Publications by authors named "Robert D Christensen"

177 Publications

Infantile Pyknocytosis: End-Tidal CO, %Micro-R Measurements, Next-Generation Sequencing, and Transfusion Avoidance with Darbepoetin.

Biomed Hub 2020 Sep-Dec;5(3):227-234. Epub 2020 Dec 11.

Division of Neonatology, Department of Pediatrics, University of Utah, Salt Lake City, Utah, USA.

Infantile pyknocytosis is a rare, self-limited, hemolytic condition of unknown pathogenesis. It is diagnosed when a neonate with Coombs-negative hemolytic anemia has abundant pyknocytes and a characteristic clinical course after other hemolytic disorders has been excluded. Previous reports suggest that transfusions might be avoidable in this condition by administering recombinant erythropoietin. We cared for a patient with this disorder where we employed novel diagnostics and therapeutics. Despite these, and a good outcome free of transfusions, we continue to consider the condition to be idiopathic.
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http://dx.doi.org/10.1159/000511388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8136318PMC
December 2020

A New Hour-Specific Serum Bilirubin Nomogram for Neonates ≥35 Weeks of Gestation.

J Pediatr 2021 May 21. Epub 2021 May 21.

Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford Medicine, and Lucile Packard Children's Hospital, Stanford, CA.

Objective: To develop a statistically rigorous, hour-specific bilirubin nomogram for newborns based on a very large data set; and use it prospectively as a replacement for the 1999 Bhutani nomogram.

Study Design: This was a retrospective analysis of first total serum bilirubin (TSB) measurements from 15 years of universal bilirubin screening during birth hospitalizations at 20 Intermountain Healthcare hospitals. Hour-specific TSB values were assembled into a nomogram by percentile, and subgroups were compared.

Results: The information obtained included robust data in the first 12 hours after birth (which was not included in the 1999 nomogram), general agreement with the 1999 nomogram for values in the first 60 hours, but higher 75th and 95th percentile TSB values thereafter in the new version, no difference in TSB between male and female infants, higher TSB values among earlier gestation neonates (35-36 weeks vs ≥37 weeks, P < .0001), and lower TSB values in neonates of Black race (P < .0001) and higher values in neonates of Asian race (P < .001).

Conclusions: An updated and more informative Bhutani neonatal bilirubin nomogram, based on 140 times the number of subjects included the 1999 version, is now in place in our health care system.
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http://dx.doi.org/10.1016/j.jpeds.2021.05.039DOI Listing
May 2021

Early iron supplementation and iron sufficiency at one month of age in NICU patients at-risk for iron deficiency.

Blood Cells Mol Dis 2021 May 6;90:102575. Epub 2021 May 6.

Department of Pediatrics, Division of Neonatology, University of Utah Health, Salt Lake City, UT, USA; Intermountain Healthcare Research, Salt Lake City, UT, USA; Center for Iron and Heme Disorders, University of Utah, Salt Lake City, UT, USA; Department of Pediatrics, Division of Hematology/Oncology, University of Utah Health, Salt Lake City, UT, USA.

In order to reduce iron deficiency in neonates at-risk for iron deficiency, we implemented a guideline to increase the consistency of early iron supplementation in infants of diabetic mothers, small for gestational age neonates and very low birthweight premature neonates. Three years following implementation we performed a retrospective analysis in order to assess adherence to the guideline and to compare timing of early iron supplementation and reticulocyte-hemoglobin (RET-He) values at one month of life in at-risk infants. Adherence with early iron supplementation guidelines was 73.4% (399/543) with 51% (275/543) having RET-He values obtained at one month. Despite good adherence, 16% (44/275) had RET-He <25 pg (5th percentile for gestational age). No infants receiving red blood cell transfusion (0/20) had RET-He <25 pg vs. 26.1% (40/153) of those treated with darbepoetin (p < 0.001). There was no evidence of increased feeding intolerance (episodes of emesis/day) with early iron supplementation.
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http://dx.doi.org/10.1016/j.bcmd.2021.102575DOI Listing
May 2021

Effect of blood transfusions on cognitive development in very low birth weight infants.

J Perinatol 2021 Apr 28. Epub 2021 Apr 28.

Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.

Objective: Preterm infants frequently receive red cell transfusions; however, the effect of transfusions on cognition is unclear. We evaluated the relationship between transfusions and cognitive outcomes in preterm infants enrolled in a randomized trial of erythropoiesis stimulating agents (ESAs).

Study Design: Preterm infants were randomized to ESAs or placebo during initial hospitalization, and transfusions recorded. Children were evaluated using standard developmental tests of cognition at 18-22 months (56 ESA, 24 placebo) and 3.5-4 years (39 ESA, 14 placebo).

