Anti-factor H antibody and its role in atypical hemolytic uremic syndrome
Atypical hemolytic uremic syndrome (aHUS) an important form of a thrombotic microangiopathy (TMA) that can frequently lead to acute kidney injury (AKI). An important subset of aHUS is the anti-factor H associated aHUS. This variant of aHUS can occur due to deletion of the complement factor H genes, CFHR1 and CFHR3, along with the presence of anti-factor H antibodies. However, it is a point of interest to note that not all patients with anti-factor H associated aHUS have a CFHR1/R3 deletion. Factor-H has a vital role in the regulation of the complement system, specifically the alternate pathway. Therefore, dysregulation of the complement system can lead to inflammatory or autoimmune diseases. Patients with this disease respond well to treatment with plasma exchange therapy along with Eculizumab and immunosuppressant therapy. Anti-factor H antibody associated aHUS has a certain genetic predilection therefore there is focus on further advancements in the diagnosis and management of this disease. In this article we discuss the baseline characteristics of patients with anti-factor H associated aHUS, their triggers, various treatment modalities and future perspectives.
Rupesh Raina
Guneive Mangat
Gordon Hong
Raghav Shah
Nikhil Nair
Brian Abboud
Sumedha Bagga
Sidharth Kumar Sethi
Front Immunol
. 2022 Aug 23;13:931210. doi: 10.3389/fimmu.2022.931210. eCollection 2022.
2022
English
The Pathway from Anatomy and Physiology to Diagnosis: A Developmental Perspective on Swallowing and Dysphagia
Dysphagia results from diverse and distinct etiologies. The pathway from anatomy and physiology to clinical diagnosis is complex and hierarchical. Our approach in this paper is to show the linkages from the underlying anatomy and physiology to the clinical presentation. In particular, the terms performance, function, behavior, and physiology are often used interchangeably, which we argue is an obstacle to clear discussion of mechanism of pathophysiology. We use examples from pediatric populations to highlight the importance of understanding anatomy and physiology to inform clinical practice. We first discuss the importance of understanding anatomy in the context of physiology and performance. We then use preterm infants and swallow-breathe coordination as examples to explicate the hierarchical nature of physiology and its impact on performance. We also highlight where the holes in our knowledge lie, with the ultimate endpoint of providing a framework that could enhance our ability to design interventions to help patients. Clarifying these terms, and the roles they play in the biology of dysphagia will help both the researchers studying the problems as well as the clinicians applying the results of those studies.
C J Mayerl
Dysphagia
. 2022 Apr 19;10.1007/s00455-022-10449-x. doi: 10.1007/s00455-022-10449-x. Online ahead of print.
2022
English