A 38-week-old male newborn weighing 2.130 g was admitted to the neonatal ICU with generalized edemas treated with furosemide infusion (1.5 mg/kg/hour) and hyperbilirubinemia did not respond to triple therapy.
After an exchange transfusion on the second and third day of birth, worsening of the edema, sustained hypertension with figures of 1426
Despite acute renal failure, peritoneal dialysis is not initiated due to diuresis between 2.5-4.5 mg/kg/hour.
On the ninth day of birth plasma was administered due to persistent hemolysis.
Platelet concentrates were transfused on six occasions, while platelet concentrates were transfused on three occasions.
PCR for cytomegalovirus and parvovirus B19 negative.
The molecular genetic study of the alternative complement pathway showed normal results and confirmed the absence of mutations in genes of factor H, I and MCP, as well as in phenotypes of CFH and MCP genes.
Despite this and remaining pending the ADAMS 13, aHUS is diagnosed due to the symptoms presented and treatment with eculizumab is initiated.
After the first dose of 300 mg, a rapid response is observed within 48 hours when hemolysis disappears and the tendency to thrombopenia (HCV normalizes, passing from 8.5 to 12.4 g/dL urine output and urine output increase).
The decrease in renal markers was insidious up to 48h post-treatment from which it became fast and sustained.
Creatinine levels (3.44 to 1.9 mg/dL) and urea (156 to 102 mg/dL) were measured.
The LDH decreased from 1640 to 625 U/L (values at 7th and 11th day of birth respectively) (VR at 4-10 days of age : 545-2000 U/L at 24 months of age: 430 U/L).
With the second dose one week later urea values of 55 mg/dL, creatinine of 0.84 mg/dL and LDH of 288 U/L were obtained. After that, eculizumab was administered every 3 weeks.
Amoxicillin clavulanic acid was used as a prophylactic measure against meningococcus during treatment.
