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Kinsler V.,Institute of Child Health
Pediatric Dermatology | Year: 2011

The term "satellite lesions" is used in many conditions in dermatology, generally to describe smaller lesions near the edges of a principal lesion. An online medical dictionary gives the definition "a smaller lesion accompanying a main one and situated nearby," and this can apply both macroscopically and microscopically. The implication is that the smaller lesions have spread from the parent lesion. Given this definition and usual understanding of the term its use is not apt in the case of congenital melanocytic nevi (CMN). In the vast majority of cases where the patient is said to have satellite lesions these are not restricted to the area around or near the edge of the principal lesion, and are not necessarily significantly smaller. It seems likely that the early use of the term in this condition is an adaptation of the established and correct use in the context of melanoma. Not only is the term not apt clinically but has no known etiological basis. This leaves us with the question of what to call these lesions in cases of CMN. This proposal is to categorise cases into single or multiple CMN, where multiple is 2 or more nevi of any size at birth. An accurate count or good estimate of the number of lesions at birth should also be recorded, and the largest lesion classified as usual with respect to projected adult size. © 2011 Wiley Periodicals, Inc.

Jones L.,Institute of Child Health
Cochrane database of systematic reviews (Online) | Year: 2010

BACKGROUND: Malnutrition can often be a consequence of cancer itself or a result of chemotherapy. Nutritional support aims to reverse malnutrition seen at diagnosis, prevent malnutrition associated with treatment and promote weight gain and growth. The most effective and safe forms of nutritional support in children and young people with cancer are unclear. OBJECTIVES: To determine the effects of any form of parenteral (PN) or enteral (EN) nutritional support in children and young people with cancer undergoing chemotherapy. SEARCH STRATEGY: We searched the following databases: CENTRAL (The Cochrane Library 2009, Issue 2), MEDLINE (1950 to 2006), EMBASE (1974 to 2006), CINAHL (1982 to 2006), the National Research Register (2007) and Dissertations & Theses (2007). We scrutinised reference lists of articles to identify additional trials. We also contacted experts in the field for information on relevant trials. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials comparing any form of nutritional support with another, or control, in children or young people with cancer undergoing chemotherapy. DATA COLLECTION AND ANALYSIS: Two authors independently selected trials. Three authors independently assessed quality and extracted data. We contacted trialists for missing information. MAIN RESULTS: We included eight trials which randomised 159 participants (< 21 years) with leukaemias or solid tumours undergoing chemotherapy. The trials were all of low quality. One small trial found that compared to EN, PN significantly increased weight (mean difference (MD) 4.12; 95% CI 1.91 to 6.33), serum albumin levels (MD 0.70; 95% CI 0.14 to 1.26), calorie intake (MD 22.00; 95% CI 5.12 to 38.88) and protein intake (MD 0.80; 95% CI 0.45 to 1.15). One trial comparing peripheral PN and EN with central PN found that mean daily weight gain (MD -27.00; 95% CI -43.32 to -10.68) and energy intakes (MD -15.00; 95% CI -26.81 to -3.19) were significantly less for the peripheral PN and EN group, whereas mean change in serum albumin was significantly greater for that group(MD 0.47; 95% CI 0.13 to 0.81, P = 0.008). AUTHORS' CONCLUSIONS: There is limited evidence from individual trials to suggest that parenteral nutrition is more effective than enteral nutrition in well-nourished children and young people with cancer undergoing chemotherapy. The evidence for other methods of nutritional support remains unclear. No studies were identified comparing the nutritional content in the PN or EN groups of studies. Further research, incorporating larger sample sizes and rigorous methodology utilising valid and reliable outcome measures, is essential.

