Acute rheumatic fever and rheumatic heart disease in children and adolescents in Victoria, Australia.

AIM: To describe the epidemiology and clinical profile of children and adolescents with acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in Victoria, Australia. METHODS: A retrospective audit was undertaken of children and adolescents with ARF and RHD attending the Royal Children's and Monash Children's Hospitals in Victoria, Australia between 2010 and 2019. Potential cases were identified by searching multiple sources for relevant ICD-10-AM codes and keywords, then reviewed manually. For confirmed cases, we collected data on patient demographics, clinical features, comorbidities and management. RESULTS: Of 179 participants included, there were 108 Victorian residents and 71 non-Victorian residents. 126 had at least one episode of ARF during the study period and 128 were diagnosed with RHD. In the Victorian resident group, the overall incidence of ARF was 0.8 per 100 000 5-14 year olds. This incidence was higher in Victorian Aboriginal and/or Torres Strait Islander (3.8 per 100 000) and Pacific Islander (32.1 per 100 000) sub-populations. Of 83 Victorian residents who had an ARF episode, 11 (13%) had a recurrence. Most Victorian residents with RHD had mixed aortic and mitral valve pathology (69.4%) and moderate to severe disease (61.9%). Most non-Victorian residents were Aboriginal and/or Torres Strait Islander people (80.3%) and were commonly transferred for tertiary or surgical management of RHD (83.1%). CONCLUSIONS: ARF and RHD continue to affect the health of significant numbers of children and adolescents living in Victoria, including severe and recurrent disease. Specialised services and a register-based control program may help to prevent complications and premature death.

Fatal ischemic stroke complicating acute multifocal placoid pigment epitheliopathy: histopathological findings.

A previously healthy 29-year-old man was admitted to a tertiary referral center with acute left hemiparesis followed shortly by de novo convulsive status epilepticus. This was in the context of a 2-month history of flu-like symptoms, severe headaches, and retinopathy recently diagnosed as acute multifocal placoid pigment epitheliopathy. Neuroimaging demonstrated bilateral, multiple territory cerebral infarction. Despite intravenous methylprednisolone and craniotomy for the management of raised intracranial pressure, the patient deteriorated and died 14 days later. At autopsy, multiple infarcts of varying ages within a 10-day period were seen in association with a segmental giant cell vasculopathy of meningeal arteries.