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1.
J Pediatr Health Care ; 38(4): 604-614, 2024.
Article in English | MEDLINE | ID: mdl-38647508

ABSTRACT

INTRODUCTION: Promoting safe sleep to decrease sudden unexpected infant death is challenging in the hospital setting. LOCAL PROBLEM: Concern for adherence to safe sleep practice across inpatient units at a large pediatric hospital. METHODS: Used quality improvement methodologies to promote safe sleep across all units. INTERVENTIONS: Development of a multidisciplinary expert group, hospital-wide guidelines, targeted interventions, and bedside audits to track progress. RESULTS: Adherence to safe sleep practices improved from 9% to 53%. Objects in the crib were a major barrier to maintaining a safe sleep environment. Safe sleep practices were less likely to be observed in infants with increased medical complexity (p = .027). CONCLUSIONS: Quality improvement methodology improved adherence to infant safe sleep guidelines across multiple units. Medically complex infants continue to be a challenge to safe sleep. Therefore, ongoing education for staff and further research into best practices for the most complex infant populations are necessary.


Subject(s)
Guideline Adherence , Hospitals, Pediatric , Quality Improvement , Sudden Infant Death , Humans , Sudden Infant Death/prevention & control , Infant , Infant, Newborn , Infant Care/methods , Infant Care/standards , Sleep/physiology , Female , Male , Practice Guidelines as Topic , Patient Safety/standards
2.
Cell Stem Cell ; 31(4): 467-483.e6, 2024 Apr 04.
Article in English | MEDLINE | ID: mdl-38537631

ABSTRACT

Brain injury is highly associated with preterm birth. Complications of prematurity, including spontaneous or necrotizing enterocolitis (NEC)-associated intestinal perforations, are linked to lifelong neurologic impairment, yet the mechanisms are poorly understood. Early diagnosis of preterm brain injuries remains a significant challenge. Here, we identified subventricular zone echogenicity (SVE) on cranial ultrasound in preterm infants following intestinal perforations. The development of SVE was significantly associated with motor impairment at 2 years. SVE was replicated in a neonatal mouse model of intestinal perforation. Examination of the murine echogenic subventricular zone (SVZ) revealed NLRP3-inflammasome assembly in multiciliated FoxJ1+ ependymal cells and a loss of the ependymal border in this postnatal stem cell niche. These data suggest a mechanism of preterm brain injury localized to the SVZ that has not been adequately considered. Ultrasound detection of SVE may serve as an early biomarker for neurodevelopmental impairment after inflammatory disease in preterm infants.


Subject(s)
Brain Injuries , Intestinal Perforation , Motor Disorders , Premature Birth , Infant , Female , Infant, Newborn , Humans , Animals , Mice , Infant, Premature , Intestinal Perforation/complications , Lateral Ventricles , Stem Cell Niche , Motor Disorders/complications , Brain Injuries/complications , Brain Injuries/diagnostic imaging
3.
J Pediatr ; 263: 113700, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37640232

ABSTRACT

OBJECTIVE: To determine the ability of the Bayley-III cognitive and language composite scores at 18-22 months corrected age to predict WISC-IV Full Scale IQ (FSIQ) at 6-7 years in infants born extremely preterm. STUDY DESIGN: Children in this study were part of the Neuroimaging and Neurodevelopmental Outcome cohort, a secondary study to the SUPPORT trial and born 240/7-276/7 weeks gestational age. Bayley-III cognitive and language scores and WISC-IV FSIQ were compared with pairwise Pearson correlation coefficients and adjusted for medical and socioeconomic variables using linear mixed effect regression models. RESULTS: Bayley-III cognitive (r = 0.33) and language scores (r = 0.44) were mildly correlated with WISC-IV FSIQ score. Of the children with Bayley-III cognitive scores of <70, 67% also had FSIQ of <70. There was less consistency for children with Bayley-III scores in the 85-100 range; 43% had an FSIQ of <85 and 10% an FSIQ of <70. Among those with Bayley-III language scores >100, approximately 1 in 5 had an FSIQ of <85. A cut point of 92 for the cognitive composite score resulted in sensitivity (0.60), specificity (0.64). A cut point of 88 for the language composite score produced sensitivity (0.61), specificity (0.70). CONCLUSIONS: Findings indicate the Bayley-III cognitive and language scores correlate with later IQ, but may fail to predict delay or misclassify children who are not delayed at school age. The Bayley-III can be a useful tool to help identify children born extremely preterm who have below average cognitive scores and may be at the greatest risk for ongoing cognitive difficulties. TRIAL REGISTRATION: Extended Follow-up at School Age for the SUPPORT Neuroimaging and Neurodevelopmental Outcomes (NEURO) Cohort: NCT00233324.


