Patient Presentation
A 28-day-old female came to clinic for her 1-month health maintenance examination. She and her family were doing well with good breastfeeding every 2.5 – 3 hours, and good urine and stooling output. She was regarding her parents’ faces, cooing, following lights and responding to sounds.
The past medical history showed a 39 4/7 week infant born to a G2P2 A+ Caucasian mother with a normal pregnancy and delivery. Her newborn screening test was also normal. She had gone home on time and at 10 days of life had regained her birth weight. At that time she was still somewhat jaundiced but her total transdermal bilirubin was decreasing and was 11.1 mg/dL. The family history was significant for Parkinson’s disease in a great grandparent, and skin cancer in the paternal grandmother.
The pertinent physical exam revealed her to be gaining weight at 29 grams/day, and all of her growth parameters were at the 75%. Her examination was normal including no hepatosplenomegaly, except for continued jaundice that was noticed over her lower abdomen. The diagnosis of a healthy breastfeeding infant with prolonged jaundice was made.
The laboratory evaluation showed her transdermal bilirubin to be 11.5 mg/dL. Because of the continued hyperbilirubinemia, the pediatrician reviewed her chart confirming her previous history, noting that she had already had one direct bilirubin of 0.6 mg/dL but the patient did not have a previous Coombs test. The father had blood type A+. The pediatrician did an additional work-up of a complete blood count with smear and reticulocytes, and another direct bilirubin. All of these were normal for age and did not reveal evidence of hemolysis. The diagnosis of breast milk jaundice was made and the infant was discharged. The patient’s clinical course at her 2-month check up showed much less jaundice but her transdermal level was still 9.8 mg/dL. At a 3-month appointment, her jaundice had resolved and her transdermal level was normal.
Discussion
Almost all infants in the first few days of life have some elevation of their bilirubin because of the various physiologic changes they undergo in the transition to extra-uterine life. This is a common problem managed by many different inpatient and outpatient healthcare providers. This hyperbilirubinemia is usually unconjugated and resolves in the first 1-2 weeks of life. However, when it does not resolve, health care providers have 3 general paths to follow when considering a cause: inadequate breast milk intake, an underlying organic cause or breast milk jaundice.
Inadequate breastfeeding is not uncommon and can be caused by many problems such as inadequate maternal education/experience or support, difficulties with latching or sucking, inadequate number of breastfeeding attempts or duration, or maternal fatigue or health issues. It is uncommon that inadequate milk supply is the cause but it does occur for some women. Often after the first 1-2 weeks, once breastfeeding appears to be established, the mother may increase the breastfeeding intervals slightly which decreases the number of attempts/day. For most healthy infants this is not a problem. For other infants especially younger ones, decreasing the number of feeds by 1-2/day may cause inadequate breastfeeding, slower weight gain and possibly prolonged hyperbilirubinemia. Some people term prolonged hyperbilirubinemia due to inadequate breastfeeding as breastfeeding jaundice. Some authors note “…this term is misleading and should be avoided. We refer to this clinical entity as ‘not enough breastfeeding jaundice,’… because the underlying aetiology is not breast feeding itself but its converse, lack of adequate breast feeding.”
Breast milk jaundice is a benign condition of prolonged unconjugated hyperbilirubinemia (usually considered less than 12 mg/dL total bilirubin) in a healthy breastfeeding infant. Infants have normal weight gain, normal urine and stooling patterns and normal physical examination. It can persist for up to 12 weeks before resolution and usually does not need treatment. In the past, breastfeeding cessation and feeding with formula was used by some as a confirmatory test, but it is not necessary nor recommended for infants suspected of having breast milk jaundice. Breast milk jaundice is considered a diagnosis of exclusion after reasonable clinical and laboratory evaluations are made. The exact cause of breast milk jaundice is not known but most likely one or more of factors within the breast milk itself and genetic variations in the newborn.
Causes of prolonged unconjugated hyperbilirubinemia include:
- Not enough breastfeeding
- Breast milk jaundice
- Hemolysis
- ABO or Rh incompatibility
- Bruising
- Cephalohematoma
- Polycythemia
- Red blood cell membrane defects – G6PD deficiency, pyruvate kinase deficiency, etc.
- Red blood cell enzyme defects – hereditary spherocytosis, etc.
- Metabolic
- G6PD deficiency
- Galactosemia
- Hypothyroidism
- Bilirubin conjugation
- Crigler Najjar syndrome
- Gilbert syndrome
- Other
- Undiagnosed conjugated hyperbilirubinemia – biliary atresia, hepatitis
- Intestinal obstruction
- Pyloric stenosis
- Medications
A review of the pathophysiology of hyperbilirubinemia and differential diagnosis of neonatal unconjungated hyperbilirubinemia can be found here
The differential diagnosis of conjugated hyperbilirubinemia can be found here
A brief review of Gilbert’s disease can be found here.
Learning Point
Additional evaluation for breast milk jaundice should be done before this diagnosis is made. A review of the medical records may provide a great deal of information before additional laboratory testing is conducted. Mother’s and infant’s blood types may be known and a Coomb’s test may already have been recorded. A cephalohematoma or bruising may have been noted in the delivery record along with potential risk factors for polycythemia (e.g. twin-twin transfusion, infant of a diabetic mother, etc.). The neonatal screening test covers many metabolic problems but hypothyroidism may need to be tested for again. A direct bilirubin test may have already been recorded too, but some providers will do a second testing out of the early neonatal period to confirm this finding. Most infants are not taking any medications other than Vitamin D but this should be reviewed with the family. If a medication is being taken then determining if there is a risk of hyperbilirubinemia should be done.
Additional testing to determine if hemolysis is occurring should be considered with a complete blood count, reticulocyte count and review of the peripheral blood smear. If these indicate potential hemolysis, appropriate consultation with a hematologist can be considered for advice about potential additional evaluation and/or treatment. Without evidence of hemolysis, most infants can be followed without additional intervention until around 12 weeks of age when breast milk jaundice should resolve. If the hyperbilirubinemia does not resolve then additional consultation with a gastroenterologist or hematologist should be considered. Evaluation for Criglar-Najjar and/or Gilbert Syndrome is often investigated at this time point.
Questions for Further Discussion
1. What are the functions of the liver? A review can be found here
2. What causes hemolytic anemia? A review can be found here
Related Cases
- Disease: Breast Milk Jaundice | Jaundice
- Symptom/Presentation: Jaundice
- Specialty: General Pediatrics | Gastroenterology| Hematology | Neonatology
- Age: Newborn
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for this topic: Jaundice
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Bratton S, Stern M. Breast Milk Jaundice. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2019. http://www.ncbi.nlm.nih.gov/books/NBK537334/. Accessed October 21, 2019.
Poddighe D, Castelli L, Marseglia GL, Bruni P. Prolonged, but transient, elevation of liver and biliary function tests in a healthy infant affected with breast milk jaundice. BMJ Case Rep. 2014;2014. doi:10.1136/bcr-2014-204124
Preer GL, Philipp BL. Understanding and managing breast milk jaundice. Arch Dis Child Fetal Neonatal Ed. 2011;96(6):F461-466. doi:10.1136/adc.2010.184416
Tan H-S, Balasubramaniam I-S, HSS A-S, et al. Impact of a standardized protocol for the Management of Prolonged Neonatal Jaundice in a regional setting: an interventional quasi-experimental study. BMC Pediatr. 2019;19. doi:10.1186/s12887-019-1550-3
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa
Pediatric Education 