Results: Cognitive scores at 18-22 months were inversely correlated with transfusion volume (p = 0.02). Among those receiving ≥1 transfusion, cognitive scores were significantly higher in the ESA-treated group (p = 0.003). At 3.5-4 years, transfusions were not correlated with cognitive scores.

Conclusions: In the placebo group, transfused children had lower cognitive scores than did non-transfused children at 18-22 months. In the ESA group, cognitive scores did not differ by transfusion status, suggesting ESAs might provide neuroprotection.
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http://dx.doi.org/10.1038/s41372-021-00997-9DOI Listing
April 2021

Maternal obesity and impaired offspring neurodevelopment: could fetal iron deficiency be a pathogenic link?

J Perinatol 2021 May 16;41(5):1199-1200. Epub 2021 Feb 16.

Department of Pediatrics, Division of Neonatology, University of Utah Health, Salt Lake City, UT, USA.

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http://dx.doi.org/10.1038/s41372-021-00951-9DOI Listing
May 2021

Is the erythropoietin-erythroferrone-hepcidin axis intact in human neonates?

Blood Cells Mol Dis 2021 May 6;88:102536. Epub 2021 Jan 6.

Division of Neonatology, University of Utah Health, Salt Lake City, UT, USA; Division of Hematology/Oncology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, USA; Center for Iron and Heme Disorders, University of Utah, Salt Lake City, UT, USA; Women and Newborns Research, Intermountain Healthcare, Murray, UT, USA.

In a two-part process, we assessed elements of the principal hormonal pathway regulating iron homeostasis in human neonates. Part 1: Quantifying erythropoietin (Epo), erythroferrone (ERFE), hepcidin, and relevant serum and erythrocytic iron-related metrics in umbilical cord blood from term (n = 13) and preterm (n = 10) neonates, and from neonates born to mothers with diabetes and obesity (n = 13); Part 2: Quantifying serum Epo, ERFE, and hepcidin before and following darbepoetin administration. Part 1: We measured Epo, ERFE and hepcidin in all cord blood samples. Epo and ERFE levels did not differ between the three groups. Preterm neonates had the lowest hepcidin levels, while neonates born to diabetic women with a very high BMI had the lowest ferritin and RET-He levels. Part 2: Following darbepoetin dosing, ERFE levels generally increased (p < 0.05) and hepcidin levels generally fell (p < 0.05). Our observations suggest that the Epo/ERFE/hepcidin axis is intact in the newborn period.
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http://dx.doi.org/10.1016/j.bcmd.2021.102536DOI Listing
May 2021

Red blood cell transfusions and potentially related morbidities in neonates under 32 weeks' gestation.

Blood Transfus 2021 Mar 14;19(2):113-119. Epub 2020 Oct 14.

Department of Women's and Children's Health, ASL Bari, Neonatal Intensive Care Unit, "Di Venere" Hospital, Bari, Italy.

Background: Preterm neonates are likely to require red blood cell (RBC) transfusion, and extremely low birth weight infants almost invariably receive multiple transfusions. Transfusion-reduction strategies can reduce transfusion rates, and might diminish certain adverse outcomes associated with transfusions.

Materials And Methods: In a single centre, we retrospectively evaluated RBC transfusion rates among preterm infants ≤32 weeks' gestational age (GA), over a 6-year period before and after adopting national transfusion-reduction strategies. We compared demographic data, adverse events, and outcomes between transfused vs not-transfused neonates. Univariate logistic regression was used to evaluate associations between dichotomous outcomes and number of transfusions, and day of first transfusion. Multivariate logistic regression evaluated the correlation between dichotomous outcomes and transfusion as an independent risk factor.

Results: During the 6 years studied, 181 infants born at ≤32 weeks' GA were admitted to our Neonatal Intensive Care Unit of whom 80 (44%) received at least one RBC transfusion. The transfusion rate tended downwards after adopting transfusion-reduction strategies, reaching 31% in 2018. The reduction was largely due to a marked fall in transfusions of neonates born at 29-32 weeks' GA (p<0.001). The number of transfusions received correlated with odds of having intraventricular haemorrhage (IVH) (OR=1.9; 95% CI: 1.3-2.7; p=0.0001) and the duration of oxygen supplementation (rho=0.51; 95% CI: 0.33-0.66; p≤0.0001). In multivariate logistic regression analysis, transfusion was an independent risk factor for IVH (adjusted OR=7.38; 95% CI: 2.24-24.30; p=0.0001).