Subject(s)
Child Development , Infant, Extremely Premature , Infant, Newborn , Infant , Humans , Child , Infant, Extremely Premature/psychology , Gestational Age , Cognition , Neuroimaging
4.
Stem Cells Transl Med ; 12(6): 355-364, 2023 06 15.
Article in English | MEDLINE | ID: mdl-37285522

ABSTRACT

Hypoxic ischemic encephalopathy (HIE) in neonates causes increased mortality and long-term morbidity in surviving babies. Hypothermia (HT) has improved outcomes, however, mortality remains high with ~half of surviving babies developing neurological impairment in their first years. We previously explored the use of autologous cord blood (CB) to determine if CB cells could lessen long-term damage to the brain. However, the feasibility of CB collection from sick neonates limited the utility of this approach. Allogeneic cord tissue mesenchymal stromal cells (hCT-MSC), cryopreserved and readily available, have been shown to ameliorate brain injury in animal models of HIE. We, therefore, conducted a pilot, phase I, clinical trial to test the safety and describe the preliminary efficacy of hCT-MSC in neonates with HIE. The study treated infants with moderate to severe HIE, treated with HT, with 1 or 2 doses of 2 million cells/kg/dose of hCT-MSC given intravenously. The babies were randomized to receive 1 or 2 doses with the first dose during HT and the second dose 2 months later. Babies were followed for survival and development with scoring of Bayley's at 12 postnatal months. Six neonates with moderate (4) or severe (2) HIE were enrolled. All received 1 dose of hCT-MSC during HT and 2 received a 2nd dose, 2 months later. hCT-MSC infusions were well tolerated although 5/6 babies developed low titer anti-HLA antibodies by 1 year of age. All babies survived, with average to low-average developmental assessment standard scores for ages between 12 and 17 postnatal months. Further study is warranted.


Subject(s)
Hematopoietic Stem Cell Transplantation , Hypothermia, Induced , Hypoxia-Ischemia, Brain , Mesenchymal Stem Cells , Hypoxia-Ischemia, Brain/therapy , Umbilical Cord , Humans , Infant, Newborn
5.
Pediatr Res ; 93(3): 689-695, 2023 02.
Article in English | MEDLINE | ID: mdl-35715492

ABSTRACT

BACKGROUND: Extremely preterm (EPT) birth has been related to dysregulation of stress responses and behavioral/learning problems at school age. Early adverse experiences can blunt HPA axis reactivity. We hypothesized that an attenuated cortisol awakening response would be associated with developmental and behavioral problems at school age in EPT children. METHODS: This secondary analysis of a sub-cohort of the SUPPORT study included children born between 24 and 27 weeks, evaluated at 6-7 years with a neurodevelopmental battery and cortisol measures. Differences were tested between EPT and a term-born group. Relationships of cortisol awakening response to test scores were analyzed. RESULTS: Cortisol was measured in 110 EPT and 29 term-born 6-7 year olds. Unadjusted WISC-IV and NEPSY-II scores were significantly worse among EPT children only. Conners Parent Rating Scale behavior scores were significantly worse among EPT children. After adjusting for covariates, blunted cortisol awakening responses were found to be associated with poorer scores on memory tests and greater problems with inattention for the EPT group (p < 0.05) only. CONCLUSIONS: Among children born EPT, we identified an association of blunted cortisol awakening response with memory and inattention problems. This may have implications related to stress reactivity and its relationship to learning problems in children born EPT. GOV ID: Extended Follow-up at School Age for the SUPPORT Neuroimaging and Neurodevelopmental Outcomes (NEURO) Cohort: NCT00233324. IMPACT: In children born EPT, stress reactivity may have a relationship to learning problems. Cortisol awakening response should be a component for follow-up in EPT born children. Components of executive function, such as memory and attention, are related to stress reactivity.


