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Similar Publications

Severe pregnancy-associated atypical hemolytic uremia syndrome in the context of the COVID-19 pandemic: a novel survival case report.

BMC Pregnancy Childbirth

January 2025

Department of Intensive Care Medicine, Army Medical Center of PLA, No. 10 Changjiang Road, Yuzhong District, Chongqing, 400010, People's Republic of China.

Background: Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy (TMA) caused by uncontrolled activation of the complement system during pregnancy or the postpartum period. In the intensive care unit, aHUS must be differentiated from sepsis-related multiple organ dysfunction, thrombotic thrombocytopenic purpura (TTP), hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. Early recognition of aHUS is critical for effective treatment and improved prognosis.

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Updates in atypical hemolytic syndrome.

Curr Opin Pediatr

January 2025

Division of Pediatric Nephrology, The University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Purpose Of Review: This review aims to summarize how scientific advances in complement biology have not only improved the diagnosis and management of aHUS but also continue to offer insights into the pathophysiology of complement-mediated disease that may be leveraged for future therapeutic developments.

Recent Findings: Updated information on the clinical and epidemiological features, pathophysiology, diagnosis, management, and potential for future therapeutic advancements in the treatment of aHUS are reviewed.

Summary: aHUS is a rare but potentially life-threatening disease that requires prompt diagnosis and treatment as well as long-term management via a multidisciplinary team providing coordination of primary and specialty care as well as outreach and education for children and families affected by this life-long disease.

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Atypical hemolytic uremic syndrome (aHUS) is an important differential diagnosis in thrombotic microangiopathy (TMA). The absence of definitive biomarkers usually allows for aHUS to be diagnosed only through a process of exclusion. Due to the unfavorable prognosis if adequate therapy is delayed or not provided, differential diagnostic considerations and initiation of treatment must occur promptly.

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Thrombotic microangiopathy (TMA) is a severe condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage, often involving the kidneys. Complement-mediated hemolytic uremic syndrome (cHUS), a rare form of TMA, arises from dysregulated alternative complement pathway activation, frequently due to genetic mutations. We report the case of a 23-year-old male presenting with TMA secondary to a heterozygous mutation in the membrane cofactor protein (MCP/CD46) gene.

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A simple, fast, sample-saving, and sensitive liquid chromatography-tandem mass spectrometry method was established with a linear range adjusted by in-source collision-induced dissociation. Notably, this could simultaneously determine busulfan, fludarabine, phenytoin, and posaconazole in plasma from children, each having unique physical and chemical properties. The procedure necessitated only 20 μL of plasma and involved a simple protein precipitation process.

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