Further investigations revealed her ADAMTS13 (a metalloproteinase that cleaves von Willebrand factor) activity to be <5, confirming the diagnosis of TTP. ADAMTS13 activity of 68-163% is considered normal and activity of less than 10% is diagnostic of TTP. She is currently monitored as an outpatient and continues to have Rituximab for subclinical relapses. Conclusion Bleeding of any form is an extremely rare and severe presentation of TTP. There are only a few case reports of TTP in children till date but there are no reported paediatric cases of symptomatic bleeding as initial presentation. Acquired TTP is mainly reported in adolescence. TTP is specifically related to severe ADAMTS13 deficiency which is also used to monitor disease activity.
Aims To assess baseline paediatric career intentions and the impact of a student-led paediatric society (PS), which aims to increase student exposure and encourage recruitment to paediatrics. The PS was established in 2009 and runs career events, paediatric/neonatal resuscitation training days, Teddy Bear Hospital (TBH) sessions and provides information for electives. Methods Clinical students (2011) joining the PS completed a baseline career choice questionnaire during their introductory week. After attending PS events, students completed anonymous feedback questionnaires to assess changes in attitudes towards paediatrics. Participation in TBH, paediatric electives and student selected components (SSC) were used as other indicators of interest in paediatrics. Results The PS has 288 members, representing 64% of clinical students. 53 students responded to the baseline careers questionnaire; of these, 7 (13%) planned to specialise in paediatrics and mean certainty about their future career choice was 2.5 (5-point Likert scale). 30 students attended our careers event in November 2011 and 16 completed the feedback questionnaire; 8 were certain they wished to specialise in paediatrics and a further 8 considering paediatrics. The mean value for certainty about their future career was 3.2 (5-point Likert scale). Students (n=30) attending our Paediatric / Neonatal resuscitation training day rated it a mean of 100% for satisfaction and 97% for increased confidence in dealing with future emergency scenarios. The PS organised 8 TBH health education events for local primary schools, involving over 100 medical student volunteers and 250 children. Over the last 4 years the number of students undertaking paediatric electives has increased from 26 to 42 and SSCs from less than 10 in 2007 to 34 in 2011. Conclusions The majority of students entering clinical medicine are unsure about their future career choice, but many consider paediatrics as an option. Events run by the PS with supportive consultants are popular. Increasing student involvement in TBH, paediatric electives and SSCs has increased the profile of paediatrics at this medical school.
Aims Growth prediction models are thought to be helpful in detecting children with Idiopathic Growth Hormone Deficiency (IGHD) with poor response to recombinant human growth hormone (rhGH) therapy. This study aims to determine adequacy of rhGH therapy responses in the first two years of treatment in children with IGHD by comparing with a validated prediction model. Methods Growth records of IGHD patients started on rhGH therapy between 1999 and 2009 at single tertiary endocrinology centre were analysed in order to calculate pre-treatment, year one and year two height velocities (HVs). HVs were expressed as standard deviation score (SDS). Growth prediction formula determinants (age at onset, birth weight SDS, height SDS, weight SDS, mid-parental height SDS, maximum GH on stimulation test and rhGH dose) were obtained. Observed and predicted HVs were compared using an unpaired t-test. Results Of 54 patients identified, 18 pre-pubertal children (median (range) age 10 (4-16) years; 13 male) had sufficient data for HV analysis (figure 1). Abstract G240(P) Figure 1 The mean HV SDS (SD) for pretreatment, year one and year two were –1.14 (2.57), 2.75 (2.61) and 2.91 (3.68) respectively In year one, 3 of the 18 patients received an rhGH dose below recommended national prescribing guidelines (0.7–1.0 mg/m2/day), increasing to 5 patients in year two. Significant differences between observed and predicted HVs were observed in year one of rhGH therapy (p=<0.01), but not in year two (p=0.19) (n=11). 55% achieved HVs below the predicted range. There was no relationship between the observed and predicted HV discrepancy and the rhGH dose, suggesting that patient compliance may be a factor in the failure to attain predicted HVs. In year one, baseline pre-treatment insulin-like growth factor-1 (IGF-1) levels were negatively correlated to the difference between observed and predicted HV (figure 2). Abstract G240(P) Figure 2 Relationship between the difference between expected and observed HVS and baseline IGF-1 (r=–0.69) Conclusion In children with IGHD receiving rhGH therapy: Improvements in HV were observed, although considerable variability is seen particularly in year two. Prediction models do not appear to give an accurate year one growth prediction. Baseline IGF-1 level may be a valuable parameter for future growth prediction formulae. Close attention to rhGH dose is needed in order to optimise growth responses. Abstract G240(P) Table 1Mean (SD) rhGH dose, mg/m2/day Year One 0.75 (0.11) Year Two 0.75 (0.15)
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.