PEDS 2 Flashcards by vanessa jasmin

EISENMENGER SYNDROME

pulmonary HTN
reversal of flow (L to R shunt becomes R to L)
cyanosis

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Investigations for CHD

2D-echocardiography

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RV (parasternal) heave
Fixed, widely split S2
Systolic ejection murmur at the pulmonic area

Ostium secundum -most common; often an isolated defect
Ostium primum - often associated with other cardiac defects and Down syndrome

ASD

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holosystolic murmur, LLSB
High pitched, harsh
The smaller the defect, the louder the murmur
Usually not audible until age 4-10 days
↑Flow through the MV: diastolic rumble, apex (large defect)

VSD

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Associated conditions with PDA

fetal alcohol syndrome: low palpebral fissure, thin lip,
VSD
congenital rubella
neonatal RDS

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Respiratory distress
Bounding arterial pulses +3 always R/O
*Paradoxical splitting of S2 during expiration
Murmur continuous, rough, machine-like (‘to and fro’)
maximal at the 2nd L ICS
*Disappears with ↑pulmonary vascular resistance

PDA

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Associated conditions with Coarctation of the Aorta

Turner syndrome

Bicuspid aortic valve

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UE hypertension systolic BP in the arms are > 20 mmHg higher than the legs
Enlarged and pulsatile collateral vessels
ICS anteriorly, in the axilla, or posteriorly in the interscapular area
(femoral < brachial pulses)
bounding pulses in UE and carotids +3 above coarctation
Cardiac: harsh systolic murmuralong the left sternal border + systolic murmuralongL and R side of the chest with thrills

Coarctation of the Aorta

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Extreme cyanosis in the first 24 hours of life without respiratory distress
Single S2
CXR: egg-on-a-string appearance

Transposition of the Great Vessels

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Downward displacement of dysplastic tricuspid valve into a hypoplastic RV
Early or late cyanosis
Right heart failure
Mom used Lithium
S1 is widely split with loud tricuspid component
Holosystolic murmur of TR

Ebstein Anomaly

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APGAR chart

pg 599 in FA

PPT SLIDE2

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No vitamin K at birth

Exclusively breastfed

Classic VKDB

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Maternal ingestion of drugs** that interfere with vitamin K metabolism

Early VKDB

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Chronic diarrhea
Prolonged antibiotic therapy
Exclusively breastfed

Late VKDB

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Necrotizing Enterocolitis (NEC) risk factors

Born at < 35 weeks

formula feeding

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NEC Clinical features

What do you see on X RAY?

*Abdominal distension
emesis
Blood in stools
*Hypothermia, temperature instability
Lethargy
Apnea, dyspnea

CXR- Pneumatosis intestinalis
AIR IN THE BOWEL WALL/ ABDOMEN

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Umbilical Hernia prognosis ?

Small umbilical hernias < 1.5 cm = Spontaneous closure
Large umbilical hernias - Spontaneous closure less likely
Surgery by age 5 if persistent

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risk factors of Gastroschisis

young maternal age
prenatal use of illicit drugs (metamphetamines, cocaine)
prenatal use of cyclooxygenase inhibitors (aspirin, ibuprofen

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associations with Gastroschisis

intestinal atresia (10-20%)
intrauterine growth retardation (IUGR)

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prenatal dx of gastroschisis

Second trimester ultrasound (>95% sensitive)
Elevated maternal serum α-fetoprotein: 
Open NTDs
Ventral wall defects
Multiple gestation

Omphalocoele risk factors

> 50% have an abnormal karyotype or other defects
Edwards syndrome (Trisomy 18)
Patau syndrome (Trisomy 13)
Congenital heart disease
Neural tube defects

Defect at linea alba covered by skin
Sometimes contains bowel
Umbilical cord inserts at apex of defect

Umbilical hernia

Defect to the right of the cord insertion
Contains bowel
Umbilical cord inserts next to defect