Discussion: The application of national, standardised transfusion-reduction strategies was associated with a lower transfusion rate in neonates born at 29-32 weeks' GA, but was less effective among neonates ≤28 weeks, in whom transfusions appeared to be an independent risk factor for severe IVH.
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http://dx.doi.org/10.2450/2020.0092-20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7925223PMC
March 2021

Reconciling markedly discordant values of serum ferritin versus reticulocyte hemoglobin content.

J Perinatol 2021 Mar 4;41(3):619-626. Epub 2020 Oct 4.

Division of Neonatology, University of Utah Health, Salt Lake City, UT, USA.

Objective: To determine why serum ferritin and reticulocyte hemoglobin (RET-He), drawn to assess neonatal iron sufficiency, sometimes have markedly discordant results.

Study Design: Retrospective records review of five NICUs over 28 months, identifying all patients with a ferritin and RET-He within 48 h. We examined records of all who had marked discordance (one value >95th % reference interval, the other <5th %).

Results: Of 190 paired ferritin and RET-He measurements, 16 (8%) were markedly discordant. Fifteen of the 16 discordant samples involved a high ferritin and a low RET-He. In these, low MCV and high %Micro-R, and low MCH and high %HYPO-He were present. In total, 8 of the 15 had laboratory or clinical evidence of an inflammatory process and five had suspicion of infection documented.

Conclusions: When ferritin and RET-He were discordant, erythrocyte microcytosis and hypochromasia suggested that the RET-He gave the more accurate interpretation; that iron deficiency was likely present.
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http://dx.doi.org/10.1038/s41372-020-00845-2DOI Listing
March 2021

A Novel Variant in G6PD (c.1375C>G) Identified from a Hispanic Neonate with Extreme Hyperbilirubinemia and Low G6PD Enzymatic Activity.

Neonatology 2020 28;117(4):532-535. Epub 2020 Sep 28.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah, USA.

We report a novel glucose-6-phosphate dehydrogenase (G6PD) variant (c.1375C>G) discovered in a 3-day-old Hispanic male child from Salt Lake City, UT, USA. This newborn presented with severe hyperbilirubinemia (29.8 mg/dL or 510 μmol/L) and marked hemolysis evidenced by elevated end-tidal carbon monoxide concentration (5.9 ppm, normal <1.7 ppm). Despite a very low prevalence of G6PD deficiency in Hispanic populations, we pursued testing for this condition and found he had low erythrocyte G6PD enzyme activity (2.8 U/g Hb, normal 9.9-16.6 U/g Hb) and a novel G6PD variant. His mother was heterozygous for this same variant, and she had a moderate decrease in G6PD enzyme activity (7.1 U/g Hb). On the basis of these findings, we propose this variant as a novel pathogenic mutation.
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http://dx.doi.org/10.1159/000510300DOI Listing
September 2020

Does heterozygosity for UGT1A1 *28 convey increased risk for severe neonatal jaundice?

J Perinatol 2021 Apr 21;41(4):658-660. Epub 2020 Sep 21.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, USA.

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http://dx.doi.org/10.1038/s41372-020-00826-5DOI Listing
April 2021

Decision Accuracy and Safety of Transcutaneous Bilirubin Screening at Intermountain Healthcare.

J Pediatr 2021 01 2;228:53-57. Epub 2020 Sep 2.

Women and Newborn's Clinical Program, Intermountain Healthcare, Salt Lake City, UT; Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT; Division of Hematology/Oncology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT.

Objective: To quantify the risk that transcutaneous bilirubin (TcB) screening would fail to recommend phototherapy for a neonate who would have qualified for it if total serum bilirubin (TSB) screening were used.

Study Design: We conducted a quality improvement project where simultaneous TcB and TSB were obtained on neonates ≥35 weeks of gestation during birth hospitalizations in our hospital system. Using our Utah bilirubin management algorithm, we quantified the risk that TcB screening would fail to identify the need for a confirmatory TSB when TSB screening alone would have revealed that phototherapy was indicated.

Results: In 3 hospitals, we obtained 727 paired TcB/TSB measurements. Two instances utilized a blood gas radiometer for TSB, and 725 utilized the clinical laboratory-based TSB method. One of the 727 instances had a TcB indicating NO PHOTOTHERAPY, when the simultaneous TSB indicated PHOTOTHERAPY NEEDED. The TSB from that instance was 1 of the 2 from the blood gas radiometer. We estimate the risk of such an error occurring is 1.4 per 1000 TcB measurements (95% CI 0.03-7.6 per 1000). When only the laboratory TSB is used, we estimate the risk of such an error occurring to be 0 per 1000 TcB measurements (95% CI 0.0-5.1 per 1000).

