ESPN 53rd Annual Meeting

ESPN 2021


 
An atypical case of typical HUS
MARINA AVRAMESCU 1 LAURA SOYER 1 ROMAIN BERTHAUD 1 MARION RABANT 1 ALINE BROCH 1 PATRICIA MARIANI 2 VERONIQUE FREMEAUX-BACCHI 3 OLIVIA BOYER 1

1- AP-HP HOPITAL NECKER
2- AP-HP HOPITAL ROBERT DEBRE
3- AP-HP HEGP
 
Abstract:

Shiga toxin-producing Escherichia coli (STEC) is the leading cause of hemolytic-uremic syndrome (HUS) in children. Patients with STEC HUS are generally ill-appearing at presentation and have the biological triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. A previously healthy 7-month-old girl presented to the emergency room with periumbilical inflammation. An abdominal ultrasound revealed the presence of a urachal cyst. Blood workup showed: hemoglobin 8.7 g/dl, white blood cell count 6 300/mm3, platelet count 287 000/mm3, C-reactive protein 3 mg/l, serum creatinine 101 μmol/l; peripheral blood smear found no schizocytes. The infant had no history of recent infection or toxic exposure. She had a normal urine output, no edema and normal blood pressure. Renal ultrasound was normal. Urinalysis revealed leukocyturia, mixed proteinuria and hematuria. Further laboratory tests were conducted and found normal values of serum albumin, LDH, haptoglobin, liver and pancreatic enzymes. Immunologic workup found normal complement levels and negative antinuclear antibodies. Creatinine levels did not decrease after IV hydration. A percutaneous kidney biopsy was performed and was in favour of a thrombotic microangiopathy. The urachal abscess was drained and culture was positive for Escherichia coli. Polymerase chain reaction identified the strain as producing shiga toxin-2. Stool samples were negative for STEC. No genetic mutation of atypical HUS was found. Evolution was favourable: at 6-month follow-up, the patient had a normal clinical examination and an improved renal function with a serum creatinine of 50 μmol/l. The abdominal ultrasound found no sign of urachus remnants. The patient is closely monitored every three months and receives an anti-proteinuric treatment with ACEi. This very atypical case raises awareness of the possibility of STEC HUS following E.Coli abcess. Our patient had no prodromal diarrhea, no signs of hemolysis, a normal platelet count, a fortuitous discovery of renal failure and STEC was identified in a urachal abscess and not in stool.