A Case of Atypical Hemolytic Uremic Syndrome Triggered by Acute Pancreatitis in a Patient with a Membrane Cofactor Protein (CD46) Genetic Variant
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- Mochizuki Kosuke
- Department of Nephrology, Kansai Electric Power Hospital, Japan
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- Toda Naohiro
- Department of Nephrology, Kansai Electric Power Hospital, Japan
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- Fujita Masaaki
- Department of Rheumatology, Kansai Electric Power Hospital, Japan
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- Kurahashi Satoshi
- Department of Nephrology, Kansai Electric Power Hospital, Japan
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- Hirashima Hisako
- Department of Nephrology, Kansai Electric Power Hospital, Japan
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- Yoshioka Kazuki
- Department of Hematology, Kansai Electric Power Hospital, Japan
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- Kitagawa Tomoya
- Department of Hematology, Kansai Electric Power Hospital, Japan
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- Ishii Akira
- Department of Nephrology, Kansai Electric Power Hospital, Japan
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- Komiya Toshiyuki
- Department of Nephrology, Kansai Electric Power Hospital, Japan
Description
<p>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. </p>
Journal
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- Internal Medicine
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Internal Medicine advpub (0), 2024
The Japanese Society of Internal Medicine