Conclusions: Using TcB for screening at the birth hospital can identify those qualifying for phototherapy, using the Utah guidelines, with 1 of 727 neonates with a blood gas bilirubin and none of 725 with a laboratory-based analysis misidentified as not needing phototherapy when by TSB they did.
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http://dx.doi.org/10.1016/j.jpeds.2020.08.079DOI Listing
January 2021

Improving thermoregulation in transported preterm infants.

J Perinatol 2021 Feb 3;41(2):356-357. Epub 2020 Sep 3.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, USA.

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http://dx.doi.org/10.1038/s41372-020-00812-xDOI Listing
February 2021

Warming blood products for transfusion to neonates: In vitro assessments.

Transfusion 2020 09 10;60(9):1924-1928. Epub 2020 Aug 10.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah, USA.

Background: Blood products may be transfused into neonates at temperatures at or below room temperature. The benefits and risks of warming blood to 37°C are not defined in this population or with the equipment used in neonates. Physiologic warming might enhance product effectiveness or decrease transfusion-associated hypothermia.

Study Design And Methods: We utilized an in vitro model of neonatal transfusions, with a syringe pump, blood tubing, and 24-gauge catheter and compared current practice (cold products) vs an inline blood warmer. Transfusions were performed rapidly (30 minutes) and slower (120 minutes) to model emergent vs routine situations. We tested red blood cells, fresh-frozen plasma, apheresis platelets (PLTs), and cold-stored low-titer group O whole blood. We used infrared detectors and inline probes to measure temperatures at the origin and at the simulated patient. We assessed warmer-induced damage by measuring plasma hemoglobin and hematocrit (seeking hemolysis), fibrinogen (seeking activation of coagulation), and PLT count and TEG-MA (seeking PLT destruction or dysfunction).

Results: The cold-stored products were 4.2 ± 1.0°C (mean ± SD) at the origin and 21.5 ± 0.1°C at the patient. With the inline warmer, products were 37.8 ± 0.6°C at the warmer and 32.6 ± 1.7°C at the patient during a 30-minute infusion, but were 34.5 ± 2.1 with a foil sheath covering the terminal tubing. We found no warmer-induced damage using any metric.

Conclusion: In simulated neonatal intensive care unit (NICU) transfusions, an inline blood warmer can deliver blood products at near-physiologic temperatures with no detected damage. We suggest in vivo testing of warmed NICU transfusions, assessing product effectiveness and hypothermia risk reduction.
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http://dx.doi.org/10.1111/trf.16007DOI Listing
September 2020

Urinary ferritin; a potential noninvasive way to screen NICU patients for iron deficiency.

J Perinatol 2020 Jul 24. Epub 2020 Jul 24.

Intermountain Healthcare, Women and Newborns Clinical Program, Salt Lake City, UT, USA.

Objective: Building on our previous study, showing a correlation between ferritin in serum and urine, we conducted a feasibility evaluation, measuring urinary ferritin as a potential noninvasive screening test for iron deficiency among NICU patients.

Study Design: This was a prospective analysis of paired serum/urine ferritin levels. We defined iron-limited erythropoiesis by a RET-He <5th percentile lower reference interval (<28 pg).

Results: We obtained 49 paired serum/urine samples from neonates judged as at-risk for iron deficiency. Urine ferritin ("corrected" for urine creatinine and specific gravity) correlated with serum ferritin (correlation coefficient of log-transformed values 0.44). A corrected urine ferritin <12 ng/mL had a sensitivity of 82% (95% CI, 67-93%) and a specificity of 100% (CI, 66-100%) for detecting iron-limited erythropoiesis, with a positive predictive value of 100% (CI, 89-100%).

Conclusions: Measuring urinary ferritin in NICU patients is feasible. Since low values identify iron-limitation, this could become a useful noninvasive screen.
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http://dx.doi.org/10.1038/s41372-020-0746-6DOI Listing
July 2020

Exchange transfusion for hemolytic hyperbilirubinemia: could some be averted by emergent administration of an inhibitor of bilirubin production?

J Perinatol 2021 Apr 15;41(4):860-864. Epub 2020 Jul 15.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, USA.

Objectives: The objective of this study is to explore the hypothetical number of neonates where an exchange transfusion (ET) could be prevented by emergency administration of an inhibitor of bilirubin production.

Study Design: We identified all neonates who received an ET in our NICUs during the past 12 years. We reviewed the indications for ET and recorded the time between ordering and beginning the exchange.

Results: Forty-six neonates underwent ET, 37 (80.4%) for hemolytic hyperbilirubinemia (36.9 ± 2.9 weeks gestation and 2.5 ± 2.1 days old at ET). The mean delay period was 7.5 ± 3.5 h. Nine (19.6%) had ET not involving bilirubin.

