Pediatric KT in children up to 15 kg: A single-center experience.

BACKGROUND: KT is the preferred treatment for ESRD in pediatrics. However, it may be challenging in those weighing ≤15 kg with potential complications that impact on morbidity and graft loss. METHODS: This retrospective review reports our experience in KT in children, weighing ≤15 kg, and the strategies to reduce morbidity and mortality. RESULTS: All patients were on RRT prior to KT. Patients reached ESRD mainly due to urologic malformations (54.54%). LD was performed in 82% of patients. The recipient's median age was 2.83 years, and median weight 12.280 kg. Male sex was predominant (73%). All patients required transfusions of PRBCs. There was a high requirement for ventilated support in patients post-KT with no relation to weight, amount of resuscitation used intra-operatively or ml/kg of PRBCs. One patient presented with stenosis of the native renal artery. No patients presented DGF, graft thrombosis, or surgical complications. No association was found between cold ischemia and eGFR at 1 year (p = .12). In univariate analysis, eGFR at 1 year is related to AR. eGFR at 3 years is related to the number of UTI. Median follow-up was 1363 days. Patient and graft survival were 100%. CONCLUSIONS: KT in children ≤15 kg can be challenging and requires a meticulous perioperative management and surgical expertise. Patient and graft survival are excellent with low rate of complications.

Uso De Eculizumab En Síndrome Urémico Hemolítico Típico: Una Opción Terapéutica En El Compromiso Neurológico Severo. Reporte De Dos Casos / Use Of Eculizumab In Typical Hemolytic Uremic Syndrome: A Therapeutic Option In Severe Neurological Compromise. Two Cases Report

Resumen El síndrome urémico hemolítico típico es una enfermedad endémica en América Latina. Argentina es uno de los países con más casos reportados, con una tasa de diez casos cada 100.000 menores de cinco años. Es la primera causa de insuficiencia renal aguda, y responsable del 9 % de los trasplantes renales. Esta patología se caracteriza por una tríada clásica: anemia microangiopática, trombocitopenia e insuficiencia renal aguda. El principal agente etiológico del Síndrome Urémico Hemolítico es la bacteria Escherichia coli, productora de la toxina Shiga. El Síndrome Urémico Hemolítico tiene una mortalidad aguda inferior al 5 %.1-2 Existe evidencia acerca del rol activo de la shiga toxina en la activación del complemento a través de su unión al factor H. El eculizumab es un anticuerpo monoclonal que inhibe la formación del complejo de ataque de membrana (C5b-9), por su alta afinidad a C5 de la cascada del complemento. Su infusión está aprobada para el tratamiento del Síndrome Urémico Hemolítico atípico, planteándose su utilidad en casos de Síndrome Urémico Hemolítico típico grave con compromiso neurológico severo como alternativa para inhibir la cascada de complemento, y así detener el daño producido por la toxina. Se presentan dos casos de pacientes pediátricos con diagnóstico Síndrome Urémico Hemolítico con rescate de Shiga toxina, con compromiso neurológico grave y que recibieron tratamiento con eculizumab con respuesta favorable.

Abstract Hemolytic Uremic Syndrome is an endemic disease in Latin America. Argentina is one of the countries where most cases are reported, with a rate of ten cases per 100,000 children under five years old. It is the first cause of acute renal failure (ARF), and responsible for 9% of kidney transplants. This pathology is characterized by a classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. The main etiological agent of HUS is the bacterium Shiga toxin-producing Escherichia coli. HUS has an acute mortality lower than 5 %. There is evidence of the active role of the Shiga toxin in the activation of the complement by binding to factor H. Eculizumab is a monoclonal antibody which inhibits the formation of the membrane attack complex (C5b-9), given its great affinity for C5 of the complement cascade. Its infusion is approved to treat atypical HUS, posing its usefulness to treat severe typical HUS with acute neurological involvement as an alternative to inhibit the complement cascade and stop toxin damage. We present two pediatric patients with SUH diagnosis with shiga toxin rescue; these patients, who showed severe neurological involvement, were treated with Eculizumab and had a favorable response.