Neonatology😊

Important terminologies

Baby born at

  • < 37 completed weeks of gestation: Preterm neonates
  • 37-42 weeks of gestation: Term neonates
  • > 42 weeks of gestation: Post term/mature neonates

Birthweight

  • <2500g:
    • Low birth weight (LBW)
    • M/c cause Prematurity > IUGR
    • Average birth weight (India): 2.8-2.9 Kg.
  • <1500g:
    • Very low birth weight (VLBW)
  • <1000g:
    • Extremely low birth weight (ELBW)

If birth weight is gestational age

  • 10th - 90th percentile of expected
    • Appropriate for GA
  • < 10th percentile of expected:
    • Small for date (SFD) or Small for gestational age (SGA)
  • > 90th percentile of expected:
    • Large for date (LFD) or Large for gestational age (LGA)
      • LFD is seen in:
        • Infant of diabetic mother,
        • congenital hypothyroidism,
        • Beckwith-wiedmann syndrome
        • Soto’s syndrome

Normal term neonate

  • Heart rate: 120-140 bpm
  • Respiratory rate: 40-60/min
  • Peripheral/ Acrocyanosis:
    • Commonly present at birth
  • Short systolic murmur:
    • Normally present,
      • pulmonary vascular resistance is ↓↓
        • sudden increase in the pulmonary blood flow

High-Risk Neonates (AIIMS Protocol)

  1. Birth weight < 1.5 kg Very Low Birth Weight (VLBW)
  1. Preterm < 32 weeks
  1. Persistent or recurrent hypoglycemia
  1. Born to HIV-positive mother

TORCH

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TOXOPLASMOSIS

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  • Primary toxoplasma infection during pregnancy.
    • Severity → more if affected earlier (11 weeks)
  • Mnemonic: Toxic killing → Kanni kude (chorioretinitis) thalayilott (cerebral calcification) inject cheyth (↑ size → hydrocephalus)
  • Definitive host: Cat.
  • Intermediate host: Human.

Mode of transmission

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  • Ingestion
    • Sporulated oocyst via contaminated soil/food/water.
    • Bradyzoites via undercooked meat.
  • Blood transfusion & Vertical
    • Tachyzoites.
  • Blood and sex - tachy
  • Relax (Brady) and eat meat
    • Beef - brady
  • spores from soil
  • Cat → Oocyst
    • Infective Stage.
    • Diagnostic Stage.
  • Diagnosis
    • Serological diagnosis.
    • Direct identification of parasite from:
      • Peripheral blood.
      • Amniotic fluid.
      • Tissue sections.

Variants of toxoplasmosis

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  • Adult toxoplasmosis
    • Asymptomatic.
    • LN enlarged.
  • HIV+ toxoplasmosis
    • Encephalitis.
    • Most commonly brainstem is affected.
  • Congenital Toxoplasmosis
    • More severe in trimester I.
    • More common in trimester III.
      • Chorioretinitis.
      • Intracerebral calcification.
      • Convulsions.
      • Microcephaly
      • Mental retardation.
      • notion image
      • Mnemonic: CHC
      • Small for gestational age.
      • Prematurity.
      • Hydrops fetalis, i.e., generalized anasarca.
      • Persistence jaundice.
      • Thrombocytopenia.
      • Focal Chorioretinitis
        • Most common manifestation
      • HYDROCEPHALUS.
        • Macrocephaly due to hydrocephalus.
      • Cerebral calcification
        • On NCCT

Important Information

  • Toxoplasma intracerebral calcification.
  • CMV periventricular calcification.

Diagnosis

  • Frenkel test
  • Sabin Feldman test: Gold standard.
    • Test uses Methylene blue and tachyzoite stage
    • If colorless - antibodies present.
    • If color is present - antibodies absent.
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Prevention and Treatment

  • Pyrimethamine + Sulfadiazine.
  • Early diagnosis of infection
  • Treatment of mothers is by sulphadiazine.

Congenital CMV Infection

  • Largest of Herpesviridae
  • SRK (salivary, resp, kidney) → Largest → lives in mannat (Monocytes) → big muscle fibre (human fibroblast culture)
  • Reservoir: Humans only
  • Transmission:
    • Common: oral, respiratory, body secretions
    • Less common: transplacental, sexual
  • Multiplication sites (mnemonic SRK in Mannat):
    • Salivary gland
    • Respiratory tract
    • Kidney
  • Latent in: Monocytes
  • M/c infection during 1st 4 months of organ transplant

Infections

Infections
Features
Congenital
M/c intrauterine infection
Defects:
mental retardation
microcephaly
periventricular calcification
chorioretinitis
Perinatal
usually asymptomatic
Immunocompromised
CMV chorioretinitis
Immunocompetent
IMN-like syndrome
Features: fever, sore throat
No lymphadenopathy, splenomegaly, or specific antibodies

Features`

  • 1st CMV infection30%.
  • 2nd CMV infection1-2% (Developing countries like India).
  • Congenital CMV
    • 90% ofasymptomatic
      • No use of routine screening.
    • Most important long-term sequelae in SYMPTOMATIC CMV
      • Sensorineural hearing loss.
      • Most common non syndromic causes of hearing loss.

Clinical features in Congenital CMV

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Urine PCR > Saliva
Urine PCR > Saliva
  • Microcephaly
    • < 3 SD of HC
  • Chorioretinitis.
  • Hepatosplenomegaly.
  • Jaundice.
  • Petechiae.
  • Periventricular calcifications.

Congenital Varicella

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Teratogenic period:

  • Infected in 1st trimester
    • 0.4% of babies
  • 2nd trimester → 12-20 weeks (after 3 months till 5 months end)
    • 2% of babies
    • Congenital / Fetal Varicella Syndrome
      • Indication for MTP

Clinical features

  • Cicatricial scarring in zoster like or
    • Dermatomal distribution around umbilicus.
  • Limb hypoplasia.
  • Low birth weight.
  • CNS involvement:
    • Microcephaly,
    • developmental delay,
    • intellectual disability,
    • seizures.
  • Eye involvement:
    • Chorioretinitis,
    • cataract,
    • microphthalmia (small eye).
  • Renal involvement:
    • Hydronephrosis.
  • Autonomic dysfunction:
    • Swallowing dysfunction,
    • neurogenic bladder.

Diagnosis of congenital varicella infection

  • history of varicella during pregnancy.
  • clinical features in a baby.
  • Anti-varicella IgM in a baby.

Neonatal Varicella Syndrome

  • Pregnant female acquires infection 5 days before to 2 days after delivery
  • Features
    • Hepatitis
    • Pneumonia
    • Skin rash
    • Meningoencephalitis
  • Treatment :
    • Give VZIG
    • IV Acyclovir to child
  • For mother :
    • Oral Acyclovir

Congenital rubella syndrome.

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  • Risk of transmission ↑↑↑ → if earliest before 11 weeks of gestation
  • Least r/o perinatal transmission
  • Virus excretion → saliva, urine
  • Mnemonic : Rubee → Blue bee( Blueberry rash)
    • Sitting in salt and pepper (Salt & Pepper Fundus)
    • Shiny Pearly eyes → Nuclear pearly cataract
    • Can't see (Small eyes) can't hear (SNHL) also had some heart disease.
    • adich Padam (PDA) aakki
  • Triad of Gregg
    • PDA
    • Cataract
    • SNHL

Complications

  1. Congenital heart diseases:
      • Most common: Patent Ductus Arteriosus > PS
      • Least common: Atrial Septal Defect.
  1. Sensorineural hearing loss.
  1. Blueberry muffin lesion:
      • Bluish red nodular lesions
      • characteristic of congenital rubella.
  1. Microcephaly, IUGR.
  1. Glaucoma.
  1. Hepatosplenomegaly.
  1. Jaundice.
  1. Thrombocytopenia.

