Chromosomal Integration of HHV-6 in a Preterm Neonate: A Rare Case of Hyperleukocytosis and Clinical Implications.

Leukocytosis in neonates can occur because of infectious, inflammatory, malignant, or physiological processes. Hyperleukocytosis is defined as a total leukocyte count (TLC) exceeding 100,000 per mm3, warranting immediate evaluation. Neonates with hyperleukocytosis are at risk of leukostasis and the associated severe complications, including respiratory distress, myocardial ischemia, hyperuricemia, acute renal failure, infarction, and hemorrhage. Differentiating leukemia and leukemoid reactions in neonates presenting with elevated TLC is challenging but critical. We present a unique case of a preterm male neonate with hyperleukocytosis, initially suspected to have an underlying malignancy. The neonate's clinical course was complicated by respiratory distress syndrome and anemia of prematurity, necessitating neonatal intensive care unit management. Further investigation revealed high human herpesvirus 6 (HHV-6) DNA levels in the whole blood, leading to a chromosomally integrated HHV-6 (ciHHV-6) diagnosis. CiHHV-6 is characterized by HHV-6 DNA integration into the host genome. Accurate diagnosis relies on whole-blood quantitative PCR, distinguishing ciHHV-6 from an active infection. The neonate remained asymptomatic, and antiviral treatment was deemed unnecessary. This case underscores the importance of recognizing ciHHV-6 as a potential cause of hyperleukocytosis in neonates and highlights the value of whole-blood PCR for differentiation. Understanding the spectrum of HHV-6 infection in neonates is vital for appropriate management and prognostication.

A Quality Improvement Initiative to Minimize Unnecessary Chest X-Ray Utilization in Pediatric Asthma Exacerbations.

Background: Current national guidelines recommend against chest X-rays (CXRs) for patients with acute asthma exacerbation (AAE). The overuse of CXRs in AAE has become a concern, prompting the need for a quality improvement (QI) project to decrease CXR usage through guideline-based interventions. We aimed to reduce the percentage of CXRs not adhering to national guidelines obtained for pediatric patients presenting to the Emergency Department (ED) with AAE by 50% within 12 months of project initiation. Methods: We conducted this study at a New York City urban level-2 trauma center. The team was composed of members from the ED and pediatric departments. Electronic medical records of children aged 2 to 18 years presenting with AAE were evaluated. Monthly data on CXR utilization encompassing instances where the ordered CXR did not adhere to guidelines was collected before and after implementing interventions. The interventions included provider education, visual reminders, printed cards, grand-round presentations, and electronic medical records modifications. Results: The study encompassed 887 eligible patients with isolated AAE. Baseline data revealed a mean preintervention CXR noncompliance rate of 37.5% among children presenting to the ED with AAE. The interventions resulted in a notable decrease in unnecessary CXR utilization, reaching 16.7%, a reduction sustained throughout subsequent months. Conclusions: This QI project successfully reduced unnecessary CXR utilization in pediatric AAE. A multi-faceted approach involving education, visual aids, and electronic reminders aligned clinical practice with evidence-based guidelines. This QI initiative is a potential template for other healthcare institutions seeking to curtail unnecessary CXR usage in pediatric AAE.

Optimizing Invasive Neonatal Respiratory Care: A Systematic Review of Invasive Neurally Adjusted Ventilatory Assist.

