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Successful treatment of neonatal atypical hemolytic uremic syndrome with C5 monoclonal antibody

Gonzalez, E
Published in Archives de pédiatrie. 2016, vol. 23, no. 3, p. 283-6
Abstract Hemolytic uremic syndrome (HUS) is rare in neonates. We report the case of atypical HUS (aHUS) revealed by neonatal seizures. This 18-day-old baby presented with repeated clonus of the left arm and eye deviation. Four days earlier, she had suffered from gastroenteritis (non-bloody diarrhea and vomiting without fever). Her work-up revealed hemolytic anemia (120 g/L), thrombocytopenia (78 g/L), and impaired renal function (serum creatinine=102 μmol/L) compatible with the diagnosis of HUS. Levels of C3 and C4 in the serum were normal. Shiga-toxin in the stools as well as the IgM and IgG against Escherichia coli O157 were negative. ADAMTS 13 deficiency, inborn error of the cobalamin pathway, deficiency in the H and I protein, and factor H antibodies were excluded and we concluded in aHUS. Genetic screening of the alternative complement pathway was normal. Cerebral magnetic resonance imaging performed after 24 h and 1 week showed restricted diffusion areas with periventricular white matter ischemic-hemorrhagic lesions. Extensive infectious work-up was negative. Upon admission the baby received antiepileptic drugs and 2 days later C5 monoclonal antibody (eculizumab) and two transfusions of packed erythrocytes because the hemoglobin value had dropped to 55 g/L. The platelet value was minimal at 30 g/L. Renal function normalized in 48 h without dialysis and neurological examination was normal in 1 week. She was discharged from the hospital at day 10 with eculizumab perfusions (300 mg) planned every 3 weeks. After 24 months, she was relapse-free and seizure-free, with a normal neurological examination.
Keywords Antibodies, Monoclonal, Humanized/therapeutic useAtypical Hemolytic Uremic Syndrome/drug therapyFemaleHumansInfant, NewbornRemission Induction
PMID: 26775886
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ANASTAZE, Karine et al. Successful treatment of neonatal atypical hemolytic uremic syndrome with C5 monoclonal antibody. In: Archives de pédiatrie, 2016, vol. 23, n° 3, p. 283-6. doi: 10.1016/j.arcped.2015.11.020 https://archive-ouverte.unige.ch/unige:96882

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Deposited on : 2017-09-19

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