title: Successful treatment of neonatal atypical hemolytic uremic syndrome with C5 monoclonal antibody.
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.
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, University Hospital of Geneva, rue Willy Donzé 6, 1211 Geneva, Switzerland. Electronic address: karine.anastazestelle@gmail.com.
authors.forename: K
authors.initials: K
authors.lastname: Anastaze Stelle
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, University Hospital of Geneva, rue Willy Donzé 6, 1211 Geneva, Switzerland.
authors.forename: E
authors.initials: E
authors.lastname: Gonzalez
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, University Hospital of Geneva, rue Willy Donzé 6, 1211 Geneva, Switzerland.
authors.forename: A
authors.initials: A
authors.lastname: Wilhelm-Bals
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, University Hospital of Geneva, rue Willy Donzé 6, 1211 Geneva, Switzerland.
authors.forename: P-R
authors.initials: PR
authors.lastname: Michelet
authors.affiliation: Pediatric Neurology, Department of Pediatrics, University Hospital of Geneva, Geneva, Switzerland.
authors.forename: C M
authors.initials: CM
authors.lastname: Korff
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, University Hospital of Geneva, rue Willy Donzé 6, 1211 Geneva, Switzerland.
authors.forename: P
authors.initials: P
authors.lastname: Parvex
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D012074:Remission Induction
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab