Management of hemolytic-uremic syndrome in children

Abstract

Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%), this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there are no proven treatment options that can directly inactivate the toxin or effectively interfere with the cascade of destructive events triggered by the toxin once it gains access to the bloodstream and binds its receptor. However, HUS is self-limited, and effective supportive management during the acute phase is proven to be a life saver for children affected by HUS. A minority of childhood HUS cases, approximately 5%, are caused by various genetic mutations causing uncontrolled activation of the complement system. These children, who used to have a poor prognosis leading to end-stage renal disease, now have access to exciting new treatment options that can preserve kidney function and avoid disease recurrences. This review provides a summary of the current knowledge on the epidemiology, pathophysiology, and clinical presentation of childhood HUS, focusing on a practical approach to best management measures.

Síndrome urémico hemolítico atípico asociado a neumococo

“…En la literatura se describe que los serotipos ligados al SUH por neumococo son el 14, 6B, 9V, 19, 3, 8 y 23F (11) . Su patogénesis está ligada a los efectos de la enzima neuraminidasa (4,6,7) . Esta enzima remueve el ácido N-acetil hialurónico de la superficie de la membrana celular de los hematíes, plaquetas y glomérulos, exponiendo al antígeno de Thomsen-Friedenreich (Ag TF) (1,6,7) .…”

“…Su patogénesis está ligada a los efectos de la enzima neuraminidasa (4,6,7) . Esta enzima remueve el ácido N-acetil hialurónico de la superficie de la membrana celular de los hematíes, plaquetas y glomérulos, exponiendo al antígeno de Thomsen-Friedenreich (Ag TF) (1,6,7) . En el ser humano, habitualmente existen anticuerpos contra el Ag TF a partir de los 3 meses de vida, debido a que existiría probablemente, flora intestinal con antígenos similares al Ag TF (1) .…”

Változások az őssejt-transzplantációhoz társult thromboticus microangiopathia diagnosztikus kritériumrendszerében

“…Gyermekkorban a leggyakoribb a hasmenést követő HUS, amelyért a Shiga toxintermelő E. coli törzsek által okozott infekciók felelősek [6,8]. A TTP és a HUS primer okainak differenciáldiagnosztikájára már részletes, a komplementdiagnosztikára és a genetikai vizsgálatokra is épülő kritériumrendszereket dolgoztak ki [9,10].…”

Critical care in the emergency department: acute kidney injury

“…And a monoclonal antibody to C5a, eculizumab, is recommended in recent NICE guidance 24. Intravenous volume resuscitation and correction of electrolyte abnormalities and anaemia are central to initial management, while urgent plasma exchange or RRT may be necessary in 50–70% of patients 25. Most patients with STEC-HUS recover renal function, although a minority may develop CKD, requiring lifelong dialysis.…”