the characterization of p-aHUS as a complement-mediated TMA. Graphic abstract

scholarly journals Preemptive Use of Eculizumab for Living-Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

2016 ◽  
Vol 30 (1&2) ◽  
pp. 22 ◽  
Author(s):  
Oleh Akchurin ◽  
Samriti Dogra ◽  
Frederick Kaskel ◽  
Dominique Jan ◽  
Stuart Greenstein ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Complement Factor ◽  
Pediatric Kidney Transplantation ◽  
Uremic Syndrome ◽  
Few Data ◽  
Stage Renal Disease ◽  
End Stage

Eculizumab is an anti-complement C5 monoclonal antibody that has recently been reported as an effective therapy for atypical hemolytic uremic syndrome. However, few data are available on the preemptive use of this medication in pediatric kidney transplantation. This report describes a successful preemptive use of eculizumab in combination with living unrelated kidney transplanta- tion in a 10-year-old child with end-stage renal disease secondary to atypical hemolytic uremic syndrome who has a complement factor H mutation that has not been previously reported. Further observations and clinical trials are required to address the challenges and areas of uncertainty related to preemptive eculizumab therapy for kidney transplantation in children and adults with atypical hemolytic uremic syndrome. 

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 Atypical hemolytic uremic syndrome: A report of two cases

2016 ◽  
Vol 9 (1) ◽  
pp. 75
Author(s):  
Md. Habibur Rahman ◽  
Morsheda Akhter ◽  
Syed Symul Haque
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Course Of Illness ◽  
Atypical Hus ◽  
Excellent Outcome ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
Insight Into

Hemolytic uremic syndrome (HUS) is one of the important cause of acute kidney injury in children. There is excellent outcome in patients with typical HUS but atypical HUS is associated with high mortality, risk of recurrence and may lead to end stage renal disease. We report two cases of 5 and 6 year old child having clinical &amp; laboratory characteristics of atypical HUS. These children had a fulminant course of illness with complications involving various systems. The report provides an insight into the etio pathogenesis, diagnoses and treatment of this condition.

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.

ATYPICAL HEMOLYTIC UREMIC SYNDROME IN CHILDREN: PRACTICAL ASPECTS OF DIFFERENTIAL DIAGNOSTICS AND TREATMENT

2021 ◽  
Vol 100 (4) ◽  
pp. 64-73
Author(s):  
S.V. Baiko ◽  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Alternative Pathway ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Differential Diagnostics ◽  
E Coli ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
The Complement System ◽  
Terminal Complement

Atypical hemolytic uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA) that develops as a result of uncontrolled activation of the alternative pathway of the complement system. In the case of late diagnosis and inadequate treatment, aHUS has an unfavorable outcome with death rates as high as 25% during the acute phase and up to 50% of cases progressing to end-stage renal disease. Clinically, aHUS is very similar to disseminated intravascular coagulation, other TMAs: HUS associated with enterohemorrhagic E. coli, thrombotic thrombocytopenic purpura, etc. The article presents the sequence and scope of studies for the differential diagnosis of aHUS. Eculizumab and its biosimilars, blocking the terminal complement complex, have changed the future of patients with aHUS, so timely diagnosis and early treatment are crucial in the outcome of the disease.

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