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1

bronchopulm displasia

chronic complication of RDS; pathologic changes that affect lung growth

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diagnosis of bronchopulm dysplasia

mechanical ventilation during the first 2 weeks of life; clinical signs of respir compromise persistent beyond 28 days of life; need for supplemental oxygen beyond 28 days of life; characteristic CXR

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prognosis for infant with RDS

with aggressive treatment in the NICU, >90% survive

4

persistent pulm hypertension of the newborn occurs most freq in what GA?

full term or post-term

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most common causes of persistent pulm hypertension of the newborn

perinatal asphyxia and meconium aspiration syndrome

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hypoxemia is a potent pulm vasoconstrictor

which explains why perinatal asphyxia is a cause of pulm hypertension

7

ECMO

extra-corporeal membrane oxygenation

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inhaled NO

may be used as a potent pulm vasodilator

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what does CXR look like in infants with meconium aspiration syndrome?

increased lung volume with diffuse patchy areas of atelectasis and pulm infiltrates alternating with areas of hyperinflation

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a couple of bad consequencees of meconium aspiration syndrome?

pneumothorax and pneumomediastinum; also persistent pulm htn of the newborn, bacterial pneumonia, and lont term reactive airway disease

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apnea of prematurity

resp pause lasting 15-20 sec OR resp pause of any duration resulting in cyanosis or ox desat as evidenced on pulse ox

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most frequent type of apnea in the newborn

mixed (of central and airway obstruction), not either one on its own

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incidence of idiopathic apenea of prematurity

frequency increases with decreasing gestational age; as high as 85% in infants less than 28 weeks and 25% in infants 33-34 weeks

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management of apnea

respiratory stimulant medications (caffeine or theophylline), ventillation as needed, CPAP as needed

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direct bilirubin

bilirubin is conjugated in the liver; most of it goes into bile and into the small intestine

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indirect (unconjugated)bilirubin

this is the bilirubin as a result of hemoglobin breakdown; it has not gone to the liver yet

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physiologic jaundice

often occurs during the first week of life; most freq caused by indirect (unconjugated) hyperbilirubinemia

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visible jaundice occurs at what bilirubin level

5 mg/dL

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direct hyperbilirubinemia

when the conjugated form is greater than 15% of the total bilirubin level; this is always pathologic in neonates

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breastfeeding jaundice

causes indirect hyperbilirubinemia; occurs within the first week; related to suboptimal milk intake; decreased stooling leads to decreased passage of bilirubin in the stool; note that breastfeeding is associated with higher peak bilirubin levels than formula feeding

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breast milk jaundice

causes indirect hyperbilirbinemia; typically occurs after the first week; likely related to breast milk's high levels of beta-glucuronidase and high lipase

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when should jaundice be evaluated

when it appears in a baby less than 24 hours after birth; when bilirubin rises more than 5-8 mg/dL in a 24 hour period; the rate of rise of bilirubin exceeds 0.5 mg/dL per hour (suggestive of hemolysis)

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exchange transfusion

performed for rapidly rising bilirubin levels secondary to hemolytic disease

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when indirect bilirubin gets so high that it passes through the BBB, where dies it most freq localize? Note that it is Indirect that gets into the brain

basal gang, hippocampus, and brainstem nuclei

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clinical features of bilirubin getting into your brain

choreoathetoid cerebral palsy, hearing loss, opisthotonus, seizures, and oculomtor paralysis

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most common signs in infants with drug abusing mothers

jitteriness and hyperreflexia, with irritability, tremulousness, feeding intolerance, and excessive wakefulness

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what is the most common type of esophageal atresia?

atresia of the esopagus (proximal pouch) with a distal tracheoesophageal fistula

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associated malformations are found in what percent of infants with esoph atresia

50%

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most cases of diaphragmatic hernias involve which side of the diaphragm?

the left

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scaphoid abdomen

abdominal contents in the abdomen; sign of diaphragmatic hernia

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omphalocele

abdominal organs covered with a peritoneal sac herniate centrally through the umbilical ring area; there is an increased risk of other congenital anomalies

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gastroschesis

fissure of the anterior abdominal wall (usually right side) ; there is no peritoneal sac covering the bowel is the only viscera that herniates; no increased risk of other congenital anomalies

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what is the most common type of intestinal obstruction?

intestinal atresia

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volvulus

loops of intestine twist, can lead to restricted circulation and gangrene;

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hirschrpung disease- more common in males or females?

males

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diagnosis of hirschsprung disease

rectal biopsy showing absence of ganglion cells

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management of hirschsprung

resection of the affected segment

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NEC clinical features

abdominal distension and tenderness; residual gastric contents, billous aspirate, bloody stools, abdominal erythema

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NEC may lead to what?

thrombocytopenai, DIC, and death

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classic radiograph findings in NEC

abdominal distension, air-fluid levels, thickened bowel walls, pneumatosis intestinalis (air in the bowel wall) and venous portal gas

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pneumoperitoneum

suggestive of NEC that perforated

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definition of hypoglycemia

serum glucose less than 40 mg/dL

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small left colon syndrome

condition that occurs exclusively in infants of diabetic mothers; they present with abdominal distension and failure to pass meconium due to decreased caliber of left colon

