Download lecture: " Neonatal jaundice "

LECTURE:
" Neonatal jaundice "
 Icteric
staining of the skin
occurs in term infants with
bilirubin levels of 80-90
mmol \l, premature - more
than 120 mmol \l , in adults
35-40 mmol \l
Classification
causes of neonatal hyperbilirubinemia
1. Konyugatsionnye jaundice:
 -physiological jaundice
 -a prolonged jaundice in premature
 -family nonhemolytic neonatal hyperbilirubinemia
ARIES-Lyutseya
2. Hemolytic jaundice:
 -hemolytic disease of the newborn
 -microspherocytic congenital hemolytic jaundice
Minkowski Chauffard
3. Mechanical jaundice:
 -biliary atresia
 -syndrome accumulation of bile
Classification
causes of neonatal hyperbilirubinemia
4. Parenchymal jaundice:
-congenital hepatitis
-toxoplasmosis
-sepsis
5. Enzymopathies:
-family Congenital nonhemolytic jaundice
Crigler-Najjar
- Galactosemia (congenital deficiency of the
enzyme-fosfogalaktozo uridemtransferazy FGVT)
- Jaundice due to congenital deficiency of
glucose 6-phosphate dehydrogenase (G-6-PD)
Physiological jaundice - transistor observed
in 86.5% of full-term newborns. Transient
hyperbilirubinemia caused by rapid hemolysis
newborn temporary immaturity
glyukouroniltransferazy enzyme.
 The general condition of the child is not
broken.Yellowness of the skin of the child
appears at 2-3 days of age and disappears at
10-14 day. Red blood cells, the size of the
liver and spleen were normal.
 Treatment: 5% glucose or 0.9% sodium
chloride, phototherapy.
 Weather favorable.
Konyugatsionnaya jaundice in
premature infants is a prolonged duration
(4-6 months), and delivers more picture.
Clinical jaundice increases slowly, with
hepatomegaly and splenomegaly are
absent, feces and urine unpainted,
anemizatsii not.
At the heart of jaundice is temporary
immaturity enzyme factors liver and
excreted bilirubin konyugiruyuschih
Neonatal hyperbilirubinemia - a disease
based on the hemolysis of the fetus and
newborn due to incompatibility of maternal
and fetal blood by erythrocyte antigens.
 Etiology: HDN razvivaetsya the result of
incompatible maternal and fetal blood Rh
factor or group antigens.
 Rhesus-conflict occurs if the Rh-negative
fetus has Rh-positive blood.
 ABO conflict develops in the presence of 0
(I) g blood of women and the fetus A (II) or
B (III) g blood.
Hemolysis of fetal and neonatal
hyperbilirubinemia and lead to anemia.
Indirect bilirubin neurotoxicity. The main
source of bilirubin in the body is the
hemoglobin.
 When a certain concentration (over 340
umol \ l) in term and (more than 200
mmol \ l) in premature infants, it can cross
the blood-brain barrier and damage the
structure of the brain, which leads to the
development of bilirubin encephalopathy
(kernicterus).
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Hemoglobin in the cells of the
reticuloendothelial system is split into
iron, bilirubin and globin. Allocated
indirect bilirubin binding to albumin and
globulins of blood to the liver, where
konyugiruet with glucuronic acid. In the
liver cells to indirect bilirubin join two
molecules of glucuronic acid. In plasma
indirect bilirubin albumin complex forms
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Indirect bilirubin - tissue poison, inhibiting
oxidative processes and in various organs
causing degenerative changes in the cells up
to necrosis. Direct bilirubin is excreted from
pechechnochnyh cells in bile capillaries. The
final products of direct bilirubin is sterkobilina
and urobilinogen.
Infants lack putrefaction in the intestines
sterkobilina not formed and released into the
bilirubin neizmennenom form. Its ability to
eliminate reduced because of the immaturity
of the excretory system and features of the
bile capillaries (narrow, a small amount). The
result appears in the bile duct cholestasis,
hepatitis, the formation of reactive
GBN Clinic
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Distinguish edema (2%), icteric (88%), anemic (10%)
forms.
Edematous form - the heaviest, develops in utero.
Massive fetal hemolysis leads to severe anemia, hypoxia,
metabolic disorders, gipoprotenemii and tissue edema.
Newborns are sluggish, sharply reduced muscle tone,
reflexes are depressed, a cardio-pulmonary insufficiency,
severe hepatosplenomegaly, belly big, barrel-shaped, Hb
concentration is less than 100 g \ l. Fetus dies before
birth or born in critical condition with massive edema.
GBN Clinic
Icteric-most common. Its main symptoms are jaundice,
anemia, hepatosplenomegaly. Jaundice with orange tint
appears at birth or for 1-2 hours.Than before jaundice
appears, the more severe the disease.
 Children lethargy, drowsiness, hyporeflexia, hypotension,
symptoms of kernicterus, neck stiffness, tonic
convulsions, "brain scream," symptom "setting sun", big
bulging fontanelle. Icteric form of HDN is accompanied
by anemia.
 Kernicterus develops in 10% of full-term at a
concentration of indirect bilirubin levels above 340
mmol \ l, 30% higher than 430 mmol \ l, 70% above
520 mmol \ l

GBN Clinic
anemic form adrift most benign.
 clinical symptoms: pale skin, anemia, enlarged
liver and spleen. Indirect bilirubin
concentration may be slightly raised.
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Antenatal
diagnosis of GBN
maternal history (previous abortions,
stillbirths, miscarriages,
 birth of sick children
 transfusion without the Rh factor)
 protivorezusnyh titration of antibodies in
the blood of women rezusotritsatelnyh
 The study of amniotic fluid obtained by
amniocentesis
 ultrasound study