Subject(s)
Hydrocortisone , Infant, Extremely Premature , Child , Female , Humans , Infant, Newborn , Executive Function , Hypothalamo-Hypophyseal System , Pituitary-Adrenal System
6.
Blood Adv ; 6(9): 2947-2956, 2022 05 10.
Article in English | MEDLINE | ID: mdl-35042231

ABSTRACT

Infantile Krabbe disease (IKD) can be treated with hematopoietic cell transplantation (HCT) if done during the first weeks of life before symptoms develop. To facilitate this, newborn screening (NBS) has been instituted in 8 US states. An application to add IKD to the recommended NBS panel is currently under review. In this report, the outcomes of newborns with IKD diagnosed through NBS and treated with HCT are presented. The unique challenges associated with NBS for this disease are discussed, including opportunities for earlier diagnosis and streamlining treatment referrals. This is a retrospective review of six infants with IKD detected by NBS who were referred for HCT. The timing from diagnosis to HCT was examined, and both HCT and neurodevelopmental outcomes are described. Neurologic testing before HCT revealed evidence of active IKD in all infants. All underwent HCT between 24 and 40 days of age, were successfully engrafted, and are alive 30 to 58 months later (median, 47.5 months). All are gaining developmental milestones albeit at a slower pace than unaffected age-matched peers. Gross motor function is most notably affected. NBS for these patients enabled early access to HCT, the only currently available treatment of infants with IKD. All children are alive and have derived developmental and neurologic benefits from timely HCT. Long-term follow up is ongoing. Optimization of HCT and further development of emerging therapies, all of which must be delivered early in life, are expected to further improve outcomes of infants with IKD.


Subject(s)
Hematopoietic Stem Cell Transplantation , Leukodystrophy, Globoid Cell , Child , Child, Preschool , Humans , Infant , Infant, Newborn , Leukodystrophy, Globoid Cell/diagnosis , Leukodystrophy, Globoid Cell/therapy , Longitudinal Studies , Neonatal Screening
7.
Am J Perinatol ; 38(13): 1366-1372, 2021 11.
Article in English | MEDLINE | ID: mdl-32485756

ABSTRACT

OBJECTIVE: Prematurity and low birth weight (LBW) are risk factors for increased morbidity and mortality in infants with congenital heart defects (CHDs). We sought to describe survival, inhospital morbidities, and 2-year neurodevelopmental follow-up in LBW infants with CHD. STUDY DESIGN: We included infants with birth weight (BW) <2,500 g diagnosed with CHD (except isolated patent ductus arteriosus) admitted January 2013 to March 2016 to a single level-IV academic neonatal intensive care unit. We reported CHD prevalence by BW and gestational age; selected in-hospital morbidities and mortality by infant BW, CHD type, and surgical intervention; and developmental outcomes by Bayley's scales of infant and toddler development, third edition (BSID-III) scores at age 2 years. RESULTS: Among 420 infants with CHD, 28 (7%) underwent cardiac surgery. Median (25th and 75th percentiles) gestational age was 30 (range: 27-33) weeks and BW was 1,258 (range: 870-1,853) g. There were 134 of 420 (32%) extremely LBW (<1,000 g) infants, 82 of 420 (20%) were small for gestational age, and 51 of 420 (12%) multiples. Most common diagnosis: atrial septal defect (260/420, 62%), followed by congenital anomaly of the pulmonary valve (75/420, 18%). Most common surgical procedure: pulmonary artery banding (5/28, 18%), followed by the tetralogy of Fallot corrective repair (4/28, 14%). Survival to discharge was 88% overall and lower among extremely LBW (<1,000 g, 81%) infants and infants undergoing surgery (79%). Comorbidities were common (35%); retinopathy of prematurity and bronchopulmonary dysplasia were most prevalent. BSID-III scores were available on 148 of 176 (84%); any scores <85 were noted in 73 of 148 (49%), with language being most commonly affected. CONCLUSION: Among LBW infants with congenital heart disease, hospital mortality varied by BW and cardiac diagnosis. KEY POINTS: · In low birth weight infants with congenital heart disease, survival varied by birth weight and cardiac diagnosis.. · Overall survival was higher than previously reported.. · There were fewer morbidities than previously reported.. · Bayley's scale-III scores at 2 years of age were <85 for nearly half..


Subject(s)
Heart Defects, Congenital/mortality , Hospital Mortality , Infant, Low Birth Weight , Infant, Premature, Diseases/mortality , Infant, Premature , Birth Weight , Cardiac Surgical Procedures , Comorbidity , Follow-Up Studies , Heart Defects, Congenital/complications , Heart Defects, Congenital/surgery , Humans , Infant, Extremely Low Birth Weight , Infant, Newborn , Infant, Small for Gestational Age , Kaplan-Meier Estimate
8.
Am J Trop Med Hyg ; 102(1): 28-30, 2020 01.
Article in English | MEDLINE | ID: mdl-31769386

ABSTRACT

Globally, diarrheal and respiratory infections are responsible for more than 24% of deaths in children aged less than 5 years. Historically, these disease entities have been studied separately; recent evidence suggests that preceding diarrheal disease may be a risk factor for subsequent respiratory illness. We used data from a community-based, prospective randomized trial of maternal influenza immunization of 3,693 pregnant women and their 3,646 infants conducted in rural Nepal from 2011 to 2014. A case-crossover design was used to determine whether the risk of respiratory infection in the 30 days following a diarrheal episode was increased compared with that 30 days prior. Diarrheal illness was a significant risk factor for subsequent respiratory illness in infants but not in women during pregnancy or in women up to six months postpartum. Diarrheal illness and respiratory infections remain important global sources of morbidity and mortality, and our study supports a causal relationship between them in infants.


