Publication date: Available online 27 February 2019
Source: The Journal of Emergency Medicine
Author(s): Sandy Chan, Adam R. Weinstein
Abstract
Background
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated disease manifesting in thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. It has a higher incidence of extrarenal manifestations, including central nervous system findings like seizure or stroke, pancreatitis, and cardiac manifestations.
Case Report
We present a case of an unimmunized 14-month-old girl presenting with generalized seizure and ultimately diagnosed with aHUS.
Why Should an Emergency Physician Be Aware of This?
These atypical neurological symptoms can cause the diagnosis to be commonly missed in the emergency department. The etiology of approximately 60% of patients with aHUS can be attributed to genetic mutations in complement regulators including factor H, membrane cofactor protein, factor I, activator factor B, or C3. Although previously treated with plasma transfusion and immunosuppressants, eculizumab is a newer treatment that has been changing prognosis and management of aHUS, but it should be administered within 48 h of symptom onset for best efficacy.
from Emergency Medicine via xlomafota13 on Inoreader https://ift.tt/2ECO9Dv
Δεν υπάρχουν σχόλια:
Δημοσίευση σχολίου