3. HISTORYy
Eight days old Male baby, Term,appropriate for gestational age, weighing
2.5 kg delivered via Normal veginal delivery at home by trained Dai
brought to emergency department on 7th day of life with complain of:
YELLOW DISCOLORATION OF WHOLE BODY
SINCE 4 DAYS.
4. HISTORY OF PRESENTING COMPLAINS
He was alright 4 days back then mother noticed yellow
discoloration of face which was progressively increase
involve whole body upto the feets within 4 days.Jaundice
was not associated with hematemesis or melena or any
petechial rash on the body.
5. BIRTH HISTORY
1) ANTENATAL HISTORY:
•Not booked case in any hospital.
• she did’nt take iron,multivitamins or any other drugs.
•No history of Pregnancy induced hypertension, maternal fever,
gestational diabetes mellitus, rash or cholestasis, Antepartum
hemorrhage or infections.
6. 2) NATAL HISTORY:
•Delivery was conducted at home by Dai.
•No History of prolonged rupture of membrane.
•Spontaneous vaginal delivery.
•No history of sedation or analgesia given to the mother
during labor and any abnormal bleeding.
7. 3) POST NATAL HISTORY:
•Baby cried immediately after birth.
•No history of cyanosis and apneic spells.
•No history of any convulsions,fever, rash after birth.
• History of jaundice on 3rd day of life after birth observed
by mother.
8. FEEDING HISTORY
Mother feeding had started soon after birth.
No formula milk given to baby.
No any iron or multivitamin supplement given to baby.
Currently baby is on direct mother feed.
9. FAMILY HISTORY
Father’s Age: 35 years
Mother’s Age: 25 years
He is the 6th product of non-consanguineous marriage.
History of 3 siblings died.
History of jaundice in one sibling.
No family History of bleeding disorder or blood transfusion,
hypertension ,diabetes mellitus, tuberculosis, thalassemia and
bleeding disorder.
10. FAMILY TREE
Died due Died due Died due Hx of Normal Patient
to B.A to B.A to B.A jaundice
12. EXAMINATION
GENERAL PHYSICAL EXAMINATION
Pale looking and deeply jaundiced 8 days old male neonate with no
dysmorphic features or signs of respiratory distress having the cannula on
the left hand.
VITALS: SUB VITALS
• R/R: 42 breaths/minute Anemia(+)
• H/R: 135 beats/minute Jaundice(+++)
• Temperature: 98.6F
• B.P: 65 mmhg (systolic on 50th percentile)
50 mmhg (diastolic on < 50th percentile)
• RBS: 89 mg/dl
• SO2: 96% at room air
14. HEAD TO TOE EXAMINATION
Deeply jaundiced from face up to the sole of both
feets.
Rest of general and systemic examination were
unremarkable.
15. CASE SUMARY
Eight days old male baby,Term,appropriate for gestational
age, weighing 2.5 kg, presented with yellow discoloration
of whole body for four days. Product of non-consangineous
marriage, delivered full Term Via normal veginal delivery
at home.
On Examination,baby was pale looking, having no any
dysmorphic features or signs of respiratoy distress. Baby
was vitally stable.
Primitive reflexes were good.
25. MANAGEMENT ON 1ST DAY OF ADMISSION
AND DURING HOSPITAL STAY
SUPPORTIVE THERAPY SPECIFIC THERAPY
Single phototherapy Injection cefotaxime 125mg IV B.D
PCV Transfused 25 ml IV injection Amikacin 20 mg IV B.D
Direct mother feed on demand
29. DEFINATION
Neonatal hyperbilirubinemia is a conndition characterized by an
excessive concentration of bilirubin in the blood.
Hyperbilirubinemia is defined as a total serum bilirubin level greater
than 1.5mg/dl(26 umol/L).
Jaundice becomes evident when total serum bilirubin level reaches
5mg/dl (86 umol/L).
Jaundice usually begins on the face and progresses in a
cephalocaudate fashion, for unknown reasons.
Jaundice is observed during the 1st week after birth in approximately
60% of term infants and 80% of preterm infants.
31. CLASSIFICATION
Hyperbilirubinemia is classfied as:
1) Unconjugated or indirect:
• which can be physiological or pathological in origin.
1) Conjugated or direct:
• which is always due to pathological causes.
32. WORKUP NEONATES WITH JAUNDICE
HISTORY
How old is the infant ?
