Background The WHO's Vision 2020 global initiative against blindness, launched in 2000, prioritises children. Progress has been hampered by the global paucity of epidemiological data about childhood visual disability. The British Childhood Visual Impairment and Blindness Study 2 (BCVIS2) was undertaken to address this evidence gap. Methods UK-wide prospective population-based observational study of all those aged under 18 years newly diagnosed with visual impairment or blindness between Oct 1, 2015 and Nov 1 2016. Eligible children were notified simultaneously but independently by their managing ophthalmologists and paediatricians via the two national active surveillance schemes, the British Ophthalmic and Paediatric Surveillance Units. Standardised detailed data were collected at diagnosis and one year later. Incidence estimates and relative rates by key sociodemographic factors were calculated. Descriptive analyses were undertaken of underlying ophthalmic disorders and nonophthalmic comorbidities. FindingsOf 784 cases, 72% had additional non-ophthalmic impairments/disorders and 4% died within the year. Annual incidence was highest in the first year of life, 5•2 per 10,000 (95% CI 4•7-5•7) with cumulative incidence by 18 years of 10•0 per 10,000 (95% CI 9•4 to 10•8). Rates were higher for those from any ethnic minority group, the lowest quintile of socio-economic status, born preterm or with low birthweight. Only 44% had a single ophthalmic condition: disorders of the brain/visual pathways affected 48% overall. Prenatal or perinatal aetiological factors accounted for 84% of all conditions. InterpretationBCVIS2 provides a contemporary snapshot of the heterogeneity, multi-morbidity and vulnerability associated with childhood visual disability in a high income country, and the arising complex needs. These findings will facilitate developing and delivering healthcare and planning interventional research. They highlight the importance of including childhood visual disability as a sentinel event and metric in global child health initiatives.
Overlap syndrome indicates the coexistence of 2 or more autoimmune liver diseases in the same individual, occurring simultaneously or sequentially. Cases of overlap of autoimmune hepatitis (AIH) with primary biliary cholangitis (PBC) and of AIH with primary sclerosing cholangitis (PSC) are known and have defined criteria for diagnosis. Overlap between PBC and PSC has been reported in only a few case reports. The cause for the rarity of this entity compared to other overlap syndromes is unclear. We present a case of an overlap syndrome of PBC with PSC in a 35-year-old woman.
Childhood visual impairment confers significant potential adversity on the individual, their family, and on wider society. 1,2 To address this at societal and individual levels, primary (preventing blinding disease from occurring), secondary (treatment of established disease to reduce negative impact), and tertiary prevention approaches are required. [3][4][5] Tertiary prevention approaches comprise interventions that mitigate the impact of established visual disability or associated disorders on the life of the child and the adult they become. These interventions may be simple, such as the provision of low vision aids, or more complex, such as the provision of parenting support, or the development of individualized 'packages' of multidisciplinary care for the additional physical, educational, psychological, and social developmental needs of the affected child. 6 In recognition of the high burden of the numerous developmental and non-ophthalmic disorders that coexist in children with impaired vision, multidisciplinary assessment of children newly diagnosed with visual disability is advocated. 1,3,7 Almost two decades ago, the British Childhood Visual Impairment and Blindness Study (BCVIS; 2003) confirmed that in the UK most children newly diagnosed with severe visual impairment and blindness (SVIBL; vision worse than 1.0 logMAR [logarithm of the minimum angle of
Renal anemia is a well recognized complication of chronic kidney disease with erythropoietin deficiency being the primary cause. Erythropoiesis-stimulating agents (ESAs), introduced almost two decades ago, have replaced blood transfusions as first line therapy for anemia. They have been shown to markedly improve the quality of life but the effects on blood pressure and rate of progression of disease are still a matter of controversy. This prospective interventional study aimed to determine the efficacy and safety of recombinant human erythropoietin therapy (rHuEPO) in correction of renal anemia, ascertain its effect on progression of renal excretory function and assess the quality of life in predialysis patients of chronic renal failure. We prospectively studied 50 anemic (hemoglobin levels <10.0 g/dL) predialysis patients of chronic renal failure (GFR between 15-90 mL/min/1.73 m2). The children, under 14 years of age, and were randomly selected in the outpatient clinic at The Children’s Hospital and Institute of Child Health Lahore, Pakistan between July 2007 to June 2008. These patients were treated with rHuEPO for 48 weeks. The maintenance dose of subcutaneous rHuEPO used was 100 U/kg/week given twice weekly along with oral iron and folate supplementation. Patients underwent clinical and laboratory evaluation during the trial period. GFR was calculated using the Schwartz formula. The mean age of the children enrolled in the study was 7.68 years at presentation with male to female ratio of 1.63. Hemoglobin and GFR values before initiation of rHuEPO treatment were 8.20±1.28 g/dL and 29.82±12.00 mL/min/1.73 m2 as compared to the final results of 10.94±1.04 g/dL and 32.36±11.24 mL/min/1.73 m2, respectively. Only 4 (8%) patients developed end – stage renal disease by the end of the study period and had to begin dialysis while others continued with the conservative management of chronic renal failure. No significant side – effects were seen in any of the study population like hypertension, seizures or hyperviscosity syndrome, except 3 (6%) children who required increase in dose of antihypertensive medication. The health – related quality of life improved in terms of physical performance and sense of well being in the majority of subjects. Our study provides conclusive evidence that rHuEPO therapy is beneficial for correction of renal anemia with substantial retardation of maintenance dialysis in the majority of predialysis patients with chronic kidney disease
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