SummaryIn a UK series, 37 of 445 (8.3%) patients with pulmonary arteriovenous malformations (PAVMs) experienced a cerebral abscess, often pre-PAVM diagnosis as first presentation. Odontogenic organisms, dental interventions, lower oxygen saturation, iron loading, and intravenous iron use were associated with abscess risk.

Background When detected early, inexpensive measures can slow chronic kidney disease progression to kidney failure which, for children, confers significant morbidity and impacts growth and development. Our objective was to determine the incidence of late presentation of childhood chronic kidney disease and its associated risk factors. Methods We searched MEDLINE, Embase, PubMed, Web of Science, Cochrane Library and CINAHL, grey literature and registry websites for observational data describing children <21 years presenting to nephrology services, with reference to late presentation (or synonyms thereof). Independent second review of eligibility, data extraction, and risk of bias was undertaken. Meta-analysis was used to generate pooled proportions for late presentation by definition and investigate risk factors. Meta-regression was undertaken to explore heterogeneity. Results Forty-five sources containing data from 30 countries were included, comprising 19,339 children. Most studies (37, n = 15,772) described children first presenting in kidney failure as a proportion of the chronic kidney disease population (mean proportion 0.43, 95% CI 0.34–0.54). Using this definition, the median incidence was 2.1 (IQR 0.9–3.9) per million age-related population. Risk associations included non-congenital disease and older age. Studies of hospitalised patients, or from low- or middle-income countries, that had older study populations than high-income countries, had higher proportions of late presentation. Conclusions Late presentation is a global problem among children with chronic kidney disease, with higher proportions seen in studies of hospitalised children or from low/middle-income countries. Children presenting late are older and more likely to have non-congenital kidney disease than timely presenting children. A consensus definition is important to further our understanding and local populations should identify modifiable barriers beyond age and disease to improve access to care.

Introduction and objectivesPulmonary arteriovenous malformations (PAVMs) are abnormal vessels that provide anatomic right-to-left shunts, and frequently result in severe hypoxaemia. Lung transplantation is sometimes considered if PAVMs are not amenable to treatment by embolization or surgical resection.MethodsA retrospective review was performed of patients with PAVMs assessed between 1999 and 2016 at a single UK institution. Characteristics of those considered and referred for lung transplantation assessment were examined.ResultsBetween May 1999 and July 2016, 707 patients with PAVMs were reviewed. Six were or had been formally considered for lung transplantation purely for PAVMs, 4 as adults (3 at our institution between 1989–1995), 2 as children. Ages ranged from 2–47 (median 22) years. Four were from the UK, two had been assessed in non UK countries. All had hereditary haemorrhagic telangiectasia (HHT). Three had suffered a cerebral abscess due to their PAVMs. The adults had undergone maximal embolisation of PAVMs, and the children were considered to have untreatable disease. The median SaO2 was 79% on air.One adult underwent lung transplantation overseas and died peri-operatively. The other five were not transplanted, in four cases at the patients’ request. Currently, their survival post assessment for lung transplant ranges from 15 to 27 (median 22) years, with four of the five still alive.All five maintained arterial oxygen content by marked secondary erythrocytotic responses when not iron deficient. Four had subsequent embolisation treatments. One had three successful pregnancies, and another is working full time with no supplementary oxygen. However, two had a cerebral abscess (one fatal); three had deep vein thromboses; two experienced long term disabling pain attributed to hypertrophic pulmonary osteoarthropathy in one; and three have exercise limitation requiring supplementary oxygen. Antiplatelet therapy was recommended for two due to ischaemic strokes; one remains anticoagulated 16 years after the insertion of a caval filter; and one is regularly evaluated for hyperviscosity symptoms that would precipitate isovolaemic venesection.ConclusionVery long term stability is evident in the five patients who did not receive a lung transplant. The cohort remain at high risk of cerebral abscess and other complications of right-to-left shunting.