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Prolonged or Persistent
rd
Day 1 of life ( < 24 hours) Day 2 – day 21 (3 week) Neonatal Jaundice
( > 3 weeks)
Always pathological [1] Physiological: 5 – 10 % of newborn
Never physiological Breast Feeding: infants are still jaundiced
↑↑ chance of (day 3 – 7) at more than 3 weeks of
Hemolytic DDx not receiving enough age.
milk
Breast Milk: This is usually
receiving enough milk unconjugated
hyperbilirubinemia , which
[2]Pathological: resolves shortly after
Usually conjugated but it wards.
may start unconjugated
then conjugated. Pathological is
"conjugated" or it might
be "unconjugated" then
conjugated caused by
liver disease.
Causes: Causes: Causes:
[unconjugated]:
[conjugated]:
(1) Rh incompatibility:
- It's the most serious of the hemolytic type, but less common than
ABO incompatibility
- It's due to blood group incompatibility between [maternal] Rh- &
[fetal] Rh+ IgG antibody reaction
- Hx of blood transfusion to mother is important, also previous
pregnancy, & previous delivery of Rh+ baby .
- O/E: The affected baby will have pallor [due to hemolytic anemia),
hepatosplenomegaly
- Investigations: +ve Coomb's test, ↑ retic count, rapidly ↑ bilirubin
with ↑ direct.
- Nowadays, this type [Rh] is not common, due to availability of
anti-Rhogam Ab / Anti-D Ab for the sensitized Rh- mother
- The mother has blood group O & the infant has either group A, B.
- Most ABO Abs are IgM & don't cross the placenta, but some group
O women have IgG anti-(either A or B)-hemolysin in the blood
which can cross the placenta & hemolyse the red cells.
- More common than Rh incompatibility, but less severe.
- Anemia is less severe
- Direct Ab test (Coomb's test) is not always +ve, weakly +ve,
might be –ve.
- Spherocytes in blood film is a feature.
- It occurs in about 60% of normal full term infants during the 1st
week of life.
- Why do we see jaundice & consider it to be normal, physiologic:
Due to:
1. ↑ RBC production catabolism of extra-hemoglobin.
2. Shorter RBC life span in neonate [70 days] whereas in
adulthood 120 days.
3. ↓ uptake by of bilirubin from plasma, due to transient
immaturity of binding proteins So, ↑ chance for
destruction
4. ↓ uridine diphosphate glucouronyl transferase enzyme
5. ↓ bacteria that convert bilirubin to uroblinigen
- It's diagnosed by exclusion:
Criteria:
1. Baby must be looking normal + active
2. No anemia or organomegaly
3. Jaundice not be seen at day 1 of life
4. Usually seen on in 3rd day in full term, & at day 5 in preterm
5. Not persistent for more than 1 week in term, & 2 weeks in
preterm
6. Bilirubin level is maximally 13 mg/dl in full term & 15 mg/dl
in preterm
7. Bilirubin daily rise should never exceed 5 mg/dl. ﻣﮭﻢ
8. There should be no family Hx of splenectomy or gall
bladder surgery
- Prolonged physiological jaundice:
1. Hypothyroidism (thyroxin is important for maturation of the
enzymes) & constipation leads to increase unconjugated
bilirubin enterohepatic circulation
2. Congenital hypertrophic pyloric stenosis (most probably due
to hypoglycemia as glucose is essential for formation of the
glucuronic acid)
3. Breast milk jaundice
4. Constipation
5. Polycythemia
6. IDM
7. SGA
(7) hypothyroidism
1) Phototherapy:
Purplish blue light wave length 540 nanometer (425 – 475)
It converts unconjugated bilirubin into polar photoisomers
which are less lipophilic
During phototherapy: temperature & hydration must be
monitored.
Side effects of phototherapy:
1. Water loss + dehydration
2. Diarrhea, over heating [Bronze baby syndrome]
3. Hyperpigmentation
4. Retinal damage especially when eyes are not covered
during ttt.
2) Phenobarbitone:
It may be used in ttt of unconjugated jaundice as it stimulate the
synthesis of bilirubin binding proteins glucuronyl transferase
enzyme.
Given in the 1st day or to the mother before delivery.
Dose: 8 mg/kg/day in 2 doses, IM
3) Exchange transfusion:
Umbilical vein catheter after cord cutting, through a special
valve, over 45 – 60 minutes period.
Aim: exchange of double the blood volume of the infant
(2 x 85 ml x body weight in kg)
In case of Rh incompatibility, blood group of the donor should
be the same of the infant but Rh-, while in ABO incompatibility
it should be group O.
Side effects of exchange transfusion:
Cardiac arrhythmias
Electrolyte disturbance
Embolism (blood clots, or air)
Portal or splenic vein thrombosis
Infection
# Stages of Kernicterus:
Categories:
Conjugated jaundice:
- Pale stool
- Dark urine
- Failure to thrive
- Bleeding tendency ↓ vit. K
(1) Biliary atresia: + case history 20.1, page 339 (illustrated textbook)
Abdominal US
Choledocal Biliary
Cyst If Atresia
Intraoperative
cholaniogram
+
Biopsy
↓
It'll show
fibrosis &
ductal
proliferation
So, intraoperative cholangiogram is diagnostic.
- Clinical features:
Prolonged neonatal jaundice
Bleeding (vit. K ↓) especially in breast-fed.
Hepatomegaly, splenomegaly
Cirrhosis
Portal hypertension
- Diagnosed by:
Enzyme level (α1-antitrypsin) in the blood
Phenotype: PiZZ on chromosome 14
(3) Galactosemia:
- Diagnosed by:
1. Tandem MS [metabolic screen]
2. Measuring the enzyme (galactose-1-phosphate uridyl
transferase) level in RBCs
- Clinical picture:
1. ∆ facies Triangular
2. Pulmonary hypertension
3. Eye defect
4. Intrahepatic biliary hypoplasia jaundice, severe pruritus +
FTT
5. Renal tubular disorders.
6. Butterfly vertebrae in X-Ray.
DISEASES OF THE LIVER
Viral Hepatitis
See Prof. Tahir Toonisi's Book (pages 68 – 70)
Causes:
Reye's $:
It's seen when giving aspirin for a child aged less that 12 years of those
who have some genetic problems.
Causes:
1. Chronic hepatitis:
Post-viral hepatitis B,C
Autoimmune hepatitis
Drugs (nitrofurantoin, NSAIDs)
Inflammatory bowel disease
Primary sclerosing cholangitis (± ulcerative colitis)
2. Wilson's disease ( > 3 years )
3. α1-antitrypsin deficiency
4. Cystic fibrosis
5. Secondary to:
Neonatal liver disease
Bile duct lesions
By: angelic_doc
Sources:
Dr. Hassan Moria summery sheet 2008, Illustrated Textbook of Pediatrics, Manual of Pediatrics, Pediatrics for
Undergraduates…