Clinical Case Presentation # 4 Building Blocks of Life

1
Clinical Case Presentation 4

• Building Blocks of Life Erythroblastosis Fetalis
• Professor Ross Kerr

2
There will be a test at the end of this
presentation!
3
13 month old femaleRussian immigrant

• Chief Complaint (CC)
• My babys teeth are green !
• History of Chief Complaint (HCC)
• Noticed color when teeth began coming in. Baby in
  no apparent distress.
• Social History (SH)
• n/a

4

• Nutritional History (NH)
• Baby is taking formula and soft foods (rice,
  peas, carrots etc)
• Family History (FH)
• Parents and older brother are healthy
• Dental History (DH)
• Mother rubs teeth daily with a damp cloth.
• Baby is taking daily fluoride drops.

5

• 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.
• Medications
• None
• Review of Systems (RS)
• Within normal limits

6
Teeth
7
Examination 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.

8
Diagnosis and Risk AssessmentAre any of the
conditions in the history connected to the green
teeth ?

• Diet eg ingestion of peas ?
• Hemolytic anemia at birth secondary to blood
  group incompatibility ?
• Fluoride treatment ?
• Blood transfusion ?
• Phototherapy ?
• Jaundice ?

9
Differential Diagnosis

• Growth of chromogenic bacteria causing extrinsic
  staining
• Erythroblastosis fetalis (Hemolytic disease of
  the new born)
• Biliary atresia
• Maternal infection affecting tooth development.

10
Diagnosis

• 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).

11
Erythroblastosis Fetalis (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 mothers 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.  

12
Clinical 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.
  

13
Pathogenesis of erythroblastosis fetalis
14
Treatment Prognosis

• 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.

15
Answer 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 ?

16
Thank You