Gastroschisis

Midline abdominal wall defect covered by peritoneum
Contains multiple abdominal organs
Umbilical cord inserts at apex of defect

Omphalocele

risk factors for Hypertrophic Pyloric Stenosis (HPS)

Firstborn male (M: F = 4:1) Caucasian Parental Hx of HPS (higher if mother affected) Use of erythromycin in the neonatal period

progressively forceful/ projectile postprandial non-bilous vomiting ‘Olive-shaped’ abdominal mass Hyperperistalsis SUNKEN FONTANELLE, JAUNDICE, LOSS OF SKIN TURGOR

Hypertrophic Pyloric Stenosis (HPS)

labs and investigation for HPS

Hypochloremic hypokalemic metabolic alkalosis ↑pH, PaCO2 and HCO3 ↓K and Cl U/S: sausage mass/donut sign

Hirschsprung Disease is Strongly associated

with Down syndrome

No meconium passage in the first 48 hours Bilious emesis Enterocolitis in 50%: Rectal exam Extremely tight anal sphincter ‘Squirt sign’ or no stool in rectal vault

Hirschsprung Disease

Signs of obstruction in Hirschsprung Disease on Xray

dilated bowel loops | absence of gas in the rectum

Suction rectal biopsy - Definitive Dx of ?

Hirschsprung Disease

``` PATH: pulmonary hypoplasia Respiratory distress at birth Asymmetric chest movements Tracheal deviation Flat/ scaphoid/ concave abdomen L-sided hernia: Absent breath sounds; may hear bowel sounds ```

Congenital Diaphragmatic Hernia (CDH)

Complications of Congenital Diaphragmatic Hernia**

In utero: polyhydramnios Postnatal: Pulmonary HTN Pneumotharax

CXR findings of Congenital Diaphragmatic Hernia

herniation of bowel loops into the left hemithorax | cardiac and mediastinal shift to the right

delayed clearance of fetal lung fluid mild pulmonary edema self-limited disease

Transient Tachypnea of the Newborn (TTN)

Risk factors of TTN (4)

full term or late preterm born following a short labor or C-section without labor diabetic mother/ BW > 4500 g maternal asthma

CXR for TTN

Interstitial and alveolar edema | Fluid in the interlobar fissures

How long does it take TTN to resolve ?

Clinical resolution in 48 to 72 hours

Congenital pneumonia causitive agents

group B streptococci Escherichia coli Ureaplasma urealyticum Staphylococcus epidermidis

Labs of congenital pneumonia

CBC: absolute neutropenia (< 2000/mL) C-reactive protein: elevated gram stain of tracheal aspirate

CXR of congenital pneumonia

nodular or coarse patchy infiltrates diffuse haziness or granularity air bronchogram signs

risk factor for Meconium Aspiration Syndrome (MAS)

post-term delivery ( ↑risk after 41 weeks AOG)

What happens when the meconium gets into the lungs of neonate ?

Deactivation of surfactant - respiratory distress

``` low APGAR score/ depression meconium + blood in amniotic fluid barrel chest - bc of obstruction crackles; coarse breath sounds meconium-stained skin ```

Meconium Aspiration Syndrome (MAS)

complication of Meconium Aspiration Syndrome (MAS)

PPHN (persistent pulmonary HTN of the newborn)

rupture of the visceral pleura

Primary Spontaneous Pneumothorax

risk factor for Primary Spontaneous Pneumothorax

intubated given too much O2 meconium aspiration

clinical feat of Primary Spontaneous Pneumothorax (4)

tachypnea hyperresonance and ↓breath sounds on the affected side heart sounds may be distant/ shifted to the opposite side subcutaneous emphysema

CXR of Primary Spontaneous Pneumothorax

``` Pneumothorax air in the pleural space hyperlucency of vascular/ lung markings Pneumomediastinum Atelectasis Tracheal/ mediastinal shifting* ``` angel wing sign