Parental holding of infants improves haemodynamics in the cardiac ICU.

We performed a single-centre, retrospective study to assess physiologic changes of infants in the cardiac ICU while being held by their parent. Continuous data streaming of vital signs were collected for infants included in the study from January 2021 to March 2022. Demographic and clinical characteristics were collected from the electronic medical record. The physiologic streaming data were analysed using mixed-effects models to account for repeated measures and quantify the effect of parental holding. Comparison analysis was also performed controlling for intubation, pre-operative versus post-operative status, and whether the holding was skin-to-skin or not. Ninety-five patients with complete physiologic data were included in the study. There were no immediate adverse events associated with holding. Heart rate decreased during the response time compared to its baseline value (p = 0.01), and this decrease was more pronounced for the non-intubated and pre-operative patients. The near-infrared spectroscopy-based venous saturation increased overall (p = 0.02) in patients while being held. We conclude that parental holding of infants in the cardiac ICU can be safely accomplished, and the haemodynamic and oximetric profile during the holding is favourable compared to the infants' baseline prior to holding.

Camptodactyly and the 22q11.2 deletion syndrome.

We describe a 5-day-old male with minor facial anomalies, a congenital laryngeal web, severe laryngomalacia, and prominent fixed flexion of the proximal interphalangeal joints of digits 2 through 5 bilaterally. A whole genome SNP microarray analysis identified a 2.55 Mb interstitial deletion of 22q11.21, typical of that seen in the DiGeorge and Velocardiofacial syndromes. A review of the literature identifies 10 other cases with camptodactyly. Camptodactyly appears to be an associated but rarely reported anomaly in patients with the 22q11.2 microdeletion syndrome. © 2016 Wiley Periodicals, Inc.

Mild achondroplasia/hypochondroplasia with acanthosis nigricans, normal development, and a p.Ser348Cys FGFR3 mutation.

Pathogenic allelic variants in the fibroblast growth factor receptor 3 (FGFR3) gene have been associated with a number of phenotypes including achondroplasia, hypochondroplasia, thanatophoric dysplasia, Crouzon syndrome with acanthosis nigricans (Crouzonodermoskeletal syndrome), and SADDAN (severe achondroplasia with developmental delay and acanthosis nigricans). Crouzon syndrome with acanthosis nigricans is caused by the pathogenic variant c.1172C>A (p.Ala391Glu) in the FGFR3 gene. The p.Lys650Thr pathogenic variant in FGFR3 has been linked to acanthosis nigricans without significant craniofacial or skeletal abnormalities. Recently, an infant with achondroplasia and a novel p.Ser348Cys FGFR3 mutation was reported. We describe the clinical history of an 8-year-old child with a skeletal dysplasia in the achondroplasia-hypochondroplasia spectrum, acanthosis nigricans, typical development, and the recently described p.Ser348Cys FGFR3 mutation.

Is Provider Training Level Associated with First Pass Success of Endotracheal Intubation in the Pediatric Intensive Care Unit?

Endotracheal intubation is a life-saving procedure in critically ill pediatric patients and a foundational skill for critical care trainees. Multiple intubation attempts are associated with increased adverse events and increased morbidity and mortality. Thus, we aimed to determine patient and provider factors associated with first pass success of endotracheal intubation in the pediatric intensive care unit (PICU). This prospective, single-center quality improvement study evaluated patient and provider factors associated with multiple intubation attempts in a tertiary care, academic, PICU from May 2017 to May 2018. The primary outcome was the number of tracheal intubation attempts. Predictive factors for first pass success were analyzed by using univariate and multivariable logistic regression analysis. A total of 98 intubation encounters in 75 patients were analyzed. Overall first pass success rate was 67% (66/98), and 7% (7/98) of encounters required three or more attempts. A Pediatric critical care medicine (PCCM) fellow was the first laryngoscopist in 94% (92/98) of encounters with a first pass success rate of 67% (62/92). Age of patient, history of difficult airway, provider training level, previous intubation experience, urgency of intubation, and time of day were not predictive of first pass success. First pass success improved slightly with increasing fellow year (fellow year = 1, 66%; fellow year = 2, 68%; fellow year = 3, 69%) but was not statistically significant. We identified no intrinsic or extrinsic factors associated with first pass intubation success. At a time when PCCM fellow intubation experience is at risk of declining, PCCM fellows should continue to take the first attempt at most intubations in the PICU.

