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Certain interventions in the neonatal intensive care unit are considered ethically obligatory, and should be provided over parental objections. After reviewing a case, comparative outcome data, and relevant ethical principles, we propose that extracorporeal membrane oxygenation for meconium aspiration syndrome may, in some cases, be an ethically obligatory treatment.
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Oxigenación por Membrana Extracorpórea/ética , Testigos de Jehová , Síndrome de Aspiración de Meconio/terapia , Consentimiento Paterno/ética , Derechos del Paciente/ética , Humanos , Recién Nacido , MasculinoRESUMEN
INTRODUCTION: Wolf-Hirschhorn syndrome (WHS) is a contiguous gene syndrome involving deletions of the chromosome 4p16 region associated with growth failure, characteristic craniofacial abnormalities, cardiac defects, and seizures. CASE REPORT: This report describes a six-month-old girl with WHS with growth failure and typical craniofacial features who died of complex congenital heart disease. Genetic studies revealed a 9.8 Mb chromosome 4p-terminal deletion. At autopsy, the liver was grossly unremarkable. Routine sampling and histologic examination revealed two hepatocellular nodular lesions with expanded cell plates and mild cytologic atypia. Immunohistochemical staining revealed these nodules were positive for glutamine synthetase and glypican 3, with increased Ki-67 signaling and diffuse CD34 expression in sinusoidal endothelium. These findings are consistent with hepatoblastoma or hepatocellular carcinoma. CONCLUSIONS: A possible association between WHS and hepatic malignancy may be an important consideration in the care and management of WHS patients.
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Hepatoblastoma/complicaciones , Hepatoblastoma/genética , Neoplasias Hepáticas/complicaciones , Neoplasias Hepáticas/genética , Síndrome de Wolf-Hirschhorn/complicaciones , Síndrome de Wolf-Hirschhorn/genética , Carcinoma Hepatocelular/complicaciones , Carcinoma Hepatocelular/genética , Carcinoma Hepatocelular/patología , Deleción Cromosómica , Cromosomas Humanos Par 4/genética , Diagnóstico Diferencial , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/genética , Hepatoblastoma/patología , Humanos , Lactante , Neoplasias Hepáticas/patologíaRESUMEN
Introduction: Bronchopulmonary dysplasia (BPD) is a chronic lung disorder affecting many premature infants. Infants with BPD have higher hospital readmission rates due to respiratory-related morbidity. We aimed to increase the rates of outpatient pulmonary follow-up and attendance of premature babies with moderate and severe BPD to above 85% within 6 months. Methods: We conducted a quality improvement project at Yale New Haven Children's Hospital. Key interventions included developing a BPD clinical pathway integrated into the electronic medical record to assist providers in correctly classifying BPD severity, assigning the appropriate International Classification of Diseases, 10th Revision code (P27.1), and providing standardized treatment options. The outcome measures included correct diagnosis and classification of BPD, the percentage of patients with BPD scheduled for pediatric pulmonology appointments within 45 days, and the percentage attending those appointments. Results: There were 226 patients in our study, including 85 in the baseline period. Correct diagnosis of BPD increased from 49% to 95%, the percentage of scheduled appointments increased from 71.9% to 100%, and the percentage of appointments attended increased from 55.6% to 87.1%. Conclusions: Our quality improvement initiative improved the accuracy of diagnosis, severity classification, and outpatient pulmonary follow-up of children with moderate and severe BPD.