Conclusions: A trial testing compounds that can inhibit bilirubin production would have about three eligible neonates/years in our system. Since our births are 1% of national, up to 300 neonates/years might qualify for such a study.
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http://dx.doi.org/10.1038/s41372-020-0736-8DOI Listing
April 2021

Dizygotic twins with prolonged jaundice and microcytic, hypochromic, hemolytic anemia with pyropoikilocytosis.

Blood Cells Mol Dis 2020 11 25;85:102462. Epub 2020 Jun 25.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, United States of America; Division of Hematology/Oncology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, United States of America.

Dizygotic twin males, born at 34 weeks gestation, had prolonged jaundice, microcytic, hypochromic anemia, FABarts hemoglobin, elevated end-tidal CO, and blood films consistent with hereditary pyropoikilocytosis. DNA sequencing revealed both had a heterozygous alpha spectrin (SPTA1) mutation (c.460_462dup) inherited from their asymptomatic mother, plus a 3-base pair duplication in alpha globin (HBA2) (c.364_366dupGTG) inherited from their asymptomatic father.
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http://dx.doi.org/10.1016/j.bcmd.2020.102462DOI Listing
November 2020

Pediatric hematology normal ranges derived from pediatric primary care patients.

Am J Hematol 2020 Jun 12. Epub 2020 Jun 12.

Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1002/ajh.25904DOI Listing
June 2020

Emergency-release blood transfusions after postpartum hemorrhage at the Intermountain Healthcare hospitals.

Transfusion 2020 07 11;60(7):1418-1423. Epub 2020 Jun 11.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah, USA.

Background: Most low-risk obstetric patients do not have crossmatched blood available to treat unexpected postpartum hemorrhage. An emergency-release blood transfusion (ERBT) program is critical for hospitals with obstetrical services. We performed a retrospective analysis of obstetrical ERBTs administered in our multihospital system.

Design And Methods: We collected data from the past 8 years at all Intermountain Healthcare hospitals on every ERBT after postpartum hemorrhage; logging circumstances, number and type of transfused products, and outcomes.

Results: Eighty-nine women received ERBT following 224,035 live births, for an incidence of 3.97 transfused women/10,000 births. The most common causally-associated conditions were: uterine atony (40%), placental abruption/placenta previa (16%), retained placenta (11%), and uterine rupture (5%). The mean number of total units transfused was 7.9 (range 1-76). The mean number of red blood cells (RBCs) transfused was 4.8, the median 4, and SD was ±4.4. Massive transfusion protocols (MTPs) for trauma recommend using a ratio of 1:1:1 or 2:1:1 of RBC:FFP:Platelets, however the ratios varied widely for postpartum hemorrhage. Only 1.5% received a 1:1:1 ratio and 7.5% received a 2:1:1 ratio. Nineteen percent (17/89) of women underwent hysterectomy, 7% (6/89) had uterine artery embolization, 36% (32/89) had an intensive care unit admission, and 1% (1/89) died.

Conclusion: Emergency transfusion for postpartum hemorrhage occurred after 1/2500 births. Most women received less FFP and platelets than recommended for traumatic hemorrhage. A potentially better practice for postpartum hemorrhage would be a balanced ratio of blood products, transfusion of low-titer, group O, cold-stored, whole blood, or inclusion in a MTP.
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http://dx.doi.org/10.1111/trf.15903DOI Listing
July 2020

Improving the Bilirubin Management Program in the Newborn Nursery: Background, Aims, and Protocol.

Neonatology 2020 7;117(3):358-364. Epub 2020 Feb 7.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah, USA.

Background: Practices to detect and manage hyperbilirubinemia in newborn nurseries are highly variable. American Academy of Pediatrics guidelines in 1999, 2004, and 2009 have generated, perhaps unintentionally, divergent practices that might not all be of equivalent value. Evidence-based progress is needed to define less invasive, less expensive, uniform, and safe methods to reduce ER visits and hospital readmissions for jaundice treatment and bilirubin encephalopathy.

Objectives: This research briefing is intended to inform readers of a new prospective quality improvement program aimed at testing the value of specific changes in newborn nursery hyperbilirubinemia detection and management. This new program includes predetermined means of assessing those specific changes, which relate to diagnosis, safety, outcomes, and cost.

Methods: In this briefing, we present the perceived problems in our present bilirubin management system, as voiced by stakeholders. We report our proposed means to test minimization of those problems utilizing already acquired data on approximately 400,000 well babies in the Intermountain Healthcare system of hospitals in the western USA. We then describe our methods of assessing specific outcomes in a pre- versus postpractice change analysis.