Key Ocular Manifestations:

  • Congenital nuclear cataract
    • Nuclear Pearly Cataract
    • Most common cataract in CRS
  • Micro-ophthalmos
    • abnormally small eyes
  • Salt & Pepper Retinopathy

NOTE:

  • Salt & Pepper Fundus
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    • Mnemonic: Salt and pepper movie
      • Ruby (Rubella) → a Star (Stargardts)
      • Rough (Refsum) Labor (Lebers) who has Syphillis (CS) Raped (RP → Retinitis Pigmentosa) her → got Rid (Thioridazine) of body
      • Thio Ridazine :
        • Thio Ridazine → rid of
        • Brown (Brown vision) Cuteee (QT prolongation) with colorful eyes (RP),
          • like Salt and Pepper () movie
        • but ejaculated retrograde ()
  • Diagnosed by:
    • Presence of IgM rubella antibodies in the infant shortly after birth
      (since IgM does not cross the placenta)
    • Persistence of IgG antibodies for >6 months
      (maternally derived antibody would have disappeared)

Zika

  • Vector borne teratogen (Aedes mosquito)
  • Associated with receptors:
    • TIM 1
    • TAM-XL
  • Features:
    • Microcephaly
    • Increased limb tone
    • Club foot
  • Chicken Tikka (Zika) from Zam Zam (TIM TAM) Club (Club foot)
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Assessment of gestational age of a neonate

Expanded new Ballard score (ENBS)

  • Between 20-44 weeks
    • Intrauterine
  • Parameters of physical maturity and neurological maturity
    • Weeks
      Score
      20
      -10
      22
      -5
      24
      0
      26
      5
      28
      10
      30
      15
      32
      20
      34
      25
      36
      30
      38
      35
      40
      40
      42
      45
      44
      50

Neurological maturity

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Upper limb

  • Square window
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  • Scarf sign
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Lower limb

  • Popliteal angle
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  • Heel to ear
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Signs of physical maturity in a preterm neonate

General

  • Small/emaciated
  • Skin
    • thin, pink and fragile
    • more chances of infections and neonatal sepsis
  • Lesser subcutaneous fat
    • more chances of developing hypothermia
  • Generalized hypotonia
    • extended posture
  • Abundant lanugo
    • but little vernix caseosa
      • Vernix caseosa
        Vernix caseosa

Head to toe

  • Head appears relatively large
  • Anterior fontanelle
    • diamond shaped area on the skull
    • large and wide open
      • other causes:
        • rickets,
        • cleidocranial dysostosis,
        • osteogenesis imperfecta,
        • congenital infections,
        • hydrocephalus
  • Ear cartilage is poorly formed
    • ear recoil is poor
  • Breast buds <5mm
  • Male:
    • Undescended testis,
    • poorly formed smooth scrotum
  • Female:
    • Labia majora is widely separated,
    • labia minora is clearly visible
  • Absent deep creases on the sole

Meconium

  • First stool of a newborn
  • Color: Greenish-black
  • Composition:
    • Amniotic fluid
    • Mucus
    • Intestinal epithelial cells
    • Bile
  • Color:
    • Due to combined components of meconium
    • Not primarily from bilirubin or biliverdin

Meconium ileus

Causes

  1. Prematurity
  1. Hypothyroidism
  1. Cystic Fibrosis
      • Present with other features of CF
      • Soap bubble appearance
      • NO AIR FLUID LEVEL (dry thick impacted meconium)
      • Bishop Koop surgery
      • Stippled calcification d/t inspissated stools
  1. Hirschsprung disease
      • present within 48hrs,
      • abdominal distension and bilious vomiting
      • On per rectal examination
        • On removal of finger
        • Sudden expulsion of meconium d/t transient dilatation
  1. Anorectal malformation
  1. Lazy Left colon syndrome
      • Infant of Diabetic mother
        • d/t ↓ gut motility → delayed passing of meconium

PASSING URINE AND MEOCONIUM

  • Meconium by 24 hours (9% pass by 48 hours)
  • Urine by 48 hours
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Umbilical cord:

  • 3 vessels
    • a. Right umbilical artery
    • b. Left umbilical artery
    • c. Left umbilical vein
  • Mnemonic: Are you Very Lost (RUV lost)
  • Presence of a single umbilical artery
    • A/w congenital anomalies
    • Like Renal agenesis
  • Normal average length is 50-60 cms
  • contains a gelatinous substance called Wharton's jelly
  • Shed off by 7 - 10 days

Hearing Test in Children

  • All newborns must be tested
    • 2 step screening process:
      • 1st: OAE testing
      • 2nd: ABR testing
  • Recommended techniques:
    • 1. Otoacoustic Emissions (OAE)
      • Detects conductive or cochlear hearing loss
      • Assesses pathway:
        • External earOuter hair cells of cochlea
    • 2. Auditory Brainstem Evoked Response (ABR/BERA)
      • More reliable test
      • Detects:
        • Conductive hearing loss
        • Cochlear/Sensorineural hearing loss
      • Assesses pathway:
        • External earBrainstem

Neonatal Hearing Screening Recommendations
(1–3–6 Rule)

  • By 1 month: Primary screening
  • By 3 months: Secondary testing
  • By 6 months: Interventions

Conditions in neonates not requiring any specific treatment

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Skin/mucosa

  • Erythema toxicum
    • Usually present in 1st week of life
    • Sterile eosinophil filled lesions
    • Reddish maculopapular lesions present mainly on the trunk
      • notion image
  • Milia:
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    • Colorless papules due to the plugging of sweat ducts
  • Mongolian spots:
    • Bluish black areas of discolouration
    • mainly on lower back, buttocks, back of thigh
  • Stork bites or Angel Kiss:
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    • Capillary hemangiomas
      • seen in between the brows, nape of the neck
  • Subconjunctival hemorrhage

Other conditions

  • Mastitis neonatorum:
    • B/l breast engorgement in both males and females
    • due to the effect of maternal hormones
  • Milk output can also be sometimes seen
  • Vaginal bleeding in female neonates:
    • Due to withdrawal of maternal hormones
  • Hymenal tags in females
  • Physiological phimosis in males
  • Physiological weight loss

Physiological weight loss

  • Term neonates:
    • loss upto 10% of their birth weight in 3-5 days
    • Regain by Day 10 of life
  • Preterm neonates:
    • Upto 15% of their birth weight in 7-10 days
    • Regain by Day 15 of life

Cephalhematoma Vs Caput Succedaneum

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Cephalhematoma
Cephalhematoma
Feature
Caput Succedaneum
Cephalhematoma
Collection
Fluid
Blood
Cause
Prolonged stagnation
of fetal head during labor
Traumatic instrumental delivery
Due to edema in the layers of scalp
Subperiosteal hemorrhage
involving cranial bones
Location
Above periosteum
(
can cross sutures/ midline)
Below periosteum
(
cannot cross suture line)
Pits on pressure
Yes
No
Associated with fracture
No
Yes
Appearance
Present at birth in its maximum size
24-48 hours to appear completely
Disappearance
Disappears in 48-72 hours
Take upto 5-7 weeks to disappear
Drainage is contraindicated
No neonatal jaundice
Predisposes to neonatal jaundice

Primitive neonatal reflexes

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Moro's reflex

  • Begins to appear: 28 weeks of gestation
  • Completely appears: 37 weeks of gestation
  • Disappears: 5-6 months post natally
    • if does not disappear beyond 6 months- Cerebral damage
  • Absent Moro's reflex:
    • Stage 3 HIE (Hypoxic ischaemic encephalopathy),
    • Down's syndrome
  • Asymmetric Moro's reflex
    • Erb's palsy
    • Congenital hemiplegia
    • Fracture of clavicle
    • Unilateral shoulder joint dislocation

