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Tips for doing well in neonatology section of Pediatric Boards

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Tips for doing well in neonatology section of Pediatric Boards Shantanu Rastogi, MD, FAAP Neonatologist, Maimonides Medical Center Assistant Professor of Pediatrics – PowerPoint PPT presentation

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Title: Tips for doing well in neonatology section of Pediatric Boards


1
Tips for doing well in neonatology section of
Pediatric Boards
  • Shantanu Rastogi, MD, FAAP
  • Neonatologist, Maimonides Medical Center
  • Assistant Professor of Pediatrics
  • Mount Sinai School of Medicine

2
Some general points
  • There are no negative markings and hence no
    questions SHOULD be left unanswered
  • When the answers are not clear in the first
    reading then try the method of exclusion to get
    to the best possible answer

3
Rastogis Rule
  • Common presentations of common diseases
  • Rare presentations of common diseases
  • Common presentations of rare diseases
  • Rare presentations of rare diseases

4
FETAL WELL BEING
5
Biophysical profile
  • 5 categories with score of 0 or 2
  • NST, fetal body movements, breathing, fetal tone,
    amniotic fluid volume
  • 10 is well fetus, 2 is certain fetal asphyxia, 4
    or 6 needs frequent reevaluation for delivery

6
Electronic Fetal Heart Rate Monitoring
  • Normal FHRv of 6-15bpm, basis of nonstress
    test-reactive positive test is normal
  • Abnormal patterns
  • Tachycardia, gt160, infection
  • Bradycardia, lt110, head compression
  • Loss of FHRv, hypoxia
  • Decelerations
  • Early, mirror image of uterine contractions, head
    compression
  • Variable, irregular, umbilical cord compression
  • Late , occur 10sec after uterine contraction and
    last longer, uterine placental insufficiency

7
RESUSCITATION
8
Resuscitation-recent changes
  • No intrapartum meconium suctioning
  • Oxygen use
  • Can use lt100 but if no improvement in
    saturations, increase to 100
  • If lt32w to use pulse oximeter keeping saturations
    between 90-95
  • Epinephrine IV is preferred but if using ET use
    x10 dose
  • Special use of laryngeal mask, CO2 monitor, and
    careful temp. control-use of clear plastic bags

9
Case
  • 28 w baby is delivered vaginally has HR of 90/m
    and irregular respiration, cyanosis, hypotonia
    with no reflexes
  • WHAT IS THE APGAR SCORE?
  • WHAT IS THE FIRST STEP?
  • WHAT IS THE SUBSEQUENT STEP?

10
Case continued
  • After 1 min of IPPV the HR is 60/m
  • WHAT IS THE NEXT STEP?

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12
INFECTIONS
13
GBS
  • The total number of cases of sepsis has gone down
    due to the decrease in GBS sepsis, but that from
    gram negative remain the same
  • CDC guideline-recent changes
  • To screen all women at 35-37 wks of gestation
  • If GBS positive prior preg. does not mean it is
    positive in present pregnancy unless there was
    invasive neonatal infection
  • No prophylaxis required for elective c-section if
    GBS positive and with no ROM/labor
  • Adequate prophylaxis is by completion of 2 doses
    of penicillin

14
GBS
  • Risk Factors
  • Intrapartum Prophylaxis to mother -if GBS
    positive OR unknown with lt35 weeks GA, ROM 18h,
    maternal fever 100.4F OR GBS bacteriuria OR
    previous invasive neonatal GBS
  • Rx to baby-Symptomatic OR if mother for IP and
    baby less than 35 wks/ROM gt18h/duration of IP
    less than 4h.
  • Clinical presentation
  • Early-lt7DOL, pneumoniagtsepsisgtCNS, ascending
    infection
  • Late onset-gt7DOL, less mortality but more CNS
    involvement and sequelae, deep infection as
    cellulitis, arthritis, osteomyelitis

15
Other bacterial infections
  • E.coli/Klebsiella sp. Absolute numbers have not
    decreased and hence the proportion of the
    neonatal sepsis cases from these organism have
    increased. Typically progress thru 3 stages of
    shock
  • Listeria- gram rod, in unpasteurized milk,
    cheese, raw vegetables and uncooked meat
  • Early onset lt7d, transplacental, chocolate
    colored amniotic fluid, preterm deliveries,
    sepsis/pneumonia
  • Late onset gt7d, nosocomial, meningitis with
    mononuclear cells

