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      Atypical hemolytic uremic syndrome after childbirth: a case report

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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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          Author and article information

          Ann Transl Med
          Ann Transl Med
          Annals of Translational Medicine
          AME Publishing Company
          January 2021
          January 2021
          : 9
          : 1
          [1 ]Department of Internal Medicine, Chonnam National University Medical School , Gwangju, Korea;
          [2 ]Department of Laboratory Medicine and Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul, Korea;
          [3 ]Veterans Medical Research Institute, Veterans Health Service Medical Center , Seoul, Korea;
          [4 ]Department of Internal Medicine, School of Medicine, CHA University, School of Medicine, Seongnam, Korea;
          [5 ]Department of Pathology, Chonnam National University Medical School , Gwangju, Korea
          Author notes
          Correspondence to: Eun Hui Bae, MD, PhD. Department of Internal Medicine, Chonnam National University Medical School, 42 Jebongro, Gwangju 61469, Korea. Email: baedak76@ 123456gmail.com .

          ORCID: 0000-0003-1727-2822.

          PMC7859817 PMC7859817 7859817 atm-09-01-79
          2021 Annals of Translational Medicine. All rights reserved.
          Case Report


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