Results And Conclusions: The University of Utah Newborn Nursery will implement a quality improvement project in bilirubin management during 2020 to test the feasibility and effectiveness of several changes to our current bilirubin management program. We maintain that the improved understanding generated by this project will be a step toward new evidence-based strategies for reducing ER visits and hospital readmissions for jaundice treatment and preventing bilirubin encephalopathy.
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http://dx.doi.org/10.1159/000505818DOI Listing
February 2020

Early Hyperbilirubinemia in Neonates with Down Syndrome.

J Pediatr 2020 04 31;219:140-145. Epub 2020 Jan 31.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT; Women and Newborn's Clinical Program, Intermountain Healthcare, Salt Lake City, UT; Division of Hematology-Oncology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT.

Objective: To compare total serum bilirubin (TSB) levels, phototherapy usage, and hospital readmission for jaundice among neonates with Down syndrome vs controls.

Study Design: A retrospective cohort study using 15 years of multihospital data. We created control reference intervals (5th, median, and 95th percentiles) for initial TSB values hourly during the first days after birth, and determined the proportion of neonates with Down syndrome whose TSB exceeded the 95th percentile control interval. We determined the proportion with an initial TSB exceeding the upper control reference interval, the highest TSB recorded, the percentage of neonates receiving phototherapy, and the rate of hospital readmission for jaundice treatment.

Results: We compared 357 neonates with Down syndrome with 377 368 controls. Compared with controls, those with Down syndrome had 4.7 times the risk (95% CI, 3.9-5.7; P < .0001) of an initial TSB exceeding the 95th percentile control interval (23.5% vs 5.0%), 8.9 times (95% CI, 8.1-9.8; P < .0001) the phototherapy usage (62.2% vs 7.0%), and 3.6 times (95% CI, 1.6-8.2; P = .0075) the readmission rate for jaundice (17.4 vs 4.8 per 1000 live births).

Conclusions: Neonates with Down syndrome have a substantial risk of early hyperbilirubinemia. The American Academy of Pediatrics currently advises obtaining an early screening complete blood count from neonates with Down syndrome. We submit that assessing their TSB is also advisable.
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http://dx.doi.org/10.1016/j.jpeds.2019.12.039DOI Listing
April 2020

Absence of severe neonatal ABO hemolytic disease at Intermountain Healthcare. Why?

J Perinatol 2020 02 3;40(2):352-353. Epub 2019 Dec 3.

Women and Newborn's Clinical Program, Intermountain Healthcare, Salt Lake City, UT, USA.

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http://dx.doi.org/10.1038/s41372-019-0553-0DOI Listing
February 2020

The fragmented red cell count can support the diagnosis of a microangiopathic neonatal condition.

J Perinatol 2020 02 6;40(2):354-355. Epub 2019 Nov 6.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, UT, USA.

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http://dx.doi.org/10.1038/s41372-019-0552-1DOI Listing
February 2020

Evaluating emergency-release blood transfusion of newborn infants at the Intermountain Healthcare hospitals.

Transfusion 2019 10 3;59(10):3113-3119. Epub 2019 Sep 3.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah.

Background: An emergency-release blood transfusion (ERBT) protocol (uncrossmatched type O-negative red blood cells, AB plasma, AB platelets) is critical for neonatology practice. However, few reports of emergency transfusions are available. We conducted an ERBT quality improvement project as a basis for progress.

Study Design And Methods: For each ERBT in the past 8 years, we logged indications, products, locations and timing of the transfusions, and outcomes.

Results: One hundred forty-nine ERBTs were administered; 42% involved a single blood product, and 58% involved two or more. The incidence was 6.25 ERBT per 10,000 live births, with a higher rate (9.52 ERBT/10,000) in hospitals with a Level 3 neonatal intensive care unit (NICU) (p < 0.001). Seventy percent of ERBTs were administered in a NICU and 30% in a delivery room, operating room, or emergency department. Indications were abruption/previa (32.2%), congenital anemia (i.e., fetomaternal hemorrhage; 15.4%), umbilical cord accident (i.e., velamentous insertion; 15.0%), and bleeding/coagulopathy (12.8%). Fifty-eight percent of those with hemorrhage before birth did not have a hemoglobin value reported on the umbilical cord gas; thus, anemia was not recognized initially. None of the 149 ERBTs were administered using a blood warmer. The mortality rate of recipients was 35%.