Subject(s)
Diarrhea/pathology , Pregnancy Complications, Infectious/epidemiology , Respiratory Tract Infections/epidemiology , Respiratory Tract Infections/etiology , Adult , Case-Control Studies , Child , Child, Preschool , Female , Humans , Infant , Nepal/epidemiology , Pregnancy , Risk Factors , Rural Population , Young Adult
9.
Dev Med Child Neurol ; 62(6): 750-757, 2020 06.
Article in English | MEDLINE | ID: mdl-31691959

ABSTRACT

AIM: To determine if genetic variation associated with decreased dopamine neurotransmission predicts a decrease in motor development in a convenience cohort study of infants born extremely-low-birthweight (ELBW). METHOD: Four hundred and ninety-eight infants born ELBW had genome-wide genotyping and a neurodevelopmental evaluation at 18 to 22 months of age, corrected for preterm birth. A polygenic risk score (PRS) was created to combine into one predictor variable the hypothesized influences on motor development of alleles at seven independent single nucleotide polymorphisms previously associated with relative decreases in both dopamine neurotransmission and motor learning, by summing the number of alleles present in each infant (range=0-14). The motor development outcome was the Psychomotor Development Index (PDI) of the Bayley Scales of Infant Development, Second Edition. The linear regression models were adjusted for seven clinical and four genetic ancestry covariates. The mean PRS of infants with cerebral palsy (CP) was compared to those without CP. RESULTS: PRS was inversely related to PDI (p=0.011). Each 1-point increase in PRS resulted in an average decrease in PDI of 1.37 points. Patients with CP did not have a greater mean PRS than those without (p=0.67), both with and without adjustment for covariates. INTERPRETATION: Genetic variation that favors a decrease in dopamine neurotransmission predisposes to a decrease in motor development in infants born ELBW, but not to the diagnosis of CP. WHAT THIS PAPER ADDS: Genetic variation in dopamine neurotransmission was associated with a decrease in motor development in infants born at an extremely-low-birthweight. It does not predispose to the diagnosis of cerebral palsy.


Subject(s)
Developmental Disabilities/genetics , Dopamine/physiology , Genetic Variation/genetics , Infant, Premature, Diseases/genetics , Motor Skills Disorders/genetics , Synaptic Transmission/genetics , Cohort Studies , Developmental Disabilities/metabolism , Female , Humans , Infant, Extremely Low Birth Weight , Infant, Extremely Premature , Infant, Newborn , Male , Motor Skills Disorders/metabolism
10.
Emerg Infect Dis ; 25(7): 1408-1411, 2019 07.
Article in English | MEDLINE | ID: mdl-31211675

ABSTRACT

Homelessness has not previously been identified as a risk factor for respiratory syncytial virus (RSV) infection. We conducted an observational study at an urban safety-net hospital in Washington, USA, during 2012-2017. Hospitalized adults with RSV were more likely to be homeless, and several clinical outcome measures were worse with RSV than with influenza.


Subject(s)
Ill-Housed Persons , Respiratory Syncytial Virus Infections/epidemiology , Respiratory Syncytial Virus Infections/virology , Respiratory Syncytial Viruses , Case-Control Studies , Female , Hospitalization , Humans , Male , Odds Ratio , Population Surveillance , Retrospective Studies , Socioeconomic Factors , Washington/epidemiology
11.
Open Forum Infect Dis ; 6(2): ofz011, 2019 Feb.
Article in English | MEDLINE | ID: mdl-30793004