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Objective: To assess the feasibility of a point-of-care 1-Tesla MRI for identification of intracranial pathologies within neonatal intensive care units (NICUs). Methods: Clinical findings and point-of-care 1-Tesla MRI imaging findings of NICU patients (1/2021 to 6/2022) were evaluated and compared with other imaging modalities when available. Results: A total of 60 infants had point-of-care 1-Tesla MRI; one scan was incompletely terminated due to motion. The average gestational age at scan time was 38.5 ± 2.3 weeks. Transcranial ultrasound (n = 46), 3-Tesla MRI (n = 3), or both (n = 4) were available for comparison in 53 (88%) infants. The most common indications for point-of-care 1-Tesla MRI were term corrected age scan for extremely preterm neonates (born at greater than 28 weeks gestation age, 42%), intraventricular hemorrhage (IVH) follow-up (33%), and suspected hypoxic injury (18%). The point-of-care 1-Tesla scan could identify ischemic lesions in two infants with suspected hypoxic injury, confirmed by follow-up 3-Tesla MRI. Using 3-Tesla MRI, two lesions were identified that were not visualized on point-of-care 1-Tesla scan: (1) punctate parenchymal injury versus microhemorrhage; and (2) small layering IVH in an incomplete point-of-care 1-Tesla MRI with only DWI/ADC series, but detectable on the follow-up 3-Tesla ADC series. However, point-of-care 1-Tesla MRI could identify parenchymal microhemorrhages, which were not visualized on ultrasound. Conclusion: Although limited by field strength, pulse sequences, and patient weight (4.5 kg)/head circumference (38 cm) restrictions, the Embrace® point-of-care 1-Tesla MRI can identify clinically relevant intracranial pathologies in infants within a NICU setting.
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OBJECTIVE: To develop a novel, rapid, and more accurate model for estimating umbilical arterial (UAC) and venous catheter (UVC) insertion length. STUDY DESIGN: We evaluated UACs and UVCs from a retrospective cohort to determine the rate of correct initial positioning based on conventional birth weight-based equations utilized in our neonatal intensive care unit. We then derived new equations, developed the mobile application, UmbiCalc, to simplify implementation of the new equations, and validated their accuracy with prospective utilization. RESULTS: The conventional equations successfully predicted insertion length in 69% (364 of 524) of UACs and only 36% (194 of 544) of UVCs. Our new model was prospectively applied to 68 UAC and 80 UVC placements with successful initial positioning achieved in 90% [95% CI, 80.2-94.9] and 76% [95% CI, 65.9-84.2], respectively. CONCLUSIONS: Our novel approach more accurately estimates UAC and UVC insertion length.
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Cateterismo Periférico , Catéteres , Humanos , Recién Nacido , Estudios Prospectivos , Estudios Retrospectivos , Arterias Umbilicales/diagnóstico por imagen , Venas UmbilicalesRESUMEN
BACKGROUND AND OBJECTIVES: Laboratory testing is performed frequently in the NICU. Unnecessary tests can result in increased costs, blood loss, and pain, which can increase the risk of long-term growth and neurodevelopmental impairment. Our aim was to decrease routine screening laboratory testing in all infants admitted to our NICU by 20% over a 24-month period. METHODS: We designed and implemented a multifaceted quality improvement project using the Institute for Healthcare Improvement's Model for Improvement. Baseline data were reviewed and analyzed to prioritize order of interventions. The primary outcome measure was number of laboratory tests performed per 1000 patient days. Secondary outcome measures included number of blood glucose and serum bilirubin tests per 1000 patient days, blood volume removed per 1000 patient days, and cost. Extreme laboratory values were tracked and reviewed as balancing measures. Statistical process control charts were used to track measures over time. RESULTS: Over a 24-month period, we achieved a 26.8% decrease in laboratory tests performed per 1000 patient days (â½51 000 fewer tests). We observed significant decreases in all secondary measures, including a decrease of almost 8 L of blood drawn and a savings of $258 000. No extreme laboratory values were deemed attributable to the interventions. Improvement was sustained for an additional 7 months. CONCLUSIONS: Targeted interventions, including guideline development, dashboard creation and distribution, electronic medical record optimization, and expansion of noninvasive and point-of-care testing resulted in a significant and sustained reduction in laboratory testing without notable adverse effects.
