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As we all know jaundice is seen when the skin and the sclera (white of the eye) becomes yellow.
Jaundice is fairly common in newborns and is seen in 90% of the babies. This is 'normal physiological jaundice' seen usually on second or third day of life and disappears by the 7th – 10th day.
In a newborn, the red cells in the blood are broken earlier and faster as compared to the adults. As a result ' bilirubin' (pigment causing jaundice)[see gi in pathology lectures] which is produced from these broken red cells accumulates in the body causing jaundice. When the body is in the womb, this bilirubin is washed away by the placenta into the mother's circulation and removed. The liver (which removes the bilirubin from the blood) in the newborn is a little immature and takes some days to start functioning properly. Hence jaundice in the baby occurs commonly.Even though there is a well established association between breast feeding and an increased risk of neonatal hyperbilirubinemia in the first week of life, this should in no way inhibit, prevent or discourage mothers from breastfeeding. In babies who nurse poorly, the likelihood of becoming jaundiced is even greater. It must also be remembered that babies of 36 to 37 weeks’ gestation (or less) do not nurse as well as more mature babies. One study has shown that infants at 37 weeks were four times more likely to have a serum bilirubin > 230 mmol/L than those of 40 weeks’ gestation. Other risk factors for developing jaundice include: [see below]ABO incompatibility, maternal diabetes, use of oxytocin in labor, Asian male babies, presence of bruising and cephalhematoma, and a family history of neonatal jaundice.Though 90% of the times, jaundice in a newborn is the physiological jaundice, some features may be suggestive of another disease. If the following signs appear,can be bad
Jaundice appearing within first 24 hours of life. Jaundice persists even after 14 days of life Baby is not feeding well. Baby's urine becomes yellow in color The stools of the baby are pale or clay colored (almost whitish) Baby appears lethargic, irritable. Prolonged jaundice may be seen in premature babies (due to immature liver), some red cell defect, infection, thyroid disorder, liver disorder etc. Hence if the baby is still jaundiced after two weeks after birth, some blood and urine tests are required to determine the cause and early treatment.Some babies may have prolonged jaundice inspite of being completely well. This is usually seen in breast fed babies. Breast-feeding is continued and the jaundice will disappear over time.Usually jaundice in newborn is not at all dangerous. Since, jaundice in babies is not usually harmful and disappears by the 10 th day, no treatment is required. However some babies may require treatment in the form of light ( phototherapy). This is done after the doctor checks the baby's blood for high bilirubin levels. It is given with the help of a special light. Baby is placed under this light completely naked except for presence of eye pads (to shield the eyes) and cloth to cover the genitals. Phototherapy helps to remove bilirubin faster from the baby. The baby should be fed properly and adequately during phototherapy to ensure a good urine output. Phototherapy is usually given for few days.Babies with hypothyroidism may have prolonged jaundice beyond 2 weeks and they should be screened for thyroid disorder and if detected, appropriate treatment should be given. Babies with red cell defects may require further treatment. If liver disease is the cause of jaundice (though very rare), early treatment should be initiated to prevent further damage to the liver.Thus, jaundice in a newborn is usually seen in 90% of babies, is not harmful and disappears by 10th day of life. Feed your baby adequately and properly and watch for the warning signs. In the neonate, dehydration results primarily from an inadequate amount of fluid intake. Breast feeding, first-time mothers and an infant who does not latch properly are risk factors for developing dehydration. Other factors include the presence of inverted nipples, previous reduction surgery, cesarean section, use of analgesics and an immature infant with a weak sucking ability. Initially a total bilirubin will suffice. If there is a suspicion of hemolytic disease or anemia (e.g. clinical jaundice at < 24 hours or a MBR > 230 mmol/L in the first 48 hours), then a blood type and Coombs test should be done. In addition a CBC, smear and reticulocyte count should be ordered. If the neonate is a male Asian or Mediterranean infant with late onset jaundice and a MBR> 260 mmol/L, a G6PD screen should be done.Types of newborn jaundice :
Physiological jaundice:
Some degree of jaundice is evident in a lot of newborns usually on the 2nd day of life. It peaks around the 4th to 5th day & the yellowness disappears by the end of the 1st week.Technically speaking the bilirubin levels are between 10-16 mg%.
