Abstract
Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease induced by many triggers, all of which produce a common end phenotype of microangiopathic hemolysis and thrombotic microangiopathy. We herein describe a 63-year-old woman with ongoing protein-losing enteropathy and frequent transudates caused by hypoalbuminemia. The patient was treated with eculizumab with a full hematologic and partial renal response. Protein-losing enteropathy is an inflammatory condition that has been linked with increased complement activation, which can trigger aHUS in patients with loss of CD55 expression. The patient in the present case had an increased estimated glomerular filtration rate but stage IV to V chronic kidney disease. One year later, she remains off dialysis with a stable estimated glomerular filtration rate. We herein report an unusual trigger of complement activation that in turn triggered aHUS in this patient.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 4027-4032 |
| Number of pages | 6 |
| Journal | Journal of International Medical Research |
| Volume | 47 |
| Issue number | 8 |
| DOIs | |
| State | Published - Aug 1 2019 |
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Keywords
- Atypical hemolytic uremic syndrome
- eculizumab
- hemolysis
- hypoalbuminemia
- protein-losing enteropathy
- thrombotic microangiopathy
ASJC Scopus subject areas
- Biochemistry
- Cell Biology
- Biochemistry, medical
Cite this
Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy. / Hanna, Ramy M.; Hasnain, Huma; Abdelnour, Lama; Yanny, Beshoy; Burwick, Richard.
In: Journal of International Medical Research, Vol. 47, No. 8, 01.08.2019, p. 4027-4032.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy
AU - Hanna, Ramy M.
AU - Hasnain, Huma
AU - Abdelnour, Lama
AU - Yanny, Beshoy
AU - Burwick, Richard
PY - 2019/8/1
Y1 - 2019/8/1
N2 - Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease induced by many triggers, all of which produce a common end phenotype of microangiopathic hemolysis and thrombotic microangiopathy. We herein describe a 63-year-old woman with ongoing protein-losing enteropathy and frequent transudates caused by hypoalbuminemia. The patient was treated with eculizumab with a full hematologic and partial renal response. Protein-losing enteropathy is an inflammatory condition that has been linked with increased complement activation, which can trigger aHUS in patients with loss of CD55 expression. The patient in the present case had an increased estimated glomerular filtration rate but stage IV to V chronic kidney disease. One year later, she remains off dialysis with a stable estimated glomerular filtration rate. We herein report an unusual trigger of complement activation that in turn triggered aHUS in this patient.
AB - Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease induced by many triggers, all of which produce a common end phenotype of microangiopathic hemolysis and thrombotic microangiopathy. We herein describe a 63-year-old woman with ongoing protein-losing enteropathy and frequent transudates caused by hypoalbuminemia. The patient was treated with eculizumab with a full hematologic and partial renal response. Protein-losing enteropathy is an inflammatory condition that has been linked with increased complement activation, which can trigger aHUS in patients with loss of CD55 expression. The patient in the present case had an increased estimated glomerular filtration rate but stage IV to V chronic kidney disease. One year later, she remains off dialysis with a stable estimated glomerular filtration rate. We herein report an unusual trigger of complement activation that in turn triggered aHUS in this patient.
KW - Atypical hemolytic uremic syndrome
KW - eculizumab
KW - hemolysis
KW - hypoalbuminemia
KW - protein-losing enteropathy
KW - thrombotic microangiopathy
UR - http://www.scopus.com/inward/record.url?scp=85071785509&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85071785509&partnerID=8YFLogxK
U2 - 10.1177/0300060519864808
DO - 10.1177/0300060519864808
M3 - Article
C2 - 31364428
AN - SCOPUS:85071785509
VL - 47
SP - 4027
EP - 4032
JO - Journal of International Medical Research
JF - Journal of International Medical Research
SN - 0300-0605
IS - 8
ER -