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Clinical Case 4 - Dr. Kerr1 Clinical Case Presentation # 4 Building Blocks of Life Erythroblastosis Fetalis Professor Ross Kerr Building Blocks of Life.

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Presentation on theme: "Clinical Case 4 - Dr. Kerr1 Clinical Case Presentation # 4 Building Blocks of Life Erythroblastosis Fetalis Professor Ross Kerr Building Blocks of Life."— Presentation transcript:

1 Clinical Case 4 - Dr. Kerr1 Clinical Case Presentation # 4 Building Blocks of Life Erythroblastosis Fetalis Professor Ross Kerr Building Blocks of Life Erythroblastosis Fetalis Professor Ross Kerr

2 Clinical Case 4 - Dr. Kerr2 There will be a test at the end of this presentation!

3 Clinical Case 4 - Dr. Kerr3 13 month old female Russian immigrant Chief Complaint (CC)Chief Complaint (CC) My babys 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

4 Clinical Case 4 - Dr. Kerr4 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.

5 Clinical Case 4 - Dr. Kerr5 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

6 Clinical Case 4 - Dr. Kerr6 Teeth

7 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 1/3 rd of the upper centrals, and in the incisal 1/3 rd of the lower centrals. The color cannot be removed by scraping teeth with a scaler.

8 Clinical Case 4 - Dr. Kerr8 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 ? 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 ?

9 Clinical Case 4 - Dr. Kerr9 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 Clinical Case 4 - Dr. Kerr10 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). Diagnosis

11 Clinical Case 4 - Dr. Kerr11 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 Case 4 - Dr. Kerr12 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 Clinical Case 4 - Dr. Kerr13 Pathogenesis of erythroblastosis fetalis

14 Clinical Case 4 - Dr. Kerr14 A pre-natal history can help to intercept Rh factor incompatibility between parents. Administration of anti-D globulins to the mother during the 3 rd 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 3 rd 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 & Prognosis

15 Clinical Case 4 - Dr. Kerr15 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 Clinical Case 4 - Dr. Kerr16 Thank You


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