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

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

Rastogi’s Rule

• Common presentations of common diseases • Rare presentations of common diseases • Common presentations of rare diseases • Rare presentations of rare diseases

FETAL WELL BEING

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

Electronic Fetal Heart Rate Monitoring

• Normal – FHRv of 6-15bpm, basis of nonstress test-reactive positive test is normal • Abnormal patterns – Tachycardia, >160, infection – Bradycardia, <110, 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

RESUSCITATION

Resuscitation-recent changes

• No intrapartum meconium suctioning • Oxygen use – Can use <100% but if no improvement in saturations, increase to 100% – If <32w 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

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?

Case continued

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

INFECTIONS

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

GBS

• Risk Factors: – Intrapartum Prophylaxis to mother -if GBS positive

OR

unknown with <35 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 >18h/duration of IP less than 4h.

• Clinical presentation – Early-<7DOL, pneumonia>sepsis>CNS, ascending infection – Late onset->7DOL, less mortality but more CNS involvement and sequelae, deep infection as cellulitis, arthritis, osteomyelitis

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: <7d, transplacental, chocolate colored amniotic fluid, preterm deliveries, sepsis/pneumonia – Late onset: >7d, nosocomial, meningitis with mononuclear cells

Other congenital infections

Clinical presentation LBW Liver/Spleen Jaundice Petechiae CHD Cataract Retinopathy Cerebral calcification Microcephaly CMV

+++ ++++ +++ +++

+ +

Periventricular+

++ Rubella ++ ++ + ++

+++ +++

Toxoplasmosis + + ++ + +

+++ cortical++

+

Hepatitis B positive mother

Mat. status Newborn >2kg + unknown HBV 3 doses, 1 st <12h 3 doses, 1 st <12h HBIG 1 dose, <12h 1 dose, <7d Newborn <2kg HBV 4doses, 1 st <12h 4doses, 1 st <12h HBIG 1dose, <12h 1dose, <12h _ 3 doses, 1 st at1-2M None 3 doses, 1 st at1-2M None

Congenital Syphilis

Mother VDRL Baby + + + +/ Mother FTA Baby + + + + Inference No or prozone False+ Mother/B disease Treated disease

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

CHROMOSOMAL ABN.

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

Trisomy 21

• Defects – Cardiac (40-50%)-Endocardial cushion defect, VSD – Extremities-single palmer crease, 5 th 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

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 2 nd over 3 rd and 5 th over 4 th finger, rocker bottom feet, small mouth/eyes/palpebral fissures, short sternum, hernia, cryptorchidism

PULMONARY

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?

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

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

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

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?

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

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

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

Apnea

• Cessation of air flow for >20s 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

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

Meconium aspiration syndrome

• Definition: MSAF+RD+CXR changes • Clinical: usually post-term, severe respiratory distress • Complications:pulmonary hypertension, airleak syndromes • CXR:snow storm appearance • Prevention: ??intrapartum suction of meconium • Rx: respiratory support, correcting acidosis, antibiotics, surfactant

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?

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

Congenital diaphragmatic hernia

• Types-Posterolateral thru Foramen of Bochdalek (L>>R) 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

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

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.

CARDIOLOGY

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- 2 nd commonest cyanotic HD in first week of life and commonest cause of cardiac mortality during that period

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

Presentation of CCHD

•

5T’s, DO, ESP

-

T

GA,

T

OF,

T

APVR,

T

ricuspid atresia,

T

runcus arteriosus,

DO

RV,

E

bstein’s Anomaly,

S

ingle ventricle and

P

ulmonary 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

PDA

• Normal course-physiological closure 12-15h , anatomic closure several months, about 4% of term, 10% of 30-37 wk and 50% of <30 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 h’age. and prolonged intubation • Treatment-Fluid restriction, maintaining hematocrit, ibuprofen (indomethacin), surgical correction

Maternal conditions and CHD

• Maternal drugs – Aspirin/Indomethacin-PH/PDA closure, Lithium Ebstein’s anomaly, Ethanol-VSD • Maternal diseases – Lupus-Cong. Heart block (anti Ro, anti La Ab), Diabetes (VSD-commonest, TGA, ventricular hypertrophy-most specific)

NEUROLOGY

S

C

a

LP

Injuries

• In Sub

C

utaneous tissue- caput succedaneum, soft, crossed midline/sutures, usually with molding, resolves over several days • Beneath Galea Aponeurotica in

L

oose areolar tissue- subgaleal, can move to neck and behind ear, can cause anemia, hypotension, jaundice, resolves in 2-4 wk • Sub

P

eriosteal- cephalhematoma, confined to suture lines, firm, 10% have skull fracture, jaundice, resolve in weeks to months

Intraventricular Hemorrhage

• From where-germinal matrix, usually involutes by 34 wk • Incidence increases with PT e.g. <1kg 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

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

Birth Asphyxia

• Definition – pH <7, AS<3 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

Cerebral palsy

• Non progressive neurological deficit • Incidence:2-5/1000, 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 U>L – 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

Brachial plexus injury

Roots Incidence Typical S/S Differentiate Associated Erb-Duchenne C5-7 Common Waiters tip Palmer grasp + C4/5 (phrenic nerve), C7 (scapular winging) Klumpke C8-T1 Rare Ape hand T1( Horner’s syndrome)

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

Hydrocephalous

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

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

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

METABOLIC/ENDOCRINE

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

Flow chart of IEM-with hyperammonemia

• Acidosis+ketonuria-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

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 LFT’s, galactose in urine (reducing substance positive with negative glucose oxidase test ) • Rx- elimination of all galactose and lactose in diet

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

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

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

Temperature Regulation

• Neonates more prone to heat loss as – Dec. skin thickness-radiant+conductive 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

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

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?

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

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

Congenital Adrenal Hyperplasia

• Commonest cause-21 hydroxylase def.

(followed by 11beta hydroxylase def.) • S/S with 21OH-salt wasting in

2 nd inc.K, dec.Na and hypotension week

with pseudohermaphroditism in females and males may have precocious puberty.

with • Diagnosis-elevated 17OHP in amniotic fluid or serum • Rx-Antenatal-maternal glucocorticoid, Postnatal-replacement of GC/MC

HEMATOLOGY

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.

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

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.

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.

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

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

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

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%

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?

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

GASTROENTEROLOGY

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.

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

Double bubble sign

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

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?

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

Congenital Hyperplastic Pyloric Stenosis

• • 3/1000 births, male x5 • Related to decreased NO production

Hypochloremic, hypokalemic, metabolic alkalosis

• Barium-string sign, US-bull’s eye sign • Rx-Pyloromyotomy

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?

Case continued

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

Hirschsprung’s Disease

• 1:5000, 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

Meconium Plug vs. ileus vs. peritonitis

• Plug – benign variation of Hirschsprung’s 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

Omphalocele vs. Gastroschises

Omphaloc.

Incidence Common Chrom. Abn. Common Midline Covering Umb.cord

Yes Yes Involved Assoc.Abn.

More M/M More Gastroch.

Rare Rare No (usu.Rt) No Normal Less intest.abn

Less

DERMATOLOGY

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

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

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-