Identification of neonatal haemolysis: an approach to predischarge management of neonatal hyperbilirubinemia

Bhutani, Vinod K.; Srinivas, Shanmukha; Cuadrado, Martin E. Castillo; Aby, Janelle; Wong, Ronald J.; Stevenson, David K.

doi:10.1111/apa.13341

Cited by 31 publications

(24 citation statements)

References 24 publications

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“…Ursodeoxycholic acid produced no decrease in the direct bilirubin level and attempts to decrease the intensity of the phototherapy consistently resulted in an increase in the total and direct bilirubin levels. Nucleated red blood cell counts remained elevated throughout the first month and, on hospital day 7, his end-tidal carbon monoxide level, corrected for ambient carbon monoxide, was significantly elevated at 4.6 ppm, confirming the presence of ongoing significant hemolysis [6]. Breast milk was introduced on day 7 and he was on full feeding from day 9.…”

Section: Case Presentationmentioning

confidence: 94%

A Newborn with a Blueberry Muffin Rash, Hepatosplenomegaly, Thrombocytopenia and Severe Cholestatic Jaundice at Birth

Lopez¹,

Pryce²,

Maisels³

2017

Clin Pediatr OA

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Neonatal cholestasis is characterized by an elevation of conjugated bilirubin. It occurs in approximately 1 in 2500 term infants. Gaucher Disease (GD) is an exceptionally rare cause of neonatal cholestasis. We report a male term newborn with a blueberry muffin rash, hepatomegaly and massive splenomegaly at birth. His total serum bilirubin (TSB)/direct was 13.5/11.9 mg/dL, and platelet count 20,000 bil/L. A leukocyte beta-glucosidase level was 0 and full sequencing of the GBA gene showed that he was homozygous for the pathogenic complex allele p.D409H and a second variant p.H255Q, consistent with the diagnosis of the severe phenotype GD type 2.

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Section: Case Presentationmentioning

confidence: 94%

A Newborn with a Blueberry Muffin Rash, Hepatosplenomegaly, Thrombocytopenia and Severe Cholestatic Jaundice at Birth

Lopez¹,

Pryce²,

Maisels³

2017

Clin Pediatr OA

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“…Parents of all participating neonates signed informed consents. Although some of the Stanford cohort data have been reported previously , here, we included additional infants enroled since that publication. Eligible infants were male and female newborns at gestational ages (GA) ≥36 weeks with birthweights (BW) ≥2000 g or at GA ≥35 weeks with BW ≥ 2500 g and at postnatal ages between 6 and 60 hours of age.…”

Section: Patient and Methodsmentioning

confidence: 99%

“…ETCOc measurements were performed using a CoSense ® CO monitor (Capnia, Inc., Redwood City, CA) as previously described . For each newborn, up to four serial ETCOc measurements were attempted in conjunction with routine TB/TcB levels during birth admission.…”

Section: Patient and Methodsmentioning

confidence: 99%

“…Therefore, any increases in bilirubin production or impairments in bilirubin elimination are associated with higher TB levels, and hence in higher percentiles on the hour‐specific bilirubin nomogram. The measurement of TB alone, however, might not be sufficient for the clinical discrimination of haemolysis in an infant . Even infants with a positive direct Coombs test and isoimmunization might have no evidence of haemolysis.…”

Section: Introductionmentioning

confidence: 99%

“…Thus, the American Academy of Pediatrics (AAP) in their 2004 Practice Guideline recommends that jaundiced infants with haemolysis (or increased bilirubin production) should be managed using lower thresholds for initiating phototherapy. The scientific basis for using ETCOc measurements as a means of identifying infants with increased bilirubin production rates has been described previously . The balance of bilirubin production and its elimination in the postnatal period determines the trajectory or ‘rate of rise’ (ROR) of total bilirubin (TB) levels over time, and thus forms the basis of the hour‐specific bilirubin or Bhutani nomogram .…”

Section: Introductionmentioning

confidence: 99%

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Identification of risk for neonatal haemolysis

et al. 2018

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History and current standard of postnatal management in hemolytic disease of the fetus and newborn

et al. 2022

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Since the discovery of the Rh blood group system in 1940, a greater understanding of hemolytic disease of the fetus and newborn (HDFN) was gained. In the years thereafter, researchers and clinicians came to the current understanding that fetal and neonatal red blood cells (RBC) are hemolyzed by maternal alloantibodies directed against RBC antigens potentially leading to severe disease. Preventative measures, such as Rhesus(D) immunoprophylaxis (RhIG), have greatly decreased the prevalence of Rh(D)-mediated HDFN, although a gap between high-income countries and middle-to low-income countries was created largely due to a lack in availability and high costs of RhIG. Other important developments in the past decades have improved the identification, monitoring, and care of pregnancies, fetuses, and neonates with HDFN. Prenatally, fetal anemia may occur and intrauterine transfusions may be needed. Postnatally, pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for anemia in the late phase of the disease. Through this review, we aim to provide an overview of important historic events and to provide hands-on guidelines for the delivery and postnatal management of neonates with HDFN. Secondarily, we aim to describe recent scientific findings and evidence gaps.Conclusion: Multiple developments have improved the identification, monitoring, and care of pregnancies and neonates with HDFN throughout the centuries. Pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for late anemia in the late phase of the disease. Future studies should be set in an international setting and ultimately aim to eradicate HDFN on a global scale. What is Known:• Developments have led to a greater understanding of the pathophysiology, an improved serological identification and monitoring of at-risk cases and the current pre-and postnatal treatment. What is New:• This review provides the pediatrician with hands-on guidelines for the delivery and postnatal management of neonates with HDFN.• Future studies should be set in an international setting with the ultimate aim of eradicating HDFN.

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Identification of neonatal haemolysis: an approach to predischarge management of neonatal hyperbilirubinemia

Abstract: Near-simultaneous ETCOc and TB measurements in infants with TB >75th percentile accurately identify haemolytic hyperbilirubinemia.

Cited by 31 publications

References 24 publications

A Newborn with a Blueberry Muffin Rash, Hepatosplenomegaly, Thrombocytopenia and Severe Cholestatic Jaundice at Birth

A Newborn with a Blueberry Muffin Rash, Hepatosplenomegaly, Thrombocytopenia and Severe Cholestatic Jaundice at Birth

Identification of risk for neonatal haemolysis

History and current standard of postnatal management in hemolytic disease of the fetus and newborn

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