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PEDIATRICS > Jaundice > Flashcards

Jaundice Flashcards

1
Q

Definition

A

Jaundice is the visible manifestation of increased levels of
bilirubin in the body.
– It is not a disease, but only a symptom

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2
Q

Values

A

*Normal BR =0,1 - 1mg/dl (2-17μmol/l)
(max 1,3-1,5mg/dl)
*Conjugated BRD =0-0,3mg/dl (0-5 μmol/l)

*In adults sclera appears jaundiced when serum bilirubin exceeds
BR>2mg/dl (5-7mg/dl in neonates)

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3
Q

Bilirubin

A

 End product of hemoglobin metabolism that is excreted in
bile.
 1g Hb ⇒ 34mg BR
 In neonates -75% : from catabolism of circulating RBCs
-25% :*from ineffective erythropoiesis
 BR has no known physiologic function
 The non-conjugated form has the potential CNS
toxicity due to fat soluble properties and
hematoencephalic barrier permeability during the
neonatal period

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4
Q
  • Unconjugated BR (indirect)
A

– Non-polar, toxic, water insoluble compound, MW 584
– Permeates easily through lipid-containing barriers (blood-brain
barrier – important risk of neurotoxicity – kernicterus)
– Requires conjugation with glucuronic acid to form a water soluble
product that can be excreted

  • It will circulate to the liver reversibly bound to albumin (MW 70,000)
  • Conjugated (direct) bilirubin - polar, high MW, water-soluble
    compound
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5
Q

Conjugation

A
  • Conjugated bilirubin crosses the placenta very little and conjugation
    is not active in the fetus (low levels of UDPGT - about 1% of the
    adult levels at 30 - 40 weeks gestation)
  • After birth, the levels of UDPGT rise rapidly but do not reach the
    adult levels until 4-6 weeks of age.
  • Ligandins, which are necessary for intracellular transport of bilirubin,
    are also low at birth and reach adult levels by 3-5 days.
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6
Q

Enterohepatic circulation

A
  • Conjugated bilirubin is unstable and easily hydrolyzed to
    unconjugated bilirubin.
  • This process occurs non-enzymatically in the duodenum and
    jejunum

!!! It occurs also in the presence of beta-glucuronidase, an enteric
mucosal enzyme having high levels:

  • in newborn infants
  • in human milk.
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7
Q

Hyperbilirubinemia

A

 Hyperbilirubinemia
-Direct (Conjugated)
-Indirect (Un-conjugated)

 Conjugated Hyperbilirubinemia is present,
* >10 -25% of total bilirubin is conjugated
* >2mg/dl is conjugated

 If neither criteria is met, hyperbilirubinemia is classified
as un-conjugated.

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8
Q

Kernicterus

A
  • “nuclear jaundice“, german term, with kern = grain,
    core, indicating the most commonly afflicted region
    of the brain (ie, the nuclear region).
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9
Q

Kernicterus or bilirubin encephalopathy

A

neurologic syndrome resulting from deposition of unconjugated (
indirect) bilirubin in the basal ganglia and brainstem nuclei

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10
Q

Pathogenesis is multifactorial Unconjugated bilirubin levels

A
  1. Albumin binding and unbound bilirubin levels
  2. Passage across the blood brain barrier
  3. Neuronal susceptibility to injury
  4. Disruption of the blood – brain barrier by : disease, asphyxia, acidoza…
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11
Q

Kernicterus factors favoring

A

– increased BR production,
– acidosis (sepsis), hypoproteinemia,
– the presence of substances that compete with the binding of
albumin BR (metabolites, drugs),
– altered hepatic metabolism of BR,
– increasing BR enterohepatic recirculation

  • Important factors in the appearance of kernicterus :
    – the BR (unconjugated, free) level,
    – the day of exposure (day3)
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12
Q

Uncojugated BR induced CNS damage (basal ganglia)

  • Clinical signs - days 2-10
A

– Severe depression: lethargy, decreased / disappearance of archaic
reflexes (sucking, Moro)
– Excitation: opistotonus, seizures, screaming, bulging AF
– Death/neurological sequelae: impaired hearing, vision, cerebral
palsy, seizures, memory loss, behavioral disorders

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13
Q

Causes of Kernicterus

A

1.Massive haemolysis
2.Criegler Najjar disease ( tip I Arias),
3.Prematurity

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14
Q

Clinical Findings

A

Acute
Lethargy
Poor feeding
Tone abnormalities
Opisthotonus
High pitched cry
Seizures
Apnea
Abnormal auditory brainstem response

Chronic
Mild neurodevelopmental delays
Choreoathetoid cerebral palsy
Paralysis of upward gaze
Sensorineural hearing loss
Dental dysplasia

