[Successful prophylactic plasma infusions in recurrent atypical hemolytic-uremic syndrome].
Atypical, recurrent hemolytic uraemic syndrome (HUS) often leads to end-stage renal disease and can relapse after a renal transplantation. Plasmapheresis and fresh frozen plasma (FFP) therapies are used to treat acute relapses. We report on the prophylactic use of FFP in a child with atypical recurrent HUS with low serum C3. The girl experienced four episodes of atypical HUS over a year from the age of 18 months. During the first relapse a decreased level of C3--0,62 g/l (normal: 0.88-2.01 g/l) was revealed. Initially daily plasma infusions were given (8-14 ml/kg for 5-15 days) during acute episodes. Following the 4th episode prophylactic FFP therapy was started. Three-times weekly plasma transfusions were continued for three months and then doses were tapered off over the next months to once-weekly FFP. During the one year follow-up no relapse has been observed. At the age of 3 1/2 years she is thriving (10th percentile for height and weight). She has severe but well controlled hypertension without end-organ involvement. The child demonstrates signs of stage 2 chronic kidney disease (CKD): serum creatinine--0.65 mg/dl, Schwartz estimated GFR--79.9 ml/min/ 1.73 m2, persistent slight proteinuria and haematuria, increased renal cortical echogenicity on ultrasound. Genetic studies for the known atypical HUS mutations and assessment of complement activation proteins (factors H and I) are under investigation.


CONCLUSIONS
Prophylactic FFP infusions can be successful in preventing relapses and further progressive renal damage in individual patients with atypical HUS associated with low C3.
Zurowska
Aleksandra
A
Klinika Nefrologii Dzieciecej, Akademii Medycznej w Gdańsku. azur@amg.gda.pl
Załuska-Leśniewska
Iga
I
Hladny-Czerska
Wanda
W
D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis / Q000172:deficiency; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000517:prevention & control* / Q000628:therapy*; D006801:Humans; D007223:Infant; D010951:Plasma Exchange; D011379:Prognosis; D055502:Secondary Prevention; D016896:Treatment Outcome
D003176:Complement C3