16
Other congenital infections
Clinical presentation CMV Rubella Toxoplasmosis
LBW
Liver/Spleen
Jaundice
Petechiae
CHD
Cataract
Retinopathy
Cerebral calcification Periventricular cortical
Microcephaly
17
Hepatitis B positive mother
Mat. status Newborn gt2kg Newborn gt2kg Newborn lt2kg Newborn lt2kg
HBV HBIG HBV HBIG
3 doses, 1stlt12h 1 dose, lt12h 4doses, 1stlt12h 1dose, lt12h
unknown 3 doses, 1stlt12h 1 dose, lt7d 4doses, 1stlt12h 1dose, lt12h
_ 3 doses, 1st at1-2M None 3 doses, 1st at1-2M None
18
Congenital Syphilis
VDRL
Inference
FTA
Mother Baby Mother Baby
- - - - No or prozone
- - False
/- Mother/B disease
- - Treated disease
19
Conjunctivitis
  • Onset will give the clue
  • Hours-chemical-silver nitrate
  • Day 2-5-Nisseria gonorrheae-purulent, emergency,
    needs IV antibiotics
  • Day 5-14-Chlamydia-bilateral, cough

20
CHROMOSOMAL ABN.
21
Trisomy 21
  • Recurrence risk- If no translocation- 1 risk
    till mat.age of 37y, if mat. translocation-10-15,
    if pat. translocation-5
  • Types- 94 non-disjunction,3-5 translocation, 2
    mosaic. Commonest cause in both old and young
    mothers is non-disjunction

22
Trisomy 21
  • Defects
  • Cardiac (40-50)-Endocardial cushion defect, VSD
  • Extremities-single palmer crease, 5th finger has
    hypoplastic middle phalange and clinodactyly
  • Face-slanting palpebral fissure, Brushfield
    spots, epicanthic folds, short neck, flat occiput
  • GI- duedenal atresia, Hirschsprung Disease
  • Neurology- hypotonia, MR,
  • Other hypothyroidism, leukemia, hip dysplasia

23
Other chromosomal anomalies
  • Trisomy 13 (Patau-MIDLINE deformities)
  • Holoprosencephaly,cleft lip/palate,coloboma,
    sloping forehead, cutis aplasia, VSD,
    polydactyly, hyperconvex nails, persistence of
    fetal Hb
  • Trisomy 18 (Edward)
  • Cardiac (common, VSD, PDA, PS),clenched hand,
    overlap of 2nd over 3rd and 5th over 4th finger,
    rocker bottom feet, small mouth/eyes/palpebral
    fissures, short sternum, hernia, cryptorchidism

24
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26
PULMONARY
27
Case
  • 28 wk preterm baby was delivered and intubated
    and placed on settings of RR 40, PIP of 25, PEEP
    of 5 and O2 of 100. Decision was made to give
    surfactant.
  • WHAT IS THE NEXT EXPECTED CHANGE IN THE
    VENT.SETTINGS?
  • If surfactant was not given as the O2 requirement
    decreased to 50 and was clinically observed.
  • WHAT CHANGE WILL NEED TO BE FOLLOWED FOR
    PREDICTING IMPROVEMENT?

28
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29
RDS-Surfactant Def.
  • Clinical course Peak-1 to 3 d and recovery
    starts with diuresis
  • Risk Factors Low GA, male, Mat. DM, perinatal
    depression
  • RDS in term SPB def, Mat. DM, Beckwith Weideman
    syndrome, congenital syphilis
  • Pathology Hyaline membrane (cellular debris in
    fibrinous matrix)
  • Treatment-surfactant replacement,
    fluid/electrolyte and respiratory management
  • Complications pneumothorax as sudden
    decompensation
  • DD for reticulogranular CXR- GBS pneumonia, PAPVR


30
Congenital malformations
  • CCAM
  • cyst of lung tissue with small communication with
    bronchial tree, blood supply from pulm.
    circulation
  • Can present IU (hydrops, polyhydramnios, pulm.
    hypoplasia) or neonatal ( resp. distress)
  • 3 types
  • I - large cyst, good prognosis
  • II- medium size cyst, 50 associated anomalies,
    prognosis ??
  • III- large lesion with multiple small cyst,
    usually present IU, poor prognosis

31
Congenital malformations
  • Congenital emphysema
  • Commonest lesion, commonest in left upper lobe
  • Over distention of lobe due to loss of cartilage
    in large airways
  • Variable clinical usually mild respiratory
    distress
  • Sequestered lung
  • Non functioning lung tissue with systemic b.s.
  • 2 types
  • Intralobar - mostly left lower, few other
    anomalies
  • Extralobar - mostly between LL and diaphragm,
    many associated anomalies
  • Often asymptomatic, rarely can present in utero

32
Case
  • FT BB delivered by stat c/s for bradycardia
    required resuscitation by IPPV. Improved but soon
    developed progressive respiratory distress and
    poor perfusion. ABG showed 7.1/72/42/-5/18 on
    100 oxygen.
  • WHAT IS THE PROBABLE CAUSE?
  • WHAT NEEDS TO BE DONE NOW?

