Severely ill pediatric patients with Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) who suffered from multi
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ORIGINAL ARTICLE
Severely ill pediatric patients with Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) who suffered from multiple organ involvement in the early stage Mariana Luna 1 & Mariana Kamariski 1 & Iliana Principi 1 & Victoria Bocanegra 2 & Patricia G. Vallés 1,3 Received: 1 June 2020 / Revised: 24 September 2020 / Accepted: 15 October 2020 # IPNA 2020
Abstract Background Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is the main cause of pediatric acute kidney injury (AKI) in Argentina. Endothelial injury is the trigger event in the microangiopathic process. The host inflammatory response to toxin and E. coli lipopolysaccharide (LPS) is involved in disease pathophysiology. Methods This retrospective study describes pediatric STEC-HUS patients with multiorgan involvement at the initial phase of disease. A retrospective study of critically ill HUS patients with evidence of E. coli infection was conducted through a period of 15 years. Results Forty-four patients 35.4 ± 4.1 months were admitted to the intensive care unit for 21 ± 2 days. Mechanical ventilation was required in 41 patients, early inotropic support in 37, and 28 developed septic shock. Forty-one patients required kidney replacement therapy for 12 ± 1 days. Forty-one patients showed neurological dysfunction. Dilated cardiomyopathy was demonstrated in 3 patients, left ventricular systolic dysfunction in 4, and hypertension in 17. Four patients had pulmonary hemorrhage, and acute respiratory distress syndrome in 2. Colectomy for transmural colonic necrosis was performed in 3 patients. Thirtyseven patients were treated with therapeutic plasma exchange, and 28 patients received methylprednisolone (10 mg/kg for 3 days). Of the surviving 32 patients, neurological sequelae were seen in 11 and chronic kidney failure in 5. Conclusions Severe clinical outcome at onset suggests an amplified inflammatory response after exposure to Shiga toxin and/or E. coli LPS. STEC-HUS associated with severe neurological involvement, hemodynamic instability, and AKI requires intensive care and focused therapy. Keywords Children . STEC-HUS patients . Multiorgan involvement . Neurological compromise . Kidney replacement therapy
Introduction Hemolytic uremic syndrome (HUS) is a disorder characterized by a prodrome of enterohemorrhagic diarrhea, followed by thrombocytopenia, microangiopathic hemolytic anemia, and
* Patricia G. Vallés [email protected] 1
Servicio de Nefrología, Departamento de Pediatría, Hospital Humberto Notti, Mendoza, Argentina
2
Instituto de Medicina y Biología Experimental de Cuyo (IMBECU), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Mendoza, Argentina
3
Área de Fisiopatología, Departamento de Patología, Facultad de Ciencias Médicas, Universidad Nacional de Cuyo, Mendoza, Argentina
acute kidney injury (AKI). Nonetheless, it is a systemic disorder in which severe forms can be associated with multiorgan involvement during the acute stage of the disease [1]. Also calle
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