Conclusion: Based on our findings, we recommend including a hemoglobin value with every cord blood gas after emergency delivery to rapidly identify fetal anemia. We also discuss two potential improvements for future testing: 1) the use of a warming device for massive transfusion of neonates and 2) the use of low-titer group O cold-stored whole blood for massive hemorrhage in neonates.
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http://dx.doi.org/10.1111/trf.15495DOI Listing
October 2019

First report of using low-titer cold-stored type O whole blood in massive postpartum hemorrhage.

Transfusion 2019 10 30;59(10):3089-3092. Epub 2019 Aug 30.

Division of Neonatology, Department of Pediatrics, University of Utah Health, Salt Lake City, Utah.

Background: In cases of massive hemorrhage in the US military, improved outcomes have been reported with the use of warm, fresh whole blood transfusions. Cold-stored low-titer type O whole blood (LTOWB) has become the preferred product for resuscitation of severe bleeding in deployed surgical units. Reports of LTOWB use in civilian trauma are becoming more frequent.

Case Report: We report our experience with emergency transfusion of LTOWB for a woman with massive postpartum hemorrhage. The patient had two previous cesarean section deliveries at term without complications. With her third elective cesarean section at term, blood loss during surgery was not excessive, but 3 to 4 hours later she had an estimated blood loss of 3600 mL. Despite measures to control the hemorrhage, she rapidly became hypotensive and tachycardic, and our massive transfusion protocol (MTP) was activated. The transfusion service had very recently incorporated LTOWB into Trauma Pack 1 of the MTP. She received two LTOWB units, after which her hemorrhaging ceased, blood pressure normalized, and she became alert. One hour later she received one unit of fresh frozen plasma and one unit of red blood cells (RBCs). The following morning she received one unit of crossmatched RBCs, for a hematocrit of 20.7%. She was discharged home on Day 4, and she remains healthy.

Conclusions: This is the first report of which we are aware of massive postpartum hemorrhage treated using LTOWB. Our positive experience leads us to speculate that this approach could have a role in massive obstetric hemorrhage.
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http://dx.doi.org/10.1111/trf.15492DOI Listing
October 2019

Neonates with suspected microangiopathic disorders: performance of standard manual schistocyte enumeration vs. the automated fragmented red cell count.

J Perinatol 2019 11 28;39(11):1555-1561. Epub 2019 Aug 28.

Department of Pathology, Intermountain Medical Center, Murray, UT, USA.

Objectives: To enhance the diagnosis of schistocyte-producing conditions, we compared routine manual schistocyte enumeration with automated fragmented red cell counts (FRCs).

Study Design: In neonates "suspected" of having sepsis, NEC, or DIC we compared manual schistocyte estimates vs. automated FRC counts. When the two disagreed, we used a "gold standard" from a  ≥ 1000 RBC differential. We also assessed the diagnostic accuracy of the FRC count in diagnosing sepsis, NEC, or DIC.

Results: We collected 270 CBCs from 90 neonates. The methods agreed in 63% (95% CI 55%-70%) of the CBCs. Among the 37% where they disagreed, the FRC count was more accurate in 100% (95% CI 88-100%). An elevated FRC count was specific for sepsis, and was sensitive and specific for necrotizing enterocolitis and DIC.

Conclusions: Automated FRC counts have advantages over routine manual evaluation, larger sample size, lower expense, and superior accuracy in diagnosing schistocyte-producing conditions.
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http://dx.doi.org/10.1038/s41372-019-0482-yDOI Listing
November 2019

Medicinal Uses of Hematopoietic Growth Factors in Neonatal Medicine.

Handb Exp Pharmacol 2020 ;261:257-283

Divisions of Neonatology and Hematology/Oncology, Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.

This review focuses on certain hematopoietic growth factors that are used as medications in clinical neonatology. It is important to note at the chapter onset that although all of the pharmacological agents mentioned in this review have been approved by the US Food and Drug administration for use in humans, none have been granted a specific FDA indication for neonates. Thus, in a sense, all of the agents mentioned in this chapter could be considered experimental, when used in neonates. However, a great many of the pharmacological agents utilized routinely in neonatology practice do not have a specific FDA indication for this population of patients. Consequently, many of the agents reviewed in this chapter are considered by some practitioners to be nonexperimental and are used when they judge such use to be "best practice" for the disorders under treatment.The medicinal uses of the agents in this chapter vary considerably, between geographic locations, and sometimes even within an institutions. "Consistent approaches" aimed at using these agents in uniform ways in the practice of neonatology are encouraged. Indeed some healthcare systems, and some individual NICUs, have developed written guidelines for using these agents within the practice group. Some such guidelines are provided in this review. It should be noted that these guidelines, or "consistent approaches," must be viewed as dynamic and changing, requiring adjustment and refinement as additional evidence accrues.
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http://dx.doi.org/10.1007/164_2019_261DOI Listing
October 2020

Platelet and red cell transfusions for neonates: lifesavers or Trojan horses?