33
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34
Air leak syndromes
  • Pneumothorax
  • Air between parietal and visceral pleura
  • Risk Factors-Aspiration synd.,lung diseases (RDS,
    MAS), ventilation (high PIP)
  • Presentation can be with tension (severe RD,
    bradycardia, apnea, hypotension with mediastinal
    shift), large leak or small leak.
  • Complication IVH by dec. venous return, SIADH
  • Diagnosis asymmetrical air entry,
    transillumination
  • Rx EMERGENCY if tension-needle aspiration
    followed by chest tube, supportive

35
Airleak Syndromes
  • Pneumopericardium- air in pericardial sac
  • Usually associated with other airleak syndromes
  • If large- muffled HS, venous congestion,
    decreased CO
  • Rx if symptomatic-pericardial aspiration, high
    mortality
  • Pneumomediastinum- air in mediastinum
  • Usually after IPPV or difficult intubation, high
    PIP
  • Muffled heart sounds, CXR-sail sign
  • Usually need supportive treatment
  • Pulmonary Interstitial Emphysema-air in
    interstitial space
  • Usually preterm with RDS and on ventilation
  • Rx decrease MAP, if unilateral-selective
    intubation/blocking of bronchus

36
Chronic lung disease Vs BPD
  • BPD- 36 w of GA with oxygen requirement
  • Mechanical trauma to susceptible lungs (preterm
    lungs) leading to inflammation, injury is
    increased with decreased antiproteases and
    antioxidants in the preterm
  • Poor compliance, increased WOB, pulmonary
    hypertension, RVH
  • Radiographic classification I to IV
  • Rx- nonspecific as supportive care, good
    nutrition (120-150cal/kg/day), diuretics,
    bronchodilators, steroids

37
Apnea
  • Cessation of air flow for gt20s with
    cyanosis/bradycardia
  • Types Central (no effort, no air flow),
    Obstructive (no airflow despite effort), Mixed
  • Cause prematurity (usually after 12h of life),
    infection, maternal med.(narcotics, magnesium),
    infant med.(Indomethacin), CNS disorders (IVH)
  • Treatment Treat underlying disease,
    methylxanthines (caffeine) , CPAP, ventilation

38
Transient Tachypnea of Newborn
  • It is a diagnosis of exclusion
  • Cause delayed clearing of lung fluid
  • Risk factors elective c/s, maternal DM,
    perinatal depression, precipitous delivery
  • Usually resolves 2-3 days
  • Treatment oxygen/ CPAP

39
Meconium aspiration syndrome
  • Definition MSAFRDCXR changes
  • Clinical usually post-term, severe respiratory
    distress
  • Complicationspulmonary hypertension, airleak
    syndromes
  • CXRsnow storm appearance
  • Prevention ??intrapartum suction of meconium
  • Rx respiratory support, correcting acidosis,
    antibiotics, surfactant

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41
Case
  • FT AGA BB was delivered to mother with GDMA2 not
    well controlled. AS were 8,9 developed
    respiratory distress with cyanosis within few
    hours of being in the WBN. Vitals are stable with
    tachypnea associated with saturation of 70 in the
    right hand and 45 in the left leg. CXR shows
    lungs with decreased blood flow.
  • HOW TO CONFIRM THE DIAGNOSIS?
  • WHAT IS THE DRUG OF CHOICE?HOW IS ITS TOXICITY
    MONITORED?