Solebo A.L.,Institute of Child Health
Cochrane database of systematic reviews (Online) | Year: 2011

Macular holes cause significant loss of central vision. With the aim of improving the outcome of surgery, a variable period of face-down positioning may be advised. To evaluate the evidence of the impact of postoperative face-down positioning on the outcome of surgery for macular hole. We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2011, Issue 8), MEDLINE (January 1950 to August 2011), EMBASE (January 1980 to August 2011), the International Standard Randomised Controlled Trial Number Register (ISRCTN Register) (http://www.controlled-trials.com), the WHO International Clinical Trials Registry Platform (ICTRP) (http://www.who.int/ictrp/search/en) and ClinicalTrials.gov (http://clinicaltrials.gov). There were no date or language restrictions in the electronic searches for trials. The electronic databases were last searched on 29 August 2011. We included randomised controlled trials (RCTs) in which postoperative face-down positioning was compared to no face-down positioning following surgery for macular holes. Data were collected and analysed independently by two authors. Three RCTs were identified, A, B and C; one of which was unpublished data. We were unable to conduct a meta-analysis due to study heterogeneity regarding duration of face-down positioning and surgical methods (use of inner limiting peel). All three studies suggested an overall beneficial effect of posturing in terms of closure of holes: (A: risk ratio (RR) 1.10; 95% confidence interval (CI) 1.00 to 1.20, P = 0.05); B: RR 1.58, CI 1.0 to 2.5, P = 0.01; C: RR 1.03, CI 0.9 to 1.17, P = 0.67). For holes which were smaller than 400 microns in size, all three studies reported that there was no significant effect of face-down positioning on successful hole closure (A: RR 1.03, CI 0.95 to 1.12; B: RR 1.0, CI 0.68 to 1.46; C: RR 1.03, CI 0.9 to 1.17). However, for holes which were larger than 400 microns in size, both of the studies which examined macular holes of this size agreed on the effectiveness of face-down positioning on hole closure following surgery (A: RR 1.2, CI 1.01 to 1.42, P = 0.04; B: RR 2.27, CI 1.04 to 4.97, P = 0.04). There is currently insufficient evidence from which to draw firm conclusions about the impact of postoperative face-down positioning on the outcome of surgery for macular hole. Of three RCTs, two suggested a benefit in larger holes but none demonstrated evidence of a benefit in smaller holes.CONSORT adherent RCTs and large scale, well designed non-randomised observational studies are needed to determine with confidence the value of this intervention.

Sewry C.A.,Institute of Child Health | Sewry C.A.,Wolfson Center for Inherited Neuromuscular Diseases
Acta Neuropathologica | Year: 2010

Muscular dystrophies are clinically, genetically, and molecularly a heterogeneous group of neuromuscular disorders. Considerable advances have been made in recent years in the identification of causative genes, the differentiation of the different forms and in broadening the understanding of pathogenesis. Muscle pathology has an important role in these aspects, but correlation of the pathology with clinical phenotype is essential. Immunohistochemistry has a major role in differential diagnosis, particularly in recessive forms where an absence or reduction in protein expression can be detected. Several muscular dystrophies are caused by defects in genes encoding sarcolemmal proteins, several of which are known to interact. Others are caused by defects in nuclear membrane proteins or enzymes. Assessment of both primary and secondary abnormalities in protein expression is useful, in particular the hypoglycosylation of alpha-dystroglycan. In dominantly inherited muscular dystrophies it is rarely possible to detect a change in the expression of the primary defective protein; an exception to this is caveolin-3. © 2010 Springer-Verlag.

Wells J.C.K.,Institute of Child Health
Reviews in Endocrine and Metabolic Disorders | Year: 2012

The consequences of fetal growth retardation remain unclear, in part because they appear to vary between industrialized and developing countries. Data on body composition offer a new opportunity to investigate this issue, and may be of particular value in addressing the controversial role of nutrition in infancy, which has been proposed by some to boost survival, and by others to increase long-term risk of chronic diseases. The uncertainty regarding the effects of post-natal nutrition is presenting challenges to nutritional policy as many countries undergo the nutrition transition, whereby the nutritional status of individuals may shift within the life-course. A theoretical model, building on the thrifty phenotype hypothesis, is presented to clarify how body composition data can address this dilemma. Measurements of body composition can now be obtained in infants and children using several different technologies, indicating that large-scale studies can now be conducted to investigate objectively the association between early growth patterns and later health. © Springer Science+Business Media, LLC 2012.

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