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- Kosuke Mochizuki, Naohiro Toda, Masaaki Fujita, Satoshi Kurahashi, Hisako Hirashima, Kazuki Yoshioka, Tomoya Kitagawa, Akira Ishii, and Toshiyuki Komiya.
- Department of Nephrology, Kansai Electric Power Hospital, Japan.
- Intern. Med. 2024 Oct 1; 63 (19): 265126542651-2654.
AbstractAtypical 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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