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Rh blood group incompatibility

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Differential diagnosis of
neonatal jaundices.
Hemolytic disease of newborn.
Lecturer:
Sakharova Inna.Ye., M.D., Ph.D.
Lecture plan:
1. Classification of neonatal jaundices.
2. Evaluation of jaundice severity.
3. Principles of the newborns management of
different types of jaundices.
4. Complications of neonatal jaundices.
5. Treatment of neonatal jaundices.
6. Hemolytic disease of the newborn
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.
Classification of jaundices:
I.
In general jaundice should be
distinguished on:
•
•
physiological
pathological.
II. According to the time from birth there are:
пЂ­ Early jaundice (< 36 hours of age)
п‚· always pathological
п‚· usually due to haemolysis, with excessive
production of bilirubin
п‚· babies can be born jaundiced with
o very severe haemolysis
o hepatitis (unusual)
п‚· causes of haemolysis (decreasing order of
probability)
o ABO incompatibility
o Rh incompatibility
o sepsis
п‚· Rare causes
пЃЇ red cell enzyme defects e.g. G6PD deficiency
пЃЇ red cell membrane defects, e.g., hereditary
spherocytosis
- 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
пЂ­ 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
пЂ­ 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.
Differential diagnostic of jaundices
Criterion
Type of jaundice
Conjugated
Appearance
Hepatosplenomegaly
Stool
Hemolytic
2-3-rd day 1-st day
—
Mecha Parenchynical
matous
1-2-nd
week
End of the
1-st week
Urea
Gradu+
ally
Yellow
Coloured Acholic Coloured or
light
Light yell. Coloured Dark
Dark
п‚­ Bilirubin
Indirect
Anemia,reticulocytosis
+ —
+
Indirect
+ +
Direct
— —
Both;direct
— —
Estimation of the risk of severe
hyperbilirubinemia development (Bhutani).
Kramer scale (jaundice appearance stages)
Zone
1
TSB 58
mg/L
2
3
4
88 117 146
5
>
146
Criteria of the “dangerous” jaundice of
newborns (WHO, 2003)
Age of
newborn (in
hours)
24
24-48
> 48
Localization
of jaundice
Any
Extremities
(zone 4)
Feet, wrists
(zone 5)
Conclusion
“Dangerous”
jaundice
The reasons of physiological jaundice
(transient jaundice) are:
пѓј increased production (1 gram of hemoglobin
produces 35 mgr of bilirubin when hemolysed)
пѓј decreased uptake and binding by liver cells
пѓј decreased conjugation ( low activity of glucuronil
transferase)
пѓј decreased excretion
пѓј increased enterohepatic circulation of bilirubin
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
Principles of the management of patient
with complicated physiological jaundice
Examination and treatment
In normal newborn’s state
Appears not earlier
than end of the second • Estimate TSB level
day of life, is present in • Decide fototherapy
the 3-4 zones
necessitivity
• Adequate brest feeding
May be worsening of
newborn’s state
• Further observation for the
child
Liver and spleen may
be enlarged
In worsening of newborn’s
state
Light-yellow urine,
normal urination,
• Immediate phototherapy
coloured stool
Clinical features
•
•
•
•
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
Principles of the management of patient
with prolonged (protracted) and late
jaundices
•
•
•
•
Examination 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
Toxic action of unconjugated bilirubin in fullterm 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.
Bilirubin staining of brain tissue
In newborns with jaundice there are specific
clinical signs, which can appear in bilirubin
encephalopathy. The early symptoms of brain
injury are poor feeding, decreased alertness,
alteration of muscle tone, and a high-pitched
cry. Later symptoms of bilirubin toxicity
include shrill cry, inability to feed, mild or deep
stupor, abnormal or uncoordinated movements,
and seizures.
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.
American Academy of Pediatrics
recommendations for healthy term
newborns (TSB, mg/dL)
Age,
hours
Consider Photo- Exchange
photo- therapy transfusion
if intensive
therapy
phototherapy fails
Exchange
transfusion and
intensive
photother
apy
25-48
п‚і 12
п‚і 15
п‚і 20
п‚і 25
49-72
п‚і 15
п‚і 18
п‚і 25
п‚і 30
> 72
п‚і 17
п‚і 20
п‚і 25
п‚і 30
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.
Hemolytic disease of the newborn (HDN,
erythroblastosis fetalis)
п‚· Common causes for HDN
- Rh blood group incompatibility
- ABO blood group incompatibility
п‚· Uncommon causes
- Kell system antibodies presence
п‚· Rare causes
- Duffy system antibodies presence
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.
HDN diagnosis criteria:
1.
2.
Family history of hemolitic disease.
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).
This photograph shows normal RBCs, damaged RBCs, and
immature RBCs that still contain nuclei.
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)
Indications for exchange blood transfusions
in term babies with HDN
Factors
Level of total bilirubin level in
umbilical blood
Bilirubin rise in serum (during
phototherapy)
- Rh incompatibility
- ABO incompatibility
Anemia in the first day of life
Indexes
> 80 mkmol/L
≥ 7 mkmol/L
≥ 10 mkmol/L
РќbпЂј 100 g/Р», Ht
<35%
Indications for exchange blood transfusions
in term babies with HDN
continuation
Factors
Ratio of TSB (mkmol/L)
and albumin (g/L)
depending on the weight
of baby
< 1250 g
1250-1499 g
1500-1999 g
2000-2500 g
> 2500 g
Indexes
Bilirubin mkmol/L
Albumin g/L
6,8
8,8
10,2
11,6
12,2
In the case of Rh blood group incompatibility
can be used Rh negative blood of the same group
(with baby) or Rh negative packed red cells Рћ (I)
in the plasma of AB (IV).
In the case of ABO blood group incompatibility
can be used the Rh same (with baby) packed red
cells Рћ (I) in the plasma of AB (IV).
In the case of both of Rh blood group
incompatibility
and
ABO
blood
group
incompatibility can be used Rh negative packed
red cells Рћ (I) in the plasma of AB (IV).
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