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Neonatal Jaundice and Hematology

Neonatal Jaundice and Hematology. Raegan Wetzel, M.D. Oct 5, 2010. Causes of Anemia. Accelerated Loss Hemorrhage, Twin-twin transfusion, early umbilical cord clamping, fetal-maternal transfusion Accelerated Destruction

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Neonatal Jaundice and Hematology

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  1. Neonatal Jaundice and Hematology Raegan Wetzel, M.D. Oct 5, 2010

  2. Causes of Anemia • Accelerated Loss • Hemorrhage, Twin-twin transfusion, early umbilical cord clamping, fetal-maternal transfusion • Accelerated Destruction • Immune hemolytic anemia, hemoglobinopathies, enzyme defects, membrane defects, mechanical destruction, infection, vit E deficiency • Diminished Production • Anemia of prematurity, Fanconi/Diamond-Blackfan anemia, parvovirus, iron deficiency

  3. Case 1 • You are called to an emergent C/S for twins now with decelerations. You are unable to get much history from L&D except that they are 33 4/7 weeks, twin A with EFW 1700 g and twin B at 1200 g. • At delivery, Twin A is a female who appears LGA with ruddy appearance. Twin B also female appears smaller with pallor and poor perfusion. • What is the diagnosis ?

  4. Twin to Twin Transfusion • Who is at risk and why ? • Monozygotic/monochornic twins • 13-33% of twin pregnancies • Donor • Anemia • Recipient • Hyperbili, Hyperviscosity

  5. Blood Loss • Fetal-placental • Infant position after delivery • Cord abnormalities • Velamentous insertion, short cords, chord rupture with precipitous delivery or entanglement, nuchal/knot • Placental abnormalities • Abruption or previa • Fetal-maternal • What should you order ? • Kleihauer-Betke

  6. Case 2 • Ex 27 week preemie, now 5 weeks old “feeding and growing” is noted to have poor weight gain, increased A/B’s, and new flow murmur. • Lab results reveal: normocytic normochromic anemia (hct 25%) with normal WBC and platelet indices. • What do you think the diagnosis is ?

  7. Anemia of Prematurity • Why does it happen ? • Blood loss • Shortened RBC lifespan • Preterm 40-60 days • Inadequate RBC production • Suboptimal erythropoiesis in response to hypoxia • Switch from hepatic to renal O2 sensor not till term

  8. Do term infants get anemic ? • YES • Physiologic anemia of infancy happens later in term infants. True or False ? • True • Term: • 8-12 weeks, Hemoglobin 9-11 g/dL • Preterm: • 3-6 weeks, Hemoglobin 7-9 g/dL

  9. Transfusion Guidlines

  10. Decreased RBC Production • Nutritional • Iron Deficiency • Bone Marrow Suppression • Rubella • Parvovirus • Aplastic/Hypoplastic Anemia • Diamond Blackfan Anemia • Fanconi Anemia

  11. Case 3 • You are notified by the state lab that a newborn has Hemoglobin Barts. What does this mean ? • Does this relate to Beta or Alpha Thalassemia ?

  12. Alpha Thalassemia • % Hgb Bart’s = number of alpha globin genes that are deleted • Silent Carrier – 1 abnormal gene • Alpha thal trait – 2 abnormal genes • Hb H disease – 3 abnormal genes • Moderate anemia • Thal Major - 4 abnormal genes • Hydrops fetalis • Transfusion dependent

  13. Beta Thalassemia • 2 genes of beta-globin production • Silent Carrier: Normal smear and electrophoresis • B-thal trait: Frequently misdiagnosed as iron deficiency anemia, mild anemia • Thal intermedia: Able to maintain Hgb > 7 without transfusion • Thal major: Require regular transfusions

  14. Case 4 • 3 day old term female presents to your office for first check up. Mom had prenatal care and decided to have the baby at home with a midwife. Uncomplicated pregnancy and delivery. Mom doesn’t believe in immunizations and didn’t want her baby to get any medicines at birth. • Baby has been having some mild bleeding around her gums with feeding and mom noticed a small amount of blood in the diaper this morning.