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Hospitales Pediátricos/normas , Unidades de Cuidado Intensivo Neonatal/normas , Laboratorios de Hospital/normas , Mejoramiento de la Calidad , Procedimientos Innecesarios/estadística & datos numéricos , Bilirrubina/sangre , Glucemia/análisis , Volumen Sanguíneo , Dióxido de Carbono/sangre , Connecticut , Hemorragia/etiología , Hemorragia/prevención & control , Hospitales Pediátricos/economía , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal/economía , Laboratorios de Hospital/economía , Monitoreo Fisiológico/efectos adversos , Dolor/etiología , Dolor/prevención & control , Pruebas en el Punto de Atención , Utilización de Procedimientos y Técnicas , Procedimientos Innecesarios/economíaRESUMEN
BACKGROUND: Listeriosis may cause severe disease in fetuses and neonates. The outcomes of critically ill neonates with early-onset listeriosis requiring extracorporeal membrane oxygenation (ECMO) from 1975 to 1991 have been reported. OBJECTIVE: To update the characteristics and outcomes of neonates with listeriosis supported by ECMO. STUDY DESIGN: Retrospective study of neonates with culture-proven listeriosis reported to the Extracorporeal Life Support Organization Registry between 1991 and 2017. Comparisons were made between this cohort and the case series from 1975-1991. RESULTS: Twenty-two neonates had culture-proven Listeria monocytogenes infection and required ECMO support. Eight-six percent survived to discharge, compared with 67% in the previous cohort (p = 0.2). The median ECMO duration was 131 h, compared with 209 h in the previous cohort (p = 0.1). Nonsurvivors had a significantly lower pre-ECMO pH (6.91 vs 7.31, p = 0.0006). CONCLUSION: The survival of neonates with listeriosis supported with ECMO is high, supporting the use of ECMO as rescue therapy for this condition.
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Oxigenación por Membrana Extracorpórea , Listeriosis/terapia , Comorbilidad , Oxigenación por Membrana Extracorpórea/efectos adversos , Femenino , Humanos , Recién Nacido , Enfermedades del Recién Nacido/epidemiología , Listeriosis/complicaciones , Listeriosis/mortalidad , Masculino , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
Defects in mevalonate kinase, a critical rate-limiting enzyme in cholesterol and isoprene metabolism, have been associated with 2 clinical phenotypes: mevalonic aciduria, which presents in infancy or early childhood with growth failure, dysmorphic features, and neurologic disease; and hyperimmunoglobulinemia D and periodic fever syndrome, which usually presents outside the neonatal period as an autoinflammatory periodic fever syndrome. This report describes a kindred with 2 siblings affected by severe mevalonate kinase deficiency (mevalonic aciduria) with perinatal onset. Dysmorphic and central nervous system abnormalities, anemia, and cholestasis were prominent features in 1 sibling. Both cases were fatal, 1 in the immediate neonatal period and 1 in utero. The small number of cases of mevalonate kinase deficiency presenting in the perinatal period have typically been severely affected, with signs and symptoms of a severe multisystem disorder. Predominant features of perinatal onset mevalonate kinase deficiency include intrauterine growth restriction, cerebral ventriculomegaly, dysmorphic features, skeletal abnormalities, dyserythropoietic anemia with extramedullary erythropoiesis, thrombocytopenia, cholestatic liver disease, persistent diarrhea, renal failure, recurrent sepsis-like episodes, and failure to thrive. Clinical findings may mimic severe intrauterine viral infection, a chromosomal abnormality, or an acute sepsis syndrome, potentially contributing to delays in diagnosis of this rare condition. Perinatal onset mevalonate kinase deficiency is associated with a very poor prognosis, with death in utero or in early infancy. Detailed autopsy findings in mevalonate kinase deficiency have rarely been reported.
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Deficiencia de Mevalonato Quinasa/genética , Deficiencia de Mevalonato Quinasa/patología , Secuencia de Aminoácidos , Secuencia de Bases , Resultado Fatal , Femenino , Humanos , Masculino , Datos de Secuencia Molecular , Mutación Missense , Linaje , Reacción en Cadena de la Polimerasa , EmbarazoRESUMEN
Based at least in part on concerns for patient safety and evidence that long shifts are associated with an increased risk of physician error, residents' and fellows' work hours have been strictly limited for the past several years. Little attention has been paid, however, to excessive attending physician shift duration, although there seems to be no reason to assume that this common practice poses any less risk to patients. Potential justifications for allowing attending physicians to work without hourly limits include physician autonomy, workforce shortages in certain communities or subspecialties, continuity of care, and financial considerations. None of these clearly justify the apparent increased risk to patients, with the exception in some settings of workforce shortage. In many hospital settings, the practice of allowing attending physicians to work with no limit on shift duration could pose an unnecessary risk to patients.