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The article presents a case study of a pediatric patient with hemolytic uremic syndrome (HUS) encephalopathy, where high b-value diffusion-weighted imaging (DWI) was crucial in identifying subtle thalamic lesions. Topics discussed include the utility of high b-value DWI in diagnosing HUS encephalopathy, the advantages and disadvantages of high b-value imaging, and the potential prognostic value of lesions detected only on high b-value DWI in pediatric encephalopathy.

The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

In 2011, a large outbreak of Shiga toxin-producing enterohemorrhagic Escherichia coli (EHEC) infections occurred in northern Germany, which mainly affected adults. Out of 3842 patients, 104 experienced a complicated course comprising hemolytic uremic syndrome and neurological complications, including cognitive impairment, aphasia, seizures and coma. T2 hyperintensities on magnet resonance imaging (MRI) bilateral in the thalami and in the dorsal pons were found suggestive of a metabolic toxic effect. Five of the 104 patients died because of toxic heart failure. In the present study, the post-mortem neuropathological findings of the five EHEC patients are described. Histological investigation of 13 brain regions (frontal, temporal, occipital cortex, corpora mammillaria, thalamus, frontal operculum, corona radiata, gyrus angularis, pons, medulla oblongata, cerebellar vermis and cerebellar hemisphere) showed no thrombosis, ischemic changes or fresh infarctions. Further, no changes were found in electron microscopy. In comparison with five age-matched controls, slightly increased activation of microglia and a higher neuronal expression of interleukin-1β and of Shiga toxin receptor CD77/globotriaosylceramide 3 was observed. The findings were confirmed by Western blot analyses. It is suggested that CD77/globotriaosylceramide upregulation may be a consequence to Shiga toxin exposure, whereas increased interleukin-1β expression may point to activation of inflammatory cascades. [ABSTRACT FROM AUTHOR]

The American Society for Apheresis (ASFA) JCA Special Issue Writing Committee is charged with reviewing, updating and categorizating indications for therapeutic apheresis. Beginning with the 2007 ASFA Special Issue (Fourth Edition), the committee has incorporated systematic review and evidence-based approach in the grading and categorization of indications. This Sixth Edition of the ASFA Special Issue has further improved the process of using evidence-based medicine in the recommendations by consistently applying the category and GRADE system definitions, but eliminating the 'level of evidence' criteria (from the University HealthCare Consortium) utilized in prior editions given redundancy between GRADE and University HealthCare Consortium systems. The general layout and concept of a fact sheet that was utilized in the Fourth and Fifth Editions, has been largely maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. This article consists of 78 fact sheets (increased from 2010) for therapeutic indications in ASFA categories I through IV, with many diseases categorized having multiple clinical presentations/situations which are individually graded and categorized. J. Clin. Apheresis 28:145-284, 2013. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

The classification of thrombotic microangiopathy has evolved and expanded due to treatment and mechanistic advances. The two basic clinical forms of thrombotic microangiopathy (excluding disseminated intravascular coagulation [DIC]), thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS) encompass a wide range of primary and secondary forms. The advent of plasma therapy and the identification of an inhibitor to ADAMTS13 in the idiopathic or acute forms of TTP and its absence in diarrheal HUS have had a major impact on our current classification of thrombotic microangiopathy. In adults, the difficulty of differentiating TTP, which is much more common than HUS and the need for a speedy diagnosis to provide life-saving plasma therapy has resulted in the term TTP/HUS for adult forms of thrombotic microangiopathy that present with unexplained thrombocytopenia and microangiopathic hemolytic anemia without a DIC. In this adult population a primary idiopathic and hereditary form as well as eight known secondary categories or clinical forms of TTP/HUS have been identified. HUS also embraces a primary (atypical HUS) and secondary forms (majority, diarrheal HUS secondary to Escherichia coli 0157:H7). In children, who present with HUS with no preceding history of diarrhea, plasma therapy is also offered on an urgent basis and studies are carried out to determine if they are suffering an abnormality in complement activation that may require eculizumab therapy. The advent of plasma therapy in the treatment of thrombotic microangiopathy has led to a clearer understanding of the role of ADAMTS13, both short- and long-term outcomes and the need for future surveillance and intervention. [ABSTRACT FROM AUTHOR]