Search

ObjectivesGrowth impairment is a common manifestation in Noonan syndrome (NS). Recombinant human GH (rhGH) treatment has been shown to increase growth and adult height (AH) in a few studies. We aimed to evaluate the growth trajectory towards the AH, and the effects of rhGH treatment in a large cohort of NS children.MethodsRetrospective, multicenter, cohort study including subjects with genetic diagnosis of NS. A total of 228 NS patients, 154 with PTPN11 mutations, 94 who reached AH, were recruited. Auxological data were collected at 2, 5, and 10 years, at pubertal onset, at AH. Sixty-eight NS subjects affected with GH deficiency (GHD) were treated with rhGH at a mean dose of 0.24 mg/kg per week until AH achievement.ResultsANOVA analysis showed a significant difference between birth length and height standard deviation scores (HSDS) at the different key ages (p

Objectives: Growth impairment is a common manifestation in Noonan syndrome (NS). Recombinant human GH (rhGH) treatment has been shown to increase growth and adult height (AH) in a few studies. We aimed to evaluate the growth trajectory towards the AH, and the effects of rhGH treatment in a large cohort of NS children. Methods: Retrospective, multicenter, cohort study including subjects with genetic diagnosis of NS. A total of 228 NS patients, 154 with PTPN11 mutations, 94 who reached AH, were recruited. Auxological data were collected at 2, 5, and 10 years, at pubertal onset, at AH. Sixty-eight NS subjects affected with GH deficiency (GHD) were treated with rhGH at a mean dose of 0.24 mg/kg per week until AH achievement. Results: ANOVA analysis showed a significant difference between birth length and height standard deviation scores (HSDS) at the different key ages (p<0.001), while no significant differences were found between HSDS measurements at 2, 5, and 10 years, at pubertal onset, and at AH. HSDS increased from −3.10 ± 0.84 to −2.31 ± 0.99 during rhGH treatment, with a total height gain of 0.79 ± 0.74, and no significant difference between untreated and treated NS at AH. Conclusions: rhGH treatment at the standard dose used for children with GH idiopathic deficiency is effective in improving growth and AH in NS with GHD. Further studies are needed to assess genotype-specific response to rhGH treatment in the different pathogenic variants of PTPN11 gene and in the less common genotypes. [ABSTRACT FROM AUTHOR]

The article presents offers information on a study on quaternion-based coordinates and equations of motion and the formulation on the low-thrust orbit-raising problem. Topics covered include the equations of motion for a single particle in orbit about the Earth, formulations using spherical coordinates, and how singularities can be removed by using quaternions to replace the usual two angle coordinates.

Introduction An institutional Burn Intensive Care Unit (ICU) Rounding Tool was implemented for both quality improvement data collection and trainee education. Forms are completed by trainee team members daily for all critically ill patients prior to teaching rounds. Gathered data are structured to facilitate evaluation of patient volume status, sepsis risk and ventilator management. The forms are then used to guide discussion among students, residents, mid-level providers, fellows, attending surgeons and other members of the multi-disciplinary rounding team. We conducted a series of interviews with trainees regarding their user experience to assess the tool's educational utility. Methods A convenience sample of residents who had recently completed their Burn Surgery rotation were interviewed in a structured format. Questions focused on the form's ease of use and comprehensibility. Emphasis was placed on the extent to which it stimulates learning while rounding. Additional feedback was sought for the purpose of improving the tool for continued use. Results Participants were post-graduate year one or two resident physicians training in General Surgery, Urology, Emergency Medicine or Anesthesia. Prior experience and comfort with ICU level care ranged from very little to moderate. All interviewees found the tool helpful in their patient assessments, though the current layout of the form was a frustration for many. Overall, they unanimously found the forms beneficial for preparation of patient presentations and felt that utilization during rounds facilitated learning. Conclusions Interviews with trainees on their burn surgery rotation demonstrate that implementation of an ICU Rounding Tool has provided educational benefit, particularly for those less experienced in intensive care. Feedback from this cohort will be used to improve the tool's usability. Our next steps will include a more formal survey of all trainees involved since the inception of this project. [ABSTRACT FROM AUTHOR]

Introduction Sepsis following critical burn injury is an ominous development that can lead to death. Most patients will manifest a systemic inflammatory response syndrome (SIRS), even without being septic. This may obscure the clinical recognition of developing sepsis and delay the initiation of effective treatment. We developed a burn sepsis screening tool (BSST) to facilitate the recognition of developing burn sepsis. The purpose of this study was to review the utility of that tool. Methods The BSST was based on several clinical signs, laboratory values, and changes in physiologic support modalities associated with sepsis. It consisted of nine parameters that could be scored as indicating or not indicating sepsis or not applicable. If three were positive, the patient was identified as septic, and a search for a septic source was undertaken and treatment initiated. The BSST was completed on patients judged to be critically ill during morning rounds over a period of nine months. The values were transcribed into a secure web database and analyzed using SAS 9.4. Results There were 593 individual encounters on 31 critically ill patients with burns and/or inhalation injury for which the BSST was completed. The mean age of the patients was 57 ± 4 years (Mean ± SEM), and the burn size was 24 ± 15 % TBSA. Eleven patients were women (36%) and 7 patients had inhalation injury (23%). The expected case fatality was 21 - 30% depending on the statistical model used. Six patients (19%) died. The length of stay was 64 ± 10 days and ranged from 3 to 267 days. A patient was judged to be septic in only 45 of the daily encounters (8.0 % ± 1.1). There were 21 instances of a new septic event made in 12 patients. Episode of sepsis separated by at least 5 days of no sepsis, were considered to be a new septic event. There was a substantial amount of data that was missing or not applicable. There were no significant differences in the septic parameters on days with new sepsis diagnosis when compared to the day prior, or compared to all encounters in patients that were never septic. Patients deteriorated acutely between BSST completions on only two occasions and both were stabilized. Conclusions The BSST was used consistently to help evaluate the daily status of critically ill burn injured patients. The expected case fatality of the group was moderately high, based on statistical models derived from the ABA Burn Registry. The observed outcome was as good as or better than predicted. Acute decompensation was rare. The BSST added daily administrative work to rounds, and the data recorded were often incomplete. Although the BSST did not demonstrate any single clinical feature that identified the transition from SIRS to sepsis, it did add structure and rigor to daily rounds. That contributed to the effectiveness of rounds, and it may have been responsible, in part, for the favorable outcomes. [ABSTRACT FROM AUTHOR]