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pediatrics > Hyperbilirubinemia in the newborn. > Flashcards

Hyperbilirubinemia in the newborn. Flashcards

1
Q

where does haemoglobin get catabolised ?

A

in the reticuloendothelial system

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2
Q

how much more bilirubin do newborn produce compared to adults

A

two to 3 times moe

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3
Q

what is the cause of increased bilirubin production in newborns ?

A

increased RBC , or hematocrit ,

shorter erythrocyte life span

inefficient erythropoesi

decreased albumin binding = lower albumin con

decreased ligandin so less hepatic uptake

decreased secretion

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4
Q

indirect bilirubin is particularly harmful to which part of the body

A

toxic to the CNS

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5
Q

what can increase indirect bilirubin rather than liver and spleen and reticuloendothelial probes

A

organic acids such as fatty acids and drugs can displace the bilirubin from albumin

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6
Q

which enzyme represents the rate limiting step of bilirubin conjugation

A

glucuronosyltransferase

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7
Q

for hepatic conjugation to occur bilirubin myst dissociate from albumin and bind to which liver protein

A

ligandin

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8
Q

conc of ligandin and glucuronosyltransferase is particularly low in whom

A

newborn and preterm

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9
Q

can indirect bilirubin cross the placenta

A

yes it can cross the placenta and become conjugated by the maternal hepatic enzymes

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10
Q

clinical pattern of physiological jaundice is what

A

hyperbilirubinemia can start no before than 24 hrs and no more than 12MG/DL in the 3rd day of life

in preterm = 15mg/dl in the fifth day of life

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11
Q

jaundice is unphysiological if what ?

A

appears on the first day of life

bilirubin levels increases more than 0.5MG/DL/HR

if the peak bilirubin is greater than 13mg/dl in term infants

if direct bilirubin fraction is greater than 1.5MG/DL

heaptosplenomegaly and anemia is present

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12
Q

what is crigler-najjar syndrome ?

A

autosomal dominant disease
or autosomal recessive

rare but permanent deficiency of gluconosyltransferase

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13
Q

what can treat AUTOSOMAL DOMINANT crigler-najjar syndrome ?

A

some variety respond to enzyme induction by phenobarbitural = producing and increase in enzyme activity and reduction of bilirubin lives

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14
Q

what can treat autosomal recessive format of the crigler-najjar syndrome ?

A

it does not respond to phenobarbitutal and manifest as perisirant indirect hyperbilirubinemia

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15
Q

what is gilbert disease ?

A

mutation of the promoter region of glucuronsyltransferase = mild indirect hyperbilirubinemia results

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16
Q

if gilbert disease was concomitant with what other disease can lead to severe jaundice formation

A

other icterogenic factor such as hemolysisi

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17
Q

breast milk jaundice have appearance when in a baby’s life

A

first or second week

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18
Q

what is a sign and symptom of breast milk jaundice ?

A

bilirubin rarely increases more than 20mg/dl and interruption of breast feeding for 1 or 2 days results in rapid decline of bilirubin which do not increase significantly after breastfeeding resumes

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19
Q

why is there breast milk jaundice ?

A

breast milk may contain the inhibitor of bilirubin conjugation

increase the enterohepatic recirculation of bilirubin because of breast milk glucuronidase

20
Q

early onset of jaundice scubas in the first days are due to ?

A

hemolytic diseases
internal haemorrhage = cephalohematoam ,, hepatic or splenic hematoma
OR infection sepsis
delayed cord clamping
Inherited red cell enzyme, membrane defects
(e.g., spherocytosis, glucose 6-phosphate-dehydrogenase deficiency,

21
Q

physical evidence of jaundice is brough about when bilirubin levels reach what level

A

5-10mg/dl

unlike 2 mg/dl in adults

22
Q

bilirubin greater than what in first day of life and second day of life should be evaluates

A

first day of life greater than 5mg/dl

second day greater than 13mg/dl

23
Q

what are the diagnostic pathways to determine the etiology of the bilirubin

A

test direct and indirect bilirubin levels

Coombs test - for autoimmune haemolytic anemia

complete blood count

blood smear

reticulocyte count

24
Q

what are the hemolysis absent causes of jaundice which is common

A

physiological

breast milk
internal haemorrhage
polycethma in diabetic mother

Mutations of glucuronyl transferase enzyme (Crigler- Najjar syndrome,
Gilbert isease),
pyloric stenosis, hypothyroidism

25
Q

what are the hemolysisi present causes of jaundice

A

ABO blood group incompatibility
rh incompatibility
kell
duffy

Red blood cell enzyme defects: glucose-6- phosphate dehydrogenase, pyruvate kinase Red blood cell membrane disorders: spherocytosis, ovalocytosis
hemoglobinopathy - thalessemia

26
Q

bilirubin encephalopathy or kernicterus is caused by bilirubin deposited where ?