ABSTRACT

BACKGROUND: Adverse birth outcomes, including low birthweight, small for gestational age (SGA), and preterm birth, contribute to 60%-80% of infant mortality worldwide. Little published data exist on the association between diarrhea during pregnancy and adverse birth outcomes. METHODS: Data were used from 2 community-based, prospective randomized trials of maternal influenza immunization during pregnancy conducted in rural Nepal from 2011 to 2014. Diarrheal illnesses were identified through longitudinal household-based weekly symptom surveillance. Diarrhea episodes were defined as at least 3 watery bowel movements per day for 1 or more days with 7 diarrhea-free days between episodes. The Poisson and log-binomial regression were performed to evaluate baseline characteristics and association between diarrhea during pregnancy and adverse birth outcomes. RESULTS: A total of 527 of 3693 women in the study (14.3%) experienced diarrhea during pregnancy. Women with diarrhea had a median of 1 episode of diarrhea (interquartile range [IQR], 1-2 episodes) and 2 cumulative days of diarrhea (IQR, 1-3 days). Of women with diarrhea, 85 (16.1%) sought medical care. In crude and adjusted analyses, women with diarrhea during pregnancy were more likely to have SGA infants (42.6% vs 36.8%; adjusted risk ratio = 1.20; 95% confidence interval, 1.06-1.36; P = .005). Birthweight and preterm birth incidence did not substantially differ between women with diarrhea during pregnancy and those without. CONCLUSIONS: Diarrheal illness during pregnancy was associated with a higher risk of SGA infants in this rural South Asian population. Interventions to reduce the burden of diarrheal illness during pregnancy may have an impact on SGA births in resource-limited settings.

12.
Early Hum Dev ; 125: 1-7, 2018 10.
Article in English | MEDLINE | ID: mdl-30144709

ABSTRACT

BACKGROUND: Therapeutic hypothermia reduces the risk of death, or moderate to severe neurodevelopmental impairment (NDI) in term infants with hypoxic-ischemic encephalopathy (HIE). Reports of its safety and efficacy in preterm infants are scarce. OBJECTIVE: Report short and long-term outcomes of preterm infants with HIE who received therapeutic hypothermia. METHODS: A retrospective cohort analysis of all preterm infants <36 weeks' gestation with HIE who received whole body hypothermia in a single center from January 2007 to April 2015. The primary outcome was death or moderate to severe NDI defined by moderate or severe cerebral palsy, severe hearing or visual impairment, or cognitive score < 85 on the Bayley Scales of Infant Development III (BSID III) at 18-24 months' adjusted age. RESULTS: 30 infants with a median gestational age and birthweight of 35 weeks' (range; 33-35) and 2575 g (1850-4840) and a median first postnatal blood pH of 6.81 (6.58-7.14). Complications included coagulopathy (50%), early clinical seizures (43.3%), arterial hypotension (40%), persistent metabolic acidosis (37%) and thrombocytopenia (20%). Four infants died before or soon after discharge (18.2%). Eighteen surviving infants (69.2%) had follow up data; 7 of them had moderate to severe NDI (38.9%). Cognitive, motor and language mean composite BSID III scores were 84 (54-110), 83 (46-118), and 78 (46-112). Death or moderate to severe NDI occurred in 11/22 (50%) infants with known outcomes. CONCLUSION: Large randomized trials on efficacy and safety are needed in this highly vulnerable population as the incidence of complications and the combined outcome of death and NDI is concerning.


Subject(s)
Asphyxia Neonatorum/therapy , Hypothermia, Induced/methods , Hypoxia-Ischemia, Brain/therapy , Asphyxia Neonatorum/complications , Birth Weight/physiology , Developmental Disabilities/etiology , Female , Humans , Hypoxia-Ischemia, Brain/complications , Infant, Newborn , Infant, Premature , Male , Retrospective Studies , Treatment Outcome
13.
Pediatrics ; 142(1)2018 07.
Article in English | MEDLINE | ID: mdl-29945955

ABSTRACT

BACKGROUND AND OBJECTIVES: Children born extremely preterm are at risk for cognitive difficulties and disability. The relative prognostic value of neonatal brain MRI and cranial ultrasound (CUS) for school-age outcomes remains unclear. Our objectives were to relate near-term conventional brain MRI and early and late CUS to cognitive impairment and disability at 6 to 7 years among children born extremely preterm and assess prognostic value. METHODS: A prospective study of adverse early and late CUS and near-term conventional MRI findings to predict outcomes at 6 to 7 years including a full-scale IQ (FSIQ) <70 and disability (FSIQ <70, moderate-to-severe cerebral palsy, or severe vision or hearing impairment) in a subgroup of Surfactant Positive Airway Pressure and Pulse Oximetry Randomized Trial enrollees. Stepwise logistic regression evaluated associations of neuroimaging with outcomes, adjusting for perinatal-neonatal factors. RESULTS: A total of 386 children had follow-up. In unadjusted analyses, severity of white matter abnormality and cerebellar lesions on MRI and adverse CUS findings were associated with outcomes. In full regression models, both adverse late CUS findings (odds ratio [OR] 27.9; 95% confidence interval [CI] 6.0-129) and significant cerebellar lesions on MRI (OR 2.71; 95% CI 1.1-6.7) remained associated with disability, but only adverse late CUS findings (OR 20.1; 95% CI 3.6-111) were associated with FSIQ <70. Predictive accuracy of stepwise models was not substantially improved with the addition of neuroimaging. CONCLUSIONS: Severe but rare adverse late CUS findings were most strongly associated with cognitive impairment and disability at school age, and significant cerebellar lesions on MRI were associated with disability. Near-term conventional MRI did not substantively enhance prediction of severe early school-age outcomes.


