Craniosynostosis With Preoperative Iron Supplementation Exposure: A Retrospective Cohort Study Examining Preoperative Iron Supplementation and Transfusion Practice in a National Paediatric Craniofacial Centre.

BACKGROUND: Blood loss and subsequent transfusion are key concerns in the surgical management of craniosynostosis, and have been associated with increased morbidity, requirement for intensive care admission and increased length of hospital stay. Patient blood management guidelines advocate treatment of anemia before elective surgical procedures where significant blood loss is anticipated. At present there is little evidence in the literature investigating the clinical value of this practice in pediatric craniofacial surgery. AIMS: The authors examined the effect of preoperative oral iron supplementation on blood loss and transfusion rates in a national pediatric craniofacial unit. METHODS: A total of 157 patients were included in a retrospective and prospective observational cohort study conducted between July 2011 and November 2016. Eighty-five (85) patients included in the preoperative iron supplementation group were prescribed oral ferrous fumarate before total cranial vault reconstruction, frontal-orbital advancement or extended strip cranial vault remodeling procedures. This cohort was retrospectively compared to seventy-two (72) consecutive patients who did not receive iron supplementation. RESULTS: Calculated blood loss was 51.3 mL/kg in the intervention group, and 56.65 mL/kg in the control group. Transfusion rate and mean volumes for the intervention group were 85.9% and 25 mL/kg. The control group had transfusion rate of 86.1% with mean transfused volume of 24.7 mL/kg. These differences were not statistically significant. Intraoperative tranexamic acid was associated with significantly reduced transfusion volumes overall. CONCLUSIONS: This study did not show a statistically significant benefit to preoperative iron supplementation. Secondary outcomes of this study showed a statistically significant difference in estimated versus calculated intraoperative blood loss. Further research in to specific iron supplementation protocols is indicated.

Maple syrup urine disease: Clinical outcomes, metabolic control, and genotypes in a screened population after four decades of newborn bloodspot screening in the Republic of Ireland.

Since 1972, 18 patients (10 females/8 males) have been detected by newborn bloodspot screening (NBS) with neonatal-onset maple syrup urine disease (MSUD) in Ireland. Patients were stratified into three clusters according to clinical outcome at the time of data collection, including developmental, clinical, and IQ data. A fourth cluster comprised of two early childhood deaths; a third patient died as an adult. We present neuroimaging and electroencephalography together with clinical and biochemical data. Incidence of MSUD (1972-2018) was 1 in 147 975. Overall good clinical outcomes were achieved with 15/18 patients alive and with essentially normal functioning (with only the lowest performing cluster lying beyond a single SD on their full scale intelligence quotient). Molecular genetic analysis revealed genotypes hitherto not reported, including a possible digenic inheritance state for the BCKDHA and DBT genes in one family. Treatment has been based on early implementation of emergency treatment, diet, close monitoring, and even dialysis in the setting of acute metabolic decompensation. A plasma leucine ≥400 µmol/L (outside therapeutic range) was more frequently observed in infancy or during adolescence, possibly due to infections, hormonal changes, or noncompliance. Children require careful management during metabolic decompensations in early childhood, and this represented a key risk period in our cohort. A high level of metabolic control can be achieved through diet with early implementation of a "sick day" regime and, in some cases, dialysis as a rescue therapy. The Irish cohort, despite largely classical phenotypes, achieved good outcomes in the NBS era, underlining the importance of early diagnosis and skilled multidisciplinary team management.

Opportunities taken: the need for and effectiveness of secondary care opportunistic immunisation.

AIM: To evaluate the effectiveness of a formalised opportunistic immunisation (OI) system in a hospital setting. METHODS: Pre-post implementation audit of missed immunisation opportunities. RESULTS: Of 5583 children in the National Immunisation Register cohort seen in a hospital setting, 1641 (29.4%) were under-immunised, compared with the concurrent regional cohort of 15%. Maori children were less likely to be age-appropriately immunised (36.9% under-immunised, P < 0.0005, χ(2) = 41.4). Of the 1641 under-immunised children, 337 (20.5%) were deemed to have current medical reasons not to be immunised acutely, and of the remaining 1304, 244 (18.7%) declined immunisations. This left 1060 for whom immunisation was possible, and we immunised 880 (83.0%) of these. All children were re-engaged with primary care services. CONCLUSIONS: Children in contact with secondary care services have low immunisation rates with ethnic disparity. Appropriately resourced formalised OI is effective, with potential for further improvement. The system we have implemented enhances primary care involvement.

Post-operative opiate requirements after hip arthroscopy.