BACKGROUND: Mechanical ventilation in preterm neonates aims for synchrony, preventing complications such as lung injury. Neurally Adjusted Ventilatory Assist (NAVA) is a unique mode relying on diaphragmatic electrical signals for synchronization. We conducted a review focusing on the long-term consequences of using invasive NAVA in neonates with a focus on bronchopulmonary dysplasia (BPD). METHODS: A systematic review following PRISMA explored invasive NAVA in preterm neonates. Primary objectives compared NAVA to conventional ventilation, assessing BPD incidence, ventilation duration, length of stay, and adverse events. Secondary objectives analyzed ventilator parameters. RESULTS: After screening 282 records, the review incorporated two randomized controlled trials for primary outcomes and seven trials for secondary outcomes, including two randomized crossovers, four prospective crossovers, and one retrospective study. NAVA showed reduced oxygen requirement at 28 days but no significant differences in oxygen need at 36 weeks postmenstrual age, total length of stay, or ventilator days. Substantial variations were not observed in adverse events. Ventilator variables favored NAVA, indicating decreased peak inspiratory pressure, tidal volume, work of breathing, and respiratory severity score. CONCLUSION: Our study found no significant reduction in BPD with NAVA despite short-term benefits. Future large-scale trials are essential to assess NAVA's impact on long-term outcomes comprehensively.

A Quality Improvement Initiative to Increase Skin-to-Skin Care Duration in Preterm Neonates.

BACKGROUND: Skin-to-skin (STS) care effectively improves neonatal outcomes, particularly for preterm neonates. However, utilization of STS remains suboptimal for the most vulnerable preterm neonates in the first 4 weeks of life. This quality improvement (QI) project aimed to increase STS duration for neonates under 35 weeks gestation. METHODS: The QI initiative was conducted in a 35-bed level IV NICU within a teaching hospital in New York City from July 2021 to January 2023. Six months of baseline data and a staff survey determined interventions across "Plan, Do, Study, Act" cycles. Interim analyses guided interventions using run charts. Interventions included parental counseling and information leaflets, discussion during rounds on STS eligibility, STS education for residents, nurses' feedback, and visual reminders. The primary outcome measure was the mean duration of STS per eligible patient day. The process measures were the age at first STS and documentation of eligibility for STS care in the electronic medical records. Balancing measures included adverse events such as apnea, bradycardia, desaturation, hypothermia, and inadvertent dislodgement of central lines and endotracheal tube. RESULTS: The study included 185 infants with a mean gestational age of 29.1 weeks. The mean STS duration per eligible patient day increased from a baseline of 13.3 minutes to 32.4 minutes without significantly increasing adverse events. CONCLUSIONS: The QI interventions implemented have successfully increased the duration of STS in preterm infants. Our interventions combined into an STS bundle can be a potential model for other NICUs to improve STS practice.

Unveiling triglyceride levels in preterm neonates: a study to guide targeted monitoring.

BACKGROUND: Mixed lipid emulsion (MLE), most commonly soybean, medium chain triglycerides, olive, and fish oils (SMOF), has replaced soybean-based lipid emulsions in many neonatal intensive care units. Only a few studies report the triglyceride (TG) trajectory in neonates receiving MLE. We designed a study to compare TG levels in neonates receiving MLE stratified by gestational age (GA), birth weight (BW), and growth restriction status. METHODS: We included neonates born at <32 weeks GA or with BW <1500 gm. SMOF is started on admission, and plasma TG levels are measured 24 hours after 2 gm/kg/day and 24 hours after 3 gm/kg/day. TG levels were compared across groups defined by GA (<28 weeks vs. 328 weeks), BW (<1000 gm vs. 31000 gm), and small for GA (SGA) vs. appropriate plus large for GA groups using the Wilcoxon rank sum test. RESULTS: From 2018 to 2021, 427 infants met the inclusion criteria. TG levels were significantly higher in neonates with GA <28 weeks, BW <1000 grams, and SGA with a notable broad distribution of TG levels. Logistic regression analysis confirmed SGA and BW as significant independent predictors of hypertriglyceridemia after SMOF at 2 gm/kg/day and 3 gm/kg/day, respectively. CONCLUSIONS: The study emphasizes the importance of TG monitoring for neonates with GA <28 weeks, BW <1000 grams, and SGA. Conversely, it is advisable to individualize TG monitoring for infants with GA>28 weeks, BW>1000 grams, and non-SGA status. Prospective studies with larger sample sizes are warranted to validate our findings.

Unveiling Peripherally Inserted Central Catheter Fractures and Related Complications in the Neonatal Intensive Care Unit: A Concise Review.