Rooting reflex

  • Begins to appear: 32 weeks of gestation
  • Disappears: 1 month post natally

Suckling reflex

  • Begins to appear: 32 weeks of gestation
  • Disappears: 3 months post natally

Palmar grasp reflex

  • Begins to appear: 28 weeks of gestation
  • Disappears: 3 months post natally

Plantar grasp reflex

  • Begins to appear: 35 weeks of gestation
  • Disappears: 3-5 months post natally

Asymmetric tonic neck reflex

  • When head of the neonate is turned to one side,
    • the upper and lower limbs of
      • same side are extended
      • opposite side are flexed
  • Begins to appear: 35 weeks of gestation
  • Disappears: 3-5 months post natally

Appearing after birth

  • Baby Landed symmetrically in Parachute

STNR (Symmetric Tonic Neck Reflex)

  • Begins to appear: 4-6 months post natally
  • Disappears: 8-12 months

Landau reflex

  • Begins to appear: 4-6 months post natally
  • Disappears: 12-24 months

Parachute reflex

  • Begins to appear: 7-8 months post natally
  • Persists throughout life

Neonatal resuscitation

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Dysrhythmias
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  • Ideal temperature of delivery room:
    • 25°C - 28°C
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Suction during neonatal resuscitation

  • Suction of all babies after delivery is not required
    • only when there is a lot of secretions
  • Mouth 1st > then nose (to prevent aspiration)
  • NOT intratracheal / intrapartum suctioning

Routine care provided if

  • Term gestation
  • Good muscle tone
  • Normal breathing/ crying

Components

  • Provide warmth: Radiant warmer/ skin contact
  • Clear airway if required
  • Dry the baby
  • Ongoing evaluation- for respiratory distress

Indications of PPV during neonatal resuscitation

  • Apnea
  • Gasping
  • HR < 100bpm

Process

  • Given using a self inflating bag and mask
    • notion image
    • rate of 30-40 breaths/ min
  • +/- Oxygen
  • Reservoir:
    • Increases the FIO2
  • In term babies
    • start with room air (FIO2-21%)
  • In preterm babies
    • start with 30% oxygen using a blender

Absolute contraindications to bag and mask ventilation

  • TEF
  • CDH

Congenital Diaphragmatic Hernia

  • Absence of the pleuroperitoneal membrane.
  • Leads to persistence of the pleuroperitoneal canal (Bochdalek foramen)
  • M/c → Left > right
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  • Clinical Features:
    • Scaphoid abdomen with respiratory distress.
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Type
Morgagni Hernia
Bochodalek Hernia
Location
Right anteromedial/Retrosternal
Most common
Left posterolateral
Defect Development
Central tendon of diaphragm

D/t enlarged Space of Larry
(Contain Superior Epigastric Artery)
(space between sternum § costal origins of diaphragm.
Pleuroperitoneal canal/membrane
Herniating Structures
Transverse colon
Stomach, spleen, transverse colon
Mnemonic
Boche → CPM → Left
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Diagnosis:

  • Prenatal detection can be done.
    • scaphoid abdomen
  • Bowel gas shadows are present in the thorax
  • Diaphragmatic outline is not clearly visible
  • Heart shadow is not visualized due to mediastinal shift

Complications

  • 1st most common cause of death:
    • Pulmonary hypoplasia
      (due to reduced space for lung development)
      • scaphoid abdomen
      • respiratory distress and
      • features of mediastinal shift
  • 2nd most common cause of death:
    • Pulmonary hypertension (PPHN).
      • Managed with inhaled nitrates.

Management (Mx)

  • Best ventilation: IPPV (Intermittent Positive Pressure Ventilation).
  • ExUtero Intrapartum Treatment Procedure (EXIT)
    • Airway is ensured before the infant is separated from Placenta
    • Also done in Laryngeal atresia, Stenosis, Teratoma, Hygroma, Oral tumors
  • Resuscitation: 
    • with Bag and mask ventilation C/I
  • If there is severe respiratory distress
    • Intubation and bag and tube ventilation needs to be done
  • Surgical Management (Sx):
    • Circular incision around the diaphragm.
    • Bowel reduced back into abdominal cavity.
    • Mesh placed to reinforce the repair.
Congenital Pulmonary Airway Malformation → D/d for CDH
Congenital Pulmonary Airway MalformationD/d for CDH

Eventration of diaphragm

  • Similar to CDH but not a true hernia.
  • Thinning of pleuroperitoneal membrane
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      Congenital weakness in muscles of diaphragm
      Congenital weakness in muscles of diaphragm
Which of the following is the most reliable indication of successful ventilation effort in neonatal
resuscitation?
A. Colour change
B. Rise in heart rate
C. Air entry
D. Chest rise
ANS
Rise in heart rate

Chest compressions in neonatal resuscitation

  • Indication: HR < 60/min, despite effective PPV
  • Ratio
    • CC : PPV = 3 : 1
  • Site:
    • Lower 1/3rd of body of sternum
    • just below the midline line joining the 2 nipples
  • Use of O2:
    • 100% O2

Inj. Adrenaline

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  • Indication:
    • HR < 60/min
      • despite 60 seconds of chest compressions and PPV
  • Dose:
    • 0.02 mg/kg/dose or
    • 0.2 ml/kg/dose of 1:10,000
      • (0.1 mg/mL)
    • can be repeated 3 times
  • Preferred route:
    • IV through the Umbilical venous catheterisation (UVC)
    • Intratracheal (only if vascular access cannot be secured)
    • Mnemonic: LUV → Left umbilical vein (not right)

Resuscitation of a baby born through meconium stained liquor (MSL)

Latest recommendations

  • Continue resuscitation as per neonatal resuscitation protocol
  • Routine endotracheal intubation and tracheal suction
    • of all non-vigorous neonates is not required
  • At least 1 person skilled in endotracheal intubation should be available during resuscitation

Delayed cord clamping

  • For at least 30 sec in all stable term and preterm neonates

Advantages

  • Higher Hb level
  • Lesser need for blood transfusion
  • Lesser risk of hypotension
  • There is a slight higher risk of neonatal jaundice
    • but the advantages outweigh the risk

Essential Newborn care

  • Keep baby warm and dry
  • Resuscitation, if required
  • Care of eyes
    • two separate sterile gauze swiped from medial to lateral
  • Care of cord
    • use of sterile cord clamp and cord tie;
    • nothing to be applied to the umbilical cord
  • Initiation of breastfeeding

Feeding of preterm neonates

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Gestational age- recommended initial mode of feeding

  • <28 weeks:
    • IV fluids +/- TPN
  • 28-31 weeks:
    • Orogastric or Gavage feeding
  • 32-34 weeks:
    • Katori spoon or Paladai feeding
  • 34 weeks:
    • Direct breastfeeding

Feed for Term baby in first 24 hours after delivery

  • 60 ml/kg
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Diseases of the newborn

Neonatal sepsis

M.c. organism responsible for neonatal sepsis

  • In India: Acinetobacter (AIIMS answer) > Klebsiella
  • Early onset sepsis: Group B streptococcus (Agalactiae)
    • Meningitis
    • Pneumonia
    • Septicemia
  • Overall, throughout the world: Group B streptococcus
  • In hospitals across the world: E.coli