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15
Q

KERNICTERUS – PREVENTION

A

Universal screening for hyperbilirubinemia in the first 24 -48 hr of life
to detect infants at high risk for severe jaundice and bilirubin –
induced neurologic dysfunction

Any infant who is jaundiced before 24 hr requires measurement of
the serum bilirubin level and if it is elevated - infant should be
evaluated for possible hemolytic disease

Follow –up should be provided within 2 – 3 days of discharge to all
neonates discharged earlier than 48 hr after birth
Early follow –up is particularly important for infants younger than 38
weeks gestation

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16
Q

Increased bilirubin production

A

– Daily 6-10mg/kg/day BR (3-4mg/kg/day adult)
– Larger circulating red blood cell volume
– Shortened RBC lifespan (70-90 days vs 120 days in adult)
– Substantial production from other sources

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17
Q

Lower plasmatic transport

A

In newborn the albumin affinity for bilirubin is low

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18
Q

Limited hepatic capacity of bilirubin metabolism

A

– Defective uptake from plasma into liver cell (deficiency of
LIGANDIN)
– Defective conjugation (UDP-glucuronosyl transferase: <1% of adult
activity during the first 10 days of life)
– Decreased excretion

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19
Q

Increased entero-hepatic circulation

A

– 6 times higher than in the adult
– Higher in intestinal obstructions, decreased intestinal motility,
fasting

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20
Q

INCREASED PRODUCTION OF BILIRUBINE
Increased erythrocyte turnover

A
  • NORMAL - Shortened RBC lifespan
  • PATOLOGIC - HEMOLYSIS - blood group incompatibilities
    (ABO/Rh), enzyme deficiencies (G6PD, piruvatkinaza),
    structural anomalies(sferocytosis…)
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21
Q

Blood sequestered (hemoragies)

A
  • Hematoma /cephalhematoma, hemangiomas
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22
Q

Encreased enterohepatic circulation of BR

A
  • NORMAL: newborn in the first week
  • PATOLOGICAL : mechanical obstructions (intestinal atresia,
    meconium plug), neurologic cause, ileus paralytic
23
Q

Neonatal jaundice (BRI) mechanism

A
  • Low hepatic ligandins
  • Decreased GLUCURONONYL TRANSFERASE ACTIVITY
    – Temporary in every newborn
    – Far low in CrieglerNajjar sdr, LuceyDriscoll sdr
    – Moderately low in Gilbert sdr,
    – Low in hypopituitarism, hypotiroidism
  • MULTIFACTORIAL
    – Sepsis
    – Prematurity
    – Breast milk jaundice
  • Intra and extrahepatic CHOLESTASIS
    An abnormal reduction in bile
    formation/excretion
    – Neonatal hepatitis
    – Biliary atresia
  • CONGENITAL CAUSES
    – Sdr. Dubin-Johnson
    – Sdr. Rotor
24
Q

Neonatal jaundice generalities
HISTORY

A

– Incompatibility ABO/Rh with the mother
– Maternofetal infection, fetal distress

25
Q

Neonatal jaundice
CLINICAL EXAM

A

– JAUNDICE :difficult to appreciate the intensity
!!! more important the onset (definitely pathological if <36 hours);

– Appearance of stool and urine
– Signs of abnormal hemolysis: pallor, edema, serous effusion (ascites,
hepatosplenomegaly), extramedullary hematopoiesis, hemoglobinuria
(cherry red urine)
– Signs of infection (especially neurological signs)

26
Q

NEONATAL JAUNDICE
LABORATORY

A

– CBC (peripheral blood smear - red blood cell
morphology, reticulocyte count
– ABO group, Rh
– Coombs tests
– BRT/BRD/ BRI
– Albumin level (index of saturation with bilirubine)

27
Q

NEONATAL JAUNDICE
CLASSIFICATION
ONSET

A
  • Onset :
    – Early (in the first 24-36hours),
    – “Normal ” – at 48-72 hours after birth,
    – At any other time (generally > 72 hours after birth)
  • BR Type : with predominance of BRI or BRD
  • Congenital / acquired
  • NB abnormal if: the onset is < 36 hours, the BR level
    rises rapidly (>0,5mg/dl/day), the duration is
    extended or there are other changes associated
28
Q

NEONATAL JAUNDICE
CLASSIFICATION

A
  • Physiological Jaundice (premature newborns)
  • Hemolytic Jaundice (early onset, risk of kernicterus,
    rapid raise in BR>0,5mg/kg/hour)
  • Infectious Jaundice (possible onset from birth, sepsis)
  • Prolonged Jaundice
    – With IBR
    – With DBR
29
Q

PHYSIOLOGICAL JAUNDICE

A

 First appears - day 2-3
 Maximum intensity seen on 4-5th day in term and 7th day
in preterm neonates
 Does not exceed 15 mg/dl
 Clinically undetectable after 14 days (21 in prematures).
 Normal urine and stools
 No hepatosplenomegaly, no signs of infection

 No treatment is required but baby should be observed
closely for signs of worsening jaundice.