42
Pulmonary Hypertension
  • Cause
  • Maladaptation- normal vasculature but
    vasoconstriction (hypoxia, hypothermia,
    polycythemia, pneumonia)
  • Maldevelopment-abnormal structure of pulmonary
    vascular bed (chronic intrauterine hypoxia,
    pulmonary hypoplasia)
  • Rx- respiratory support (no hyperventilation),
    correcting acidosis, NO, ECMO

43
Congenital diaphragmatic hernia
  • Types-Posterolateral thru Foramen of Bochdalek
    (LgtgtR) and central thru Foramen of Morgagni
  • In-utero as hydrops, after birth as RD due to
    lung hypoplasia, scaphoid abdomen
  • Complications-related to lung hypoplasia and
    pulmonary hypertension (PH)
  • Treatment strategy-IMMEDIATE intubation,
    stabilizing the PH and delayed surgical repair
  • Usually delivered in ECMO centers as may be
    needed for treating PH

44
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45
Hypoplastic lungs (Potters sequence)
  • Causes
  • Intrathoracic compression (CDH, CCAM)
  • Thoracic (neurological ds. as Werdnig Hoffman
    syndrome)
  • Extrathoracic (common, causes of oligohydramnios
    specially kidney diseases)
  • Clinical- related to primary cause but usually
    present with severe respiratory distress
  • Rx respiratory support and treatment of the cause

46
Neurological ds. causing RD
  • Werdnig Hoffman- usually in neonatal period as
    hypoplastic lungs/hypoventilation
  • Brachial plexus injury- with h/o shoulder
    dystocia and LGA, can be associated with Horner's
    Syndrome, chest fluoroscopy -eventration of the
    diaphragm
  • Injury after cardiac surgery-recurrent laryngeal
    nerve injury mild distress with stridor, direct
    laryngoscopy will show ipsilateral vc in
    cadaveric position with no movement with crying.

47
CARDIOLOGY
48
Congenital Heart Disease-Some Facts
  • Incidence 8/1000 live births (excluding PDA in PT
    newborns) with 25 with other associated
    abnormalities
  • VSD- commonest CHD
  • TOF- commonest cyanotic HD beyond neonatal period
  • TGA-commonest cyanotic HD in first week of life
  • HLHS- 2nd commonest cyanotic HD in first week of
    life and commonest cause of cardiac mortality
    during that period

49
Congenital cardiac disease
  • When to suspect-murmur, cyanosis with minimal
    respiratory distress
  • IMMEDIATE need for ECHO, consider starting
    PROSTAGLANDIN. 3-5 babies with PG have apnea-
    might require intubation

50
Presentation of CCHD
  • 5Ts, DO, ESP- TGA, TOF, TAPVR, Tricuspid
    atresia, Truncus arteriosus, DORV, Ebsteins
    Anomaly, Single ventricle and Pulmonary atresia
  • HLHS Vs sepsis usually HLHS presents after the
    duct is closed by 48-72 h and baby presents with
    cardiac failure to the ER with no murmur. If no
    high risk factors for infection always consider
    the diagnosis of HLHS

51
PDA
  • Normal course-physiological closure 12-15h ,
    anatomic closure several months, about 4 of
    term, 10 of 30-37 wk and 50 of lt30 wk do not
    close by 72 h and considered PDA
  • S/S-
  • Term-asymptomatic, machinery murmur, if large
    have bounding pulse, CHF
  • Preterm can also have decreased bf to the
    gut-NEC, Pulmonary hage. and prolonged
    intubation
  • Treatment-Fluid restriction, maintaining
    hematocrit, ibuprofen (indomethacin), surgical
    correction

52
Maternal conditions and CHD
  • Maternal drugs
  • Aspirin/Indomethacin-PH/PDA closure,
    Lithium-Ebsteins anomaly, Ethanol-VSD
  • Maternal diseases
  • Lupus-Cong. Heart block (anti Ro, anti La Ab),
    Diabetes (VSD-commonest, TGA, ventricular
    hypertrophy-most specific)

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55
NEUROLOGY
56
SCaLP Injuries
  • In SubCutaneous tissue- caput succedaneum, soft,
    crossed midline/sutures, usually with molding,
    resolves over several days
  • Beneath Galea Aponeurotica in Loose areolar
    tissue- subgaleal, can move to neck and behind
    ear, can cause anemia, hypotension, jaundice,
    resolves in 2-4 wk
  • SubPeriosteal- cephalhematoma, confined to suture
    lines, firm, 10 have skull fracture, jaundice,
    resolve in weeks to months

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59
Intraventricular Hemorrhage
  • From where-germinal matrix, usually involutes by
    34 wk
  • Incidence increases with PT e.g. lt1kg 30,
    1-1.25kg 15, 1.25-1.5kg 8
  • Timing- 50 in 24h and 90 in 72h present as S/S
    of anemia CNS involvement.
  • Grading- I thru IV
  • Prognosis- Poor in grade III/ IV
  • Complication- hydrocephalous/PVL

