Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Atypical Hemolytic Uremic Syndrome Triggered by Acute Pancreatitis in a Patient with a Membrane Cofactor Protein (CD46) Genetic Variant
Kosuke Mochizuki, Naohiro Toda, Masaaki Fujita, Satoshi Kurahashi, Hisako Hirashima, Kazuki Yoshioka, Tomoya Kitagawa, Akira Ishii, Toshiyuki Komiya
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Keywords: atypical HUS, MCP variant, multiple hit theory, pancreatitis
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2024 Volume 63 Issue 19 Pages 2651-2654

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Abstract

Atypical hemolytic uremic syndrome (aHUS) is a type of HUS. We herein report a case of aHUS triggered by pancreatitis in a patient with a heterozygous variant of membrane cofactor protein (MCP; P165S), a complement-related gene. Plasma exchange therapy and hemodialysis improved thrombocytopenia and anemia without leading to end-stage kidney disease. This MCP heterozygous variant was insufficient to cause aHUS on its own. Pancreatitis, in addition to a genetic background with a MCP heterozygous variant, led to the manifestation of aHUS. This case supports the "multiple hit theory" that several factors are required for the manifestation of aHUS.

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© 2024 by The Japanese Society of Internal Medicine

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