Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
What Is Hyperbilirubinemia?
Hyperbilirubinemia (also known as jaundice) is an
increased level of bilirubin in the blood bilirubin : A substance in the blood that is produced by the breakdown of red blood cells. It is released in the unconjugated form. Unconjugated bilirubin is fat-soluble. It cannot be excreted in bile or urine and is absorbed by the SQ fat, causing a yellowish discoloration of the skin Jaundice is observed during the 1st wk in approximately 60% of term infant and 80% of preterm infant. Hyperbilirubinemia can be toxic, with high levels resulting in an encephalopathy known as kerni-cterus.
2
Conjugation of bilirubin
The liver must conjugate the bilirubin (change
it into a water-soluble form) in order for the body to excrete it through the biliary tree into the gut.
Conjugated bilirubin is further catabolised by
intestinal flora & It forms a major component of bile in faeces (This gives the characteristic orange colour to faeces).
A small amount is passed in the urine
3
mucosa and returned to the circulation. Travels in plasma, bound to albumin. Enters the liver cells with the aid of Y & Z carrier proteins Becomes conjugated with glucoronic acid by an enzyme Glucuronyl Transferase Hypoxia or hypothermia may compromise bilirubin conjugation Total serum bilirubin is the sum of conjugated (direct) and unconjugated (indirect) bilirubin.
BILIRUBIN
UNCONJUGATED Final product of heme degeneration Water-insoluble Tightly complexed to serum albumin Cannot be excreted in urine Free form is toxic Lab test: Total bilirubin minus direct bilirubin Normal values 0,2-1.4 mg/dl
CONJUGATED Water-soluble Loosely bound to serum albumin Excess amounts are excreted in urine Nontoxic Lab test: measured by direct bilirubin Requires O2 and glucose
J - aundice within first 24 hrs of life A - sibling who was jaundiced as neonate U - nrecognized hemolysis N on-optimal sucking/nursing D - eficiency of G6PD I - nfection C ephalhematoma /bruising E - ast Asian/North Indian
6
Types of Jaundice
Physiologic Jaundice. Breast milk Jaundice
Pathologic Jaundice
OR
Early Onset Late Onset
Physiologic Jaundice
Peak bilirubin concentration in Full-term infant <12mg/dl (205.2 umol/L) Peak bilirubin concentration in Premature infant <15mg/dl (257umol/L)
Clinical jaundice is resolved by 2 weeks in the term infant by 3-4 weeks in the Preterm infant.
8
and the lifespan of an RBC is shorter in neonates ( 80 days). Increased RBCs and a shorter lifespan leads to increased destruction of RBCs, which leads to more bilirubin in the blood, which leads to hyperbilirubinemia
hyperbilirubinemia due
to: Immature hepatic system Delayed enteral feedings (Hypoglycaemia ) Decrease in serum albumin levels Hypoxia /asphyxia, Hypothermia
Breastfeeding Jaundice
Due to insufficient milk production by
mom or intake by baby; Supplemental water or dextrose-water administration may decreases breast milk production Decreased volume and frequency of feedings Decrease gut motility & delayed stooling
per day Formula supplement if infant has decline in weight gain, delayed stooling, and continued poor caloric intake Breastfeeding should be continued to maintain breast milk production Neutral thermal environment Prevent hypoglycaemia & hypoxia Avoid constipation Phototherapy for bilirubin 17-22mg/dl
BreastMILK Jaundice
Occurs later in life (Day 6 to 14) Bili 12 to 20 May stay high for 1-2 months Etiology: Unclear; Substances in maternal milk,
such as alpha glucuronidases, and nonesterified fatty acids, may inhibit normal bilirubin metabolism
Management
may be temporarily interrupted -With formula substitution, the total serum bilirubin level should decline rapidly over 48 hours,confirming the diagnosis
-Breastfeeding
Pathologic Jaundice
Anything OTHER THAN physiologic or
breastfeeding or breastmilk jaundice!! Jaundice within 24 hours after birth Rapidly rising total serum bilirubin concentration level higher than 17 in a fullterm newborn Other features of concern include prolonged jaundice Evidence of underlying illness Elevation of the serum conjugated bilirubin level to >2 mg per dL or more than 20% of the total serum bilirubin
Rh hemolytic disease
People who are Rh+ have the Rh antigen on their RBCs. People who are Rh- do not. If blood that is Rh+ enters the body of a person who is Rh-, the body reacts as it would to any foreign substance, and develops antibodies that destroy the invading antigen (this is called sensitization). Rh incompatibility is when the mother lacks the Rh factor on the surface of her red blood cells and her baby is born with the Rh factor on his or her red blood cells It does not occur with the first born child Increased destruction of red blood cells leads to increased bilirubin in the blood; therefore, leading to hyperbilirubinemia
Clinical Presentation
Varies from mild jaundice and
