Differential diagnosis of neonatal jaundices. Hemolytic disease of newborn.
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Transcript of Differential diagnosis of neonatal jaundices. Hemolytic disease of newborn.
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Differential diagnosis of Differential diagnosis of neonatal jaundices.neonatal jaundices.
Hemolytic disease of newborn.Hemolytic disease of newborn.
Lecturer:
Prof. H.A. Pavlyshyn
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Neonatal jaundice (jaundice of newborns) – appearance of a yellowish coloration of the skin, sclerae and/or mucouses of the infant because of serum bilirubin level increase.
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Classification of jaundices:I. In general jaundice should be distinguished on:
• physiological • pathological. II. According to the time from birth there are: Early jaundice (< 36 hours of age) always pathological usually due to haemolysis, with excessive production of bilirubin babies can be born jaundiced with o very severe haemolysis o hepatitis (unusual) causes of haemolysis (decreasing order of probability) o ABO incompatibility o Rh incompatibility o sepsis
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- Physiological (appears after 36 hours of age, usually on the 3-5 th day, lasts up to 14-th day of life)
Total serum bilirubin concentration doesn’t exceed 205 mkmol/L (12 mg/dL). This type of jaundice can be complicated and uncomplicated, that is why observation and bilirubin level control are very important.
Nota bene – 1 mg/dL of bilirubin = 17,1 mkmol/L of bilirubin
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Prolonged (protracted) jaundice is present after 14 days of life in term newborns and after 21 days of life in premature infant.
• breast milk jaundice (diagnosis of exclusion, cessation of brest feeding not necessary)
• continued poor milk intake • haemolysis • infection (especially pre-natal) • hypothyroidism Late jaundice which appears after 7-th day of life.• It is necessary to perform careful inspection of the newborn to
find the reason of this jaundice.
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Estimation of the risk of severe hyperbilirubinemia development (Bhutani).
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Kramer scale (jaundice appearance stages)
Zone 1 2 3 4 5
TSB
mg/L
58 88 117 146 > 146
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Criteria of the “dangerous” jaundice of newborns (WHO, 2003)
Age of newborn (in
hours)
Localization of jaundice
Conclusion
24 Any “Dangerous” jaundice
24-48 Extremities (zone 4)
> 48 Feet, wrists (zone 5)
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Сomplications HDN
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The reasons of physiological jaundice (transient jaundice) are:
increased production (1 gram of hemoglobin
produces 35 mgr of bilirubin when hemolysed) decreased uptake and binding by liver cells decreased conjugation ( low activity of glucuronil
transferase) decreased excretion increased enterohepatic
circulation of bilirubin
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Principles of the management of patient
with physiological jaundice Clinical features
• Appears not earlier than end of the second day of life, is present in the 1-2 zones only
• Active baby• Liver and spleen not
enlarged• Light-yellow uria,
normal urination, coloured stool
Examination and
treatment• Transcutaneous bilirubinometry
(level of skin bilirubin
• Adequate brest feeding
• Further observation for the child
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Principles of the management of patient with complicated physiological jaundice
Clinical features• Appears not earlier than
end of the second day of life, is present in the 3-4 zones
• May be worsening of newborn’s state
• Liver and spleen may be enlarged
• Light-yellow urine, normal urination, coloured stool
Examination and treatmentIn normal newborn’s state
• Estimate TSB level• Decide fototherapy
necessitivity• Adequate brest feeding• Further observation for the
childIn worsening of newborn’s
state• Immediate phototherapy
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Principles of the management of patient with early or “dangerous” jaundice
• To start phototherapy immediately• To estimate total and conjugated serum bilirubin
concentration • Baby's blood group, direct antiglobulin
(Coombs') test (detects antibodies on the baby's red cells), and elution test to detect anti-A or anti-B antibodies on baby's red cells (more sensitive than the direct Coomb's test)
• Full blood examination, looking for evidence of haemolysis, reticulocytes level, unusually-shaped red cells, or evidence of infection
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Principles of the management of patient with prolonged (protracted) and late
jaundicesExamination and
treatment• To estimate total and
conjugated serum bilirubin concentration (TSB and CSB)
• In hepatomegaly to estimate AlT, AsT
• Adequate brest feeding• Further observation for
the child
Immediate hospitalization in the case of:
• Worsening of newborn’s state
• TSB > 11,7 mg/dL• CSB > 1,9 mg/dL (> 20 %
of TSB)• Liver or spleen
enlargement• Dark urine and/or acholic
stool
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Toxic action of unconjugated bilirubin in full-term newborns appears in 18-20 mg/dL(in premature newborns – in 12-14 mg/dL), it can lead to the bilirubin encephalopathy and kernicterus.
Kernicterus is a preventable neurologic disorder caused by newborn jaundice that can result in cerebral palsy, mental development retardation, auditory processing problems (AN), gaze and vision abnormalities, and dental enamel
hypoplasia.
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Bilirubin staining of brain tissue
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Risk Factors for High Bilirubin Levels:• Blood group incompatibility
• Gestational age less than 37 weeks • Previous sibling received phototherapy/family history of jaundice • East Asian ethnicity • Presence of bruising or cephalohematoma • Exclusive breastfeeding, particularly if nursing is not going well and weight loss is excessive (> 10% of birth weight)
Risk factors for kernicterus appearance:• Asphyxia• Acidosis• Prematurity• Acute hemolysis• Not effective therapy of jaundice• Hypoalbuminemia.
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Preterm baby
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Baby with asphyxia
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There are several types of phototherapy:
- fiber-optical (using of special matress or diaper), - classic (ultra-violet lamps), - spotted (local) - intensive. Intensive phototherapy suggests at
least two sources of light: photomattress and lamp. Intensive phototherapy should produce a decline of TSB of 1-2 mg/dl within 4-6 hours, and the TSB level should continue to fall. If this doesn’t occur, it’s considered a failure of phototherapy.
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Hemolytic disease of the newborn (HDN, erythroblastosis fetalis)
Common causes for HDN
- Rh blood group incompatibility
- ABO blood group incompatibility Uncommon causes - Kell system antibodies presence
Rare causes- Duffy system antibodies
presence
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Clinical types of HDN:Icteric type is the most frequent type of jaundice.
Clinical feature is jaundice of skin and mucoses.
Anemic type is present in 10-20 % of newborns. Diagnostic criteria are paleness, HB level <120 g/L, haematocrit < 40% in birth.
Hydropic type (hydrops foetalis) is the most severe type, approximately always is connected with Rh blood group incompatibilitiy. Clinical features are generalized edemas and anemia in birth.
Mixed type.
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HDN diagnosis criteria:
1. Family history of hemolitic disease.
2. Generalized edemas, HB level <120 g/L, haematocrit < 40% in birth, reticulocytosis
3. Onset of jaundice before 24 hours, positive direct antiglobulin (Coombs') test.
4. Level of unconjugated bilirubin in umbilical blood > 2,9 (50 mkmol/L) mg/dL, bilirubin rise in serum > 0.5 mg/dL/hour (> 8,55 mkmol/L).
5. Changes in peripheral smear (microspherocyrosis, anisocytosis, terget cells).
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This photograph shows normal RBCs, damaged RBCs, and immature RBCs that still contain nuclei.
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Principles of the management of the newborn with hemolytic disease
• To start phototherapy immediately
• To estimate total and conjugated serum bilirubin concentration (TSB and CSB)
• To decide exchange blood transfusions necessitivity according to special tables
• In the case of intensive phototherapy fails after 4-6 hours to performe exchange blood transfusions (under the control of TSB according to special tables)
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