Peripheral nervous system manifestations of Shiga toxin-producing E. coli-induced haemolytic uremic syndrome in children

Italian Journal of Pediatrics

Elena Bresin¹  Maria Chironna²  Giuseppe Stefano Netti³  Elena Ranieri³  Federica Spadaccino³  Leonardo Milella⁴  Maria Luigia Lasorella⁴  Vincenza Carbone⁵  Giovanni Piscopo⁵  Diletta Domenica Torres⁵  Mario Giordano⁵  Luisa Santangelo⁵  Pasquale Conti⁶  Delio Gagliardi⁶  Luciana Losito⁷  Antonio Trabacca⁷ 

[1] Clinical Research Center for Rare Diseases ‘Aldo e Cele Daccò’, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy;Department of Biomedical Sciences and Human Oncology, Hygiene Unit, University of Bari “Aldo Moro”, Bari, Italy;Department of Medical and Surgical Sciences, Clinical Pathology Unit and Center for Molecular Medicine, University of Foggia, Viale Luigi Pinto -, 71122, Foggia, Italy;Intensive Care Unit, Pediatric Hospital “Giovanni XXIII”, Bari, Italy;Pediatric Nephrology and Dialysis Unit, Pediatric Hospital ‘Giovanni XXIII’, Bari, Italy;Pediatric Neurology Unit, Pediatric Hospital “Giovanni XXIII”, Bari, Italy;Scientific Institute I.R.C.C.S. “E. Medea”- Unit for Severe disabilities in developmental age and young adults (Developmental Neurology and Neurorehabilitation), Brindisi, Italy;

关键词: Hemolytic uremic syndrome;    Escherichia Coli;    Peripheral nervous system;    Eculizumab;    Plasma exchange;    Neurorehabilitation;   

DOI  :  10.1186/s13052-021-01133-1

来源: Springer

【 摘 要 】

BackgroundThe Neurological involvement is the most common extra-renal complication of Shiga toxin-producing E. coli-hemolytic uremic syndrome (HUS) or typical HUS. On brain magnetic resonance examination, main neurological signs encompass acute lesions of the basal ganglia and the white matter, which could usually regress after Eculizumab infusion. In contrast, peripheral nervous system (PNS) manifestations in typical HUS are very rare and, when occurring, they require a careful management of neurological sequelae and an intensive multidisciplinary neuro-rehabilitation program.Case presentationHere, we present two pediatric cases of severe and complicated typical HUS with PNS manifestations who required therapeutic treatment and an intensive multidisciplinary neuro-rehabilitation program.In both cases, PNS manifestations were followed by the recovery from typical HUS-related severe central neurological damage and manifested mainly with marked bilateral motor deficit and hyporeflexia/areflexia in the lower limbs. The peripheral polyneuropathy was treated with immunosuppressive therapy (methylprednisolone boluses, i.v. immunoglobulins, plasma exchange), followed by a prolonged intensive neuro-rehabilitation program. After 8 months of rehabilitation, both patients gained complete functional recovery.ConclusionsPNS manifestations during typical HUS are a rare event and potentially leading to severe disability. A timely clinical assessment is mandatory to set up a prompt therapeutic and rehabilitation program and to obtain a complete clinical and functional recovery.

【 授权许可】

CC BY