CASE PRESENTATION

BY DR ABIYYA JAHANGIR
HO(PAEDS 2)
 PRESENTING COMPLAINTS

• 22 day old male twin 1 born via csection at 34 weeks

gestation, wt 1.2kg pre term presented with complaints of
• jaundice since 1st DOL
• Loose motions 1 day
• shallow breathing 1 day
 HOPC
• According to the mother baby delivered via csection at 34
weeks of gestation, cried immediately, kept in NICU for 30
hrs for observation after which they were handed over to
the parents. Since 1st DOL baby developed jaundice
initially noticed in eyes then gradually progressed to
involve whole body. mother ws giving the neonate sun
bath only for jaundice.No history of reluctance to feed.
Since afternoon he developed loose motion,5-6 times
yellow in color. No blood or mucus in stool. He also
developed shallow breathing. When the condition did not
improve he was brought in CHK.
 BIRTH HISTORY

• It was a booked case

• mother had regular hospital visits and regular u/s were
done.
• No history of PIH, GDM, maternal fever or any vaginal
discharge.
• History of PROM due to which c-sec was performed.
 NATAL HISTORY

• Baby born via csection due to PROM, at 34 weeks

gestation.
• Baby cried immeditely after birth.
• birth weight 1.7 kg
 POSTNATAL HISTORY

• No history of cyanosis after birth.

• No history of respiratory distress
• No history of head trauma at birth.
• History of jaundice since day 1 of life.
 FEEDING HISTORY

• Initially given meig-3 in 30 ml water and 2 scoops of milk.

• DMF started after 2 days,3-4 times per day, frequently
bottlefed.
 FAMILY HISTORY

No history of any misscarriage, non consanginos marriage.

 SOCIOECNOMIC HISTORY

• Father works in zong company, ecnomic conditon

average.
 EXAMINATION

vitals: Anthroprometry:
• HR 168 weight: 1.2 kg below 3rd
• R/R 40 br/m percentile
• TEMP 37.6 length: 42 cm below 3rd
• CRT > 3 SEC OFC: 30 cm below 3rd
• PULSES PALPABLE. percentile
• rbs 80mg/dl

• -, -
A J +++
,D ,E ,Cy
++ -
 HEAD TO TOE EXAMINATION

• no obvious dysmorphism visible

• Anterior frontanelle open and flat
• wasted appearence.
• back and spine normal.
• fully deeply jaundiced
• male gentalia seen
• dry pale lips
• oral thrush.
• patent anal and urethral opening.
 SYSTEMIC EXAMINATION
• CNS:
• tone inceased
• moro : incomplete
• sucking : poor
• grasping : poor
• pupils: bilaterally reactive.
• CVS.
• S1+ S2+0
• normal chest with no
visible pulsations
• apex beat in 5th
intercostal space at
midclavicular line.
• central and peripheral
pulses palpable.
 SYSTEMIC EXAMINATION

• RESP:
• bilateral equal air entry.
• harsh vesicular breathing
• no added sounds
• ABDOMEN
• normal shape,with no
distention, soft
nontender, no
viseromegaly, gut
sounds audible. skin
pinch was delayed.
• after arrival to the hospital baby threw a fit which lasted
for few seconds with starring gaze and tonic posture. fits
were aborted with inj phenytoin 30 mg I/V loading dose.
 CASE SUMMARY

• 22 day old male twin delivered via csection at 34 weeks

gestation, presented to us with jaundice since 1st DOL,
loose motions and shallow breathing since 1 day.
• On examination child was sick looking , deeply jaundiced
and dehydrated also actively throwing a fit.
 DIFFRENTIAL DIAGNOSIS

presumed sepsis with kernicterus sec to

hemolytic anemia (abo incompatibility, rh incompability,
g6pd deficiency)
TORCH infection
congenital hypothyroism
 INITIAL MANAGEMENT

• 1 liter O2 via nasal prongs @ 4L/min

• NPO
• Inj R/L 105ml I/V over 6 hrs @ 17 ml/hr
• Inj N/S 0.9% 20 ml/kg I/V bolus
• Inj 10% D/W 6 ml/kg I/V
• Inj phenytoin 30 mg I/V loading dose
• cbc, crp, uce, btd, bcs, blood group were sent.
 LABS
DATE 4/1 6/1 7/1 8/1 9/1 10/1 12/1 14/1 17/1 18/1
HB 8.3 10.4 12.1 9.2 10.1 10.1 8.5 7.9
MCV 92.0 75.1 72.7 71.9 68.6 73.4 73.5 73
HCT 24.0 31.4 36.3 28.1 29.30 30.3 26.6 24
TLC 17.4 15.0 13.5 13.0 17.5 8.4 73.5 8.1
NEU 32 34.7 48.6 49.7 56 31 0 30
LYM 65 34 27 35 32 56 0 69
PLT 527 285 223 409 351 114 96 116
UREA 53 63 50 42 15 8 6 3
CRE 1.5 2.4 1.4 1.0 0.4 0.3 0.3 0.2
NA 138 129 127 129 131 130 128 137
K 2.1 2.3 3.1 2.6 2.6 2.4 4.1 3.8
CL 98 94 94 93 96 95 94 103
CAL 9.9 10.4 10.2
MAG 2.6 2.7 2.1
PHO 5.0 4.5 4.9
DATE 4/1 6/1 7/1 8/1 9/1 10/1 12/1 14/1 18/1