Ophthalmia Neonatorum

  • Onset & Cause:
    • Time of Onset
      Cause
      Notes
      Within first 6 hours
      Chemical conjunctivitis
      • Due to (silver nitrate)
      Rx: Eye lubricant
      24–48 hours
      Neisseria gonorrhoeae
      Most severe
      DOC: Ceftriaxone or cefotaxime
      Around 1 week
      Chlamydia trachomatis (D–K)
      Most common
      DOC: Oral erythromycin
  • Prevention:
    • Erythromycin eye ointment (first 2 hrs).
    • Crede’s method (obsolete; used silver nitrate).
  • Ophthalmia Neonatorum Child → (Neisseria Chalmydia)

Meningitis cause in Neonates

  • Escherichia coli (most common)
    • 0 - 2 months
  • Group B Streptococcus (most common)
    • > 2 months
  • Listeria monocytogenes
    • PALCAM media,
    • tumbling motility
    • treat with ampicillin

Meningitis cause in Children, Adults, and Elderly

  • Streptococcus pneumoniae (most common)
  • Neisseria meningitidis (meningococcus)
  • Haemophilus influenzae type B (Hib)
    • risk of deafness if no Hib vaccine

Candida parapsilosis

  • Significance:
    • Most common non-albicans cause of invasive candidiasis in neonates.
  • Transmission:
    • C. parapsilosis:
      • Primarily nosocomial, total parenteral nutrition (TPN);
      • associated with colonization of healthcare workers’ hands.
    • C. albicans:
      • Usually acquired from maternal genital tract.

Risk factor for early onset sepsis (< 72 hrs)

  • Maternal fever
  • Foul smelling liquor
  • Multiple PV examinations during pregnancy
  • Low birth weight
  • Prematurity

Risk factor for late onset sepsis (< 72 hrs)

  • Lack of breastfeeding
  • Skin infection of the neonate
  • Infection of the umbilical cord stump
  • Multiple interventions

Most common method to prevent neonatal sepsis

  • Proper hand washing of the caregivers (for 3 minutes)

Investigations

  • Most effective method for prevention: Proper Handwashing
  • Earliest C/F: Baby not feeding well
  • Initial Ix: Sepsis screen
  • IOC: Culture
  • R/F:
    • Preterm
    • ROM >18hrs
    • PPV

Sepsis screen

  • Positive when any 2 / 5 parameters are abnormal
      1. TLC (< 5000)
      1. ANC (< 1800)
      1. IT ratio (> 0.2)
      1. CRP (positive)
      1. Micro ESR (> 15 mm in 1st hr)
  • Confirmatory test to diagnose neonatal sepsis:
    • Blood culture
  • In Early: Additionally take
    • CSF analysis
    • Chest Xray
    • Usually the infection is Meningitis or pneumonia
      • Urine C/S not much role in early neonatal sepsis

Treatment of choice

  • IV broad spectrum empirical antibiotics

Duration of antibiotic therapy

  • Blood c/s negative:
    • 5-7 days
  • Blood c/s positive:
    • 10 days
  • CSF s/o meningitis:
    • 21 days

Neonatal hypothermia

  • Definition: Axillary temperature <35.5°
    • Min 3 mins
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In AHA 2020 Guidelines :

  • Hypothermia in NB is defined as temp. <36°c
  • Cold stress:
    • 36 - 36.4 °C
  • Moderate hypothermia:
    • 32 - 35.9 °C
  • Severe hypothermia:
    • < 32°C

Most important mechanism for protection against hypothermia in neonates:

  • Non shivering thermogenesis
  • Due to the presence of brown fat
    • Around scapula, axilla
    • lipid deposits rich in mitochondria
    • Release of norepinephrine and uncoupling of beta oxidation of fat → heat production
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  • Mnemonic: AD ThyLI (adi thyli)
  • Newborns: Interscapular, perirenal, mediastinal, perivascular, supraclavicular.
  • Adults: Less, but persists in supraclavicular and paravertebral regions.

Maximum heat loss:

  • via head of the baby
  • Recommended to keep the head of the baby covered
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  • Measurement: Rectal temperature (best for core temperature)
  • Rewarming: Cardiopulmonary bypass machine (best for severe hypothermia)

Classification in adults:

  1. Cold Stress: > 35°C.
  1. Mild: 32 - 35°C.
  1. Moderate: 28 - 32°C.
      • <30°C hypothalamus stops functioning.
      • Shivering stops
      • Reflexes are low.
      • Osborn J waves in ECG → Indicate severity
      • Paradoxical undressing 
        • Severe cold exposure → failure of thermoregulation → ↑ blood flow → failure of Vasoconstriction → ↑ sense of warmth → undressing → death
        • Kills the patient & can mimic sexual offense
      • Hide & die syndrome/terminal burrowing.
  1. Severe: <28°C.
      • No motion, Absent reflexes & shivering, Coma
      • Osborn J waves in ECG → Indicate severity
      notion image

Autopsy findings:

  • White deaths.
  • Pink hypostasis.
  • Wischnewski’s bleeding spot in stomach.
  • Note : Pink hypostasis also seen in refrigerated body.
    • notion image

Frost Bite

Progression: Pallor → Erythema → blisters → Gangrene.
Progression: Pallor Erythema blisters Gangrene.
notion image
  • Cause: Prolonged exposure to dry cold
  • Pathophysiology:
    • Ice crystals form in tissue
    • Membrane damage + microvascular damage
  • D/t exposure to freezing temperature.
  • Site: Periphery (Finger, feet, tip of nose/ear lobes).
  • Rx: Gradual rewarming at 40°C.
    • Rewarming Re-perfusion injury
  • Grading after rewarming
    • notion image

Trench Foot

  • Cause: 
    • Prolonged exposure to cold + moisture (tissue remains wet)
  • Pathophysiology:
    • Microvascular damage
    • Stasis & occlusion

Management of Frost Bite and Trench Foot

  • Rapid re-warming of affected leg (water: 40°C)
    • Beware of re-perfusion injury
  • Do not rub the tissues (causes severe pain)
  • Hyperkalemia, acidosis can occur
  • If gangrene + Wait for demarcation line Amputation
notion image

Clinically cold stress is

  • abdomen is warm
  • soles are cold

Areas rich in brown fat

  • Axilla
  • Groin
  • Nape of neck
  • Inter scapular area
 

Under-Five Clinics:

  • Elements:
    • Promotive care
    • Core
    • Family planning
    • Curative care
    • Preventive care
    • Health Education
notion image

Kangaroo mother care

  • Elements (SAANS):
    • Skin-to-skin (Hypothermia prevention)
    • Airway disease prevention
    • Ambulatory support
    • Nutritional support (Exclusive breastfeeding)
    • Support (Emotional)

Indication

  • All stable low birth weight neonates

Components

  1. Kangaroo position:
      • Skin-to-skin contact
  1. Kangaroo nutrition:
      • Exclusive breastfeeding,
      • no supplementation
  1. Early discharge from hospital

Advantages

  • Lesser chances of:
    • Sepsis,
    • Hypothermia
    • Neonatal mortality
  • Higher weight gain (better gain of anthropometric parameters)
  • Earlier discharge from hospital
  • Higher breastfeeding rates

STOP KMC

  • baby attains 2.5 Kg or 37 weeks.