  15. What is her diagnosis ? • Hemorrhagic Disease of the Newborn • How could this have been avoided ? • 0.5 to 1 mg IM of vitamin K

  16. Hemorrhagic Disease of the Newborn Why are Newborns deficient in Vit K ? • Placental transfer is poor • Breast milk is a poor source • GI tract is sterile at birth • What lab values are associated ? • Platelet Count • Normal • Fibrinogen • Normal • PT • prolonged

  17. 3 Types of HD of N • Early Disease • 1st 24 hours • Maternal use of anticoagulant/anticonvulsant • Severe bleeding/intracranial hemorrhage • Classic Disease • 1-7 days • Cutaneous, GI or circumcision site bleeding • Late-onset • Beyond 1 week • Associated with exclusively breast-fed

  18. Case 5 • Term male infant noted to be bleeding from the umbilical stump in WBN. Physical exam otherwise unremarkable. • Family history of maternal uncle with frequent nose bleeds. • Labs: • Platelet count : 200 • PTT : prolonged • PT : normal • Bleeding time normal • Fibrinogen level : normal • Factor IX and VII : pending

  19. Hemophilia A and B • X linked disorder • Hemophilia A (factor VIII) : 5x more common • Hemophilia B (factor IX) : milder • Bleeding less common in newborn period • Common bleeding sites ? • Circumcision, umbilical bleeding, subdural > IVH

  20. How is this different than Von Willebrand’s disease ? • V W is most common heritable bleeding disorder • V W is autosomal dominant • V W will have prolonged bleeding time • What test should you order for diagnosis ? • von Willebrand factor activity (ristocetin cofactor • von Willebrand factor antigen

  21. Case 6 • Newborn male in WBN noted to be oozing from umbilical stump. Physical exam significant for petechiae. Infant born to a primagravida with no prenatal problems. Maternal CBC is normal. • Laboratory on baby: • Platelet count 20 • Normal PT,PTT • Normal fibrinogen

  22. What is the diagnosis ? • Neonatal Alloimmune Thrombocytopenia • What is the pathophysiology ? • Placental transfer of maternal antibodies against paternally inherited antigens on fetal platelets. • HPA-1a (78%) and HPA-5b alloantigens • Can the disease occur in the first pregnancy ? • Yes • Antigenic determinants expressed on placental endothelium • Extended window of time for sensitization

  23. BlueBerry Muffin Rash Petechiae

  24. Bilirubin Metabolism

  25. Case 7 • 72 hr old infant who was delivered after an uncomplicated pregnancy @ 39 WGA appears clinically jaundiced at discharge. Baby is bottle feeding, no family history of jaundice and MBT O+. You get the following lab values: • Total bilirubin 11mg/dl • Direct fraction 0.8 mg/dl • Infant Hct 52% • IBT O+ • Does this seem physiologic or pathologic ? • Physiologic

  26. Physiologic Elevation is universal and transient Mechanisms: Increased RBC destruction Decreased uptake and conjugation Ineffective excretion Pathologic Jaundice that varies significantly from physiologic expectations in: Time of appearance Duration Pattern of serially determined concentrations Pathologic Vs. PhysiologicWhat’s the difference ?

  27. Pathologic Jaundice • Occurs in the first ____ hrs of life • 24 hrs of life • Rate of bilirubin rise > ____ mg/dl/day • > 5mg/dl/day • Clinical jaundice > ____ days in duration • > 1 week duration • Direct bilirubin > _____ • > 2mg/dl or > 15% of total

  28. Term Infant Peak day 3-5 Peak Level 8-13 mg/dl Decline day 5-10 Preterm Infant Peak day 5-7 Peak level 12-15 mg/dl Decline day 7-15 Natural History

  29. Case 8 • Term AGA male born to a 35 yo G3P3 without any prenatal/neonatal complications, presents for 2 week check. Mom is exclusively breastfeeding without any difficulty and infant is gaining weight. He looks a little jaundiced. • Lab results: • H/H and platelet count WNL • Total Bilirubin 19mg/dl • Direct Bilirubin 0.8 mg/dl

  30. Breast Milk Jaundice • 10-30 % of breast fed infants • After first 5 days • Peaks at 2 weeks • Gradual decline over months • Cause ? • Progesterone Metabolite • Fatty acids

  31. Breastfeeding Failure Jaundice • First few days of life • First time moms • Poor enteral intake → delayed meconium→ increased enterohepatic uptake of bilirubin • Prevention: • Lactation consultant • At least 8-12 feeds/day • Avoid supplements

  32. Why would these neonates be at risk for hyperbili ?

  33. Case 9 • 2 week old infant here for routine check. Uncomplicated delivery and WBN stay. Vit K given at birth, discharged at 72 hrs, breastfeeding well. Feeding every 3 hrs, stooling 6 x/day and voiding 9x/day. Exam significant for clinical jaundice. • What labs would you order ? • What maternal blood type would you be concerned about ?

