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Genetic disorders are a leading contributor to mortality in neonatal and pediatric intensive care units (ICUs). Rapid whole-genome sequencing (rWGS)-based rapid precision medicine (RPM) is an intervention that has demonstrated improved clinical outcomes and reduced costs of care. However, the feasibility of broad clinical deployment has not been established. The objective of this study was to implement RPM based on rWGS and evaluate the clinical and economic impact of this implementation as a first line diagnostic test in the California Medicaid (Medi-Cal) program. Project Baby Bear was a payor funded, prospective, real-world quality improvement project in the regional ICUs of five tertiary care children's hospitals. Participation was limited to acutely ill Medi-Cal beneficiaries who were admitted November 2018 to May 2020, were

Pediatric palliative home-based care has been shown to improve symptoms, quality of life, and coordination of care. Despite these successes, hospital utilization in our own palliative home-based care population remained high as some caregivers lacked confidence to manage symptoms at home and had difficulty in recalling or accessing "sick care plans." Our team developed the Symptom Management Plan (SMP), a multi-system "sick care plan," as a quality improvement project with the aim of improving caregiver confidence to manage symptoms at home. An Electronic Health Record-based SMP template was created for common symptoms: respiratory distress, seizures, feeding intolerance, and constipation with core subspecialists' input. Individualized SMPs were created and reviewed with caregivers at every subsequent palliative home nursing visit. Caregivers were surveyed on their confidence 3 and 6-months post-implementation. Resource utilization was analyzed throughout implementation. At 6 months, 73% of caregivers reported "better" or "much better" confidence in managing their child's symptoms after using the SMP, and 76% of caregivers perceived the SMP prevented urgent care or emergency department (ED) visits. After the SMP was launched, the rate of ED visits decreased from 0.86 to 0.47 per 100 patient-days, and admissions decreased from 0.56 to 0.39 per 100 patient-days. These rates further decreased to 0.31 ED visits and 0.19 admissions per 100 patient-days within 4 and 6 months. Introducing the SMP for our home-based palliative care patients was associated with improved caregiver confidence in managing acute symptoms at home and a reduction in hospital utilization.

Objectives:Genetic disorders are a leading contributor to mortality in the neonatal ICU and PICU in the United States. Although individually rare, there are over 6,200 single-gene diseases, which may preclude a genetic diagnosis prior to ICU admission. Rapid whole genome sequencing is an emerging me

Background: Rady Children's Hospital (RCH) offers an outpatient pediatric palliative clinic that began offering telepalliative care in 2016. Objectives: This study describes demographics of parents...

Children and adolescents living with primary or secondary immunodeficiencies often have an unpredictable prognosis and benefit from palliative care as a standard part of their regular care. This chapter illustrates the challenges faced by children and adolescents living with HIV, which have parallels to issues encountered by children with other immunodeficiencies. The nuances of palliative care management for children living with HIV are detailed according to various aspects of care: physical; structure and process of care of patients and families; psychological and psychiatric; social, cultural, spiritual, religious, and existential; ethical and legal, and care of the patient nearing the end of life.

The diagnostic and clinical utility of rapid whole genome sequencing (rWGS) for critically ill children in the intensive care unit (ICU) has been substantiated by multiple studies, but comprehensive cost-effectiveness evaluation of rWGS in the ICU outside of the neonatal age group is lacking. In this study, we examined cost data retrospectively for a cohort of 38 children in a regional pediatric ICU (PICU) who received rWGS. We identified seven of 17 patients who received molecular diagnoses by rWGS and had resultant changes in clinical management with sufficient clarity to permit cost and quality adjusted life years (QALY) modeling. Cost of PICU care was estimated to be reduced by $184,846 and a total of 12.1 QALYs were gained among these seven patients. The total cost of rWGS for patients and families for the entire cohort (38 probands) was $239,400. Thus, the net cost of rWGS was $54,554, representing $4,509 per QALY gained. This quantitative, retrospective examination of healthcare utilization associated with rWGS-informed medicine interventions in the PICU revealed approximately one-third of a QALY gained per patient tested at a cost per QALY that was approximately one-tenth of that typically sought for cost-effective new medical interventions. This evidence suggests that performance of rWGS as a first-tier test in selected PICU children with diseases of unknown etiology is associated with acceptable cost-per-QALY gained.