GOBI FFF

  • Given by WHO/UNICEF.
  • Aim: Promote child survival
  • Components:
    • Growth monitoring
    • Oral rehydration solution
    • Breastfeeding
    • Immunization
    • Female fertility
    • Female literacy
    • Food (Female nutrition)

BFHI, MAA, CLMC:

  • Baby Friendly Hospital Initiative (BFHI):
    • UNICEF initiative to promote child survival
  • Mother’s Absolute Affection (MAA):
    • Gov of India, MoHFW initiative to promote breastfeeding
      • Normal delivery: Within 1 hour
      • C-section: Within 4 hours
  • Comprehensive Lactational Management Centers (CLMC):
    • Promote breast milk donation
    • CHCs & District hospitals:
      • Breast pumps & breastmilk storage facilities (+)

Important Dates & Weeks

Date/Period
Event
April 14th
Ayushman Bharat Health & Wellness Centre Day
Last week of April
World Immunization Week
May 28th - June 8th
Intensified Diarrhoea Control Fortnight
August 1st - 7th
World Breastfeeding Week
September 1st - 7th
National Nutrition Week
November 15th - 21st
Newborn Week

Devices used to keep neonates warm

Radiant warmer → Radiation
Radiant warmer → Radiation
Incubator → Convection
Incubator → Convection

1. Radiant warmer

  • Open care system
  • Method by which neonate is kept warm:
    • Radiation

2. Incubator

  • Baby is kept inside a closed box
  • Method by which neonate is kept warm:
    • Convection
  • Preferred for preterm neonates
    • helps to maintain humidity to prevent further losses due to evaporation

Neonatal hypoglycemia

Definition

  • Blood glucose <40 mg/dl or
  • plasma glucose <45 mg
  • M.c: Jitteriness/ tremors
    • (when limbs are held, jitteriness stops and is not associated with up rolling of eyes; whereas in seizures, movements do not stop when limb is held)
  • Others:
    • Apnea, seizures, increased sweating, lethargy

Treatment

  • If asymptomatic:
    • Offer a feed and
      • Frequent feeding
    • Regular monitoring of blood glucose
  • If symptomatic or <20 mg/dl:
    • IV 10% dextrose 2 ml/kg
      • Continuous glucose infusion
        • GIR → glucose infusion rate (6 mg/kg/min)
notion image

Important causes of persistent hypoglycemia during infancy

Endocrine causes

  • Congenital hyperinsulinemia
    • most common cause
    • earlier known as Nesidioblastosis
    • currently it is known as PHHI- persistent hyperinsulinemic hypoglycemia of infancy
    • Mnemonic: HI → HyperInsulinemia
  • Congenital hypopituitarism
  • Congenital adrenal insufficiency

Metabolic disorders

  • Glycogen storage disease
  • Galactosemia
  • Hereditary fructose intolerance
  • Mitochondrial disorders
  • Fatty acid oxidation defects

Problems in infants of diabetic mother

Large for date/ macrosomia:

  • ↑↑ chances of obstructed/prolonged labor
    • Use of instruments
    • Increased chances of birth trauma
    • Increased chances of birth/perinatal asphyxia

Metabolic

  • Hypoglycemia
  • Hypocalcemia
  • Polycythemia
  • Hyperbilirubinemia

Respiratory system

  • Increased risk of
    • Respiratory distress syndrome/
    • Hyaline membrane disease
      • due to delayed maturation of surfactant
      • Even in term babies
  • Note:
    • Usually RDS is disease of preterm neonates,

Cardiovascular system

  • M.c. CHD: VSD
  • Most specific: Transposition of great arteries

Neurologic system

  • M.c: Neural tube defects
  • Most specific: Sacral agenesis/ Caudal regression syndrome

APGAR score

notion image
Component
0
1
2
Appearance
(Color)
Blue, pale
Pink body,
blue extremities
Completely pink
Pulse
(Heart rate)
Absent
<100 bpm
>100 bpm
Grimace
(Reflex irritability)
No response
Grimace
Cough, sneeze, cry
Activity
(Muscle tone)
Limp and flaccid
Some flexion of extremities
Flexed posture
with
actively moving limbs
Respiratory effort
Absent
Slow and irregular
Normal/ strong

Question Trick

  • If given MINIMAL RESPONSE
    • ⇒ Reflex irritability (Grimace) + Some muscle tone
      ⇒ 1 + 1 =
      2
  • Total score: 10
  • Minimum score: 0
  • APGAR score > 7 - normal
  • APGAR score 0-3 - severely depressed neonate
  • Note
    • Nasogastric tube is used to irritate nasal mucosa to elicit grimace.
    • Normal newborn heart rate : 130-160/min.
 
What is the Apgar score for a newborn baby that is assessed 5 minutes after birth and found to be
cyanosed with irregular gasping respiration, a heart rate of 60 beats/min, and minimal response to
stimulation?
A. 2
B. 5
C. 3
D. 4
ANS
4
  • Heart rate: 60 beats/min (score of 1)
    • Respiratory effort: Irregular gasping respiration (score of 1)
    Muscle tone: Some flexion of extremities(Minimal response) (score of 1)
    • Reflex irritability: Shows minimal response to stimulation (score of 1)
    Color: Cyanosed (score of 0)
    Adding up the scores for each component, we get a total Apgar score of 4.
A baby assessed five minutes after birth is found to be blue, Absent pulse, Minimal response to stimulation, flexed arms and leg, and slow irregular movement. The APGAR score for this newborn is.
A. 2
B. 3
C. 4
D. 5
ANS
3
Flexed arm and leg but without active movement = 1
What is the Apgar score for a baby that is evaluated 5 minutes after birth and exhibits a pink body
color, blue extremities, irregular breathing, a heart rate of 80 beats per minute, grimacing, and some
flexion?
A. 2
B. 3
C. 4
D. 5
ANS
5

Perinatal asphyxia

  • Definition: Inability to initiate or sustain breathingdamage to brain (HIE)

CEREBRAL PALSY

  • NON PROGRESSIVE, NON REVERSIBLE
  • DPT vaccine can be given

Parts of brain most commonly affected due to asphyxia

  • In term neonate with HIE
    • Parasagittal area
      • Spastic quadriplegia
      • Multicentric encephalomalacia
      • Status marmoratus:
        • Basal galglia affected
        • m/c Choreo-athetoid type
  • In preterm neonates:
    • Periventricular area
      • normal intelligencescissoring + W-sitting + commando crawl
      • Spastic diplegia
      • Periventricular encephalomalacia
      • Best prognosis (because intellect is spared)
  • Spastic hemiplegia
    • Porencephalic cyst

Diagnosis

  • APGAR score:
    • 0-3 for > 5 mins
  • Cord blood pH:
    • pH < 7
  • CNS dysfunction:
    • Seizures,
    • tone abnormalities,
    • abnormal Moro's
    • Scissoring gait
    • Commando crawl
  • Neonatal multiorgan dysfunction:
    • Acute kidney injury,
    • liver dysfunction
  • Cortical thumb
    • notion image

Neonatal seizures

DOC
Phenobarbitol
MC TYPE
Subtle seizures
MC CAUSE
HIE
CAUSES
B6 - Pyridoxine deficiency
Hypo/Hyper Na
↓ Calcium
↓ Glucose
Hypoglycemia:
BG < 40 mg/dl
Plasma < 45 mg/dl
Best prognosis
Focal clonic
Worst prognosis
Myoclonic
BEDSIDE MONITOR
Amplitude Integrated EEG
INITIAL IX
Transcranial USGto r/o PVH
(Probe around Anterior Frontanelle)
IOC
MRI (DWI)
notion image

Staging of HIE (Hypoxic Ischemic encephalopathy)

Sarnat staging

  • if Sar nuts → HIE
Parameter
Stage I (Mild)
Stage II (Moderate)
Stage III (Severe)
Consciousness
Alert
Lethargic
Comatose
Muscle Tone
Normal
Hypotonia
Flaccid
Tendon Reflexes
Normal
Normal
Absent
Moro's Reflex
Normal
Exaggerated
Absent
Seizures
Absent
Often present
Persistent and refractory
EEG
Normal
Low voltage
Burst suppression
Prognosis
99% Normal
80% Normal
50% Death,
50% Severe sequelae
  • Treatment: Mainly supportive care
  • Therapeutic hypothermia may be tried in moderate HIE

NOTE

  • EEG shows burst suppression
    • SSPE
    • HIE stage 3

Respiratory distress syndrome

notion image
White out lungs
White out lungs
  • M.c cause of respiratory distress is a preterm neonate
  • Basic defect: deficiency of mature surfactant
1 → Type 1 pneumocytes
2 → Type 2 pneumocytes
1 → Type 1 pneumocytes
2 → Type 2 pneumocytes
 
  • Type 1 pneumocytes:
    • Thin squamous cells
    • Predominant cells (cover 95% of alveolar surface).
    • Flat cells.
    • Primary site for gas exchange.
  • Type 2 pneumocytes:
    • Cuboidal cells (cover 5% of alveolar area).
    • Have microvilli on their surface.
    • Secrete surfactant.
    • Appear larger with big, rounded nuclei in sections.
      • Described as mimicking Clara cells.

