ESPN 50th Annual Meeting

ESPN 2017


 
Neonatal onset of atypical Hemolitic Uremic Syndrome (aHUS) treated with Therapeutic Plasma Exchange (TPE) using CARPEDIEM machine
MATTIA PAROLIN 1 ENRICO VIDAL 1 FRANCESCO GARZOTTO 3 GIUSEPPE REMUZZI 4 LUISA MURER 1 CLAUDIO RONCO 2

1- PEDIATRIC NEPHROLOGY DIALYSIS AND TRANSPLANTATION UNIT, DEPARTMENT FOR WOMAN AND CHILD HEALTH, UNIVERSITY OF PADUA
2- DEPARTMENT OF NEPHROLOGY, SAN BORTOLO HOSPITAL, VICENZA
3- INTERNATIONAL RENAL RESEARCH INSTITUTE (IRRIV), VICENZA
4- IRCCS-ISTITUTO DI RICERCHE FARMACOLOGICHE MARIO NEGRI, CLINICAL RESEARCH CENTER FOR RARE DISEASES ALDO E CELE DACCò, RANICA, BERGAMO, THE AZIENDA OSPEDALIERA PAPA GIOVANNI XXIII, UNIT OF NEPHROLOGY AND DIALYSIS, BERGAMO, AND THE DEPARTMENT OF BIOMEDICAL AND CLINICAL SCIENCES, UNIVERSITY OF MILAN, MILAN , ITALY
 
Introduction:

 

aHUS is a potentially life-threatening rare disease characterized by the triad:Coombs negative microangiopathic anemia,thrombocytopenia and acute kidney injury(AKI).It can be sporadic or familial,and its often associated with genetic complement abnormalities/anti-complement factor-H antibodies.

Material and methods:

 

A 42-day-old male was referred to our center because of AKI,anemia,and thrombocytopenia.He was a term neonate(bw.2,765g)with an uneventful course of pregnancy.On the 2nd day of life,the patient experienced abdominal distension,oliguria,anemia and thrombocytopenia.An abdominal X-ray showed a necrotizing enterocolitis,but,despite the treatment he became hypotensive,with a remarkable increase in inflammatory markers,and finally reached a 30%fluid overload(3,490g).At 6 weeks of life,he was transferred to our unit.Anemia,thrombocytopenia and AKI suggested a thrombotic microangiopathy(TMA).The blood tests showed a consumption of haptoglobin,increase in LDH,schistocytes with negative Coombs test and a reduction of C3 with normal fibrinogen and coagulation tests.Potential causes of TMA were investigated and the overall picture was then suggestive for a complement-mediated aHUS.Due to the abdominal condition and the infectious risk,no complement-blocker was used.

Results:

 

TPE was then initiated using the CARPEDIEM machine and the Plasmart05 filter,with fresh frozen plasma(FFP)as replacement fluid and an exchange volume of 200ml.The circuit was primed with 50mL of 4%albumin.The blood pump rate was set at 10mL/min,with an exchange rate of 1mL/min and a total session time of 3.5h.He received 5 consecutive daily sessions of TPE,without any clinical/technical problem.The first 2 sessions of TPE were followed by a CVVH.Afterwards,an increase in urinary output was observed,with restoration of normal fluid status,renal function and blood counts.At 2 months of age,after complete resolution of the abdominal and infectious condition,Eculizumab was started.Molecular analysis resulted as negative,but an increase in the amount of C5b9 on human microvascular endothelial cells was found during the acute phase.

Conclusions:

 

Few reports have described the experience of using Eculizumab,and still less TPE in neonates,with aHUS.We would like to underline the efficacy of TPE with CARPEDIEM when Eculizumab is contraindicated.