Comfort and Coordination among Interprofessional Care Providers Involved in Intubations in the Pediatric Intensive Care Unit.

Background: Successful execution of invasive procedures in acute care settings, including tracheal intubation, requires careful coordination of an interprofessional team. The stress inherent to the intensive care unit (ICU) environment may threaten the optimal communication and planning necessary for the safe execution of this complex procedure. The objective of this study is to characterize the perceptions of interprofessional team members surrounding tracheal intubations in the pediatric ICU (PICU). Methods: This is a single-center survey-based study of staff involved in the intubation of pediatric patients admitted to a tertiary level academic PICU. Physicians, nurses, and respiratory therapists (RT) involved in tracheal intubations were queried via standardized, discipline-specific electronic surveys regarding their involvement in procedural planning and overall awareness of and comfort with the intubation plan. Qualitative variables were assessed by both Likert scales and free-text comments that were grouped and analyzed thematically. Results: One hundred and eleven intubation encounters were included during the study time period, of which 93 (84%) had survey responses from at least 2 professional teams. Among those included in the analysis, the survey was completed 244 times by members of the PICU teams including 86 responses from physicians, 76 from nurses, and 82 from RTs. Survey response rates were >80% from each provider team. There were significant differences in interprofessional team comfort with nurses feeling less well informed and comfortable with the intubation plan and process compared to physicians and RTs (p < 0.001 for both). Qualitative themes including clear communication, adequate planning and preparation prior to procedure initiation, and clear definition of roles emerged among both affirmative and constructive comments. Conclusions: Exploration of provider perceptions and emergence of constructive themes expose opportunities for teamwork improvement strategies involving intubations in the PICU. The use of a preintubation checklist may improve organization and communication amongst team members, increase provider morale, decrease team stress levels, and, ultimately, may improve patient outcomes during this high stakes, coordinated event.

Neurodevelopmental Outcomes in Infants With Cardiac Surgery Associated Acute Kidney Injury.

BACKGROUND: Infants who undergo surgery for congenital heart disease are at risk of neurodevelopmental delay. Cardiac surgery-associated acute kidney injury (CS-AKI) is common but its association with neurodevelopment has not been explored. METHODS: This was a single-center retrospective observational study of infants who underwent cardiac surgery in the first year of life who had neurodevelopmental testing using the Bayley Scale for Infant Development, third edition. Single and recurrent episodes of stages 2 and 3 CS-AKI were determined. RESULTS: Of 203 children with median age at first surgery of 12 days, 31% had one or more episodes of severe CS-AKI; of those, 16% had recurrent CS-AKI. Median age at neurodevelopmental assessment was 20 months. The incidence of delay was similar for patients with and patients without CS-AKI but all children with recurrent CS-AKI had a delay in one or more domains and had significantly lower scores in all three domains, namely, cognitive, language, and motor. CONCLUSIONS: This study has assessed the association of CS-AKI with neurodevelopmental delay after surgery for congenital heart disease in infancy. Infants who have recurrent CS-AKI in the first year of life are more likely to be delayed and have lower neurodevelopmental scores.

The Neglected Price of Pediatric Acute Kidney Injury: Non-renal Implications.

Preclinical models and emerging translational data suggest that acute kidney injury (AKI) has far reaching effects on all other major organ systems in the body. Common in critically ill children and adults, AKI is independently associated with worse short and long term morbidity, as well as mortality, in these vulnerable populations. Evidence exists in adult populations regarding the impact AKI has on life course. Recently, non-renal organ effects of AKI have been highlighted in pediatric AKI survivors. Given the unique pediatric considerations related to somatic growth and neurodevelopmental consequences, pediatric AKI has the potential to fundamentally alter life course outcomes. In this article, we highlight the challenging and complex interplay between AKI and the brain, heart, lungs, immune system, growth, functional status, and longitudinal outcomes. Specifically, we discuss the biologic basis for how AKI may contribute to neurologic injury and neurodevelopment, cardiac dysfunction, acute lung injury, immunoparalysis and increased risk of infections, diminished somatic growth, worsened functional status and health related quality of life, and finally the impact on young adult health and life course outcomes.