Pathological jaundice:
If the bilirubin builds up too high in the body that is more than 17mg% within the first week then it may be toxic to the brain and it can cause permanent brain damage. This can occur due to some infection to the baby, mismatch between the baby’s and mother’s blood group or due to some structural defects in the liver.
Jaundice of prematurity:
It occurs frequently in premature babies because the liver takes much more time to be able to handle the bilirubin levels.
Breast Feeding
Breast milk jaundice:
A particular compound in the mother’s milk can lead to jaundice in a few babies. It usually occurs the 4th day onwards. However sometimes it can reach high levels be prolonged.
Stopping breast feeding & feeding only formula for 2 days subsides the jaundice and then breast feeding can be resumed .
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Physiologic Breast milk jaundice Intestinal obstruction Hemolytic disease:Resorption of extravascular bloodPolycythemia Hypothyroidism Hereditary causes: Criggler-Najjar syndrome MANAGEMENT OF HYPERBILIRUBINEMIA IN THE HEALTHY TERM NEWBORN (1)
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Total Serum Bilirubin Level in mmol/L
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Age(hours)
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Consider phototherapy (2)
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Phototherapy (3)
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Exchange transfusion if intensive phototherapy fails (4)
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< 24 (5)
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-
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-
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-
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25-48
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> 170
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> 260
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> 340
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49-72
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> 260
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> 310
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> 430
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> 72
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> 290
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> 340
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> 430
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PEARLS
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High risk factors in the jaundiced neonate
 Clinical jaundice first 24 hours Jaundice in the < 37 week gestation infant Male, oriental, family history of neonatal jaundice |
Timely follow-up for infants discharged at < 48 hours after birth
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Monitor feeding closely: High risk factors
Weight loss > 10% of birth weight Expect > 6 wet diapers in 24 hours Expect > 2 to 3 stools/24 hours |
JAUNDICE - NEONATAL
DEFINITION:
A disorder caused by an excess of unconjugated or conjugated bilirubin in the newborn period.
EPIDEMIOLOGY:
incidence: ? age of onset: newborn period risk factors: see differential diagnosis DIFFERENTIAL DIAGNOSIS FOR UNCONJUGATED HYPERBILIRUBINEMIA:
1. Increased Production
1. Hemolytic Diseases Intrinsic Membrane Enzyme Hemoglobin Synthesis Extrinsic Immune Non-immune 2. Infection Sepsis TORCH infections 3. Extravesated bruising cephalohematoma delayed cord clamping transfusion (twin-twin, maternal-fetal) 4. Enterohepatic Circulation Increase delayed feeding delayed stooling dehydration GI obstruction 5. Polycythemia 2. Decreased Conjugation
1. Crigler-Najjar Syndrome - I 2. Gilbert Syndrome 3. Lucy-Driscoll Syndrome 4. Breast Milk Jaundice DIFFERENTIAL DIAGNOSIS OF CONJUGATED HYPERBILIRUBINEMIA:
1. Intrahepatic Cholestasis
1. Persistent Alagille Syndrome (arteriohepatic dysplasia) Benign Recurrent Intrahepatic Cholestasis Byler Disease Cholestasis - Lymphedema Neonatal Hepatitis NSP of Interlobular Bile Ducts 2. Acquired Infections Toxoplasmosis Others - Echovirus, Leptospirosis, Syphilis, TB, Varicella Rubella CMV, Coxsackievirus Hepatitis B (?C), HSV Drug-Induced 3. Metabolic/Genetic Disorders Alpha-1-Antitrypsin Deficiency Cystic Fibrosis Dubin-Johnson Syndrome Familial Hepatosteatosis Rotor Syndrome Trihydroxycoprostanic Acidemia Zellweger's Syndrome Trisomies 18 and 21 2. Extrahepatic Obstruction
