Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome

Abstract

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case.

Keywords: Hemolytic uremic syndrome; Intestinal perforation; Shiga toxin; Typical.

Fig. 1

Renal ultrasonographic findings showing diffusely increased parenchymal echogenicity with decreased perfusion in both the kidneys. (A) Right kidney. (B) Left kidney.

Fig. 2

X-ray scans (A, supine view; B, cross-table lateral view) and computed tomography scan (C) of the abdomen showing a suspected intestinal perforation seen on the day of operation.

Fig. 3

Gross specimen of the resected intestine. A perforated site is seen in the center of the specimen.