anemia to hydrops fetalis Erythroblastosis fetalis: This occurs as the baby's organs are unable to handle the anemia. The heart begins to fail and large amounts of fluid build up in the baby's tissues and organs. A fetus with hydrops is at great risk of being stillborn. RBC destruction and anemia cause bone marrow to release erythroblasts, hence the name erythroblastosis fetalis)
Postnatal problems include: Pulmonary hypertension Pallor (due to anemia) Edema (hydrops, due to low serum albumin) Respiratory distress Coagulopathies ( platelets & clotting factors) Jaundice Kernicterus (from hyperbilirubinemia) Hypoglycemia (due to hyperinsulinemnia from islet cell hyperplasia)
globulin which is given to pregnant women at 28 weeks of pregnancy and within 72 hours of delivering an infant who is born Rh positive This injection prevents the mothers body from forming antibodies against the Rh factor found on fetal red blood cells If the mother is already sensitized, meaning her body has already made antibodies against the Rh factor, the injection will be ineffective
18
however, it is more common if the mother has type O blood and the infant has blood type A or B Fetal cells cross the placenta and enter the mothers bloodstream When this occurs the mothers body forms antibodies against the fetal cells Those antibodies are then small enough to cross back through the placenta into the babys circulation and cause destruction of red blood cells Increased destruction of red blood cells leads to increased bilirubin in the blood; therefore, leading to hyperbilirubinemia
19
Clinical Presentation
The first fetus can be affected.
IgG are the only antibodies that can
cross the placental barrier, and usually most of the antibodies formed are IgM, so this condition is usually much milder than the Rh issue. When delivered, infants may present with mild anemia or normal hemoglobin levels
Rh
Unusual
Marked Marked
ABO
Common
Minimal Minimal to moderate
Hydrops
Hepatosplenomegaly Kernicterus
Common
Marked Common
Rare
Minimal Rare
Rh negative
Rh positive Positive
O
A or B Negative
Positive
marked
Usually positive
Variable
Coombs test
Negative -Cephalhematoma -Breast milk jaundice -Infection -Asphyxia -RDS -Galactosemia -Rare disorders 23
the amount of maternal antibody coating the infants red blood cell If the antibody is present, the test is positive
sample of the infants serum (which is thought to contain maternal antibodies) on unrelated adult RBCs If the infants serum contains antibodies, they will interact with and coat these adult RBCs (positive test)
oxidation/reduction reaction. Enzyme to maintenance of RBC life) Oxidation is the loss of electrons and reduction is the gain of electrons which leads to hemolysis of red blood cells Increased hemolysis of red blood cells leads to increased levels of bilirubin, which then leads to hyperbilirubinemia
26
Clinical Manifestations
Usually no evidence of hemolysis is apparent until 4896 hours after the patient has ingested a substance which has oxidant properties (Antipyretic,Sulfonamide, Anti malarial producing an acute and severe hemolytic syndrome called FAVISM, Hb level become very low, and increased reticulocyte count - Jaundice - Anemia - Hydrops - Massive enlargement of the liver & spleen
- Bilirubin encephalopathy (Kernicterus)
Kernicterus
Kernicterus is a rare, irreversible
complication of hyperbilirubinemia If bilirubin levels become markedly elevated, the unconjugated bilirubin may cross into the blood brain barrier and stain the brain tissues If staining of the brain tissues occurs there is permanent injury sustained to areas of the brain which leads to neurological damage
28
Complication
Kernicterus:
Phase 1 ( 3-4 day): Hypotonia Decreased alertness; Poor feeding Diminished Moro reflex High pitched cry Phase 2 ( 1 wk): Hypertonia, Irritability or Seizures; muscle spasms & back arching Phase 3 ( by 3 yrs of age): Hypotonia
G 6 P D deficiency
Diagnosis Low G6PD activity in red blood cells (normal range, 4.613.5 U/g Hb). Treatment When hemolysis has occurred => Red blood cell transfusion Prevention Avoiding oxidant substances
Jaundice-Diagnosis
Visual assessment (least reliable) Check bilirubin level: Total bilirubin at birth is less than 3mg/dL.
Not visible if bili <4 Visual assessment of jaundice is most accurate when the infants skin is blanched with light digital pressure in a well-lit room As bilirubin levels rise, the accuracy of visual assessment decreases
32
Treatments
Intrauterine transfusion Early Delivery
If labor is induced, fetal lung maturity must be determined using the lecithin/sphingomyelon (L/S) ratio to avoid respiratory distress syndrome Encourage frequent feedings Intravenous hydration Intravenous immune globulin (IVIG) has been used to decrease bilirubin levels due to hemolytic anemia; 1 gm/kg inhibits AB that cause RBC destruction Phototherapy Exchange transfusion
Maisels Chart
Sr Bilirubin (mg/dl) Birth weight Age in hrs
< 24
24 48
49 72
>72
<5 5-9
10-14
> 2500g
15-19 > 20
< 2500g
> 2500g All
Phototherapy
The therapy uses a blue light (420-470 nm) that converts bilirubin
Phototherapy- Cont.