To describe the concerns, confidence, and barriers of practicing hospitalists around serious illness communication.Hospitalist physicians are optimally positioned to provide primary palliative care, yet their experiences in serious illness communication are not well described.Web-based survey, conducted in May 2016. The survey link was distributed via email to 4000 members of the Society of Hospital Medicine. The 39-item survey assessed frequency of concerns about serious illness communication, confidence for common tasks, and barriers using Likert-type scales. It was developed by the authors based on prior work, a focus group, and feedback from pilot respondents.We received 332 completed surveys. On most or every shift, many participants reported having concerns about a patient's or family's understanding of prognosis (53%) or the patient's code status (63%). Most participants were either confident or very confident in discussing goals of care (93%) and prognosis (87%). Fewer were confident or very confident in responding to patients or families who had not accepted the seriousness of an illness (59%) or in managing conflict (50%). Other frequently cited barriers were lack of time, lack of prior discussions in the outpatient setting, unrealistic prognostic expectations from other physicians, limited institutional support, and difficulty finding records of previous discussions.Our results suggest opportunities to improve hospitalists' ability to lead serious illness communication by increasing the time hospitalists have for discussions, improving documentation systems and communication between inpatient and outpatient clinicians, and targeted training on challenging communication scenarios.

Boron- and silicon-containing conjugated homo- and copolymers could be synthesized using acyclic diene metathesis (ADMET) condensation of bis-styryl monomers. Both, tri-and tetra-coordinated boron monomers were successfully polymerized forming homopolymers, or random copolymers (if polymerized together with a silicon containing co-monomer). Polymer molecular weights Mn were measured at ∼6000 to 15,000 g/mol (NMR end group analysis) with molecular weight distributions Mw/Mn ∼1.8 to 2.2. The polymers absorbed at λmax ∼317 to 406 nm and emitted at λmax ∼370 to 494 nm with fluorescent quantum efficiencies ∼24 to 48%. The copolymer with tri-coordinate boron showed highly efficient fluorescence quenching in the presence of fluoride ions at ratios boron/fluoride ∼5/1, demonstrating its potential as anion sensor. © 2015 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2015, 53, 1707–1718

BACKGROUND No standard management plan for infants with an apparent life-threatening event (ALTE) currently exists. These infants are routinely hospitalized. Benefits of hospitalization of ALTE patients with gastroesophageal reflux disease (GERD) need definition. OBJECTIVES The study's objectives were to determine the accuracy of a working diagnosis of GERD in infants admitted with ALTE and to describe the history and hospital course of infants with both working and discharge diagnoses of GERD. METHODS Authors retrospectively reviewed records from a large children's hospital of infants aged 1 year old and younger hospitalized from January 1, 2004, to March 1, 2007, with an admission diagnosis of ALTE. Demographics, clinical presentation, testing, hospital course, and 6-month postdischarge visits were abstracted. Intensive care admissions were excluded. Univariate and multivariate analyses identified factors associated with a discharge diagnosis of GERD. RESULTS Three hundred thirteen infants met inclusion. Mean age was 2.1 months; mean length of stay was 2.5 days. A discharge diagnosis of GERD was most common (n = 154, 49%); 138 (89%) were initially well appearing, 10 (6%) had in-hospital events, and only 20 (13%) had upper gastrointestinal series performed. Concordance of initial working to discharge diagnosis of GERD was 96%. Nonconcordant diagnoses evolved within 24 hours. Rescue breaths and calling 911 were independently associated with a discharge diagnosis of GERD. Within 6 months, 14 patients (9%) with a discharge diagnosis of GERD had recurrent ALTE, and 5 (3%) had significant new diagnoses. CONCLUSIONS Concordance of initial working diagnosis with discharge diagnosis of GERD in ALTE patients is high. However, in hospital events, evolution to new diagnoses and recurrent ALTE suggest that hospitalization of these patients is beneficial. Diagnostic studies should not be routine but should target concerns from the history, examination, and hospital course.