1. Infantile Obstructive Cholangiopathy Biliary Atresia Bile Plug Syndrome Choledochal Cyst Choledocholithiasis extrinsic bile duct compression spontaneous bile duct perforation 3. Metabolic Disorders
1. Disorders of Carbohydrate Metabolism Hereditary Fructose Intolerance Galactosemia-I+III Glycogen Storage Diseases - Types I,III,IV,VI,IX 2. Disorders of Amino Acid Metabolism Tyrosinemia 3. Disorders of Lipid Metabolism Gaucher Disease Niemann-Pick Disease Wolman Disease Cholesteryl Ester Storage Disease PATHOPHYSIOLOGY:
1. Bilirubin Metabolism:
end product of heme degradation majority derived from RBC's 8-10 mg/kg/day produced 1g Hb = 35 mg bilirubin two types: unconjugated (indirect) - prehepatic, enterohepatic conjugated (direct) - hepatic 2. Physiological Jaundice:
1. Elevated Bilirubin Load increased RBC volume decreased RBC survival increased enterohepatic circulation 2. Decreased Hepatic Uptake of Bilirubin low level of ligandin competition for binding to intracellular proteins 3. Defective Bilirubin Conjugation decreased UDP glucuronyl transferase activity 4. Defective Bilirubin Excretion 3. Non-Physiological Jaundice:
see differential diagnosis CLINICAL FEATURES:
1. Physiologic Jaundice
1. Features
appears on Day 2 peaks on Days 2-5 disappears after Day 7 (term infants) disappears after Day 14 (premature infants) of term babies: 60% are jaundiced within the first week 95% have a bilirubin < 260 umol/L visible jaundice occurs at levels > 85 umol/L 2. Non-Physiologic Jaundice
1. Features
within 24 hours total bilirubin > 225 umol/L direct bilirubin > 35 umol/L or > 30-40% of total rate of rise > 85 umol/L in a 24 hour period persists > 7 days (term) or > 14 days (preterm) 3. Approach
1. History
1. Maternal History blood type past obstetrical history abortions (spontaneous or induced) jaundiced sibs past medical history IDDM family history inborn errors of metabolism, cystic fibrosis congenital hepatic diseases congenital hemolytic anemia jaundice, anemia, splenectomy, gallbladder disease 2. Pregnancy History complications infections (TORCH), illnesses, PIH, GDM sepsis - premie, fever, increased WBC, PROM, amnionitis, etc 3. Labour and Delivery History gestational age - ? premie method of delivery - ? traumatic APGAR or cord gases - ? birth asphyxia ingestion of maternal blood delayed cord clamping 4. Post Natal History characteristics of jaundice - ? onset, duration, etc. method of feeding - ? breast milk jaundice infants blood group - ? Rh or ABO incompatability evidence of underlying illness: delayed passage of meconium, nausea/vomiting, sepsis, failure to thrive, decreased caloric/fluid intake 2. Physical
1. Vitals temperature (sepsis) weight/length (SGA, LGA) head circumferance (decreased size with TORCH infections) 2. HEENT cephalohematoma bruising 3. Gastrointestinal hepatosplenomegaly (hemolytic anemia, congenital infections, inborn errors of metabolism) mass/distention (obstruction, adrenal hematoma) 4. Skin plethoric (polycythemia) pallor (anemia) petchechiae (sepsis, congenital infections, severe hemolytic anemia) INVESTIGATIONS:
1. First Line
bilirubin (total and direct) blood groups and Rh (mother and infant) CBC (Hct, Hb, Platelets) Coombs test peripheral blood smear/morphology reticulocyte count 2. Second Line
septic work-up cultures (blood, CSF, urine), chest x-ray screen for hypothyroidism TSH, T4 screen for inborn errors of metabolism urine for organic and amino acids plasma for amino acids alkaline denaturation of Hb test of emesis - PT, PTT, haemoglobin electrophoresis MANAGEMENT:
1. Alter Feeding
interrupt breast-feeding stimulate enterohepatic circulation 2. Phototherapy
based on birth weight and age of patient white or blue lights, single or double bank principle: photoisomerization 3. Exchange Transfusion
direct removal of bilirubin 4. Medications
phenobarbital trial to induce glucuronyl transferase activity |