Quality Indices and Outcomes of a Neonatology Telerounding Program in a Level II Neonatal Intensive Care Unit: Single-Center Experience during the COVID-19 Pandemic.

OBJECTIVE: The objective of this program evaluation was to describe the outcomes of daily neonatologist telerounding with the onsite advanced practice provider (APP) in a Level II neonatal intensive care unit (NICU), before and during the coronavirus disease 2019 (COVID-19) pandemic. STUDY DESIGN: Bedside telerounding occurred with an onsite APP using a telehealth cart and paired Bluetooth stethoscope. Data collected by longitudinal and cross-sectional surveys and chart review before (May 2019-February 2020) and during (March 2020-February 2021) the COVID-19 pandemic were analyzed using descriptive statistics and thematic analysis. RESULTS: A total of 258 patients were admitted to the Level II NICU before (May 2019-February 2020) and during (March 2020-February 2021) the COVID-19 pandemic. Demographic characteristics and outcomes, including breastfeeding at discharge and length of stay were similar pre- and postonset of the COVID-19 pandemic. Postrounding surveys by 10 (response rate 83%) neonatologists indicated parents were present in 80 (77%) of rounds and video was at least somewhat helpful in 94% of cases. Cross-sectional survey responses of 23 neonatologists and APPs (response rate 62%) indicated satisfaction with the program. Common themes on qualitative analysis of open-ended survey responses were "need for goodness of fit" and "another set of eyes" and "opportunities for use." CONCLUSION: Daily telerounding with neonatologists and APPs in a Level II NICU supported neonatal care. Quality metrics and clinical outcomes are described with no differences seen before and during the COVID-19 pandemic. KEY POINTS: · Little is known about Level II NICU quality metrics and outcomes.. · Daily bedside telerounding with neonatologists and APPs is described.. · Telerounding supported neonatal care before and during the COVID-19 pandemic.. · Neonatologists found visual exam helpful in the majority of cases.. · No differences in NICU clinical outcomes were seen during the COVID-19 pandemic..

CHRNB1-associated congenital myasthenia syndrome: Expanding the clinical spectrum.

CHRNB1 encodes the ß subunit of the acetylcholine receptor (AChR) at the neuromuscular junction. Inherited defects in the neuromuscular junction can lead to congenital myasthenia syndrome (CMS), a clinically and genetically heterogeneous group of disorders which includes fetal akinesia deformation sequence (FADS) on the severe end of the spectrum. Here, we report two unrelated families with biallelic CHRNB1 variants, and in each family, one child presented with lethal FADS. We contrast the diagnostic odysseys in the two families, one of which lasted 16 years while the other, utilizing rapid exome sequencing, led to specific treatment in the first 2 weeks of life. Furthermore, we note that CHRNB1 variants may be under-recognized because in both families, one of the variants is a single exon deletion that has been previously described but may not easily be detected in clinically available genetic testing.

Utility of Formulas Using Fetal Thigh Soft Tissue Thickness in Estimating Weight in Gastroschisis.

OBJECTIVES: To evaluate the utility of the fetal thigh soft tissue thickness (STT) in calculating the estimated fetal weight (EFW) in fetuses with gastroschisis versus the standard formula of Hadlock et al (Am J Obstet Gynecol 1985; 151:333-337) compared to the actual birth weight (ABW). METHODS: A retrospective study of neonates born with gastroschisis delivered at our institution was performed. Two reviewers measured the fetal thigh STT on saved images. The estimated gestational age, fetal biometric measurements, and ABW were abstracted. In addition to the Hadlock formula, 3 STT-based formulas reported by Scioscia et al (Ultrasound Obstet Gynecol 2008; 31:314-320) and Kalantari et al (Iran J Reprod Med 2013; 11:933-938) were used to calculate the EFW. RESULTS: Eighty-two patients with gastroschisis qualified for inclusion in our study. The mean STTs ± SD as measured by readers 1 and 2 were 10.9 ± 2.7 and 10.6 ± 2.7 mm, respectively. Seventeen (21%) fetuses were small for gestational age at birth. The Hadlock formula underestimated the EFW relative to the ABW, with an average difference of -97 g (-3.9%) and - 5.1% in terms of growth percentiles. All of the STT-based EFW formulas overestimated the EFW on average by 327 to 701 g (13%-24%) in terms of weight and 26% to 52% in terms of growth percentiles. The Hadlock formula classified 22 as having intrauterine growth restriction (sensitivity, 65%; specificity, 83%, based on the ABW). None of the STT-based formulas classified any fetuses as intrauterine growth restricted. CONCLUSIONS: In a group of patients with gastroschisis, we found that the EFW by the fetal thigh STT calculation overestimated the average fetal weight in all of our cases.