Postnatal
diagnosis of GBN
The clinical symptoms (jaundice , anemia ,
hepatosplenomegaly )
 Increase in blood indirect bilirubin
fraction erythroblastosis , reticulocytosis ,
positive Coombs in rhesus conflict.
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Conservative treatment
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Phototherapy - Phototherapy for photo-oxidation
of indirect bilirubin is found in the skin to form a
water-soluble isomers that are excreted in urine
and feces
inducers of microsomal liver enzymes,
phenobarbital
Vitamin E, B1, B2, B6, C and cocarboxylase
improves liver metabolism and stabilizes
in order to inhibition of absorption of bilirubin in
the gut cleansing enemas administered activated
charcoal
accumulation of bile syndrome cropped
appointment cholagogue (magnesium sulfate,
drotaverine, noshpy)
Surgeries : replace blood transfusions
hemosorbtion , plasmapheresis
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Absolute indications for exchange transfusion of
blood:
-indirect bilirubin concentration in the umbilical
cord blood of more than 60 mmol\ l
-hour increase in indirect bilirubin
concentrations over 6.10 mmol\ l
-indirect bilirubin concentration in the
peripheral blood of more than 340 mmol\ l
-severe anemia (hemoglobin less than 100g\ l)
For use exchange transfusion of blood
Rh (-) of the same blood group as the
baby's blood
 Currently not whole blood transfusions
and Rh (-) red cell mass, mixed with fresh
frozen plasma.
 If HDN due to incompatibility group, then
use the red cell mass 0 (I) group, plasma
or AB (IV) group.
 The volume of transfused blood is from
170 to 180 ml \ kg body weight of the
child.
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Prophylaxis
Nonspecific:
 -carrying blood transfusions only given Rh-factor
 -prevention of abortion
 -range of social protection measures for women's health
Specific:
 -introduction of Rh (antidepressant) Lg in the first 24 hours
after the birth of a healthy Rh (+) child, and abortion, which
facilitates rapid elimination of red blood cells from the blood
of mother of the child, preventing the synthesis of Rh
antibodies mother
 -high titer of Rh antibodies in the blood during pregnancy
make the replanting of the skin on her husband 16-32 weeks
pregnant 2-3 times at intervals of 4-6-8 weeks
 -delivery to spend 37-39 weeks of gestation by cesarean
section
Congenital hemolytic jaundice
Minkowski Chauffard newborns
The disease runs hard with severe jaundice.
Reinforced hemolysis of red blood cells due to a
defect in the maturation of the violation in these
carbohydrate metabolism, lack of ATP in the stroma
of erythrocytes.
 Clinic: jaundice, hyperbilirubinemia, anemia,
enlargement of the liver, especially the spleen,
decreasing the osmotic resistance of red blood
cells, red blood cells microspherocytic grit,
reticulocytosis and normablastozom
 Treatment: The treatment of the same, as in the
HDN
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Biliary atresia - a birth defect
Clinic: jaundice appearing in the first days of
life and gradually increases to 1-1.5 months,
discolored stools, bloating, enlarged liver
and spleen, dark urine, (+) response to bile
pigments (-) reaction to urobilinogen, the
level of direct fraction increases, signs of
intoxication increases, signs of portal
hypertension, blood clotting
 Treatment: Surgery
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Accumulation of bile syndrome , due
to total or partial blockage of the bile
duct bile plugs
Clinic: jaundice, discolored stools, dark
urine, (+) response to bile pigments,
enlarged liver, blood appears in indirect
bilirubin fraction
 Treatment: 25% magnesium sulfate solution
inside of 5ml 3 times, a heating pad or
diathermy to the liver
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Jaundice due to congenital
enzymopathies
Congenital nonhemolytic jaundice family - CriglerNajjar syndrome, is a rare enzimopaty caused by
congenital deficiency of the enzyme glucuronyl
transferase in the liver cells, which leads to
disruption of binding and release of bilirubin
 Clinic: jaundice at birth, continues throughout the
child's life, the amount of indirect bilirubin from 256
to 765 mmol \ l, urine and feces normally painted,
there is no hepatosplenomegaly
 Treatment: symptomatic, in severe cases, Plant. Most
of the children die before their first birthday due to
CNS.
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Jaundice due to congenital
enzymopathies
Galactosemia - due to congenital deficiency of the
enzyme fosfogalaktozauridil transferase, which
regulates the exchange of galactose
 Clinic: jaundice with one day of life, an increase in
the direct fraction of bilirubin, hepatomegaly,
vomiting, general intosikatsiya, glycosuria
 The diagnosis is the detection of high content of
galactose in the blood and the detection
galactosuria.
 Treatment: elimination of milk from the diet,
replacing the artificial mixture containing galactose
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Jaundice due to congenital deficiency of the
enzyme glucose -6- phosphate
dehydrogenase
The lack of this enzyme leads to a violation of
exchange in red blood cells and to their breakdown.
Children with deficiency of this enzyme are basically
healthy
Clinic: severe jaundice in the first days, with
anizopoykilotsitozom anemia, reticulocytosis,
normablastoz, indirect hyperbilirubinemia
Diagnosis: Determination of glucose-6-phosphate
dehydrogenase deficiency in red blood cells
Treatment: symptomatic, with high
hyperbilirubinemia shown exchange transfusion of
blood
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