30
Q

PHYSIOLOGICAL JAUNDICE IS EXLUDED IF

A
  • The onset is in the first 24 hours of life
  • TBR rises with > 5mg/dl/day
  • TBR >12,9mg/dl (15mg/dl in a premature newborn)
  • DBR >1,5-2mg/dl
  • It lasts >1-2 weeks in the newborn at term /// 2-3
    weeks in a premature newborn
31
Q

PHYSIOLOGICAL JAUNDICE
TREATMENT

A

– Fototherapy (at term >13-14mg/dl, premature >10mg/dl)
– Exchange transfusion
– Fluid therapy, iv albumin

32
Q

Phototherapy

A

has been the mainstay of treating
hyperbilirubinemia since the 1960s.

  • Phototherapy causes structural isomerization, forming
    lumirubin, which is then excreted in the bile and urine.
  • Since photoisomers are water soluble, they should not
    be able to cross the blood-brain barrier, so starting
    phototherapy should decrease the risk of kernicterus
  • To be effective, bilirubin must be present in skin, hence
    no role for prophylactic phototherapy
33
Q

HEMOLYTIC JAUNDICE

A
  • The onset is < 36 hours of life
  • Risk of kernicterus
  • Causes
    – Blood Group Incompatibilities (ABO, Rh)
    – Structurally Abnormal Red cells
    – Non-immune Hemolytic Anemias (G6PD deficiency)
    – Severe sepsis
  • Clinic
    – Jaundice (variable as intensity)
    – Normal Urine and stool
    – Hepatosplenomegaly
34
Q

HEMOLYTIC JAUNDICE DIAGNOSIS

A
  • Diagnosis
    – History, Clinical exam
    – TBR/ IBR/ DBR
    – CBC with peripheral blood smear
    – Blood group (ABO, Rh) – mother and child
    – Coombs Test
    – Evaluation for sepsis /TORCH/associated pathology
35
Q

HEMOLYTIC JAUNDICE TREATMENT

A

– In severe forms - exchange transfusion
– Supportive treatment, parenteral nutrition
– Sepsis treatment - antibiotherapy

36
Q

BREAST MILK/FEEDING JAUNDICE

A
  • BRI jaundice
  • Healthy newborn, no signs of infection, no signs of
    hemolysis
  • Normal urine and stool
  • Onset from the first week; can last until 8 weeks of age
  • 50-70% of newborns have jaundice
  • Moderate (>12 mg/dL) develops in 4% of bottlefed compared to 14%
    of breastfed
  • Severe jaundice (>15 mg/dL) occurs in 0.3% bottlefed vs

2% of breastfed

37
Q

Breastfeeding Jaundice vs Breastmilk Jaundice

A

Lecture

38
Q

JAUNDICE IN NEWBORNS AND INFANTS

A
  • PHYSICAL EXAMINATION
    – Jaundice
    – Dark urine, acholic stools
    – Pruritus/itch (after 4-5 months)
    – Hepatomegaly
  • BIOLOGIC
    – DBR > 10-25% TBR (sometimes it reaches 50-90%)
    – Hyperlipemia (total colesterol)
    – Elevated alkaline phosphatase, 5 nucleotidaza
    – Steatorrhea
39
Q

PATHOLOGICAL JAUNDICE

A
  • Clinical jaundice detected before 24 hours of age
  • Rise in serum total bilirubin by more than 5 mg/dl/ day (>5mg/dl on first
    day , 10 mg/dl on second day and 12- 13 mg/dl thereafter in term
    babies)
  • Serum bilirubin more than 15 mg/dl
  • Clinical jaundice persisting beyond 14 days of life
  • Clay/white colored stool and/or dark urine staining the nappy yellow
  • Direct bilirubin >2 mg/dl at any time.
  • Treatment is required in the form of phototherapy or exchange blood
    transfusion.
    – ! You should investigate to find the cause of a pathological jaundice.
40
Q

INTRAHEPATIC CHOLESTASIS: normal extrahepatic
biliary tract, no obstruction

A

– Infectious diseases (sdr TORCH, HVB, sifilis, lysteria)
– Genetic causes (Down’s)
– Metabolic causes (galactosaemia, glycogenosis, CF, α1-
antitrypsin deficiency)
– Toxic causes (sepsis with endotoxinemia, TPN
associated cholestasis)