Surfactant

  • Produced by- type II alveolar cells or type 2 pneumocytes
  • Synthesis begins in fetal lungs at- 20 weeks
  • Begins to appear in amniotic fluid- 28 weeks
  • Mature and in adequate amount- > 35 weeks
  • Most important component of surfactant:
    • DPPC (Dipalmitoyl Phosphatidyl Choline) > Lecithin
  • Most important surfactant protein:
    • Surfactant Protein B

Function of surfactant- to decrease the surface tension of alveoli

  • Prevents alveoli from collapsing
  • Increases lung compliance

When mature surfactant is deficient

  • Alveolar collapse
  • Interstitial edema
  • Alveolar damage
  • Decreased lung compliance
  • Deposition of fibrin
    • hyaline membrane disease
  • Small volume lungs with ground glass haziness of lungs is seen
  • Sometimes air bronchogram is seen
    • radiolucent areas

Ways to detect adequacy of mature surfactant in amniotic fluid

  • L:S ratio (Lecithin: Sphingomyelin ratio):
    • >2:1
  • Estimation of phosphatidylglycerol level
  • Nile blue sulphatase test
    • > 50% orange cellsimmature surfactant
  • Shake test:
    • Equal volumes of amniotic fluid and ethyl alcohol taken
    • shook together
    • stand for few hour
    • if complete ring of bubbles is present on top (≥1)
      • indicates mature surfactant is present

Silverman score

  • Only Preterm
Parameter
0
1
2
Upper chest retraction
Chest and abdomen move equally with respiration
Chest wall lags behind abdomen
See-saw pattern
(
paradoxical breathing)
Lower chest retraction
Absent
Minimal
Marked
Xiphisternal retractions
Absent
Minimal
Marked
Nasal flare
Absent
Minimal
Marked
Grunt
None
Audible only with stethoscope
Loud,
audible
without stethoscope
  • Grunt is a noise due to expiration against a partially closed glottis

Downe's score

  • For term and preterm neonate
    • Parameter
      0
      1
      2
      Cyanosis
      None
      Peripheral cyanosis
      Central cyanosis
      Retractions
      None
      Mild
      Moderate → Severe
      Grunting
      None
      Audible only with stethoscope
      Audible without stethoscope
      Air entry
      Normal
      Decreased
      Absent
      Respiratory rate
      <60
      60-80
      >80 or Apnea
  • Maximum score: 10
  • Minimum score: 0
  • Score < 3 - Normal
  • Score > 7 - Severe respiratory distress

Treatment of respiratory distress syndrome

Mild

  • CPAP (continuous positive airway pressure)
    • prevents alveoli from collapsing during expiration
    • +/- O2

Moderate to severe

Intratracheal surfactant

  • INSURE:
    • Intubate give SurfactantExtubate
    • Indication
      • RDS + > 30% need for oxygen
  • LISA:
    • Less Invasive Surfactant Administration
  • LAST RESORT → Respiratory support:
    • Mechanical ventilation

Prevention of RDS

Antenatal corticosteroids

Drug
Administration
Total Dose
Total Duration
Betamethasone
IM 12 mg every 24 hrs x 2 Doses
24 mg
48 hrs
more neuroprotective
Dexamethasone
IM 6 mg every 12 hrs x 4 Doses
24 mg
48 hrs
Cheaper and easily available

Benefits

  • decrease the risk of:
    • RDS
    • IVH, NEC
    • Neonatal mortality
  • Note: Does not decrease the risk of neonatal jaundice
  • Administered to all pregnant women between 24 to 34 weeks of gestation

Contraindication

  • Clinical chorioamnionitis

Replacement:


  • Hydrocortisone
    • Acute Adrenal insufficiency / Addisonian crisis (IV).
      • approximately 10-12 mg/m²/day.
    • Chronic Adrenal Insufficiency / Addison’s disease (Oral).
      • 20 mg/day TDS 10 mg 5 mg 5 mg doses

Other Uses:

  • Inflammation
  • Autoimmune diseases
  • Transplantation
  • Anticancer therapy except in Kaposi sarcoma
  • Asthma

Meconium aspiration syndrome

notion image
  • C/F:
    • SGA/IUGR baby
    • meconium stained liquor
    • respiratory distress soon after birth
    • Green staining of vocal cord
  • On examination: Increased antero-posterior diameter of the chest due to hyperinflation

CXR

  • Obstructive emphysema
    • Hyperinflated lungs (appear more black, flattening of diaphragm)
  • Chemical pneumonitis
  • Segmental collapse

Treatment:

  • Supportive
notion image

Transient tachypnea of newborn (TTNB)

  • Also called delayed adaptation
  • Due to delayed clearance of lung fluid
  • M.c in babies born through
    • C section

CXR

  • Fluid in interlobar fissure
  • Perihilar streaking/ prominent broncho vascular markings
  • Pleural effusion
    • notion image

Management:

  • Self-limiting

Neonatal apnea

  • Definition: Cessation of breathing for
    • at least 20 seconds or
    • for any duration + either bradycardia/cyanosis

Causes

  • Neonatal sepsis,
  • Neonatal jaundice,
  • Polycythemia,
  • Hypoglycemia,
  • Hypocalcemia,
  • Anemia,
  • Apnea of prematurity

Treatment

  • Drug of choice for apnea of prematurity:
    • Inj caffeine citrate
  • Treat the cause
  • Respiratory support

Bronchopulmonary dysplasia

  • Baby on persistent O2 support
  • Baby received > 21 % O2 for > 28 days
  • Then developed RDS

Necrotising enterocolitis

Important risk factors

  • Prematurity,
  • Lack of breastfeeding/ use of formula feeding,
  • 10% cases can occur in term neonates

Stages of NEC → BPG

notion image
Pneumatosis intestinalis
Pneumatosis intestinalis
Stage
Systemic Signs
Treatment
Ia
Occult blood in stool

BAT
• Bradycardia
• Apnea
• Temperature instability
• NPO, antibiotics 3 days
Ib
Fresh blood in stool
• Same as IA
IIa
Pneumatosis intestinalis in X ray
• NPO, antibiotics 7 to 10 days
IIb
Portal vein gas (Pneumatosis portalis)
• NPO, antibiotics 14 days
IIIa
Peritonitis
DIC
• NPO, antibiotic 14 days,
• fluid resuscitation,
• inotropic support
IIIb
Gas under diaphragm/pneumoperitoneum
FOOTBALL SIGN
• Surgery

Pneumoperitoneum

Signs on X ray:

notion image
notion image
  • Air in peritoneal cavity due to ruptured hollow viscus organ (perforation, post laparoscopy).
  • On CT, jet black appearance shows air.


1. On erect chest X ray:

  • Free air under the diaphragm.