Subject(s)
Brain/diagnostic imaging , Developmental Disabilities/diagnostic imaging , Echoencephalography/methods , Magnetic Resonance Imaging/methods , Child , Child, Preschool , Cognition , Developmental Disabilities/epidemiology , Disability Evaluation , Follow-Up Studies , Humans , Infant , Infant, Newborn , Infant, Premature , Neuroimaging/methods , Prognosis , Prospective Studies
14.
J Gerontol B Psychol Sci Soc Sci ; 73(3): 421-431, 2018 03 02.
Article in English | MEDLINE | ID: mdl-27522086

ABSTRACT

Objectives: Sleep problems increase in later life. Studies have linked sleep with marital satisfaction, yet mechanisms, such as mood, have not been explored. The current study is innovative in examining sleep and marital interactions among older couples in a daily context, exploring mood as a potential mediator. Method: Data were taken from the Life and Family Legacies Daily Experiences Study, involving 191 older couples surveyed across 14 days. Multivariate (dyadic) multilevel models were used to address our research questions. Results: Findings indicated significant associations between daily sleep hours, sleep quality, and feeling rested with daily marital interactions. These associations were most consistent for wives. Mediation analyses indicated that positive mood was a common mechanism linking sleep with marital interactions. Also, in some cases, spouse sleep and mood reports were associated with partner marital interactions. Discussion: Improving sleep quality among older couples could lead to better daily marital interactions through changes in mood.


Subject(s)
Affect , Marriage/psychology , Sleep , Female , Humans , Male , Marriage/statistics & numerical data , Middle Aged , Sleep Wake Disorders/complications , Sleep Wake Disorders/psychology , Surveys and Questionnaires
15.
Adv Neonatal Care ; 17(6): 461-469, 2017 Dec.
Article in English | MEDLINE | ID: mdl-29077581

ABSTRACT

BACKGROUND: Financial obligations serve as an added source of stress and burden for parents of medically complex infants that have extended hospitalizations in the neonatal intensive care unit. Financial resources and support personnel are available to assist parents, but systems must be in place to help access these services. When neonatal intensive care unit nurses work collaboratively with financial support personnel, they improve families' access to financial resources. PURPOSE: The purpose of this quality improvement initiative was to increase and facilitate timely parent referrals to health benefits coordinators (HBCs). METHODS/SEARCH STRATEGY: Utilizing the Plan-Do-Study Act framework, the hospital's current system for HBC referrals was revised utilizing 3 Plan-Do-Study Act cycles. FINDINGS/RESULTS: A substantial increase in the percentage of HBC referrals, from preimplementation of less than 5% to a sustained average of 90% was observed. IMPLICATIONS FOR PRACTICE: A simple, sustainable screening process was successfully created to identify families with primary health insurance who qualified for coordination of benefits. This resulted in a significant increase in the number of HBC referrals. Minimal time is now required for the multidisciplinary team to ensure that parents, eligible for referral, are identified as soon as possible. Early identification and timely referral to the HBC may lessen the financial burden for families caring for children with medically complex long-term care needs by securing secondary insurance and other resources. IMPLICATIONS FOR RESEARCH: Research focused on the financial impact of the HBC role is needed.


