Retinal disease in the C3 glomerulopathies and the risk of impaired vision

Background: Dense deposit disease and atypical hemolytic uremic syndrome are often caused by Complement Factor H (CFH) mutations. This study describes the retinal abnormalities in dense deposit disease and, for the first time, atypical haemolytic uremic syndrome. It also reviews our understanding of drusen pathogenesis and their relevance for glomerular disease. Methods: Six individuals with dense deposit disease and one with atypical haemolytic uremic syndrome were studied from 2 to 40 years after presentation. Five had renal transplants. All four who had genetic testing had CFH mutations. Individuals underwent ophthalmological review and retinal photography, and in some cases, optical coherence tomography, and further tests of retinal function. Results: All subjects with dense deposit disease had impaired night vision and retinal drusen or whitish-yellow deposits. Retinal atrophy, pigmentation, and hemorrhage were common. In late disease, peripheral vision was restricted, central vision was distorted, and there were scotoma from sub-retinal choroidal neovascular membranes and atypical serous retinopathy. Drusen were present but less prominent in the young person with atypical uremic syndrome due to a heterozygous CFH mutation. Conclusions: Drusen are common in forms of C3 glomerulopathy caused by compound heterozygous or heterozygous CFH mutations. They are useful diagnostically but also impair vision. Drusen have an identical composition to glomerular deposits. They are also identical to the drusen of age-related macular degeneration, and may respond to the same treatments. Individuals with a C3 glomerulopathy should be assessed ophthalmologically at diagnosis, and monitored regularly for vision-threatening complications.     

Racial matching and adolescent self-disclosure of substance use and mental health symptoms

Obtaining accurate assessment data from adolescents in treatment aids clinical decision making and facilitates more accurate outcome evaluations. However, findings could be biased due to underreported substance use and mental health symptoms. This article compares self-reports of youth in non-White matched client–assessor dyads and those in nonmatched dyads. There were no differences on self-reported substance use, but matched youth reported significantly fewer attention deficit/hyperactivity disorder symptoms versus the comparison group. One possible reason for these findings is the effect of in-group stereotype threat. Future studies should examine the potential effect that in-group stereotyping and perceived racism have on the therapeutic relationship.     

Relation between active mediation,exposure to Daniel Tiger’s Neighborhood,and US preschoolers’ social and emotional development

This study explored the relationship between active mediation, exposure to Daniel Tiger’s Neighborhood, and key indicators of preschoolers’ social and emotional development. One hundred and twenty-seven children aged 2–6 either watched or did not watch 10 episodes of Daniel Tiger’s Neighborhood over a two-week period. Results revealed that preschoolers who watched the program exhibited higher levels of empathy, self-efficacy, and emotion recognition when their regular TV-watching experiences are frequently accompanied by active mediation. This was especially true for younger preschoolers and preschoolers from low-income families. Implications for policy-makers, parents, producers of prosocial programming, and educators are discussed.     

Pioglitazone has a dubious bladder cancer risk but an undoubted cardiovascular benefit

On 8 April 2014, a US jury ordered Takeda and Eli Lilly to pay $9 bn in punitive damages after finding that they had concealed the cancer risks associated with pioglitazone. By contrast, on 28 August 2014, the long‐awaited outcome of the 10‐year Kaiser Permanente Northern California study was announced. That study was specifically designed to investigate whether patients exposed to pioglitazone were at an increased risk of bladder cancer and found no association; thus, at last, the controversial issue has been resolved. A review, in retrospect, of the story of the proposed link between pioglitazone and bladder cancer reveals flaws at every stage. In 2012, a BMJ editorial, in keeping with some other contemporary reports, stated ‘it can confidently be assumed that pioglitazone increases the risk of bladder cancer’. Examination of the information which led to such a statement shows that: 1) the pre‐clinical findings of bladder cancer in male rats is not indicative of human risk; 2) there is no association between bladder cancer and pioglitazone in randomized controlled trials, once cases that could not plausibly be related to treatment are removed; and 3) the observational studies that have suggested a link have over‐extrapolated from the data: pioglitazone‐treated patients had more risk factors for bladder cancer than those not treated with pioglitazone. Meanwhile careful study of randomized controlled trials shows evidence of cardiovascular benefit from pioglitazone in Type 2 diabetes, a condition which results, more than anything, in premature cardiovascular death and morbidity.