60
Periventricular Leukomalacia
  • Where- periventricular white matter usually focal
  • Risk factors-prematurity, hypotension, IVH
  • US shows bilateral periventricular echodensities
  • Outcome usually as spastic diplegia with
    associated cognitive and visual defects

61
Birth Asphyxia
  • Definition
  • pH lt7, ASlt3 at 5min, neurological sequelae (HIE),
    multiple organ dysfunction
  • HIE staging ( Sarnat Stages)
  • Stage 1-usually hyperactive CNS with
    sympathomimetic activity, 100 normal
  • Stage 2-decreased CNS activity, lost reflexes,
    parasympathomimetic activity, seizures, 80
    normal
  • Stage 3- variable presentation, seizures rare,
    burst suppression EEG, 100 severe sequelae

62
Cerebral palsy
  • Non progressive neurological deficit
  • Incidence2-5/1000, increase with dec. GA
  • Clinicopathological correlation
  • Selective neuronal necrosis-common after HIE,
    diffuse damage, Quadriplegia, MR, seizures
  • Parasagittal cerebral injury-due to dec.
    perfusion, necrosis in watershed areas of
    carotids, weakness of proximal muscles UgtL
  • Focal or multifocal ischemia- usually in FT,
    meningitis, trauma, thrombotic syndromes, as
    hemiplegias, seizures, cognitive defects
  • Status Marmoratus- kernicterus, HIE, basal
    ganglia, spasticity, choreoform movements

63
Brachial plexus injury
Erb-Duchenne Klumpke
Roots C5-7 C8-T1
Incidence Common Rare
Typical S/S Waiters tip Ape hand
Differentiate Palmer grasp -
Associated C4/5 (phrenic nerve), C7 (scapular winging) T1( Horners syndrome)
64
Neural Tube Defect
  • Incidence variable geographically, more with
    folic acid def., maternal diabetes, valproate
    intake
  • Intrauterine diagnosis- inc. alpha fetoprotein
    and cholinesterase in amniotic fluid
  • Prognosis-level of involvement e.g. no ambulation
    with cervical, thoracolumbar involvement and with
    sacral can ambulate without braces

65
Hydrocephalous
  • 2 types
  • Obstructive- common, commonest cause is post
    hemorrhagic HC, others are aqueductal stenosis,
    Dandy-Walker Syndrome (cystic dilatation of 4th
    ventricle, 70 have other abnormalities), rarely
    masses
  • Communicating-usually after bleeds, infections,
    NTD, Arnold Chiari malformation

66
Case
  • Mother is rushed in for stat c/s for abruption.
    Apgars are 2, 3, 8- improving after IPPV, chest
    compression and fluid resuscitation. Improves and
    admitted to NICU. Mother had uneventful antenatal
    course. Baby develops generalized tonic clonic
    seizures and bradycardia at 12h of life-
    controlled by phenobarb.
  • WHAT IS THE CAUSE OF THE SEIZURES

67
Siezures
  • Types
  • Subtle-most frequent, oral, facial ocular
    activity, may be associated with changes in HR,
    resp. BP and sats.
  • Multifocal clonic-one limb migrating to another
  • Focal clonic- may represent focal disease
  • Tonic-change in posture, more in preterm
  • Myoclonic
  • Many causes- usual asphyxia, metabolic (Ca,
    glucose etc), infection, trauma, malformation
  • Initial drug of choice is phenobarb

68
METABOLIC/ENDOCRINE
69
Inborn errors of metabolism
  • When to suspect just about any S/S specially if
    the initial usual diagnosis e.g. sepsis is not
    responding to the usual forms of treatment
    e.g.antibiotics.
  • Specific smells
  • Sweaty feet-Isovaleric acidemia/Glutaric aciduria
  • Male cat urine-Glycinuria
  • Maple syrup odor-Branched chain aa(MSUD)
  • Musty odor-PKU

70
Flow chart of IEM-with hyperammonemia
  • Acidosisketonuria-MSUD, Lactic acidemia,
    glutaric aciduria, glycinuria
  • Acidosis without ketonuria-Fatty acid oxidation
    problem
  • Without acidosis or ketonuria-urea cycle defects,
    check citrulline
  • Very high-Argininosuccinic Acid (ASA) Synth. Def.
  • Absent- check urine orotic acid
  • High-Ornithine transcarboxylase def.
  • Low/Normal-Carbamyl phosphatase synthetase def.,
    N-acetyl glutamate def.
  • Normal or slight increase-check ASA
  • Present-AS acid lysase def
  • Absent-check arginine, if inc.-arginase def., if
    normal or low-transient neonatal hyperammonemia

