Reduction of adult height in childhood acute lymphoblastic leukemia survivors after prophylactic cranial irradiation

Background. Impaired linear growth is a well-recognized complication in long-term childhood ALL survivors who received cranial irradiation. However, as many patients achieve a final height between the 5th and the 95th centile, the true incidence of linear growth impairment might be underestimated. Methods. Reduction of adult height (RAH) was estimated in adult childhood ALL survivors with and without cranial irradiation. RAH was calculated as the difference between target height (TH) and final height (FH). TH was calculated according to the formula TH{[(height father + height mother +/- 12)/2] + 3}. RAH was assessed in 79 adult childhood ALL survivors in first complete rernission who had received cranial irradiation 25 Gy (Group I, n=53), 18 Gy (Group II, n=10) or chemotherapy alone (controls, n=16). Results. RAH was 8.6 +/- 8.2 cm in Group I (P=0.001 vs. controls), 6.2 +/- 3.2 cm in Group II (P=0.01 vs. controls), and 1.7 +/- 4.6 in controls (chemotherapy only). There was no significant difference between Group I and Group II. In Group I females had more RAH than males (P=0.02). RAH was related to younger age at diagnosis (P=0.001). Conclusions. The deficit between target height and final height highlights the reduction of adult height in the majority of male and female childhood ALL Survivors who had received prophylactic cranial irradiation, in particular in those who were diagnosed at a younger age. This reduction would have been masked if patients FH was only compared with standard methods. RAH might be a sensitive predictor for growth hormone deficiency as these results suggest that radiation-induced growth hormone deficiency in these patients is the rule rather than the exception. (c) 2005 Wi ley-Liss, Inc

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

A deficiency of essential fatty acids (EFA) is frequently described in cystic fibrosis (CF), but whether this is a primary consequence of altered EFA metabolism or a secondary phenomenon is unclear. It was suggested that defective long-chain polyunsaturated fatty acid (LCPUFA) synthesis contributes to the CF phenotype. To establish whether cystic fibrosis transmembrane conductance regulator (CFM) dysfunction affects LCPUFA synthesis, we quantified EFA metabolism in cftr(-/-CAM) and cftr(+/+CAM) mice. Effects of intestinal phenotype, diet, age, and genetic background on EFA status were evaluated in cftr(-/-CAM) mice, DeltaF508/DeltaF508 mice, and litter-mate controls. EFA metabolism was measured by C-13 stable isotope methodology in vivo. EFA status was determined by gas chromatography in tissues of cftr(-/-CAM) mice, DeltaF508/DeltaF508 mice, littermate controls, and C57B1/6 wild types fed chow or liquid diet. After enteral administration of [C-13]EFA, arachidonic acid (AA) and docosahexaenoic acid (DRA) were equally C-13-enriched in cftr(-/-CAM) and cftr(+/+CAM) mice, indicating similar EFA elongation/desaturation rates. LA, ALA, AA, and DHA concentrations were equal in pancreas, lung, and jejunum of chow-fed cftr(-/-CAM) and DeltaF508/DeltaF508 mice and controls. LCPUFA levels were also equal in liquid diet-weaned cftr(-/-CAM) mice and littermate controls, but consistently higher than in age- and diet-matched C57B1/6 wild types. We conclude that cftr(-/-CAM) mice adequately absorb and metabolize EFA, indicating that CFTR dysfunction does not impair LCPUFA synthesis. A membrane EFA imbalance is not inextricably linked to the CF genotype. EFA status in murine CF models is strongly determined by genetic background.-Werner, A., M. E. J. Bongers, M. J. Bijvelds, H. R. de Jonge, and H. J. Verkade. No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

Patient and public engagement in decision-making regarding infectious disease outbreak management: an integrative review.

INTRODUCTION: Worldwide, people experience the effects of infectious disease outbreaks on a regular basis. These effects vary from direct impact of the virus on health, to indirect impact of control measures on day-to-day life. Yet, incorporating the experiences, views and ideas of patients and the public in decision-making in managing outbreaks does not take place on a structural basis. However, this might be beneficial. We examined the current incorporation of patient and public engagement (PPE) in decision-making regarding outbreak management (OM). METHODS: A systematic search was executed in PubMed, Embase, APA PsycInfo, Web of Science, Scopus and other literature sources. Papers describing PPE in decision-making regarding OM on a collective level (group-level) were included. Relevant information about study characteristics, methods, impact and embedment of PPE in decision-making in OM was collected. RESULTS: The search yielded 4186 papers of which 13 were included. The papers varied in study context and design. Remarkably, no substantial patient engagement was identified. Overall, public engagement (PE) in decision-making regarding OM was mostly executed by a mix of methods, for example, workshops, interviews and surveys. Knowledge and idea sharing between the public and experts was deemed beneficial for establishing well-informed discussions. The efforts resulted in either direct implications for practice or recommendations in policy papers. Most papers described their efforts as a first step. No structural embedment of collective PE in decision-making regarding OM was identified. Furthermore, the quality of most papers was low to moderate due to insufficient description. CONCLUSION: Overall, various practices for PE can be potentially valuable, but structural embedment in OM decision-making on a collective level was low. Before PPE can be permanently embedded in OM, more evidence on its impact needs to be collected. Furthermore, reporting on the engagement process and used terminology needs to be harmonised to ensure reproducibility and transparency

Review about the impact of growing up with a chronic disease showed delays achieving psychosocial milestones

Aim: This review aimed to provide a comprehensive overview of the psychosocial developmental trajectory of various diseases during childhood and adolescence. Methods: Studies of Dutch young adults aged 18-35 years, who had grown up with a chronic disease, were included if the Course of Life Questionnaire had been used to assess psychosocial developmental milestones in three domains: social, autonomy and psychosexual. Differences between the disease groups and the general population were presented as Cohen's d and odds ratios. Results: We included 17 studies comprising 1899 young adults, who had grown up with 18 different paediatric diseases. Psychosocial development was delayed in all three questionnaire domains. Remarkable findings with regard to specific milestones were as follows: less participation in sports clubs in the social domain, less likely to have had paid jobs in the autonomy domain and later sexual intimacy in the psychosexual domain. End-stage renal disease, galactosaemia (males), childhood cancer and orthotopic liver transplants were the most affected disease groups. Conclusion: Children and adolescents with chronic diseases risked delays in psychosocial development. This should be addressed by healthcare providers, along with the physical aspects of diseases, and they should focus on the optimal psychosocial development of the patient