Peripherally inserted central catheters (PICCs) have become popular over tunneled catheters in neonatal intensive care units (NICUs) due to their ease of use and convenience. Although rare, a PICC fracture can be a severe and potentially fatal complication. This narrative review aims to identify factors predisposing neonates to PICC fracture and related complications, such as catheter jamming, and explore strategies for preventing and detecting this complication. A thorough search of PubMed and Google Scholar was conducted using relevant keywords to identify articles discussing PICC fracture in neonates. The review encompassed English-language literature on PICC fracture in neonates, with additional pertinent publications identified through citation searching. The incidence of PICC fracture in neonates varies from less than 1% to 10%, with a higher risk associated with prolonged catheterization, lower gestational age and lower birth weight, and the use of multi-lumen catheters. PICC fractures can occur during insertion, maintenance, or removal. Factors such as catheter duration, gestational age, birth weight, and catheter type increase the risk of PICC fracture. Excessive syringe pressure, securement failure, and excessive force during removal are contributing factors. Catheter fatigue and thin-walled catheter design are common causes of breakage. Preventive measures include proper training of healthcare providers, regular monitoring, early recognition, and prompt catheter removal upon fracture. Preventing and detecting PICC fractures is crucial for neonatal safety. Vigilance during insertion, maintenance, and removal, along with care to avoid excessive force during removal and high pressure during flushing, can help prevent catheter breakage. More research is required to improve prevention strategies for PICC fractures in neonates.

A Improving Birth-dose Hepatitis-B Vaccination in a Tertiary Level IV Neonatal Intensive Care Unit.

Background: Perinatal hepatitis B is a global public health concern. To reduce perinatal hepatitis B and its complications, the Hepatitis B vaccine (HBV) is recommended by the New York State Department of Health and Advisory Committee on Immunization Practices within 24 hours of life for infants born with a birth weight ≥2000 g. Infants admitted to the neonatal intensive care unit (NICU) weighing over 2000 g missed their birth dose HBV frequently, which prompted the implementation of a quality improvement initiative to increase birth dose HBV immunization in a level IV NICU in New York. Methods: May 2019 to April 2021 baseline data showed the birth dose HBV rate of infants born ≥2000 g at 24% and 31% within 12 and 24 hours, respectively. The multidisciplinary QI team identified barriers using an Ishikawa cause-and-effect diagram. Our interventions included multidisciplinary collaboration, electronic medical record reminders, education, posters, and improved communication between staff and parents. We aimed to achieve a 25% improvement from the baseline. Results: After 19 months of QI interventions (four Plan-Do-Study-Act cycles), the rate of administering birth dose HBV within 12 hours of life increased from 24% to 56% and within 24 hours from 31% to 64%. Process measure compliance improved, exceeding the 25% target, and showed sustained improvement. Conclusion: This QI initiative improved the rate of eligible infants receiving HBV within the first 24 hours of life in the NICU. This work can serve as a model for other healthcare institutions to improve HBV immunization rates in NICUs.

Unraveling Leukocyte Profile Shifts and Platelet Dynamics Following Leukoreduced Packed Red Cell Transfusions in Very Low Birth Weight Preterm Neonates.

