Transcript Clinical Case Presentation

Clinical Case Presentation
#4
Building Blocks of Life
Erythroblastosis Fetalis
Professor Ross Kerr
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There will be a test at the end of this
presentation!
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13 month old female
Russian immigrant
• Chief Complaint (CC)
• My baby’s 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
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•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.
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• 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
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Teeth
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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.
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Diagnosis and Risk Assessment
Are 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 ?
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Differential 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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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).
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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 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 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 fullterm 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 fetalis
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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.
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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 ?
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Thank You
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