Expert Rev Hematol 2019 10 29;12(10):797-800. Epub 2019 Aug 29.

Department of Pediatrics, Division of neonatology, Leiden University Medical Center , Leiden , the Netherlands.

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http://dx.doi.org/10.1080/17474086.2019.1657824DOI Listing
October 2019

Molecular diagnostic update in hereditary hemolytic anemia and neonatal hyperbilirubinemia.

Int J Lab Hematol 2019 May;41 Suppl 1:95-101

Department of Pathology, University of Utah Health, Salt Lake City, Utah.

Hereditary hemolytic anemia (HHA) is a group of genetically and phenotypically heterogeneous disorders characterized by premature destruction of red blood cells (RBCs) with clinical manifestations ranging from asymptomatic to marked hemolytic anemia. There are three main categories of HHA: (a) RBC membrane defects; (b) hemoglobinopathies/thalassemias; and (c) RBC enzyme deficiencies. Hyperbilirubinemia is a frequent consequence of hemolytic anemia and can lead to bilirubin-associated neurotoxicity in neonates and to jaundice, and formation of gall stones in adults. Hyperbilirubinemia can also be caused by impaired bilirubin conjugation due to polymorphisms and mutations in genes involved in bilirubin metabolism (eg, UGT1A1). Neonates with HHA and co-inherited variants impairing bilirubin conjugation are at increased risk of bilirubin-associated toxicity. Prior to the advent of next-generation sequencing (NGS), molecular diagnosis of these disorders was limited to targeted single gene Sanger sequencing. However, NGS is making its way into the standard diagnostic workup of complex and multigene disorders like HHA. This review will focus on the molecular updates of HHA with particular focus on the neonatal and pediatric population.
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http://dx.doi.org/10.1111/ijlh.13014DOI Listing
May 2019

Screening umbilical cord blood for congenital Iron deficiency.

Blood Cells Mol Dis 2019 07 8;77:95-100. Epub 2019 Apr 8.

The Statistical Data Center, Intermountain Healthcare, Salt Lake City, UT, USA.

Objectives: Small for gestational age infants (SGA), infants of diabetic mothers (IDM), and very low birth weight infants (VLBW) are at risk for congenital iron deficiency. We evaluated the iron status of SGA, IDM, and VLBW neonates at birth and sought mechanistic explanations in those with iron deficiency.

Methods: This was a prospective study. If congenital iron deficiency was present, maternal iron studies were obtained. When neonates were two weeks old, their iron status was reevaluated.

Results: Sixteen of 180 neonates screened were iron deficient at birth. The Body Mass Index of the 16 mothers was high. These mothers often had mild iron deficiency and measurable hepcidin levels. Two weeks after birth, neonates had improved iron measurements.

Conclusions: Among SGA, IDM, and VLBW neonates, maternal obesity is a risk factor for congenital iron deficiency. We speculate that elevated hepcidin levels in obese pregnant women impede iron absorption and interfere with transplacental iron transfer.
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http://dx.doi.org/10.1016/j.bcmd.2019.04.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6573027PMC
July 2019

Ferritin in serum and urine: A pilot study.

Blood Cells Mol Dis 2019 05 8;76:59-62. Epub 2019 Feb 8.

Division of Hematology/Oncology, University of Utah, Salt Lake City, UT, USA.

Serum ferritin reflects total body iron stores, thus a low serum ferritin is used as a parameter of iron deficiency. In healthy adults in Japan, urine ferritin levels were about 5% of serum ferritin levels, with a correlation coefficient of 0.79. It is not known whether a low urine ferritin could serve as a non-invasive screen for iron deficiency. If so, this might be useful for neonates and young children, avoiding phlebotomy to screen for iron deficiency. However, for urinary ferritin screening to be feasible, ferritin must be measurable in the urine and correlate with serum ferritin. Testing should also clarify whether the iron content of ferritin in serum and urine are similar. In this pilot feasibility study we measured ferritin in paired serum and urine samples of healthy adult males, healthy term neonates, growing preterm neonates, and children who had very high serum ferritin levels from liver disorders or iron overload. We detected ferritin in every urine sample, and found a correlation with paired serum ferritin (Spearman correlation coefficient 0.78 of log-transformed values). These findings suggest merit in further studying urinary ferritin in select populations, as a potential non-invasive screen to assess iron stores.
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http://dx.doi.org/10.1016/j.bcmd.2019.02.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420832PMC
May 2019