Subject(s)
Cost of Illness , Intensive Care Units, Neonatal/classification , Intensive Care, Neonatal/economics , Quality Improvement , Fees and Charges , Humans , Parents , Patient Care Team/economics
16.
Stem Cells Transl Med ; 6(12): 2071-2078, 2017 12.
Article in English | MEDLINE | ID: mdl-29080265

ABSTRACT

Cerebral palsy (CP) is a condition affecting young children that causes lifelong disabilities. Umbilical cord blood cells improve motor function in experimental systems via paracrine signaling. After demonstrating safety, we conducted a phase II trial of autologous cord blood (ACB) infusion in children with CP to test whether ACB could improve function (ClinicalTrials.gov, NCT01147653; IND 14360). In this double-blind, placebo-controlled, crossover study of a single intravenous infusion of 1-5 × 107 total nucleated cells per kilogram of ACB, children ages 1 to 6 years with CP were randomly assigned to receive ACB or placebo at baseline, followed by the alternate infusion 1 year later. Motor function and magnetic resonance imaging brain connectivity studies were performed at baseline, 1, and 2 years post-treatment. The primary endpoint was change in motor function 1 year after baseline infusion. Additional analyses were performed at 2 years. Sixty-three children (median age 2.1 years) were randomized to treatment (n = 32) or placebo (n = 31) at baseline. Although there was no difference in mean change in Gross Motor Function Measure-66 (GMFM-66) scores at 1 year between placebo and treated groups, a dosing effect was identified. In an analysis 1 year post-ACB treatment, those who received doses ≥2 × 107 /kg demonstrated significantly greater increases in GMFM-66 scores above those predicted by age and severity, as well as in Peabody Developmental Motor Scales-2 Gross Motor Quotient scores and normalized brain connectivity. Results of this study suggest that appropriately dosed ACB infusion improves brain connectivity and gross motor function in young children with CP. Stem Cells Translational Medicine 2017;6:2071-2078.


Subject(s)
Blood Transfusion/methods , Cerebral Palsy/therapy , Connectome , Fetal Blood/transplantation , Motor Skills , Brain/diagnostic imaging , Brain/physiology , Child , Child, Preschool , Female , Humans , Infant , Male , Movement
17.
Article in English | MEDLINE | ID: mdl-28729373

ABSTRACT

GLE1 encodes a protein important for mRNA export and appears to play roles in translation initiation and termination as well. Pathogenic variants in GLE1 mutations have been associated with lethal contracture syndrome and lethal arthrogryposis with anterior horn cell disease; phenotypes reported in individuals include fetal akinesia and a severe form of motor neuron disease, typically presenting with prenatal symptoms and perinatal lethality. In this article, we identified biallelic missense mutations in GLE1 by trio whole-exome sequencing in an individual affected with congenital motor weakness and contractures as well as feeding and respiratory difficulties. Muscle biopsy was consistent with anterior horn cell disease and supported the pathogenicity of the sequence variants. Importantly, this individual survived past the perinatal period with respiratory support and currently demonstrates age-appropriate cognition and slow but steady motor developmental progress. We propose that pathogenic variants in GLE1 can be associated with a nonperinatal lethal motor phenotype, and affected individuals can demonstrate motor skill progression, unlike prototypical anterior horn cell diseases such as spinal muscular atrophy.


Subject(s)
Arthrogryposis/genetics , Nucleocytoplasmic Transport Proteins/genetics , Contracture/genetics , Female , Humans , Infant , Infant, Newborn , Muscular Atrophy, Spinal , Mutation , Mutation, Missense/genetics , Nucleocytoplasmic Transport Proteins/metabolism , Pedigree , Phenotype , RNA Transport , RNA, Messenger/metabolism , Exome Sequencing
18.
Pediatrics ; 139(6)2017 Jun.
Article in English | MEDLINE | ID: mdl-28562255

ABSTRACT

BACKGROUND: Behavior and socioemotional development are crucial aspects of child development . METHODS: A total of 2505 children born at <27 weeks' gestation was evaluated at 18 to 22 months' corrected age between January 1, 2008 and December 12, 2012 (86% follow-up). The Brief Infant and Toddler Social and Emotional Assessment was used to evaluate behavioral and socioemotional problems. Cognition and language were evaluated by using the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Logistic regression analysis was used to evaluate for perinatal and demographic factors associated with behavioral problems (≥75th percentile) and delayed socioemotional competence (≤15th percentile). Structural equation modeling with bootstrapping was used to identify possible associated risk factors and Bayley-III scores as mediators. RESULTS: Thirty-five percent (873) of children had behavioral problems, and 26% (637) displayed deficits in socioemotional competence. Male sex, public insurance, mothers with less than a high school education, and lower maternal age were associated with behavioral problems. Deficits in competence were associated with lower birth weight, public insurance, mothers with less than a high school education, and abnormal neuromotor exam. Bayley-III language and cognitive scores were significant mediators of the relationships between risk factors and both behavioral and competence scores (P < .05). CONCLUSIONS: Extremely premature children are at risk for behavioral problems and deficits in socioemotional competence. Sociodemographic factors were associated with both socioemotional competence and behavioral problems. Deficits in socioemotional competence were also associated with neuromotor abnormalities and cognitive and language function.