2. Decubitus abdomen sign:

notion image
  • Left lateral decubitus position with horizontal X-ray beam.
  • Provides good contrast against liver.
  • Air around lesser sac can escape through epiploic foramen.
  • Black air seen above liver.

3. Football sign:

  • Patient supine.
  • In neonate
  • Air beneath anterior abdominal wall.
  • Suggestive of Massive pneumoperitoneum.
    • notion image

4. Rigler's sign

  • Double bowel sign.
  • Air inside and outside the bowel makes bowel loops clearly visible.
    • notion image
  • Important Information:
    • Rigler's triad: Seen in gall stone ileus.

5. Cupola sign:

  • Air beneath the central diaphragm.
    • notion image

Ligament sign:

  • Falciform ligament seen
    • notion image
    • Falciform ligament connects anterior abdominal wall and liver.
    • Ligament visualized due to air on either side.
    • All ligament signs are seen in pneumoperitoneum.

7. Inverted V sign:

  • Paired umbilical ligament sign (seen due to air around the ligament).
    • notion image

Pseudo Pneumoperitoneum

notion image
  • Air in the bowel beneath the diaphragm.
  • Bowel markings are seen.

Chilaiditi syndrome:

  • Presence of colonic loop between diaphragm and liver.
  • Colonic interposition.

Systemic/lab features (seen in advanced disease):

  • Thrombocytopenia → due to sepsis/DIC
  • Hypoglycemia → due to sepsis and poor perfusion
  • Metabolic acidosis
  • Neutropenia or leukocytosis

Treatment

  • Mainly supportive
  • NPO, IV fluids, IV antibiotics
  • Surgery (stage IIIb)

Neonatal jaundice

PHYSIOLOGIC JAUNDICE
PATHOLOGIC JAUNDICE
Not present until 72 hours after birth.
Present in the first 24 hours of life.
Bilirubin ↑ < 5 mg/dL/day.
Bilirubin ↑ > 0.2- 0.5 mg/dL/hour.
Bilirubin peaks at < 14–15 mg/dL.
Bilirubin ↑ to > 15 mg/dL.
Direct bilirubin is < 10% of total.
Direct bilirubin is > 10% of total.
Resolves by one week in term infants &
two weeks in preterm infants.
Persists beyond > 14 days
one week in term infants &
two weeks in preterm infants.
ABO > HS/G6PD
  • Prolonged jaundice
    • Persists beyond > 14 days
  • Clinical jaundice
    • 60% of term neonates
    • 80% of preterm neonates
    • Bilirubin >4-6 mg/dl
    • Serum bilirubin>15mg/dl → stains palms and soles

Physiological Jaundice

notion image
  • Appears after 24 hrs
  • Always unconjugated
  • No dark urine / pale stools
  • Palms and soles not yellow
  • Resolves within 3 weeks

Breastfeeding Jaundice

  • Cause: Inadequate breastfeeding
    • Dehydration → relative polycythemia → hyperbilirubinemia
  • Rx:
    • Frequent breastfeeding
    • Supplement feeding
      • BM Bank > Formula milk

Breastmilk Jaundice

  • Cause: Pregnanediol & Free fatty acids (↓ UDP GT)
  • Rx: Continue breastfeeding
    • Stop only if bilirubin >20 mg/dl
 
What is a characteristic feature observed in physiological jaundice?
A. Increased indirect bilirubin, increased urobilinogen

Pathological Jaundice

  • May appear within 24 hrs
  • May be conjugated or unconjugated
  • Dark urine / pale stools present
  • Palms and soles may be yellow
  • May persist >3 weeks

EARLY NEONATAL JAUNDICE

  • Onset <24 hrs

CAUSES

  1. Haemolytic disease
      • RH incompatibility
      • ABO incompatibility
      • G6PD deficiency
      • Heriditary spherocytosis
  1. Congenital infections
      • toxoplasmosis
      • rubella
      • CMV
      • herpes simplex
      • syphilis
      • postnatal infections that develop into sepsis
  1. Crigler-Najjar $ or Dubin-Johnson $
  1. Gilbert's $

Hyperbilirubinemia Syndromes: Comparison

Feature
↑ Unconjugated Bilirubin Syndromes
Increased Conjugated Bilirubin Syndromes
UGT Enzyme Status
Conjugation by UGT deficient
(
UDP Glucoronyl transferase)
Conjugation by UGT is normal.
Defect Cause
UGT deficiency.
Defect in excretory proteins
Syndromes
Crigler-Najjar Type 1
Dubin-Johnson Syndrome
Crigler-Najjar Type 2
Rotor Syndrome
Gilbert Syndrome
Mnemonic: Doctors always united or
Doctors always conjugated (D+R)

Unconjugated Hyperbilirubinemia Disorders

Characteristic
Crigler-Najjar Type 1
Crigler-Najjar Type 2
Gilbert Syndrome
Genetics
AR
Autosomal dominant
AR
UGTA1 Deficiency
Complete
Partial
Very Mild defect
Clinical Features
Intrauterine deaths/Stillbirths

100% fatal
Patients survive
Asymptomatic

Manifests under stress:
-
Fever
-
Pregnancy
Treatment
Exchange transfusion

intensive phototherapy

Orthotopic liver transplant

Phenobarbitone has no role
Phenobarbitone:
* Increases UGT activity
No treatment needed
Koch indirect ayitt swapnathil 2 (CN T2) pambine kand karanju
(cry Naja)
Apo, Baribie (
Phenobarbitone) kanich samadanipichu
notion image
What is the most probable diagnosis for a neonate who continues to have unconjugated hyperbilirubinemia after three weeks of birth, with normal liver enzymes, PT/INR, and albumin levels, no evidence of hemolysis on a peripheral blood smear, and a decrease in bilirubin levels following treatment with phenobarbital?
A. Rotor syndrome
B. Crigler Najjar type 2
C. Dubin Johnson syndrome
D. Crigler Najjar type 1

Conjugated Hyperbilirubinemia

  • Mnemonic: Direct → Dr → Doctor → Need both
    • D → DJ → MRP (money) → MRP2
      • Dubin is dark
    • R → Rotar → ATP → OATP
      • Raw ATP
Condition
Defect in
Key Features
Dubin Johnson Syndrome
ABCC2 gene
(ATP Binding Cassette)
MRP2 protein
- Dark pigmented liver
- Pigment is epinephrine

Mnemonic: Dubin is dark
Dubbing Johnson
→ A busy (ABC) dubbing artist
→ needs MRP (MRP2)
Rotor Syndrome
Organic anion transporter protein
- No pigmentation in liver
Row cheyyan ATP venam

ABC Terms
Seen in
ABCG2
• Marker for Limbus/Pterygium (with CD34)
ABCA4 gene mutation
Stargardt Disease
Juvenile boy (Juvenile hereditary macular dystrophy)
Star (stargardts) → studies ABC (ABCA4 gene mutation)
At night (bcz blind during day → Hemeralopia)
Eat fish (fish flecks) & bulls eye (Bulls eye maculopathy)
Everyone beat him (copper beaten on Fundus exam)
Became Dark & Silent (dark/silent choroidal sign on FFA)
ABC1 (ATP Binding Cassette transporter 1) Mutation
Tangier's Disease
Reduced levels of apo A1very low HDL levels
Features
Greyish-orange tonsils
Hepatosplenomegaly
Mononeuritis multiplex
ABC students drink Tang → don't get A1 → cant multiply
ABCC2 gene mutation /
MRP2 protein
Dubin Johnson Syndrome
Dark pigmented liver
Pigment is epinephrine