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definition of polycythemia

central venous hematocrit greater than 65% (22-ish hgb)

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increased risk of NEC with polycythemia

TRUE

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management of polycythemia

partial exchange transfusion, in which blood is removed and replaced with saline

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cause of direct hyperbilirubinemia

choledochal cyst (cystic dilation of the bile duct)

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soap bubble on abdominal radiograph

meconium ileus

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fragile x syndrome

unstable repeat sequences (number of specific nucleotide copies within a gene increases)

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genomic imprinting

the expression of the gene depends on which parent you inherited that chromosome from; example is 11q region on chrom 15 (prader willi vs angelman)

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marfan syndrome

autosomal dominant connective tissue disorder; affects the ocular, cardiovascular, and skeletal systems; gene defect is fibrillin on chrom 15; decreased upper to lower segment body ratio

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ocular findings in marfans syndrome

upward lens subluxation and retinal detachment

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diagnosis of marfans?

mostly on clinical findings, but, interestingly, homocystinuria has similar findings so you need to rule that out first;

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marfans syndrome patients are at increased risk for endocarditis

right

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prader willi genetics

absence of paternally derived part of chrom 15

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craniofacial findings of prader willi

almond shaped eyes and fishlike mouth

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growth problems in prader willi syndrome

failure to thrive initially followed by obesity as a result of hyperphagia; also short stature with small hands and feet

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other features of prader willi

hypogonadism, hypotonia (in infancy that can lead to poor feeding), mental retardiation

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what percent of alveolar development occurs after birth?

90 percent

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alveoli increase in number until what age

8 yo

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three reasons that infants are more prone to resp problems than older kids and adults

smaller air passages, less compliant lungs with more compliant chest wall, less efficient pulm mechanisms

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inspiratory stridor suggests what kind of obstruction?

extrathoracic

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two examples of extrathoracic obtruction

croup and laryngomalacia

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what is laryngomalacia

softening of the laryngeal cartilage that collapses into the airway, esp when patient is in a supine position

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expiratory wheezing suggests what?

intrathoracic obstruction like asthma and bronchiolitis

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crackles or rales suggest what

parenchymal disease like pneumonia or pulm edema

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epiglottitis most common in what age? What gender?

2-7 year olds; equal in boys and girls

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epiglottitis is caused by what?

HIB (hemophilus influenzae type B) was most common; also group A strep, strep pneumonia, and staph

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clinical features of epiglottitis

abrupt onset of upper airways disease without prodrome

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what do lab studies of epiglottitis reveal?

leukocytosis with left shift; blood culture will show HIB

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what is classix xray finding of epiglottitis?

thumb print epiglottis on lateral x ray

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what should the airway look like?

cery red swollen epiglottis

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management of epiglottitis

medical emergency! Controlled nasotracheal intubatio; avoid examining the throat as this might cause resp distress

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antibiotic therapy for epiglottitis

2nd or 3rd gen cephalosporin; if epiglottitis is secondary to HIB then give rifampin to non-immunized house-hold contacts less than 4 yo

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laryngotracheobronchitis (aka croup)

inflamm and edema of the subglottic larynx, trachea and bronchi

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two forms of croup

viral and spasmodic

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what is the most common cause of stridor

viral croup

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age of croup

3 months to 3 years; male to female ratio is 2:1

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season for croup

late fall and winter

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most common cause of viral croup

parainfluenza; but can also be caused by RSV, rhinovirus, adeno , influenza A and B, and mycoplasma pneumoniae

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cause of spasmodic croup

hypersensitivity reaction

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clinical feature of viral croup

begins with URI for 2-3 days followed by stridor and cough; eventually barky cough; can last 3-7 days

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what does xray show for croup?

"steeple sign" of subglottic narrowing

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treatment for spasmodic croup

resolves without treatment

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management of viral croup

supportive care (col mist and fluids), systemic corticosteroids such as IM dexamethasone, nebulized budesonide, or oral corticosteroids

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children with resp distress from croup get treated how?

should be hospitalized; racemic epinephrine aerosols, which vasoconstrict subglottic tissue

87

bacterial tracheitis

uncommon, but can be a cause of stridor

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causes of bacterial tracheitis

staph aureus, strep, HIB

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clinical features of bacterial tracheitis

abrupt onset; high fever, and mucous and pus in the trachea

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management of bacterial tracheitis

anti-staph agent and airway support

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bronchiolitis

inflamm bronchiolar obstruction

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most common cause of bronchiolitis

RSV

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less common causes of bronchiolitis

parainflluenza, adeno, rhino, mycoplasma pneumoniae

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bronchiolitis predominantly affects what age

kids less than 2 yo

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clinical features of bronchiolitis

onset is gradual with URI sx then tachypnea, fine rales, wheezing; liver and spleen may be enlarged due to lung hyperinflation

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cxr for bronchiolitis

hyperinflation with air trapping; patchy infiltrates and atelectasis

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will babies have recurrent wheezing after they recover from bronchiolitis?

yes

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when do babies with bronchiolitis usually recover by?

2 weeks

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what is a complication of bronchiolitis?

apnea

100

management of bronchiolitis

primarily supportive; you can try nasal bulbs, nebulized racemic epinephrine may be helpful, aerosolized ribavirin (against RSV) may possibly help but evidence is lacking