Identification of a deletion containing TBX4 in a neonate with acinar dysplasia by rapid exome sequencing.

We describe a neonate with severe respiratory failure due to acinar dysplasia found by rapid exome sequencing (rES), to have a deletion containing the TBX4 gene. rES can affect patient management in the intensive care unit and should be considered in concert with lung biopsy in neonates with undifferentiated respiratory failure.

Acute Funisitis Correlates With the Risk of Early-Onset Sepsis in Term Newborns Assessed Using the Kaiser Sepsis Calculator.

BACKGROUND: The risk of neonatal early-onset sepsis (EOS) is traditionally assessed on maternal signs of clinical chorioamnionitis. Recently, an online EOS risk calculator was developed by Kaiser Permanente using maternal and neonatal clinical parameters. We were interested in whether an increased Kaiser sepsis risk score correlates with histologic acute chorioamnionitis or acute funisitis. DESIGN: Included in this retrospective review are 119 chorioamnionitis-exposed term neonates from January 1, 2015 and December 31, 2016. Clinical charts from mother-baby pairs were reviewed. An EOS risk score was obtained using the online Kaiser Sepsis Calculator. The presence and severity of acute chorioamnionitis and acute funisitis were recorded. A SPSS software was used for statistical analysis (IBM, New Jersey, USA). RESULTS: The Kaiser Sepsis Calculator could identify 97 of 119 (81.5%) neonates without increased risk for sepsis. Histologic acute chorioamnionitis was present in 100 of 119 cases (84%), in which 44 cases (44%) show severe acute chorioamnionitis. Acute funisitis was recognized in 87 of 119 (73.1%) cases, all of which had concurrent acute chorioamnionitis. Severe funisitis was seen in 38 of the 87 cases (43.7%). The Kaiser Sepsis risk score correlates with the presence and severity of acute funisitis (P = .037 and P = .044, respectively) but not with the presence or the severity of acute chorioamnionitis (P = .105 and P = .672, respectively). CONCLUSION: Our study provides histological evidence to support that the Kaiser Sepsis Calculator may help to effectively reduce unwarranted blood culture, antibiotics exposure, and neonatal intensive care unit admission in term neonates.

Limited Ultrasound Protocol for Upper Extremity Peripherally Inserted Central Catheter Monitoring: A Pilot Study in the Neonatal Intensive Care Unit.

OBJECTIVES: To assess whether a limited ultrasound (US) scanning protocol to monitor the upper extremity peripherally inserted central catheter (PICC) location in neonates is feasible for experienced US operators. METHODS: A radiologist, who was blinded to the PICC location on chest radiography, performed 14 US scans on 11 neonates with upper extremity PICCs. A US machine with 13-6-MHz linear and 8-4-MHz phased array transducers was used for the examinations. RESULTS: The study population included 54% (n = 6) preterm infants, with 72% (n = 8) weighing less than 1500 g. The US location of the PICC was the same as the chest radiographic report in all 14 scans. A subclavicular long-axis view of the anterior chest visualized all PICCs in the subclavian or brachiocephalic veins. A parasternal long-axis right ventricular inflow view was able to visualize PICCs in the superior vena cava (SVC), and a subcostal long-axis view evaluated PICCs in the lower SVC and heart. The scanning time was location dependent: less than 5 minutes for PICCs in the brachiocephalic or subclavian vein and 5 to 10 minutes for PICCs in the SVC or heart. There were no desaturations below 90%, increases in the fraction of inspired oxygen need, or hypotension episodes during scanning. CONCLUSIONS: A limited US scanning protocol to determine the upper extremity PICC location is feasible. Our protocol needs to be tested in neonatal providers before further dissemination.

Point-of-care ultrasound for peripherally inserted central catheter monitoring: a pilot study.

