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

Article Article scientifique Article Successful treatment of neonatal atypical hemolytic uremic syndrome with C5 monoclonal antibody eng 2016 author Anastaze Karine 582988 author Gonzalez E author Wilhelm-Bals Alexandra Marina 105558 author Michelet Pierre-Raphaël 503154 author Korff Christian 0000-0002-3328-7680 30053 author Parvex Paloma Maria 36046 283-286 10.1016/j.arcped.2015.11.020 26775886 0929-693X 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. Antibodies, Monoclonal, Humanized/therapeutic use Atypical Hemolytic Uremic Syndrome/drug therapy Female Humans Infant, Newborn Remission Induction Dewey 618 58 Département de pédiatrie, gynécologie et obstétrique 223 false Archives de pédiatrie 23 3 287831b6-1181-4b17-a6af-c6f0694251e0 Article (Published version) Restricted 1-s2.0-S0929693X15004522-main-1.pdf 380451 application/pdf restrictedAccess Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique

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Michelet, Pierre-Raphaël