The effect of the binding of pyridine ligands to pentafluorophenyl copper, [C6F5Cu]4 (1), on structural features and photophysical properties has been investigated through a combined multinuclear NMR, X-ray crystallography, and photoluminescence study. Reaction of 1 with 2 equiv of pyridine yields a novel pyridine complex, 3, in which the tetranuclear framework of 1 is retained. Complex 3 features a rhombus-shaped tetracopper core with a short diagonal Cu···Cu distance of 2.5941(6) A between the dicoordinate copper centers and a longer distance between the pyridine-coordinated copper centers of 4.178(1) A. In contrast, treatment of 1 with 4 equiv of pyridine results in complete breakdown of the tetranuclear aggregate to give the formally dicoordinate species C6F5Cu(py) (4-H). Reaction of 1 with 2,2′-bipyridine results in formation of the tricoordinate complex C6F5Cu(2,2′-bipy) (5). Aggregate breakdown in species 4 and 5 is reflected in a significantly reduced chemical shift difference Δδ(19Fmeta/para) and...

Polystyrene was functionalized with luminescent fluorenyl- and carbazolylborane pendant moieties. Because of an interaction of the empty p orbital on boron with the extended π-systems of fluorene or carbazole, the resulting polymeric materials exhibit intense blue emission with maxima in the range 390–420 nm. The solution quantum yields were 65% for the fluorene derivative and 68 and 11% for two different carbazole derivatives, respectively. The stability of the borylated polymers was enhanced by attachment of a bulky triisopropylphenyl group to each of the boron centers. Thus, the polymers were found to be stable for a period of over 1 month. Thermal stability up to ca. 250 °C was confirmed by thermogravimetric analysis.

Reaction of the bidentate Lewis acid 1,2-Fc(SnMe 2 Cl)(B(Me)Cl) ( 1 , Fc = 1,2-ferrocenylene) with 2-trimethylstannylpyridine gave a complex ( 2 ), in which the 2-pyridyl group forms a bridge between the Lewis acidic tin and boron centers. The structure of 2 was confirmed by multinuclear and two-dimensional NMR, single crystal X-ray diffraction, and elemental analysis. The crystal structure shows that the pyridyl moiety is positioned endo relative to the substituted Cp ring and UV–Vis spectroscopy revealed an enhancement of the ferrocene-centered dd transition in comparison to related complexes with pyridine as a terminal ligand.

Resolution of the planar-chiral bidentate Lewis acid Fc[B(Cl)Me][SnMe2Cl] (Fc = 1,2-ferrocenediyl) (1) by complexation with pseudoephedrine derivatives was studied. Compound 1 was first converted t...

A series of mixed-valent (MV) complexes [(FeCp) 2 (μ-C 10 H 6 -(BPh) 2 )] + X ([1 + ]X; X=I 5 , PF 6 , SbF 6 , B(C 6 F 5 ) 4 ) were prepared by oxidation of diboradiferrocene [(FeCp) 2 (μ-C 10 H 6 -(BPh) 2 )] (1) with I 2 , AgPF 6 , and AgSbF 6 , respectively, and through anion exchange of the I 5 - salt with [Li-(Et 2 O) x ][B(C 6 F 5 ) 4 ] in the case of X=B-(C 6 F 5 ) 4 . The MV state of the cation was investigated in solution by multi-nuclear NMR spectroscopy, CV, and UV/Vis-NIR absorption spectroscopy, and in the solid state by IR spectroscopy, single-crystal X-ray crystallography, and Mossbauer spectroscopy. The cyclic voltammogram of 1 shows two distinct redox waves with a large redox splitting of ΔE=510 mV in CH 2 Cl 2 and the NIR spectrum for the mono-oxidized species displays an intervalence charge-transfer band at around 1500 to 1700 nm depending on the specific counterion present. The X-ray crystal structures of [1 + ]X show inversion-symmetric cations with X=I 5 and B-(C 6 F 5 ) 4 and unsymmetric valence-trapped structures composed of one ferrocene and one ferrocenium moiety with X=PF 6 and SbF 6 . Mossbauer data for X=PF 6 are consistent with valence trapping at all temperatures between 90 and 343 K. In comparison, fast electron transfer is evident on the Mossbauer timescale for X=I 5 and temperature-dependent behavior is observed for X=B(C 6 F 5 ) 4 . The anion dependence of the X-ray structural and Mossbauer data is discussed in the context of crystal symmetry and the possibility of static and dynamic disorder effects is considered.