A

basal ganglia

27
Q

how can the indirect bilirubin cross the blood brain barrier

A

because of its lipid solubility and water insolubility

28
Q

kernicterus usually develop in term infants when the bilirubin is ?

A

above 25mg/dl

In extremely immature infants weighting less than 1000 g when bilirubin
levels are less than 10 mg/dL because of
a more permeable bloodbrain barrier
associated with prematurity

29
Q

the risk of kernicterus increases in the presence of what concomitant diseases

A 
meningitis 
hemolysisi 
asphyxia 
hypoxia 
hypothermia 
hypoglycaemia 
bilirubin displacing drugs
30
Q

what are the earliest clinical manifestation of kernicterus ?

A 
lethargy 
hypotonia 
irritability 
poor moro reflex 
poor feeding 
high pitched cry 
emesis
31
Q

when are the early signs of kerincterus noted ?

A

4 days of life

32
Q

what are the late signs of kerincterus

A

bulging fontanel

opisthotonos posturing = when the bad becomes extremely arched due to muscle spasm seen in serious brain conditions such as meningitis and tetanus
pulmonary haemorrhage

fever

hypertonicity

paralysis of upward gaze

seizures

33
Q

what are the late complication of kernicterus

A 
spasticity resolves 
however nerve deafness 
choreoathetoid cerebral palsy 
mental retardation 
enamel dysplasia 
discolouration of teeth
34
Q

early signs of kernicterus may be reversed using what

A

institute change transfusion

35
Q

what is the treatment for hyperbilirubinemia ?

A

blue light phototherapy = esp when reaching between 16-18mg/dl

bilirubin is converted into isomers which are water soluble and easily excreted

36
Q

infants with mild haemolytic disease can be managed how ?

A

occasionally by blue light phototherapy

37
Q

when is exchange transfusion given ?

A

dangerously high levels of

as a rule of thumb 10 percent of the baby’s weight is used as the change number for indirect bilirubin

20mg/dl of bilirubin can be exchanged for a baby weight 2000g

depending on size of baby 5-20ml of of blood is withdrawn and new is infused per cycle the procedure lasting 45-90 minutes

total amount of blood exchanges = twice the infants blood vol
calculated

weight (kg) x85ml/kg x 2

this volume should remove 85 percent of infants RBC source of bilirubin , maternal antibodies

38
Q

exchange transfusion is usually performed through what ?

A

umbilical venous catheter placed in the inferior end cava or the confluence of the umbilical vein and portal system

39
Q

what are the complications of transfusion ?

A

transfusion reaction
metabolic instability
infection

vessel perforation - haemorrhage

hypotension
nectrositing enterocolitis

40
Q

transfusion is also conjugated with what therapy o reduce the amount of transfusion needed ?

A

phototherapy

41
Q

at what level is there direct conjugated hyperbilirubinemia

A

more than 2mg/dl or more than 20 percent of total bilirubin

42
Q

is direct conjugated hyperbilirubinmia physiological

A

it is NEVER physiological

43
Q

what causes direct conjugated hyperbilirubinemia ?

A 
cholestasis
Choledochal cysts
billary atresia 
hepatocellular injury 
alpha 1 antitrypsin def
CMV infection 
TORCH 
neonatal hepatitis 
sepsis 
cystic fibrosis 
inborn errors of metabolism 
neonatal iron storage disease
44
Q

what is the diagnosis of conjugated direct hyperbilirubinemia ?

A

measure liver enzymes

bacterial and viral cultures

metabolic screening tests

hepatic ultrasound

sweat chloride test

45
Q

what is a strong sign of binary atresia

A

dark urine

grey white stool after th second week

46
Q

is direct bilirubin neurotoxic ?

A

Direct bilirubin cannot cross BBB and thus is not neurotoxic to the infant

pediatrics (19 decks)

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