ALB 3.5 3.6 3.6

GLO 1.0 1.6

A/G RATIO 3.46 2.19

T. BILI 44.3 35.0 26.1 21.1 13.7 23.3 15 6.7

B.DIRECT 24.7 24.8 19.3 18.4 11.2 18.1 12.5 5.6

B.INDIREC 19.6 10.2 6.8 1.1

T
SGPT 36

ALP 232

PT 11.8

INT 1.13

APTT 19.5

CRP 45.0 40.4 45.4 16.0 10.1 149.8 119.1 116

 INVESTIGATION
• retic count high
• COOMBS TEST : NEGATIVE
• G6PD : DEFICIENT
• BBG A+ve
• MBG B+ve
• Free T3 2.8
• Free T4 0.92
• TSH decreased.
• peripheral film was sent but sample was hemolysed.
• TORCH profile was planned but not sent
• blood c/s no growth
 INVESTIGATIONS
• ULTRASOUND ABDOMEN:
• liver, pancreas, spleen normal.
• gallbladder normal
• no evidence of biliary atresia
• ULTRASOUND BRAIN;
• normal parenchyma
• no solid or cystic lesion seen
• no vntricular dilatation seen
• no midline shift seen
• no ventricular, parenchymal or choroid plexus
hemorrhage seen.
 HOSPITAL COURSE
• 4/1 presented as above and then was recieved in nicu due to shallow breathing, fits nd deep
jaundice. bilirubin came out to be 44.3 ( ernicterus was presumed to be the cause of fits and
irritability). hb was 8.3 so hemolytic workup was sent, coombs came out to be negative. g6pd
levels were sent which were deficient. inj meropenem 50 mg i/v started.
• 5/1 u/s abd done due to acholic stools and dark urine and came out to be normal. tsh levels
were dec but FT3 and FT4 were normal
• 6/1 inj colomycin was added with mero due to static crp
• 8/1. BTD dec to 2.1
• 10/1 o2 tappered to 0.5 lit/min, fluid stopped, DMF started.
• bilirubin dropped to 11. hb was 8.3 pcvs transfused.
• 11/1 shifted to paeds 2 nursery upon improving labs.
• 13/1 inj linzolid 15 mg was added due to rise in crp
• oral phenobrbitone 8 mg hs was added.
• baby was shifted to post nursery,upon completion of antibiotics he was discharged, with
proper counselling of parents done regarging prevention of further hemolytic episodes. They
were given the list of medication which were to be avoided throughout his life.
 G6PD DEFICIENCY ANEMIA
• G6PD deficiency is one the common causes of hemolytic
anemia.
• This disorder has x linked recessive inheritance.
Pathophysiology
G6pd deficient cells donot generate adequate amount of
glutathione→ red cells are not protected from oxidant
agents→exposed sulfhydyrl groups of hemoglobin are
oxidized→denaturation of hemoglobin→heme and globin
dissociate ,hemolysis occurs→globin precipitates into heinz
bodies→damaged cells removed by reticuloendothelial
system→severely damaged cells may lyse intravenously.
 AGENTS PRECIPITATING HEMOLYSIS IN G6PD
DEFICIENCY..
• MEDICATIONS: • CHEMICALS:
Sulfonamides Vit k analogs
dapsone methylene blue
probenecid
trimethoprim
nalidixic acid • ILLNESS
chloramphenicol diabetic acidosis
primaquine hepatitis
chloroquine sepsis
 CLINICAL FINDINGS

• Neonates with g6pd deficiency may have

hyperbilirubinemia.
• older children with g6pd deficiency are asymptomatic and
appear normal between episodes of hemolysis, the
triggering event is intake of antipyretics or antimalarials.
• about 24 hrs after ingestion of oxidant substance, pallor,
jaundice, hemoglobinemia and hemoglobinuria develops
and there is intavascular hemolysis.
• hemolytic episodes may be triggered by infections.
 INVESTIGATIONS

• Hemoglobin levels: reduced

• urine examination: hemoglobinuria
• Bilirubin levels: raised
• Perpheral blood smear: heinz bodies
• DIAGNOSIS:
• based on history of previous episode, family history.
• Demonstration of low g6pd levels.
 MANAGEMENT

• Prevention of hemolysis by avoiding and discontinuing

oxidant drugs.
• infections are treated and antiobiotics are given.
• most episodes of hemolysis are self limiting
• after hemolysis has occured only supportive therapy has
occured.
• Blood transfusion in case of severe hemolysis.