A polystyrene derivative in which the para-positions of the phenyl rings are selectively functionalised with BH2 moieties (PS-BH2) has been prepared from poly(4-trimethylsilyl styrene) through a series of polymer analogous reactions. The polymer was successfully isolated in the form of its polymeric acid-base complexes PS-BH2 • D (D = tBuPy, PPh2Me). The selective and nearly quantitative placement of the BH2 functionalities was confirmed by multi-nuclear NMR spectroscopy and IR spectroscopy. The thermal properties of the polymer complexes were investigated by differential scanning calorimetry and thermogravimetric analysis.

This chapter provides an overview of the most significant developments in the area of boron-containing polymers over the past about 10 years. We describe the synthesis, characterization, and applications of new polymeric materials that feature boron as an integral component with a clear effect on the chemical or physical properties. These include (1) organoborane polymers, (2) polymers that contain conjugated boron heterocyles, such as borazane, pyrazabole, bipyridylboronium, boron quinolate, boron diketonate, and 4,4-difluoro-4-bora-3a,4a-diaza- s -indacene moieties, (3) boronic acid and boronic ester-functionalized polymers and related materials, and (4) polymers that feature carboranes and other boron clusters.

Enantiomerically pure metalated 2-(1-naphthyl)ferrocene (NpFc) derivatives NpFcM (M=SnMe 3 , HgCl) were prepared and characterized by multinuclear NMR and UV/Vis spectroscopy, cyclic voltammetry, and elemental analysis. Optical rotation measurements were performed and the absolute configuration of the new planar chiral ferrocene species was confirmed by single-crystal X-ray diffraction analysis. The mercuriated species NpFcHgCl proved suitable as a reagent for the preparation of the chiral organoborane Lewis acid NpFcBCl 2 , which can in turn be converted to other ferrocenylboranes by replacement of Cl with nucleophiles. The highly Lewis acidic perfluoroarylborane derivatives NpFcB(C 6 F 5 )Cl and NpFcB(C 6 F 5 ) 2 were successfully prepared by treatment with CuC 6 F 5 . The structures were studied by single-crystal X-ray diffraction and variable-temperature 19 F NMR spectroscopy, which suggested that π stacking of a C 6 F 5 group on boron with the adjacent naphthyl group is energetically favorable. UV/ Vis absorption spectroscopy and cyclic voltammetry measurements were performed to examine the electronic properties of these novel redox-active chiral Lewis acids.

A series of micro2-fluoro-bridged heteronuclear bidentate Lewis acid complexes [K(18-crown-6)THF]+ [Fc(BMeF)(SnMe2Cl)F]- (1-2F), [K(18-crown-6)THF]+ [Fc(BMeF)(SnMe2F)F]- (1-3F), [K(18-crown-6)THF]+ [Fc(BMePh)(SnMe2Cl)F]- (2-F), and [K(18-crown-6)THF]+ [Fc(BMePh)(SnMe2F)F]- (2-2F) (Fc=1,2-ferrocenediyl) was prepared. Compounds 2-F and 2-2F were obtained as a mixture of diastereomers, which arise due to the generation of a stereocenter at boron in addition to their inherent planar chirality. All compounds have been studied in the solid state by single-crystal X-ray diffraction analysis and by multinuclear NMR spectroscopy in solution. As a result of bridging-fluoride interactions, tetrahedral boron and distorted trigonal-bipyramidal tin centers are observed. Comparison with the corresponding monofunctional ferrocenylborates further supports the bridging nature of the fluoride anion. Two-dimensional exchange spectroscopy 19F NMR studies provide evidence for facile intermolecular and intramolecular fluorine exchange processes. All complexes display reversible one-electron oxidation events at lower potentials than those of the tricoordinate ferrocenylborane precursors, which is typical of ferrocenylborate complexes.

The first examples of organoboron star polymers were prepared by arm-first RAFT polymerization using a novel luminescent organoboron crosslinker in combination with PS, PNIPAM, P4VP, and PNIPAM-b-PS as linear polymer precursors. Well-defined star architectures with a luminescent core were successfully obtained as confirmed by GPC-MALLS, NMR, DLS, and TEM analysis. The PNIPAM-b-PS stars are amphiphilic and undergo self-assembly in water with formation of super-aggregates that exhibit strong green fluorescence. The P4VP stars lend themselves to further functionalization at the periphery.