Dubbing Johnson
Dubin is dark
A busy (ABC) dubbing artist
needs MRP (MRP2)
ABC Pump
Digoxin dosage is adjusted based on loss via efflux (GI)
Loperamide does not cross BBB (no CNS S/E)
Bacteria / Tumor Cells: Drug resistance
ABC Pump Inducer
(CRP in CRAP GPs)
Cause Drug Failure
Rifampicin
Digoxin failure
Phenytoin
Carbamazepine
ABC Pump Inhibitor
(CAVE Q itra neram)
Cause Toxicity
Cyclosporine
Cholestatic jaundice
Amiodarone
Verapamil
Reversal of drug resistance
Verapamil → Vera kalayan → Bacteria kalayan
• (cancer, bacteria)
Erythromycin / Clarithromycin
Digoxin toxicity
Quinidine
Loperamide-induced central S/E
Itraconazole
Neratinib

Etiology of Unconjugated Hyperbilirubinemia

1. Increased Production

  • Hemolytic disease of newborn (ABO / Rh incompatibility)
  • Hereditary spherocytosis
  • G6PD deficiency
  • Cephalhematoma

2. Decreased Conjugation

  • Crigler Najjar syndrome (↓ UDP glucuronyl transferase)

Etiology of Conjugated Hyperbilirubinemia

  • Conjugated if:
    • >2 mg/dl or
    • >20% of total bilirubin

1. Non-obstructive

  • Neonatal sepsis (bacterial, viral, parasitic)
  • UTIs
  • Viral infections
  • Metabolic disorders:
    • Hereditary Fructose Intolerance (HFI)
    • Alpha-1 antitrypsin deficiency
    • Galactosemia
  • Idiopathic neonatal hepatitis
    • Giant cells

2. Obstructive

Intrahepatic

  • Congenital hepatic fibrosis
  • Progressive familial intrahepatic cholestasis
  • Alagille syndrome
  • Dubin-Johnson syndrome
  • Rotor syndrome

Extrahepatic biliary atresia

  • Jaundice at birth
  • Periductal fibrosis and proliferation
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  • Japanese & Anglo-Saxon:
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    • Type 1: Atresia restricted to CBD.
    • Type II: Atresia of Common Hepatic Duct (CHD).
    • Type III: Atresia of right, left hepatic ducts & entire extrahepatic biliary tree.
  • D/ds
    • Neonatal hepatitis
    • Alagille syndrome:
      • Biliary atresia
      • Congenital heart disease
      • Skeletal abnormalities
    • Mnemonic: Alanjappo → slipped on Bile, got Skeletal fracture + Heart attack
  • A/w
    • Cardiac lesions
    • Polysplenia
    • Situs inversus
    • Absent vena cava
    • Preduodenal portal vein
  • Initial: Fasting USG (Gold standard): Shows atretic biliary tree.
    • Triangular Cord sign
      • fibrosed bile duct seen anterior to portal vein
        • Triangular cord sign
          Triangular cord sign
    • Ghost GB triad
      • GB become small and atretic
  • Screen: HIDA scan ??
    • Highest Negative Predictive Value → rule out
  • MRCPSensitive and specific.
  • Liver Biopsy: Confirmatory.
  • Tx:
    • Kasai procedure (H - J)
      • within first 60 days OR
      • NO cirrhosis
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    • Otherwise → Liver Transplant
      • M/c indication for liver transplant in children.

Choledochal Cysts

PATHOPHYSIOLOGY

  • ABNORMAL PANCREATICO BILIARY JUNCTION
Identify
Identify

Todani/Modified Alonso-Lej Classification:

Type I (M/c): 

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  • Diffuse dilatation of CBD.
  • Treatment: 
    • Cyst resection +
    • Roux-en-Y hepaticojejunostomy.
    • Single → Cyst

Type II: 

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  • Diverticulum of CBD.
  • Treatment: Diverticulum resection & repair.
  • 2 → Di → Diverticulum

Type III: 

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  • Dilatation of intraduodenal portion of CBD (Choledochocele).
    • Treatment: 
      • ERCP +
      • Sphincterotomy +
      • Removal of abnormal mucosa.
  • 3 → 3C → CholedoChoCele

Type IV A: 

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  • Intrahepatic + Extrahepatic biliary tree dilatation.
    • Treatment: Liver transplant.
  • 4 A → A → All involved

Type IV B: 

  • Only extrahepatic biliary tree dilatation.
    • Treatment: Kasai procedure
      • Portoenterostomy

Type 5: 

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  • Dilatation of only intrahepatic biliary tree (CarolI’s disease).
    • DUCT DILATED WITH A DOT (VENOUS RADICLE ) IN CENTRE
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  • Treatment: Liver transplant.
  • 5 carolI teams → I → Intrahepatic

IOC:

  • MRCP

Caroli disease

  • Rare congenital disorder
  • Saccular dilatation of intrahepatic bile ducts
  • Autosomal recessive
  • Non-obstructive
A/W
  • Congenital hepatic fibrosis
  • Often with polycystic kidney disease
Imaging sign
  • Central dot sign on CT
    • Central dot sign
      Central dot sign
  • MRI
    • IOC
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Clinical Features

Modified Krammer's Rule

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  • Face: 7 mg/dl
  • Upper trunk: 9 mg/dl
  • Lower abdomen & thigh: 11 mg/dl
  • Arms & legs: 13 mg/dl
  • Palms & soles: 17 mg/dl

Kernicterus / Biliary Induced Neurological Damage (BIND)

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  • Aka Biliary encephalopathy
  • MC site: Basal gangliaKernicterus
  • Type of CP: Extrapyramidal

Acute Bilirubin Encephalopathy

  • Early:
    • Hypotonia
    • Lethargy
    • Poor feeding
  • Late:
    • Fever
    • Seizures
    • Hypertonia
    • Opisthotonus

Chronic Bilirubin Encephalopathy

  • Sensorineural hearing loss (SNHL)
  • Athetosis
  • Dental enamel hypoplasia
  • Upward gaze palsy
  • Intellectual disability

Treatment of neonatal jaundice

  • First line: Phototherapy
  • Sn Mesoporphyrin
    • Competitive inhibition of heme oxygenase (Heme ⇏ Bilirubin)
  • Exchange transfusion (severe cases)
  • IV Ig (ABO or Rh incompatibility)

NOTE:

  • Exchange transfusion
    • A/w ↑ risk of Portal Vein thrombosis,
    • child presents with hematemesis, splenomegaly
    • d/t development of collaterals

Phototherapy

  • Most effective wavelength: 460-490 nm
  • Use of LED lamps
  • Irradiance using flux meter: 30 uW/cm2/nm
  • The mechanisms involved in phototherapy are
      1. Irreversible structural isomerization
        1. bilirubin is converted to Lumirubin
            • which is excreted via kidneys.
            • Most important mechanism.
      1. Reversible photo-isomerization
      1. Photo-oxidation

Ways to increase effectiveness of phototherapy

  • Decrease distance between baby and phototherapy unit
  • Increased exposed surface area
  • NO effect from skin pigmentation

Adverse effects of phototherapy

  • Bronze baby syndrome
    • color of the baby becomes dark
    • transient
  • Watery diarrhea
  • Dehydration
  • Hypocalcemia
  • Retinal toxicity
  • Gonadal toxicity/ mutations
  • Impaired maternal child bonding

When to start phototherapy?

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In an otherwise well term neonate

  • Phototherapy cut-off (mg/dl): 12-15
  • Exchange transfusion cut-off (mg/dl): 20-25
  • In high risk and preterm babies, cut offs will be lower

Indication of exchange transfusion in a baby with Rh incompatibility

  • Cord blood bilirubin:
    • > 5 mg/dl
  • Cord blood
    • Hb: <10 g/dl /
    • PCV: <30