Subject(s)
Child Behavior Disorders/epidemiology , Child Development , Developmental Disabilities/epidemiology , Cognition , Female , Gestational Age , Humans , Infant , Infant, Extremely Premature , Infant, Newborn , Logistic Models , Male , Risk Factors
19.
J Obstet Gynecol Neonatal Nurs ; 45(5): 661-70, 2016.
Article in English | MEDLINE | ID: mdl-27497030

ABSTRACT

OBJECTIVE: To evaluate the effect of parent presence during multidisciplinary rounds on NICU-related parental stress. DESIGN: Quasi-experimental study. SETTING: University-affiliated, 24-bed NICU located within a children's hospital that admits infants from birth to 6 months of age. PARTICIPANTS: One hundred thirty-two parents of infants admitted to the NICU for the first time. METHODS: All parents completed the Parent Stressor Scale: NICU (PSS:NICU) on Study Days 0 and 3. In addition to usual family communication practices, parents in the experimental group were offered the opportunity to participate in multidisciplinary rounds on their infants. RESULTS: A total of 132 parents completed the study; the first 46 parents were enrolled in the control group, and the subsequent 86 parents in the experimental group. Overall PSS:NICU scores decreased significantly in the experimental group between Study Days 0 and 3 (mean ± standard error [SE] = -0.24 ± 0.07, p < .001), but the change was not significantly different between the control and experimental groups (mean ± SE = -0.12 ± 0.10, p = .25). The PSS:NICU Parental Role Alteration subscale decreased by the largest margin in the experimental group (mean ± SE = -0.42 ± 0.09, p < .0001), but the change was not significantly different between groups (mean ± SE = -0.26 ± 0.14, p = .06). Overall PSS:NICU stress scores were higher in mothers than fathers (mothers, mean ± SE = 3.4 ± 0.81; fathers, mean ± SE = 2.7 ± 0.67; p < .001). CONCLUSION: Providing parents with the opportunity to participate in multidisciplinary rounds did not affect NICU-related parental stress. Mothers reported higher levels of stress than fathers.


Subject(s)
Intensive Care Units, Neonatal , Parents , Stress, Psychological , Teaching Rounds , Adult , Child , Fathers , Female , Humans , Infant, Newborn , Male , Mothers , Pregnancy
20.
Early Hum Dev ; 95: 5-8, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26900775

ABSTRACT

BACKGROUND: Infants born near the limit of viability are at high risk for death or adverse neurodevelopmental outcomes. It is unclear whether these outcomes have improved over the past 15 years. AIM: To determine if death and neurodevelopmental impairment have declined over the past 15 years in infants born at 22 to 24 weeks' gestation. STUDY DESIGN: Retrospective cohort study. SUBJECTS: We identified infants born at 22 to 24 weeks' gestation in our center in two epochs: 1998-2004 (Epoch 1) and 2005-2011 (Epoch 2). OUTCOME MEASURES: The primary outcome, death or neurodevelopmental impairment, was evaluated at 17-25 months' corrected gestational age with neurologic exams and Bayley Scales of Infant Development. Perinatal characteristics, major morbidities, and outcomes were compared between epochs. RESULTS: Birth weight and gestational age were similar between 170 infants in Epoch 1 and 187 infants in Epoch 2. Mortality was significantly lower in Epoch 2, 55% vs. 42% (p=0.02). Among surviving infants, late-onset sepsis (p<0.01), bronchopulmonary dysplasia (p<0.01), and surgical necrotizing enterocolitis (p=0.04) were less common in Epoch 2. Neurodevelopmental impairment among surviving infants declined from 68% in Epoch 1 to 47% in Epoch 2, p=0.02. Odds of death or NDI were significantly lower in Epoch 2 vs. Epoch 1, OR=0.31 (95% confidence interval; 0.16, 0.58). CONCLUSION: Risk of death or neurodevelopmental impairment decreased over time in infants born at 22 to 24 weeks' gestation.


Subject(s)
Bronchopulmonary Dysplasia/epidemiology , Developmental Disabilities/epidemiology , Enterocolitis, Necrotizing/epidemiology , Infant Mortality , Infant, Extremely Premature/growth & development , Bronchopulmonary Dysplasia/diagnosis , Developmental Disabilities/diagnosis , Enterocolitis, Necrotizing/diagnosis , Female , Gestational Age , Humans , Infant , Infant, Newborn , Male
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