Clinical Case 4 - Dr. Kerr 1

Clinical Case Presentation# 4

Clinical Case Presentation# 4

Building Blocks of Life Erythroblastosis Fetalis

Professor Ross Kerr

Building Blocks of Life Erythroblastosis Fetalis

Professor Ross Kerr

Clinical Case 4 - Dr. Kerr 2

There will be a test at the end of this presentation!

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13 month old femaleRussian immigrant

• Chief Complaint (CC)Chief Complaint (CC)

• My baby’s teeth are green !

• History of Chief Complaint (HCC)History of Chief Complaint (HCC)

• Noticed color when teeth began coming in. Baby in no apparent distress.

• Social History (SH)Social History (SH) • n/a

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•Nutritional History (NH)Nutritional History (NH)• Baby is taking formula and soft foods (rice, peas, carrots etc)

•Family History (FH)Family History (FH)• Parents and older brother are healthy

•Dental History (DH)Dental History (DH)• Mother rubs teeth daily with a damp cloth. Baby is taking daily fluoride drops.

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• Medical History (MH) Medical History (MH) • Developed hemolytic anemia secondary to blood

group incompatibility with her mother. She received an in utero blood transfusion. At birth she had jaundice and received phototherapy. She did not start feeding for 5 days.

• MedicationsMedications• None

• Review of Systems (RS)Review of Systems (RS)• Within normal limits

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TeethTeeth

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Examination FindingsExamination Findings• Extra-oral exam WNL

• Deciduous central incisors have a light green color. There is 1 mm wide band of hypoplastic enamel in the middle 1/3rd of the upper centrals, and in the incisal 1/3rd of the lower centrals. The color cannot be removed by scraping teeth with a scaler.

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Diagnosis and Risk AssessmentAre any of the conditions in the history connected

to the green teeth ?

Diagnosis and Risk AssessmentAre any of the conditions in the history connected

to the green teeth ?

1. Diet eg ingestion of peas ?2. Hemolytic anemia at birth secondary to

blood group incompatibility ?3. Fluoride treatment ?4. Blood transfusion ?5. Phototherapy ?6. Jaundice ?

1. Diet eg ingestion of peas ?2. Hemolytic anemia at birth secondary to

blood group incompatibility ?3. Fluoride treatment ?4. Blood transfusion ?5. Phototherapy ?6. Jaundice ?

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Differential DiagnosisDifferential DiagnosisDifferential DiagnosisDifferential Diagnosis

• Growth of chromogenic bacteria causing extrinsic staining

• Erythroblastosis fetalis (Hemolytic disease of the new born)

• Biliary atresia• Maternal infection affecting tooth

development.

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• Blood test at birth revealed baby has O+ (Rhesus positive), and mother has O- blood types.

• Unconjugated bilirubin levels at birth were high (12 mg/dL).

DiagnosisDiagnosis

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Erythroblastosis Fetalis Erythroblastosis Fetalis (Hemolytic Disease of the Newborn)(Hemolytic Disease of the Newborn)

Erythroblastosis Fetalis Erythroblastosis Fetalis (Hemolytic Disease of the Newborn)(Hemolytic Disease of the Newborn)

• Antibody-induced hemolytic disease in the newborn.• Caused by blood group incompatibility when fetus inherits red cell

antigenic determinants from the father that are foreign to the mother.

• Important in this respect are the ABO, Rhesus, Kell, Lutheran and Kidd blood group antigens.

• Patients are Rh-positive and Rh-negative according to the presence or the absence of the major D antigen on the surface of their erythrocytes.

• The mother’s antibodies (only IgG antibodies can cross the placent, hence sensitization must take place) attach to the Rh+ erythrocytes and cause hemolysis

• Hemoglobin is broken down to unconjugated bilirubin.  

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Clinical manifestationsClinical manifestations• The anatomic findings in erythroblastosis fetalis vary with the severity of the

hemolytic process.• Infants may be stillborn, die within the first few days, or recover completely. • More severe hemolysis gives rise to jaundice, hepatomegaly, splenomegaly,

and other features associated with hemolytic anemias • In very severe cases, hypoxic injury to the heart and liver may lead to

circulatory and hepatic failure, with resultant generalized edema. This pattern is known as hydrops fetalis.

• There is increased hematopoietic activity leading to circulation of large numbers of immature red cells, including reticulocytes, normoblasts, and erythroblasts (hence the name erythroblastosis fetalis).  

• When hyperbilirubinemia is marked (usually above 20 mg/dl in full-term infants), the CNS system may be damaged (kernicterus) due to uptake of circulating unconjugated bilirubin by the brain.

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Pathogenesis of erythroblastosis fetalisPathogenesis of erythroblastosis fetalis

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• A pre-natal history can help to intercept Rh factor incompatibility between parents.

•Administration of anti-D globulins to the mother during the 3rd trimester can easily prevent the occurrence of Rh erythroblastosis.

•If administration of anti-D globulin is impossible, early recognition of the disorder is imperative e.g., rapidly rising Rh antibody titers in the mother during pregnancy, increasing bilirubin levels in amniotic fluid, or a positive human antiglobulin test (Coombs' test).

• Treatment by exchange transfusion of the infant is an effective form of therapy. Postnatally, phototherapy is helpful because visible light converts bilirubin to readily excreted dipyrroles.

• A pre-natal history can help to intercept Rh factor incompatibility between parents.

•Administration of anti-D globulins to the mother during the 3rd trimester can easily prevent the occurrence of Rh erythroblastosis.

•If administration of anti-D globulin is impossible, early recognition of the disorder is imperative e.g., rapidly rising Rh antibody titers in the mother during pregnancy, increasing bilirubin levels in amniotic fluid, or a positive human antiglobulin test (Coombs' test).

• Treatment by exchange transfusion of the infant is an effective form of therapy. Postnatally, phototherapy is helpful because visible light converts bilirubin to readily excreted dipyrroles.

Treatment & PrognosisTreatment & Prognosis

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Answer the followingAnswer the following

•Incompatibility of which blood groups may lead to erythroblastosis fetalis ?•What causes a change in the teeth in erythroblastosis fetalis ?• How can erythroblastosis fetalis be prevented ?

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Thank YouThank You