Objective To assess the feasibility and accuracy of point-of-care ultrasound (POCUS) in monitoring peripherally inserted central catheter (PICC) location in neonates by non-radiologist physicians. Methods A prospective cohort study compared PICC localization by ultrasound in neonates with a recent radiograph. The ultrasound exam was performed using a standardized protocol with 13-6 MHz linear and 8-4 MHz phased array transducers by a neonatal-perinatal fellow who was blinded to PICC location on the radiograph. Results Of the 30 neonates included, 96.6% (n = 29) were preterm, with 63.3% (n = 19) weighing <1500 g. Nighty-four percent (n = 94) of ultrasound scans matched the radiograph report. The protocol had a sensitivity of 0.97, specificity of 0.66 and positive predictive value of 0.98. Conclusion Limited ultrasound exams to monitor PICC position in neonates using a standardized protocol by non-radiologist physicians are feasible and accurate in a single ultrasound user. Further study in multiple providers is needed before widespread use.

Implication of antibodies against human leukocyte antigen in simultaneous presentation of fetal and neonatal alloimmune thrombocytopenia and neutropenia.

Fetal and neonatal alloimmune thrombocytopenia (FNAIT) and neonatal alloimmune neutropenia (NAN) are two rare complications of newborns caused by antibodies against paternal inherited antigens. Human platelet (HPA) and neutrophil antigens (HNA) are the common targets. Human leukocyte antigen (HLA) class I proteins are also expressed on platelets and neutrophils and anti-HLA antibodies have occasionally been implicated in these complications. We report a premature twin infant who presented with severe thrombocytopenia and neutropenia clinically compatible with FNAIT and NAN, from a mother with no identifiable HPA or HNA antibodies, but with very high levels of complement-fixing antibodies against paternal inherited HLA. These antibodies were also detected in the infant. HLA antibodies are commonly present in multiparous women who deliver healthy infants. They can, however, be cytotoxic and cause clinical complications after blood products transfusion (TRALI and becoming refractory to platelets transfusion) and after organ transplantation (allogeneic organ rejection).

Evolution of Delivery Room Management for Meconium-Stained Infants: Recent Updates.

BACKGROUND: The approach to intrapartum and postnatal management of an infant born through meconium-stained amniotic fluid (MSAF) in the delivery room (DR) has changed several times over the last few decades, leading to confusion and anxiety among health care providers (nurses, nurse practitioners, respiratory therapists, midwives, and physicians). This article provides state-of-the-art insight into the evidence or lack thereof for the changes in guidelines. PURPOSE: To discuss the evidence for evolution of DR management of vigorous and nonvigorous infants born through any type of MSAF. METHODS: Review of guidelines from the Neonatal Resuscitation Program of the American Academy of Pediatrics and the American College of Obstetricians and Gynecologists, the International Liaison Committee on Resuscitation, Consensus on Science and Treatment Recommendations, and textbooks of neonatal resuscitation and research using MEDLINE via PubMed. FINDINGS: In pregnancies complicated by MSAF, intrapartum suctioning of the oro- and nasopharynx and postnatal intubation with tracheal suctioning of infants have been traditionally used to clear the airway and decrease meconium aspiration syndrome. The recommendations for these perinatal practices have changed several times due to some evidence that the procedures are not beneficial and may even be harmful. IMPLICATIONS FOR PRACTICE: Intrapartum suctioning and postnatal tracheal suctioning of infants (vigorous or nonvigorous) born through MSAF are not recommended. This is a "high-risk" delivery requiring 2 team members to be present at birth-one with full resuscitation skills including tracheal intubation. IMPLICATIONS FOR RESEARCH: Need to evaluate effects of discontinuing the practice of tracheal suctioning in nonvigorous infants on the incidence of meconium aspiration syndrome and neonatal mortality.

Trends in incidence and outcomes of gastroschisis in the United States: analysis of the national inpatient sample 2010-2014.

