title: Subarachnoid haemorrhage and cerebral vasculopathy in a child with sickle cell anaemia.
abstract: Stroke in sickle cell anaemia (SCA) is either infarctive or haemorrhagic in nature. In childhood, over 75% of strokes in SCA are infarctive. We present an adolescent with SCA who developed hypertension at the age of 13, and was treated with lisinopril. Sixteen months later she was found in cardiorespiratory arrest and died on arrival in hospital. The last transcranial Doppler scan performed 6 months before her death and a brain MRI were reported normal. The autopsy discovered massive subarachnoid haemorrhage in association with vascular damage in the circle of Willis arteries. The case highlights a cause of haemorrhagic stroke, the first reported association between hypertension, SCA and a histopathologically proven cerebral vasculopathy. The difficulties in the management of haemorrhagic stroke and the poor outcome in SCA are discussed.
authors.affiliation: Department of Paediatrics, Evelina Children's Hospital, London, UK.
authors.forename: Baba
authors.initials: B
authors.lastname: Inusa
authors.affiliation: Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Seconda Università di Napoli, Naples, Italy.
authors.forename: Maddalena
authors.initials: M
authors.lastname: Casale
authors.affiliation: Evelina London Children's Hospital, Guy's and St Thomas NHS Foundation Trust, London, UK.
authors.forename: Caroline
authors.initials: C
authors.lastname: Booth
authors.affiliation: Department of Pathology, King's College London, London, UK.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Lucas
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D002561:Cerebrovascular Disorders / Q000209:etiology* / Q000473:pathology; D002941:Circle of Willis / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications / Q000188:drug therapy; D017706:Lisinopril / Q000627:therapeutic use; D013345:Subarachnoid Hemorrhage / Q000209:etiology*
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D017706:Lisinopril