POS-190 RENAL PROGNOSIS OF HEMOLYTIC UREMIC SYNDROME IN ADULTS

Abstract

Hemolytic uremic syndrome (HUS) is caused by intrarenal thrombotic microangiopathy, leading to high blood pressure and acute renal failure. The most common form of HUS is secondary to shiga toxin-producing bacteria, typically Escherichia coli. Atypical HUS (aHUS) has been used to classify any HUS not caused by shiga toxin. It is characterized by the triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The aim of our study was to determinate the clinical features and the renal prognosis of the HUS occurring in adults. We conducted a retrospective study of 19 patients over a period of 27 years (1990-2017) in our nephrology department. We defined hypertension as an increase in systolic blood pressure > 140 mmhgand / or diastolic blood pressure > 90 mmhg on the basis of the Europeandefe society of cardiology We defined anemia as a hemoglobin levels lower than 13 g/100ml for adult males, 12 g/100ml for adult females non pregnant, 11 g/100ml for adult females pregnant. We defined thrombocytopenia as platelets levels lower than 150000/mm3. AKI is defined as any of the following (Not Graded): Increase in SCr by X0.3 mg/dl (X26.5 lmol/l) within 48 hours or Increase in SCr to X1.5 times baseline, which is known or presumed to have occurred within the prior 7 days or Urine volume o0.5 ml/kg/h for 6 hours on the basis of KDIGO. We included patients aged over 16 years old with biopsy proven thrombotic microangiopathy. We collected the following parameters: age, gender, co morbidities, clinical exam, biological assessment, treatment We included 19 patients. There were 15 men (79%) and 4 women (21%), sex ratio 3,75 with a median age 36 years old (19- 60). The inaugural symptoms were dominated by headaches in 52% of cases. It was associated to malignant high blood pressure. The median of PAS was 178mmHg. And the median of PAS was 120 mmHg, the median of PAM was 143 mmHg. The median creatinine at diagnosis was 786 μmol /l (350 – 1940μmol /l). The blood smear revealed schizocytes in 11 cases (58%). The median hemoglobin was 6,3 g/dl (4,5 – 7,8g/dl). The haptoglobin level in 7 patients was low in 3 cases. The median platelets was 110000 /mm3 (45000 – 143000). The serum complement C3 was consumed in 64% of cases. A glomerular thrombotic microangiopathy was objectified in 42% of cases. For the etiologies, the HUS was related to gene mutations, pregnancy, infections, cancer in respectively 21%, 5%, 10% and 5% of cases. For the other cases the cause remains unknown. The treatment combined corticosteroid therapy (1mg/Kg/j) in all cases with plasma exchanges in 26% of cases. The evolution has been marked by a progressive improvement in renal function in 37% of cases but 74% of patients maintained chronic kidney failure then requiring hemodialysis in 58% of cases after a mean of follow-up of 14 months (3 to 32 months) Three patients had kidney transplant. One death occurred and was secondary to acute pulmonary edema. The diagnosis of aHUS is complex and challenging, but is essential because of the devastating consequences of inadequate treatment. The prognosis of HUS has been improved by plasma exchange but chronic renal insufficiency is still common.