May be provided using lightweight, fiber-optic
blankets ( bili blankets). The baby is wrapped in the blanket. The eyes are not covered.
A combination of a fiber-optic light source in
the mattress under the baby and a standard phototherapy light source above
Nursing diagnoses:
Body temperature, risk for imbalanced
phototherapy.
Family processes, interrupted, related to
39
Jaundice
Nursing Assessment and Diagnosis Identify the newborn at risk: Nursing Plan and Implementation Monitor temperature for hyperthermia or hypothermia. Apply eye patches over newborns closed eyes. Turn off lights when drawing blood for repeat bilirubin. Maintaining a neutral thermal environment. Observe for signs of dehydration and perineal excoriation. Weigh daily
related to parenting a newborn with jaundice. Risk for Injury related to use of phototherapy. Fluid Volume Deficit related to increased insensible water loss and frequent loose stools.
40
while the blood is drawn. Cluster care activities Discontinue phototherapy & remove eye patches at least once per shift. Also discontinue phototherapy and remove patches when 41 feeding the infant & when the parents visit.
Nursing consideration
Accurate charting is important nursing responsibility and
includes: 1. Times that phototherapy is started and stopped. 2. Proper shielding of the eyes. 3. Type of fluorescent lamp. 4. Number of lamps. 5. Distance between surface of lamps and infant 6. Use of phototherapy in combination with an incubator or open bassinet. Occurrence of side effects .as: loose, greenish stools, transient skin rashes, hyperthermia, increase metabolic rate, dehydration, electrolyte disturbances as hypocalcemia, Oxidize essential fatty acids, decreases vitamins and calcium in premature infants lead to adverse effect on cell 42 growth Tanning/Bronze Baby Syndrome
Phototherapy Precautions
Ensure patent airway
Maintain constant body temperature by using incubator Maintain fluid balance by increasing intake and minimizing loss
frequent diaper changes ( perianal excoriation due to irritating effect of diarrhea stools). water baths no lotions or oils on skin position to avoid skin irritation Careful technique when repeatedly drawing labs Warm foot before procedure 43 Avoid areas of previous puncture
procedure in babies with, or who are likely to develop, neurotoxic levels of bilirubin. Historically it was one of the most common procedures performed in neonatal services. Three factors have led to exchange transfusions becoming less commonly performed, namely: 1. The effective prevention of rhesus iso-immunisation with prophylactic Anti D for rhesus negative mothers; 2. Foetal intrauterine transfusions; and, 3. The recognition that well babies born at term with physiological jaundice can tolerate higher levels of bilirubin than previously thought
Exchange Transfusion
An exchange transfusion is used only in extreme
cases when phototherapy has failed The process for an exchange transfusion involves small amounts of blood being removed from the infant and then replaced with the same amount of donor RBCs and plasma The exchange replaces ~ 87% of the circulating blood volume and decreases the bilirubin level by ~ 55% Mechanism: removes bilirubin and antibodies from circulation and correct anemia
45
suction airway prior Informed consent signed by parents Check blood typing Restrain infant & Place under radiant warmer Exchange transfusions are performed using a one catheter or two catheter push-pull method under a septic technique N.B: Blood Volume = 70-90 ml/kg for term and 85-110 ml/kg for preterm infants
46
In Umbilical vein
OR
OR OR
Umbilical vein
Peripheral vein Peripheral vein
Umbilical artery
Peripheral artery Umbilical artery
venous catheter. Exceptionally, an umbilical artery catheter can be used. Ideally, the tip of the UVC should be in the IVC/right atrium (at or just above the diaphragm), position should be checked by an X-ray. Catheter is usually removed after each exchange. Withdraw blood over 2 minutes, infuse slightly faster.
Irradiated
Fresh Whole Blood (to avoid Ca++),
Infarction or arrythmias
Infection Death Thrombocytopenia
50
Transfusion Reactions
Rare in neonates
Signs & symptoms in neonates Temperature instability Reaction at infusion site, flushing of skin, urticaria, rash Vomiting or diarrhea Wheezing or acute respiratory distress Hypotension, shock, tachycardia Sudden rise in bilirubin Acute bleeding from venipunctures or surgical sites
Transfusion Reactions
What to do if you suspect a transfusion
reaction
Stop the transfusion immediately Begin treatment for any new, emergent problems Notify the physician Notify the blood bank Blood bank may request a fresh blood sample