Is the infant being breast-fed ?
What is the gestational age of the infant ?
Ask about jaundice in previous siblings?
Is there family history of significat hemolytic disease?
Is the urine dark or what color is the stool?
Is the infant lethargic or irritable?
I s the infant receiving total parenteral nutrition?
Is the infant gaining weight?
Is there consanguinity?
Is the infant being treated for jaundice for another condition and not improving?
33. PHYSICAL EXAMINATION
Vital signs,weight assessment,general assessment of nutrition and
observation for any signs of sepsis.
Check for cephalhematoma/caput saccudum/ sub-aponeurotic
hemmorhage,bruises and petechiae on the skin.
Attension should be given to examination of the abdomen. Is it
distended? Palpate for enlarged liver or spleen or for any masses.
34. CLINICAL ASSESSMENT OF JAUNDICE
Total bilirubin level roughly correlates with progression of jaundice.
FACE = 4-8mg/dl (68-137 umol/L)
UPPER TRUNK = 5-12 mg/dl (86-205 umol/L)
LOWER TRUNK = 8-16 mg/dl (137-274 umol/L)
SOLES OF THE FEET = >16 mg/dl (>257 umol/L)
35.
36. GLUCOSE-6-PHOSPHATE DEHYDROGENASE
(G6PD) DEFICIENCY
Glucose-6-phophate dehydrogenase (G6PD) is the most
frequent disease involving enzymes of the hexose
monophosphate pathway.
The disorder has X-linked recessive inheritance
The deficiency is caused by inheritance of any of a large
number of abnormal alleles of the gene responsible for
synthesis of the G6PD protein.
39. Glucose-6-phosphate dehydrogenase (G6PD) Deficiency responsible
for two clinical syndromes:
1)Episodic or induced Acute hemolytic Anemia:
Acute condition is common and is manifested by hemolytic episodes
which are induced by infections or some drugs.
2)Chronic nonspherocytic hemolytic anemia:
chronic condition is rare and has been associated with profound
deficiency of G6PD caused by enzymes variants,particularly those
defective in quantity,activity,or stability.
40.
41. CLINICAL MANIFESTATIONS
Most individuals with Glucose-6-phosphate dehydrogenase (G6PD)
deficiency are asymptomatic, with no clinical manifestations of illness
unless triggered by infection, drugs or ingestion of fava beans.
Typically hemolysis ensues in about 24-48 hr after a patient has
ingested a substance with oxidant properties.
In severe cases hemoglobinuria and jaundice result and the
hemoglobin concentration may fall precipitously.
The degree of hemolysis varies with inciting agent,amount ingested
and severity of enzyme deficiency.
42. INVESTIGATIONS
HEMOGLOBIN LEVEL is reduced ( may be as low as 2-5g/dl).
URINE EXAMINATION reveals hemoglobinuria.
BILIRUBIN LEVEL is raised.
PERIPHERAL BLOOD SMEAR may reveal Heinz bodies in the first 3-4
days of illness because they are rapidly cleared from blood.
Also,Blood film may contain red cells with what appears to be bite
taken from their periphery (‘‘bite cells’’) and polychromasia( evidence
of bluish, larger RBCs), representing reticulocytosis.
44. DIAGNOSIS
The diagnosis depends on direct or indirect demonstration of reduced
G6PD activity in RBCs.
By direct measurement enzyme activity in affected persons is < 10% of
normal.
Immediate after hemolysis the level may be normal, because presence of
young red cells and reticulocytes, which have normal G6PD activity. Re-
testing in a few weeks may confirm the diagnosis.
Satisfactory screening test are based on decoloration of methylene blue,
reduction of methmoglobin, or flourescence of NADPH.
The diagnosis can be suspected when G6PD activity is within the low
normal range in the presence of a high reticulocyte count.
G6PD variants can also be detected by electrophoretic and molecular
analysis.
G6PD should be considered in any neonatal patients with
hyperbilirubinemia and borderline-low G6PD activity.
45. PREVENTION AND TREATMENT
Prevention of hemolysis by avoiding or discontinuing
oxidant drugs.
Infections are treated and antibiotics are given when
appropriate.
Most episodes of hemolysis are self-limiting.
After hemolysis has occurred,supportive therapy is given.
Blood transfusion in case of severe hemolysis.