BILI BOY

Kody Crowell MD PGY2

Setting: 4 day old male who presents for a newborn visit in clinic.
HISTORY: Born to a 29 year old G1 NSVD at 37 4/7 weeks. Uncomplicated delivery and 2 day hospital stay. Maternal labs were normal including GBS and Blood O+/-. Discharged at 93 % birth wt. Bili done prior to discharge was 12.8 with LL of 15.3. Fam Hx: No congenital diseases. Maternal uncle with jaundice as an infant. Social Hx: Lives with both parents who are from South Korea and PhD students at the University.

PHYSICAL EXAM
PHYSICAL EXAM: Wt: 90% Birth wt. GEN: Fussy but consolable. Not ill appearing. HEENT: Sclera icteric. CV: Clear S1 and S2 with no murmurs rubs or gallops. PULM: Moving air well with clear breath sounds. No retractions , wheezes or nasal flaring. ABD: Soft, nontender, nondistended. No palpable masses. SKIN: Jaundice to thigh. No rashes. NEURO: normal infant reflexes, facial symmetry.

INTERVAL HX: DOL 4

Breast Feeding Mom feels her breast milk had just come in. Feeding 15 minutes every 2-3 hours. Stooling 2-3 times a day of dark green stool. Bili 21.8 with LL 20. Started on phototherapy. Parents do not wish to supplement with formula as mothers breast milk is now in. Bili next day is 19.8

NEXT CLINIC VISIT: DOL 7

Gained 15g. Now at 91% of birth wt. Stool x8/day and transitioning Mom does not like leaving under bili lights because he gets fussy. PHYSICAL EXAM: facial Jaundice Bili 21.6. Sent to RTU

RTU
Started on phototherapy. Temp of 38.5 and full ROS done. Transferred to inpatient service.

DIFFERENTIAL DIAGNOSIS?

DIFFERENTIAL DIAGNOSIS
GI: Breast feeding jaundice, Breast milk jaundice, CriglerNajjar syndrome type I and II, Gilberts. Conjugated bili: Cholestasis (Biliary atresia), Dubin Johnson ID: Sepsis (viral and bacterial), Viral infection (CMV, HSV, Hepatitis, etc),parasitic infection (ascaris, lumbricoides, liver flukes) HEME: Pyruvate kinase deficiency, G6PD, Spherocytosis, ABO incompatibility. (Too early for thalassemia or sickle cell) ENDO: Hyperthyroidism CV: Heart failure TOX: Drug induced (not applicable here, but rather in older kids). Rifampin, probenecid, some herbal medicines.

LABS
Blood Cx, Urine Cx, CSF Cx: NGTD Enterovirus & HSV PCR Serum/CSF: Negative CBC: Normal CSF: Gram Stain negative, RBC 1, WBC 1 U/A: Normal BMP: Normal Hepatic function panel: Normal Reticulocytes: 1 (Normal) Bilirubin: Sun. (05:37) Neonatal = 12.2, Unconjugated = 12.2, Conjugated = 0. Sat. (06:15) Neonatal = 13.7, Unconjugated = 13.7, Conjugated = 0 Friday (16:20) Neonatal = 15.5, Unconjugated = 15.2, Conjugated = 0.3 Friday (06:25) Neonatal = 17.2, Unconjugated = 16.8, Conjugated = 0.4 Thurs. (20:05) Neonatal = 19.6, Unconjugated = 19.1, Conjugated = 0.4

DOL 10: Clinic Visit for Hospitalization follow-up.

Off Phototherapy x1 day. 96% Birth Wt. Parent s supplementing with formula 2 oz x5/day in addition to Q2-3H breast feeding. Jaundice to chest. Bili 19.8!!!

 CBC normal. Retics normal. Periphera l Smear: Target cells, Tear drop cells, reticulocytes, spherocytes, schistocytes. (normal for newborn) G6PD assay normal NMS has returned normal Sent home with phototherapy.

DOL 13
Above birth wt. Bili 18.4 Sent home with no lights and daily bili.

DOL 16
Bili 19 Pump and Dump x 2days.

DOL 18

BILI

12!!

Hyperbilirubinemia
35 weeks gestation defined as a Total Bili >95th percentile for hours of age. Bilirubin production is 2-3 times higher in infants than adults. UGT activity in term infants is 1% of the adult at 7 days and does not reach adult levels until 14 weeks of age. Bilirubin-induced Neurologic Dysfunction (BIND): Increased risk with a Total Bili >25 - 30 mg/dL.

Gilberts/Crigler-Najjar (I/II)
Gilberts:
-Most common inherited disorder of bilirubin glucoronidation. -Mutation in the promotor region of UGT 1A1 gene resulting in a reduced production of UGT. -Breast milk jaunidice during second week of life may be a result of a concurrent manifestation of Gilberts.

CN-I:
-More sever than CN-II. -UGT is present, but there is little to no activity. -Severe hyperbilirubinemia during the first 2-3 days. -Lifelong phototherapy or Liver transplant is needed for treatment.

CN-II:
-UGT is present and has low activity.

Breast Feeding Jaundice

Due to Inadequate oral intake by the infant leading to hypovolemia.

Breast Milk Jaundice

Persistence of physiologic jaundice beyond the first week of age. Usually begins after first 3-5 days of life. Peaks within 1-2 weeks after birth and may peak again at 3 weeks of life. May take up to 3 months to completely resolve.

Breast Milk Jaundice continued

Likely caused by deconjugation of bilirubin by Beta-Glucuronidase in breast milk. Unconjucated bili is then absorbed by the intestine causing an increase in enterohepatic circulation. Benign, but bili should be monitored regularly until normalizing.