71
Galactosemia
  • AR, galactokinase or Galactose-1-PO4ase
    uridyltransferase def.
  • Presents when feeds are introduced as lethargy,
    hepatomegaly, liver failure, renal tubular
    acidosis
  • Can have cataract at birth
  • Increased risk of infection specially E.coli
  • Lab-elevated LFTs, galactose in urine (reducing
    substance positive with negative glucose oxidase
    test )
  • Rx- elimination of all galactose and lactose in
    diet

72
PKU and Homocystinuria
  • Classic PKU-AR, def. of phenylalanine
    hydroxylase, mousy or musty urine odor, severe MR
    and siezures if untreated, diagnosed by NBS, Rx
    by low phenylalanine diet
  • Homocytinuria-AR, commonly by def. of
    cystathianine synthetase, usually asymptomatic in
    neonatal period, has downward dislocated lens
    (D/D Marfans), myopia, osteoporosis, scoliosis,
    arachnodactyly, dec.joint mobility (D\D Marfans),
    MR seizures, thrombotic episodes, Rx Dec.
    methionine, supplement cytiene, folate, pyridoxine

73
Glycogen storage disease
  • 8 types, 1,2 3 are common
  • Type1 von Gierke, Glucose 6 PO4ase def., has
    lactic acidosis, hepatomegaly, diarrhoea,
    bleeding disorder, poor prognosis
  • Type 2-Pompes, lysosomal glucosidase def., muscle
    weakness, cardiomegaly, CHF, poor prognosis
  • Type 3- Forbes, low glucose, hepatomegaly, muscle
    fatigue, usually after neonatal period, good
    prognosis

74
MPS and Lipidoses
  • MPS- dysostosis multiplex, AlderRielly bodies in
    WBC and urine MPS
  • Hurler-iduronidase def., cloudy cornea, HSM,
    coarse features, short stature, kyphosis
  • Hunters-iduronidase sulfatase def., Xlinked, only
    MPS with retinal abn.
  • Lipidoses
  • Gauchers-glucocerebrosidase def., Gaucher cell in
    bone marrow, normal retina, type I-normal CNS,
    type II, profound CNS loss
  • Niemann Pick-sphignomyelinase def., foam cells in
    bone marrow, Type A cherry red spot, profound CNS
    loss, Type B normal retina, normal CNS

75
Temperature Regulation
  • Neonates more prone to heat loss as
  • Dec. skin thickness-radiantconductive loss
  • Dec. subcut. Fat
  • Dec. peripheral vasoconstriction leading to dec.
    heat conservation
  • Immature autonomic nervous system
  • Increased BSA to wt.-radiant heat loss
  • Convective Incubators-large radiant loss(dec by
    double wall) small evaporative loss(dec by inc.
    humidity) and small conductive heat loss (dec by
    rubber mattress)
  • Radiant Warmer-large convective and evaporative
    loss, reduced by plastic sheet

76
Hypothyroidism
  • Commonest cause-thyroid dysgenesis
  • Early presentations-prolonged jaundice, large
    post. fontanelle
  • Others-umbilical hernia, macroglossia, hypotonia,
    goiter
  • Diagnosis- by newborn screening-low T4 and high
    TSH
  • Rx-levothyroxine sodium

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78
Case
  • Baby delivered after difficult vaginal delivery
    to a mother with gestational diabetes poorly
    controlled by insulin. Baby was 4300g and was
    send to WBN where he developed tachypnea with
    occasional jitteriness.
  • WHAT IS THE D/D FOR TACHYPNEA IN THIS BABY?

79
Hypoglycemia- IDM
  • Commonest presentation of IDM and can primarily
    present as tachypnea (Other causes are RDS, TTN,
    CHD (VSD) , birth asphyxia birth trauma and
    hypocalcemia)
  • Other common presentations are hypocalcemia,
    polycythemia and jaundice
  • Specific malformations-Hypertrophic Obstructive
    Cardiomyopathy d/t asymmetrical ventricular
    septal hypertrophy and caudal agenesis syndrome

80
Hypocalcemia
  • Types
  • Early (till 72h) maternal causes (DM,
    hyperparathyroidism), perinatal causes
    (prematurity, asphyxia, infections)
  • Late (after 72h) hypoparathyroidism,
    hypomagnesemia, vitamin D def.
  • S/S-If symptomatic as jitteriness, high pitched
    cry, Chvostek/Trousseau sign, siezures, prolonged
    QTc
  • Rx- Underlying cause, Ca, Vit. D, low PO4