41
Q

EXTRAHEPATIC CHOLESTASIS : extrahepatic biliary
obstruction (anatomical or mechanical)

A

– Extrahepatic biliary atresia
– Choledochal Cyst
– Tumors (rhabdomiosarcomas)

42
Q

NEONATAL CHOLESTASIS

A
  • History, Clinical exam
  • TBR, DBR, IBR + lipids (cholesterol, TG) + AP
  • TGO, TGP
  • Albumine, protrombine level (hepatic synthesis)
  • Cultures (blood, urine)
  • Serology (TORCH, agHBs, HIV, VDRL)
  • α1-antitrypsine //T4, TSH
  • Sweat test
  • Metabolic screening
  • Ultrasound (liver, biliary tract)
  • liver scintigraphy
  • Liver biopsy
43
Q

Neonatal Hepatitis

A
  • PHYSICAL SIGNS
    – Kepatic dysfunction present from the beginning
    – Clotting disorders
    – Stools : normal and acholic (alternating)
    – Variable intensity of jaundice (spontaneous
    improvements)
  • TREATMENT
    – Supportive and symptomatic
    – Diet (hypercaloric, MCT and vegetable fats)
    – Vitamin K
    – Pruritus: colistiramine, phenobarbital
    – ?? Corticosteroids ?? (improvement of bile flow)
44
Q

Extrahepatic biliary atresia

A
  • PHYSICAL SIGNS
    – Symptom-free interval (2-3 weeks)
    – Progressive jaundice (cholestasis)
    – Verdinic jaundice the skin colour turns to green
    – Progressive atrepsia (severe malnutrition)
    – Later: biliary cirrhosis (ascites, portal hypertension)
  • TREATMENT
    – Surgical treatment – the only option
    – Kasai portoenterostomy – restoration of bile flow to the intestine
    – Best results if < 60 days
    – Liver transplantation
45
Q

CHILD’S JAUNDICE

A

HISTORY:

 Age at onset of symptoms. E.g.: Wilson’s disease
commonly manifests in pre-adolescents & adolescents.
 Past/present use of any drugs
 History of blood transfusion/ dialysis
 Exposure to viral hepatitis
 Any history of chronic illness; hemoglobinopathies.
 Family history of inheritable disorders: Wilson’s.

46
Q

CHILD’S JAUNDICE
Indirect hyperbilirubinemia

A

Hemolytic
Gilbert syndrome – nonhemolytic jaundice

47
Q

Hemolytic

A

 Auto-immune hemolytic anemias
 Drug induced hemolytic anemias
 Erythrocyte membrane defects
 Erythrocyte enzyme defects
 Hemoglobinopathies
 Congestive cardiac failure
 Sepsis

48
Q

Gilbert syndrome – nonhemolytic jaundice

A

(an autosomal dominant inherited liver disorder characterized by jaundice
due to unconjugated hyperbilirubinemia, resulting from a partial
deficiency in hepatic bilirubin glucurononyl-transferase activity).

49
Q

CHILD’S JAUNDICE
Direct hyperbilirubinemia

A
  • **Infectious jaundice **– most frequent
    – HAV, HBV, EBV, leptospirosis
  • Chronic Hepatopathies
  • Toxic jaundice (drugs)
    – Overdose /side effect of a cytotoxic substance
    – Analgesics, antipyretics, antibiotics, chemotherapy,
    anticonvulsants, cytotoxic and immunosuppressive drugs
    – Mushrooms, industrial toxics….
50
Q

CHILD’S JAUNDICE
DIRECT HYPERBILIRUBINEMIA

A

Obstructive
 Gall stones
 Primary sclerosing cholangitis
 Choledochal cyst
 Tumors

Infections
 Hepatitis A, B, C, D, E
 EBV,CMV
 Varicella zoster
 HIV
 Leptospirosis

51
Q

Congenital (noncholestatic) Jaundice

A

Dubin Johnson Syndrome
Sdr. Rotor Syndrome

52
Q

Dubin Johnson Syndrome

A

a benign, inherited liver disorder (autosomal recessive) characterized clinically by chronic, predominantly conjugated, hyperbilirubinemia (without elevation of liver enzymes - ALT, AST and histopathologically by black-brown
pigment deposition in parenchymal liver cells.

53
Q

Sdr. Rotor Syndrome

A

is a benign, inherited liver disorder characterized by chronic, predominantly conjugated, nonhemolytic hyperbilirubinemia with normal liver histology.

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