PURPOSE: Gastroschisis is a severe congenital anomaly associated with a significant morbidity and mortality. There are limited temporal trend data on incidence, mortality, length of stay, and hospital cost of gastroschisis. Our aim was to study these temporal trends using the National Inpatient Sample (NIS). METHODS: We identified all neonatal admissions with a diagnosis of gastroschisis within the NIS from 2010 through 2014. We limited admission age to ≤ 28 days and excluded all those transferred to other hospitals. We estimated gastroschisis incidence, mortality, length of hospital stay, and cost of hospitalization. For continuous variables, trends were analyzed using survey regression. Cochrane-Armitage trend test was used to analyze trends for categorical variables. P < 0.05 was considered as significant. RESULTS: The incidence of gastroschisis increased from 4.5 to 4.9/10,000 live births from 2010 through 2014 (P = 0.01). Overall mortality was 3.5%, median length of stay was 35 days (95% CI 26-55 days), and median cost of hospitalization was $75,859 (95% CI $50,231-$122,000). After adjusting for covariates, there was no statistically significant change in mortality (OR = 1.13; 95% CI 0.87-1.48), LOS (ß = - 2.1 ± 3.5; 95% CI - 9.0 to 4.8) and hospital cost (ß = - 2.137 ± 10.813; 95% CI - 23,331 to 19,056) with each calendar year increase on multivariate logistic regression analysis. CONCLUSION: The incidence of neonates with gastroschisis increased between 2010 and 2014. Incidence was highest in the West. No difference in mortality and resource utilization was observed.

International Classification of Diseases, 10th Revision, coding for prematurity: need for standardized nomenclature.

The International Classification of Diseases, Ninth Revision, Clinical Modification was introduced in the United States in 1979 as a coding system to document inpatient diagnostic and procedural codes. However, transition to International Classification of Diseases, 10th Revision (ICD-10) has been federally mandated by October 2015 due to advances in medical technology and procedures in medicine. This includes ICD-10, Clinical Modification and ICD-10, Procedure Coding System. The ICD-10 coding set is more detailed and specific and will add 54 000 diagnosis codes and 83 000 procedure codes to the International Classification of Diseases, Ninth Revision, Clinical Modification code set, thereby accounting for increased granularity of codes needed for evidence-based medicine. This article describes the importance of need for specificity of the codes and emphasizes the role of training in preparing for implementation of the ICD-10 coding system. An example is made for the need for accuracy in ICD-10 codes for prematurity as regards defining the premature population using standardized nomenclature.

Characterization of Neutropenia in Preterm Neonates Following Administration of Darbepoetin Alfa.

OBJECTIVE: This study is to evaluate the effects of darbepoetin alfa (darbe) on neutrophil count in preterm neonates treated for anemia of prematurity. METHODS: This was a retrospective chart review comparing the absolute neutrophil counts (ANCs) of neonates administered 2 doses of subcutaneous darbe 10 mcg/kg to that of a randomly selected comparator group of neonates not administered the drug. Neonates <34 weeks gestational age, gestational age between 23w1d and 33w4d, born between July 2016 and June 2019, were included in the study. RESULTS: The ANCs of 45 darbe-treated neonates compared with those of 45 randomly selected comparator control neonates revealed no difference in the rate of occurrence of neutropenia (ANC ≤1000/µL) between the darbe-treated neonates (26.7%) and comparator neonates (24.4%) (p > 0.99). There was also no difference in the rate of occurrence of severe neutropenia (ANC ≤500/µL) between the darbe-treated neonates (11.1%) and comparator neonates (6.7%) (p = 0.70). Darbepoetin alfa did not lead to differences in rates of resolution of neutropenia or severe neutropenia. CONCLUSIONS: Short-term administration of darbe did not affect the ANCs of preterm neonates treated for anemia of prematurity. There was no difference in the rates of occurrence of neutropenia, severe neutropenia, or resolution of either between the darbe-treated neonates and comparator neonates.

From A to AB: A Caucasian Mother with High Anti-B Titer Causing Hemolytic Disease of the Newborn.

We report on a term infant with clinically significant hemolysis and hyperbilirubinemia. Testing revealed ABO incompatibility between maternal type A and infant type AB. The maternal alloantibody screen was negative. The infant's direct antiglobulin test was positive, and anti-B IgG was eluted off the infant's red blood cells (RBCs). Testing of the mother's plasma revealed a high anti-B titer. The infant was successfully treated with phototherapy and intravenous immunoglobulin. The bilirubin and hematocrit stabilized, and the infant was discharged home. This case was unusual because of its severity and unusual ABO constellation. Furthermore, this report is an exemplary educational case study on how effective collaboration between the clinical team and the blood bank laboratory is critical in reaching the correct diagnosis. In summary, the differential diagnosis of more unusual and atypical ABO-incompatible constellations must be considered when an infant presents with unexplained hemolysis.