PURPOSE: Although hip arthroscopy continues to evolve in its use and its popularity grows, little attention has been paid to the anaesthetic and analgesic management of patients undergoing this procedure. We aimed to report on our initial experience of the anaesthetic and analgesic requirements of a consecutive series of patients undergoing hip arthroscopy. METHODS: We reviewed the surgical and anaesthetic records of the initial, consecutive 85 patients undergoing hip arthroscopy for any reason at our hospital. Basic demographics, intra-operative findings, operative procedures, analgesic requirements in the form of opiate requirement and post-operative pain scores were reviewed. RESULTS: The mean intra-operative morphine dose was 7.1 mg (S.D. 3.2 mg). Thirty-nine patients (46%) required I.V. morphine at in the recovery room post-arthroscopy (mean 1.8 mg; S.D. 2.6 mg). Regression analysis showed that lower intra-operative opiate dose resulted in higher maximum VAS pain scores (P = 0.03) and rescue intravenous opiate (P < 0.001) requirement post-surgery. CONCLUSIONS: Adequate intra-operative morphine use can minimize post-operative pain and subsequent need for rescue analgesia.

Morphological responses of Daphnia pulex to Chaoborus americanus kairomone in the presence and absence of metals.

Daphnia pulex neonates develop neck teeth in the presence of predatory kairomone from Chaoborus americanus that are fed D. pulex. These neck teeth reduce the susceptibility of the neonates to predation. Evidence suggests that aqueous metals interfere with chemical communication in fish. The objective of our study was to determine if Cu or Ni at environmentally relevant concentrations affects predatory kairomone response in D. pulex. To test this possibility, D. pulex were placed in increasing waterborne concentrations of Cu or Ni in the presence or absence of predatory kairomone. Both Cu and Ni reduced neck tooth induction in D. pulex neonates in the presence of predatory kairomone. Copper had a significant nonlinear effect on neck tooth length consistent with a hormetic response, where neck tooth length was highest at 5 microg/L Cu, but not significantly different than 0 microg/L Cu at higher Cu concentrations. A Ni concentration of 200 microg/L caused D. pulex to become hypersensitive to Chaoborus regardless of Chaoborus' diet, leading to increased neck tooth number but decreased neck tooth length. Neither Ni nor Cu produced any significant effects on body length or brood size. These results suggest that metal inhibition of neck tooth induction probably occurs along the signal transduction pathway. Impairment of chemosensory response to predatory chemical cues may have widespread ecological consequences in aquatic systems contaminated by metals.

Analgesic control after hip arthroscopy: a randomised, double-blinded trial comparing portal with intra-articular infiltration of bupivacaine.

The optimum anaesthetic and analgesic management following hip arthroscopy is yet to be determined. There is, in addition, some concern over the use of intraarticular local anaesthetic. We compared the analgesic efficacy of intra-articular infiltration compared with portal infiltration of bupivacaine following hip arthroscopy. Patients were randomised to receive either 10 ml of 0.25% bupivacaine either into the joint or around the portal sites following completion of surgery. 73 patients were recruited (40 intra-articular). The portal infiltration group required significantly more rescue analgesia immediately after surgery (2.33 mg vs.0.57 mg, p=0.036). Visual Analogue Scale pain scores were not significantly different at 1 and 2 hours following surgery, but at 6 hours the portal group had significantly lower VAS scores (p=0.0036). We believe that the initial pain following surgery results from capsular injury and this explains the need for more rescue analgesia in the portal infiltration group. Further work is needed to establish the ideal regimen. A combination of portal and intra-articular infiltration may be the most efficacious.

Pilot survey of NICU nurses' interest in the neonatal nurse practitioner role.

PURPOSE: This descriptive, qualitative pilot study explored the interest and perceptions of neonatal intensive care unit (NICU) nurses regarding the neonatal nurse practitioner (NNP) role. Motivating factors to become an NNP, challenges facing NNPs, and rewards of the NNP role from the perspectives of NICU nurses were explored. SUBJECTS: The convenience sample was obtained using 2 survey techniques. The first sample group included nurses who were employed in Level III NICUs located within 2 major Midwestern cities. In order to confirm the data and to expand the scope, the second sample group was recruited from NICU nurses who were attending a regional educational conference. All participants were currently employed NICU nurses and were therefore potential NNP students. Combining the participants of both enrollment techniques resulted in a potential of 696 subjects. DESIGN AND METHODS: A simple self-administered survey was used to collect data. Narrative data were qualitatively analyzed. Demographic data and categorical items were quantified. RESULTS: This study achieved a total 30% response rate (n = 209). Of the total participants, only 32% of Level III NICU nurses were interested in becoming an NNP. Analysis of the data revealed 6 major categories (themes) of reasons why nurses were not interested in the NNP role. The themes most often mentioned by the participants were (1) obligations to family and/or work (46%) and (2) too much responsibility in the NNP role (30%). The data also revealed several different rewards and challenges for those in the NNP role as well as factors that may motivate nurses to become an NNP. CONCLUSIONS: Given the current NNP shortage, an increase in the supply of NNPs for the workforce is imperative. Current enrollment in NNP academic programs does not appear to be meeting the demand. Exploring the factors that influence enrollment in NNP programs from the perspective of potential NNP students is the first step towards increasing the supply of NNPs. The majority of participants were not interested in becoming an NNP for a variety of reasons. Negative perceptions of the NNP role were identified. Solutions posed from these results may provide scientifically sound solutions to help ease the shortage of NNPs. The findings of this naturalistic inquiry may be used to develop an instrument to measure interest in the NNP role.