81
Congenital Adrenal Hyperplasia
  • Commonest cause-21 hydroxylase def. (followed by
    11beta hydroxylase def.)
  • S/S with 21OH-salt wasting in 2nd week with
    inc.K, dec.Na and hypotension with
    pseudohermaphroditism in females and males may
    have precocious puberty.
  • Diagnosis-elevated 17OHP in amniotic fluid or
    serum
  • Rx-Antenatal-maternal glucocorticoid,
    Postnatal-replacement of GC/MC

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HEMATOLOGY
84
Cord compression
  • Usually presents as variable decelerations
  • Most common presentation is anemia due to mild to
    moderate compression compresses the umbilical
    vein leading to pooling of the blood in the
    placenta leading to anemia usually seen in CBC
    done 4-6h of life.

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Twin to twin transfusion(TTT)
  • Discordant twins when the weight of the twins
    differ by 15-25
  • One of the causes of DT is TTT
  • Usually TTT takes place in monochorionic, mono or
    di-amniotic twins, mortality can be 50
  • Donor twin is anemic and develops hypovolemia and
    oligohydramnios
  • Recipient twin is polycythemic and may develop
    hydrops
  • When donor dies- blood flows from recipient
    leading to its demise soon after the donors hence
    need for STAT c/s

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Isoimmunization-Rhesus factor
  • Decreased incidence due to use of Rhogam
  • Mother is Rh- i.e. dd (Rhesus An has 3 components
    C, D, E with D as the major component). Baby is
    Rh i.e.DD or Dd.
  • Initial pregnancy usually induces IgM which do
    not cross placenta, but repeat exposure induces
    IgG which crosses placenta easily causing
    hemolysis of fetal RBC.
  • Prevented by giving Rhogam to Rh-mother at 28 wk
    GA and at birth of Rh baby.

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Isoimmunization-ABO
  • Incidence same in first or subsequent pregnancies
  • Mothers with group A or B produce IgM antibodies
    and that of O produce IgG which easily crosses
    placenta
  • Usually milder than Rh as the antigen is on all
    the tissues and they capture the antibodies
    transferred from the mother
  • Usually indirect Coombs is positive
  • Has spherocytosis with B-O incomp.

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AAP guidelines for bili management
  • To measure bilirubin in hours of life
  • Aggressive phototherapy and specific follow up
    depending on the zone in the hourly bilirubin
    charts

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Phototherapy
  • Mechanism of action
  • Photo-isomerization-configurational 4Z15Z to
    4Z15E
  • Photo-isomerization-structural-lumibilirubin
  • Photo-oxidation
  • Blue light- effective wavelength (710-780nm) and
    penetrates skin well.
  • If phototherapy given to baby with high direct
    bilirubin -bronze baby syndrome

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Jaundice related to breast feeding Vs Breast
feeding jaundice
  • Jaundice related to BF
  • Usually exaggerated physiological jaundice due to
    decreased intake
  • BF jaundice
  • Prolonged with peak of 20-30mg/dl by 2 wk and
    than normalize over 4-12 wk
  • Rapid decrease after cessation of breast feeding
    and rises 2-4mg/dl after resuming BF
  • Can cause kernicterus

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Thrombocytopenia
  • Sick Vs Well baby
  • Commonest for well babies is Alloimmune and
    Autoimmune, and for sick babies is sepsis/DIC
  • Autoimmune
  • Transference of antiplatelet antibodies as that
    of lupus, ITP
  • Maternal and newborns platelets are low
  • Alloimmune
  • Transplacental transference of maternal
    antibodies ( like Rh disease), with normal
    maternal platelets
  • Severe, can have IC bleed, death in 20

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Case
  • BB delivered at home with precipitous delivery
    was admitted to WBN and discharged with mother.
    Was exclusively breast fed. Found to have fresh
    bleeding per rectum on DOL 4. There is no other
    site of bleeding or pertinent history. Clinical
    examination is normal.
  • WHAT NEEDS TO BE DONE IMMEDIATELY?