Background Packed red blood cell (PRBC) transfusions are routine in neonatal care and the most common blood product administered to sick neonates. However, their impact on leukocyte and platelet profiles in very low birth weight (VLBW) preterm infants remains largely unexplored. This study examines leukocyte profile shifts and platelet dynamics following leukoreduced PRBC transfusions in VLBW preterm infants, offering insights to improve neonatal care and reduce unnecessary interventions. Methods The study utilized a retrospective cohort design within a single center, focusing on VLBW preterm infants who received PRBC transfusions at a level 3 NICU between January 2014 and June 2019. Data collection encompassed white blood cell (WBC) and platelet count measurements taken 24 hours before and up to 72 hours after PRBC transfusion. Neonates lacking complete blood count (CBC) data within the 72-hour post-transfusion window were excluded. A subgroup analysis distinguished the outcome between the initial PRBC transfusion and subsequent ones. The statistical significance of pre- and post-transfusion laboratory data was determined using the Wilcoxon signed ranks test and paired T-test. Results A cohort of 108 VLBW preterm infants who underwent a total of 402 PRBC transfusions was included in the analysis. The subjects exhibited a mean gestational age of 27.2 ± 2.5 weeks and a mean birth weight of 913 ± 264 grams. Analysis of pre- and post-transfusion data revealed no significant differences in total white blood cell count (WBC), absolute neutrophil count (ANC), absolute monocyte count (AMC), absolute eosinophil count, and absolute lymphocyte count. Notably, the platelet count was significantly decreased in the post-transfusion group (p < 0.001). In a subset analysis limited to the first-time transfusions among the 108 infants, a statistically significant increase was observed in total WBC, AMC, and ANC following transfusion. Conclusions The findings of this study highlight that PRBC transfusions can prompt an increase in neutrophils, monocytes, and eosinophils, coupled with a decline in platelet counts, all within a 72-hour window post-transfusion. Notably, these changes were predominantly discernible after the initial transfusion, with subsequent transfusions demonstrating consistency, except for the observed platelet count reduction. Recognizing these patterns could prove instrumental in averting undue investigations for suspected sepsis, particularly following the initial transfusion event. However, further in-depth investigations are necessary to uncover the underlying factors responsible for the shifts in leukocyte and platelet profiles triggered by PRBC transfusions.

Novel TTN Mutation Causing Severe Congenital Myopathy and Uncertain Association with Infantile Hydrocephalus.

Arthrogryposis multiplex congenita (AMC) is characterized by nonprogressive symmetric contractures of multiple joints with normal intellect and normal systemic examination. AMC is often due to fetal akinesia, which has neurologic, muscular, and connective tissue etiologies. We present a case of AMC due to a variant in the titin (TTN) gene in a term neonate. The infant is homozygous for this variant, c.38442dup, which is predicted to result in a truncated protein (p.Pro12815Thr fs∗37, NM_001267550.2). A literature search (PubMed) failed to find reports of this TTN variant. The variant was classified as pathogenic and submitted to ClinVar. Titin is the body's largest protein, expressed in skeletal and cardiac muscles and encoded by the TTN gene. Due to its large size (364 exons), the TTN gene has been difficult to sequence; the number of variants in the TTN gene and the spectrum of titinopathies are probably underestimated.

A Rare Case of Lethal Neonatal Rigidity and Multi-Focal Seizure Syndrome.

We present a case of lethal neonatal rigidity and multifocal seizure syndrome (RMFSL) in an early-term female infant born to non-consanguineous parents. RMFSL is a recently discovered autosomal recessive disease caused by the BRAT1 gene mutations. The BRAT1 gene encodes the BRCA1-associated protein required for ATM activation-1, a protein that interacts with BRCA1 and ATM to initiate DNA repair in response to DNA damage. The exon sequence revealed biallelic deletions of exon 1-2 of the BRAT1 gene in our patient. There are only a few cases of RMFSL reported in the literature, and all of them have died before two years, mostly in the first six months of life. Our patient died at the age of 74 days.

Severe Rhabdomyolysis in a Pediatric Patient after Coxsackie B Virus Infection without Acute Renal Failure: A Case Report.

Rhabdomyolysis is a condition resulting from the breakdown of skeletal muscle fibers with leakage of muscle enzymes into the circulation. The degraded muscle components in the circulation can lead to lethal complications as acute renal failure (ARF). In younger children, viral infections tend to be the major cause while trauma and exercise are the important ones in adolescents. Several viruses such as influenza A & B, parainfluenza and coxsackie have been implicated in causing rhabdomyolysis. We report a case of a 14-year-old girl with severe rhabdomyolysis after recent Coxsackie B infection without acute renal failure.