  34. Labs: • WBC 9 x 10 3/mm3, Hct 26%, Plt 175k/mm3 • Retic 7% • Total Bilirubin 19mg/dl, direct 0.6mg/dl • History from the well baby chart • Maternal Blood Type O+ • Infant Blood Type A + • DAT +

  35. Maternal IgG (via placenta) mediated Maternal Coombs (indirect) + Hemolysis of fetal cells Hyperbilirubinemia, anemia Hydrops Fetalis Rh Alloimmunization Rhesus D most common and most severe Rhogam @ 28 wks and within 72 hrs of delivery Severity increases ABO Incompatibility Mild to severe Anti-A Anti-B Can occur in 1st pregnancy Immune Mediated Hemolytic Disease

  36. Case 10 • 4 day old infant presents to office with jaundice. Term male, uncomplicated delivery to a G1 with blood type B+ Prenatal labs unremarkable. Breast feeding well. Jaundiced yesterday with level of 17, home phototherapy initiated and follow up today with bilirubin of 22. • PE significant for jaundice and palpable spleen.

  37. Labs • Initial Hct 47, now 28 • Reticulocyte count 4% • Infant blood type B+, coombs – • Blood smear What specific test will be positive ? Osmotic Fragility Test

  38. Hereditary Spherocytosis • Autosomal Dominant • Northern European descent • Anemia • Jaundice • Splenomegaly

  39. Other Heritable Hemolytic Diseases • Pyruvate Kinase Deficiency • Inherited as …….. • Auto recessive • G6PD deficiency • Most common in people of what descent ? • Mediterranean, tropical African, and Asian • Inherited as …….. • X linked

  40. Case 11 • 7 day old, full term male comes to office with lethargy. Unremarkable prenatal/nursery course. Exclusively breastfeeding. No stool x 2 days and mom has to wake him up for feeds. • Physical exam: lethargic, jaundiced, large head and widely open fontanelles, low tone

  41. Congenital Hypothyroidism • Incidence 1:4000 • Usually asymptomatic at birth • Conjugating enzyme deficiency lasts weeks to months • L-thyroxine treatment by 2 weeks • Prompt treatment corrects the bilirubin

  42. Case 12 • Ex 30 week EGA preemie, now 3 weeks old. Delivered for maternal pre-e, otherwise unremarkable pregnancy with negative maternal labs. Now on RA but with frequent feeding intolerance and multiple periods of NPO. Currently on 5 q3 enteral feeds and had been on TPN since birth. Total bili today 11 mg/dl with a direct component of 5.6mg/dl • What are some general causes ? • What labs might help narrow your differential ?

  43. Hepatocellular Hepatitis TPN induced Alpha-1 antitrypsin Galacotsemia Cystic Fibrosis Biliary Tree Abnormalities Extra-hepatic BiliaryAtresia Paucity of bile ducts Choledochal cyst Bile Plug Causes of Direct Hyperbilirubinemia

  44. What tests will be helpful ? • Radiology: • Abdominal ultrasound • HIDA scan • Newborn screen: • CF results • GALT enzyme activity • Infant Labs: • Hepatitis panel • ALT,AST, GGT • Urine for reducing substances

  45. ManagementWhen to start Phototherapy

  46. ManagementWhen to consider Exchange

  47. Why do we treat ? • To prevent …… • Kernicterus • Which is deposition of unconjugated bilirubin in the ……….. Basal Ganglia

  48. Phototherapy • Photoisomerizes unconjugated bilirubin into more H2O soluble form • Excreted rapidly by liver and kidney • Does not need glucuronidation

  49. Exchange Transfusion • Double volume exchange • Replaces 85% of circulating RBC • Two neonatal blood volumes • 160ml/kg • Aliquots = 10% of total blood volume

  50. THANK YOU

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