95
Hemorrhagic Disease of Newborn (Vit.K def.)
  • Vitamin K needed for carboxylation reaction which
    activates the clotting factors
  • Newborns are predisposed as have no bacterial
    flora intestine to produce Vit.K and also has
    liver immaturity
  • Decreased placental transference if maternal
    intake of anticonvulsants, warfarin and ATT or
    when exclusively breast fed.
  • Types
  • Early-less than 24h, maternal drugs
  • Classic- 2-7days, exclusive breast feeding
  • Late-2w-6m, hepatobiliary disease, inc.ICT

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GASTROENTEROLOGY
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Esophageal atresia
  • 30-40 with associated abnormalities specially
    as VACTERL
  • 4 types-commonest upper end atresia and lower
    end with fistula to the trachea
  • H type is rare but commonest one for the exams-
    S/S as cough during feeding and recurrent
    aspirations
  • Rx-surgical-primary or delayed as in stages.

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Case
  • Baby is delivered to mother with history of
    polyhydramnios. Mother had irregular antenatal
    care. Baby did not tolerate feeds and started to
    have non bilious vomiting. OG tube could be
    passed to the stomach. AXR was ordered.
  • WHAT WOULD BE THE LIKELY AXR PICTURE

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Double bubble sign
  • Associated with duodenal atresia
  • High rate of association with trisomy 21, other
    malrotation and CHD.

101
Case
  • 24 week PT AGA BG has had relatively uneventful
    course in NICU. At 8 weeks of life when
    tolerating full feeds developed abdominal
    distention. Feeds were stopped and AXR showed
    pneumotosis intestinalis. Antibiotics were given
    and no surgical intervention was required. Baby
    recovered.
  • WHAT IS THE COMMONEST SEQUALAE THAT NEEDS TO BE
    WATCHED FOR?

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Necrotising Enterocolitis
  • 10 of those less than 1500g
  • Predisposing factors-prematurity, feeds,
    infection
  • AXR- pneumotosis intestinalis
  • Outcome-high mortality, morbidity-small gut
    syndrome if surgery is done, strictures if
    medically treated

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Congenital Hyperplastic Pyloric Stenosis
  • 3/1000 births, male x5
  • Related to decreased NO production
  • Hypochloremic, hypokalemic, metabolic alkalosis
  • Barium-string sign, US-bulls eye sign
  • Rx-Pyloromyotomy

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Case
  • 2D old FT Baby in WBN develops distention and
    has not passed meconium since birth. PMD orders
    an AXR which shows large dilated stacked loops
    with absence of air in the recto-sigmoid region.
  • WHAT IS THE NEXT STEP?

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Case continued
  • If it reveals gradual narrowing of the sigmoid
  • WHAT IS THE LIKELY DIAGNOSIS AND HOW TO CONFIRM
    IT?

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Hirschsprungs Disease
  • 15000, usually male, 80 rectosig. only
  • Associated with trisomy 21
  • Failure of cranial to caudal migration of neural
    crest cell-dec. parasymp. innervation
  • Diagnosis-XR, biopsy-absent ganglion cells
  • Complication-Acute bacterial colitis
  • Rx- single stage pull through or initial
    colostomy followed by correction

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Meconium Plug vs. ileus vs. peritonitis
  • Plug
  • benign variation of Hirschsprungs disease
  • Delayed passage of meconium
  • Usually has small colon, IDM
  • Ileus
  • 90 have CF
  • bilious vomiting, obstruction, AXR-bubbles in the
    intestinal lumen
  • enema successful in 60
  • Peritonitis
  • In utero perforation
  • Secondary to ileus, atresia, volvulus,
    gastroschisis
  • Usually seal spontaneously or can require surgery

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Omphalocele vs. Gastroschises
Omphaloc. Gastroch.
Incidence Common Rare
Chrom. Abn. Common Rare
Midline Yes No (usu.Rt)
Covering Yes No
Umb.cord Involved Normal
Assoc.Abn. More Less-intest.abn
M/M More Less
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DERMATOLOGY
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ERYTHEMA TOXICUM
  • Most common 30-70
  • Onset DOL 2-3
  • 1-3mm erythematous macule/papule-pustule
  • Fades in 5-7 days
  • May reoccur
  • Benign, has eosinophils

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PUSTULAR MELANOSIS
  • More in dark skinned
  • 3 stages-non-inflammatory pustule, ruptured
    pustule with scale, hyperpigmented macule (up to
    3m)
  • Benign, has neutrophils
  • No Rx

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Mystery Case
  • Baby boy born with oligohydramnios, Potters
    Sequence, and hypoplastic lungs requiring ECMO.
    Had B/L hydronephrosis diagnosed antenatally and
    the voiding cystourethrogram shows-

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