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Strabismus has been known to have a significant genetic component, but the mode of inheritance and the identity of the relevant genes have been enigmatic. This paper reports linkage analysis of nonsyndromic strabismus. The principal results of this study are: (i) the demonstrated feasibility of identifying and recruiting large families in which multiple members have (or had) strabismus; (ii) the linkage in one large family of a presumptive strabismus susceptibility locus to 7p22.1 with a multipoint logarithm of odds score of 4.51 under a model of recessive inheritance; and (iii) the failure to observe significant linkage to 7p in six other multiplex families, consistent with genetic heterogeneity among families. These findings suggest that it will be possible to localize and ultimately identify strabismus susceptibility genes by linkage analysis and mutation screening of candidate genes.

The preterm infant is subjected to prolonged exposure to ambient nursery illumination at levels that have been found to produce retinal damage in animals. We prospectively investigated the effect of exposure to light in two intensive care nurseries by comparing the incidence of retinopathy of prematurity among 74 infants from the standard bright nursery environment (median light level, 60 foot-candles [ftc]) with the incidence among 154 infants of similar birth weight for whom the light levels were reduced (median, 25 ftc). There was a higher incidence of retinopathy of prematurity in the group of infants who had been exposed to the brighter nursery lights, particularly in those with birth weights below 1000 g (86 per cent vs. 54 per cent, P<0.01 by chi-square test). We conclude that the high level of ambient illumination commonly found in the hospital nursery may be one factor contributing to retinopathy of prematurity and that safety standards with regard to current lighting practices should be reassessed. (N Engl J Med 1985; 313:401–4.) With the increased survival rate of premature infants, especially those of very low birth weight, there has been a concomitant increase in retrolental fibroplasia, now known as retinopathy of prematurity. Severe retinopathy of prematurity has nearly reached the epidemic frequency of the 1950s, with an estimated 500 to 600 infants blinded each year. 1 Although the duration of exposure to an elevated oxygen tension and immaturity of the retina are regarded as the principal factors associated with the disease, other factors contribute. One factor may be early exposure to light. The intensity of light in the hospital intensive care nursery has . . .

E ditor ,-Unifocal choroiditis occurs in children and adults with primary (chickenpox) and secondary varicella zoster virus (VZV) infection. 1 Current recommendations by the American Academy of Pediatrics do not include the routine use of oral aciclovir for uncomplicated varicella in otherwise healthy children; individual cases, however, may justify a \"modest clinical benefit\" from oral aciclovir therapy, provided it can be initiated within the first 24 hours of illness. 2 We describe here an otherwise healthy child with chickenpox who developed a unilateral, unifocal choroiditis with overlying serous detachment of the macula. Barondes et al have, however, described acute retinal necrosis (ARN) in a healthy man 2 weeks after diffuse varicella eruption, where aciclovir and corticosteroids were associated with a favourable outcome. 3 Kelly and Rosenthal also describe multifocal choroiditis in an otherwise healthy adult with primary VZV infection, where oral aciclovir resulted in regression of lesions. 4 The patient described here is unusual in that the choroiditis was unilateral, unifocal, and involved the macula.

In the late 1970s, universal bibliographic control seemed to be a possibility, and an online national bibliography appeared to be within reach. Now, discussions between OCLC and RLG that had the potential to lead to a transparent linking of their databases into a unified national online bibliography have collapsed. This situation indicates that many library directors and consortia heads are not committed to contributing records to a national bibliographic database, nor do they understand why the concept is important. No library is serving its local patrons well unless it is sharing bibliographic information at the national level. Support is being withdrawn from commitment to a national bibliographic database at a time when 2 important new conceptual developments in librarianship have made an online national bibliography critical.

ObjectiveThis study describes core outcomes of Hirschsprung’s disease (HD) in a UK-wide cohort of primary school-aged children.DesignA prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5–8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis.SettingAll 28 UK and Irish paediatric surgical centres.ParticipantsChildren with histologically proven HD diagnosed at <6 months of age.Main outcome measuresNETS1HD core outcomes.ResultsData were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age.Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex.Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD.ConclusionThis study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority.