Proposed Screening for Congenital Hyperinsulinism in Newborns: Perspective from a Neonatal-Perinatal Medicine Group.

This perspective work by academic neonatal providers is written specifically for the audience of newborn care providers and neonatologists involved in neonatal hypoglycemia screening. Herein, we propose adding a screen for congenital hyperinsulinism (CHI) by measuring glucose and ketone (i.e., ß-hydroxybutyrate (BOHB)) concentrations just prior to newborn hospital discharge and as close to 48 h after birth as possible, at the same time that the mandated state Newborn Dried Blood Spot Screen is obtained. In the proposed protocol, we do not recommend specific metabolite cutoffs, as our primary objective is to simply highlight the concept of screening for CHI in newborns to newborn caregivers. The premise for our proposed screen is based on the known effect of hyperinsulinism in suppressing ketogenesis, thereby limiting ketone production. We will briefly discuss genetic CHI, other forms of neonatal hypoglycemia, and their shared mechanisms; the mechanism of insulin regulation by functional pancreatic islet cell membrane KATP channels; adverse neurodevelopmental sequelae and brain injury due to missing or delaying the CHI diagnosis; the principles of a good screening test; how current neonatal hypoglycemia screening programs do not fulfill the criteria for being effective screening tests; and our proposed algorithm for screening for CHI in newborns.

That head lag is impressive! Infantile botulism in the NICU: a case report.

BACKGROUND: Infantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, descending paralysis, and, potentially, respiratory failure. Botulism is a diagnosis that is more commonly seen in the pediatric intensive care unit (PICU) or on the general pediatric wards and would not typically be managed in the neonatal intensive care unit (NICU), and therefore requires high clinical suspicion to ensure prompt diagnosis and treatment. CASE PRESENTATION: We discuss a case where an infant from central Pennsylvania presented to a Level IV NICU rather than to the PICU for an evaluation for sepsis and was uniquely diagnosed with IB. The infant presented with poor oral feeding and reduced oral intake, hypothermia, and lethargy. His symptoms progressed into hypoxia and acute respiratory failure. Interestingly, this infant had no known exposure to honey or any other identifiable sources of botulism contact. The infant's twin brother and the other infants who attended the mother's in-home daycare remained asymptomatic. This infant was initially evaluated and managed for a potential infectious etiology. However, a diagnosis of IB was suspected, and was later confirmed through the detection of botulinum toxin in the infant's stools. A high level of suspicion allowed for timely treatment with Botulism Immune Globulin neutralizing antibodies (BabyBIG), even prior to confirmatory testing. We describe the process of obtaining BabyBIG, as well as the natural course of illness after treatment in our patient who ultimately made a complete recovery. CONCLUSIONS: This case highlights the importance of considering infantile botulism as a diagnostic possibility even in the absence of risk factors, and the need for vigilance in diagnosing and treating this rare but potentially life-threatening condition. With timely recognition, subsequent treatment with BabyBIG, and supportive care, infants with infantile botulism can be expected to recover completely. This information is particularly important for neonatologists providing care for infants outside the neonatal period, especially during times of high patient census and resulting overflow of pediatric admissions in the NICU.

Successes of a Focused Mothers' Own Milk (MOM) Program in Counteracting Unintended Effects of a Donor Milk Program on MOM Rates at Discharge.

Background: Avoidance of early formula feeding (EFF) and advancement of mother's own milk (MOM) in very low birth weight (VLBW) infants are important health influencers to decrease serious morbidities. Objective: To present the challenges and feeding strategy successes implemented to counteract a decline in MOM at discharge after initiation of donor milk (DM) to avoid EFF in racially and ethnically diverse VLBW infants. Patients and Methods: Retrospective review of prospectively tracked inborn surviving VLBW infants and their mothers admitted to neonatal intensive care unit from 2010 to 2020 during three feeding strategy implementations baby friendly (BF), DM program, and MOM bundle. Analysis included type of feeding (MOM, DM, or formula) and maternal with descriptive and comparative statistical analysis as indicated. Results: Analysis included 616 VLBW infants. Initiation of BF program resulted in 58.5% of infants discharged on MOM with 41.5% exposed to EFF. Initiation of the DM program resulted in a decline in EFF to 5% and decline in MOM at discharge to 26%. MOM bundle strategy resulted in an increase in MOM at discharge to 41% with sustained EFF exposure 0%. MOM at discharge varied among maternal racial and ethnic backgrounds in all epochs. Early DM use was not different among mothers by race or ethnicity with DM by African American (AA) mothers 89% > White mothers 83% > Other/Hispanic mothers 75%. MOM at discharge was lowest for AA mothers 33% < Hispanic mothers 40% < White mothers 55% < Asian/Other mothers at 60%. Conclusion: Changes in VLBW feeding strategies to avoid EFF utilizing DM can be successful among diverse maternal racial and ethnic populations. Nursing and maternal education coupled with early lactation support and attention to maternal individual long-term feeding plans were critical to improve MOM at discharge among mothers of all racial-ethnic backgrounds for successful attainment of MOM utilization in term corrected VLBW infants at discharge.

Predictors of circuit health in neonatal patients receiving extracorporeal membrane oxygenation (ECMO).

To identify predictors of neonatal ECMO circuit health, a retrospective analysis of circuit functional pressure and flow parameters as well as infant clotting values were collected 48 h prior to and 24 h post circuit change. Circuit impairment was defined as need for partial or total circuit change. Statistical analysis used multivariate statistics and non-parametric Mann-Whitney U-test with possible non-normality of measurements. A total of 9764 ECMO circuit and clotting values in 21 circuits were analyzed. Circuit delta-P mean, and maximum values increased from 8.62 to 48.59 mmHg (p < 0.011) and 16.00 to 53.00 mmHg (p < 0.0128) respectively prior to need for circuit change. Maximum and mean Pump Flow Revolutions per minute (RPM) increased by 75% (p < 0.0043) and 81% (p < 0.0057), respectively. Mean plasma free hemoglobin (pfHb) increased from 26.45 to 76.00 mg/dl, (p < 0.0209). Sweep, venous pressure, and clotting parameters were unaffected. ECMO circuit delta-P, RPM, and pfHb were early predictors of circuit impairment.