peds4 Flashcards
bronchopulm displasia
chronic complication of RDS; pathologic changes that affect lung growth
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
prognosis for infant with RDS
with aggressive treatment in the NICU, >90% survive
persistent pulm hypertension of the newborn occurs most freq in what GA?
full term or post-term
most common causes of persistent pulm hypertension of the newborn
perinatal asphyxia and meconium aspiration syndrome
hypoxemia is a potent pulm vasoconstrictor
which explains why perinatal asphyxia is a cause of pulm hypertension
ECMO
extra-corporeal membrane oxygenation
inhaled NO
may be used as a potent pulm vasodilator
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
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
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
most frequent type of apnea in the newborn
mixed (of central and airway obstruction), not either one on its own
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
management of apnea
respiratory stimulant medications (caffeine or theophylline), ventillation as needed, CPAP as needed
direct bilirubin
bilirubin is conjugated in the liver; most of it goes into bile and into the small intestine
indirect (unconjugated)bilirubin
this is the bilirubin as a result of hemoglobin breakdown; it has not gone to the liver yet
physiologic jaundice
often occurs during the first week of life; most freq caused by indirect (unconjugated) hyperbilirubinemia
visible jaundice occurs at what bilirubin level
5 mg/dL
direct hyperbilirubinemia
when the conjugated form is greater than 15% of the total bilirubin level; this is always pathologic in neonates
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
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
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)
exchange transfusion
performed for rapidly rising bilirubin levels secondary to hemolytic disease
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
clinical features of bilirubin getting into your brain
choreoathetoid cerebral palsy, hearing loss, opisthotonus, seizures, and oculomtor paralysis
most common signs in infants with drug abusing mothers
jitteriness and hyperreflexia, with irritability, tremulousness, feeding intolerance, and excessive wakefulness
what is the most common type of esophageal atresia?
atresia of the esopagus (proximal pouch) with a distal tracheoesophageal fistula
associated malformations are found in what percent of infants with esoph atresia
50%
most cases of diaphragmatic hernias involve which side of the diaphragm?
the left
scaphoid abdomen
abdominal contents in the abdomen; sign of diaphragmatic hernia
omphalocele
abdominal organs covered with a peritoneal sac herniate centrally through the umbilical ring area; there is an increased risk of other congenital anomalies
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
what is the most common type of intestinal obstruction?
intestinal atresia
volvulus
loops of intestine twist, can lead to restricted circulation and gangrene;
hirschrpung disease- more common in males or females?
males
diagnosis of hirschsprung disease
rectal biopsy showing absence of ganglion cells
management of hirschsprung
resection of the affected segment
NEC clinical features
abdominal distension and tenderness; residual gastric contents, billous aspirate, bloody stools, abdominal erythema
NEC may lead to what?
thrombocytopenai, DIC, and death
classic radiograph findings in NEC
abdominal distension, air-fluid levels, thickened bowel walls, pneumatosis intestinalis (air in the bowel wall) and venous portal gas
pneumoperitoneum
suggestive of NEC that perforated
definition of hypoglycemia
serum glucose less than 40 mg/dL
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
definition of polycythemia
central venous hematocrit greater than 65% (22-ish hgb)
increased risk of NEC with polycythemia
TRUE
management of polycythemia
partial exchange transfusion, in which blood is removed and replaced with saline
cause of direct hyperbilirubinemia
choledochal cyst (cystic dilation of the bile duct)
soap bubble on abdominal radiograph
meconium ileus
fragile x syndrome
unstable repeat sequences (number of specific nucleotide copies within a gene increases)
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)
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
ocular findings in marfans syndrome
upward lens subluxation and retinal detachment
diagnosis of marfans?
mostly on clinical findings, but, interestingly, homocystinuria has similar findings so you need to rule that out first;
marfans syndrome patients are at increased risk for endocarditis
right
prader willi genetics
absence of paternally derived part of chrom 15
craniofacial findings of prader willi
almond shaped eyes and fishlike mouth
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
other features of prader willi
hypogonadism, hypotonia (in infancy that can lead to poor feeding), mental retardiation
what percent of alveolar development occurs after birth?
90 percent
alveoli increase in number until what age
8 yo
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
inspiratory stridor suggests what kind of obstruction?
extrathoracic
two examples of extrathoracic obtruction
croup and laryngomalacia
what is laryngomalacia
softening of the laryngeal cartilage that collapses into the airway, esp when patient is in a supine position
expiratory wheezing suggests what?
intrathoracic obstruction like asthma and bronchiolitis
crackles or rales suggest what
parenchymal disease like pneumonia or pulm edema
epiglottitis most common in what age? What gender?
2-7 year olds; equal in boys and girls
epiglottitis is caused by what?
HIB (hemophilus influenzae type B) was most common; also group A strep, strep pneumonia, and staph
clinical features of epiglottitis
abrupt onset of upper airways disease without prodrome
what do lab studies of epiglottitis reveal?
leukocytosis with left shift; blood culture will show HIB
what is classix xray finding of epiglottitis?
thumb print epiglottis on lateral x ray
what should the airway look like?
cery red swollen epiglottis
management of epiglottitis
medical emergency! Controlled nasotracheal intubatio; avoid examining the throat as this might cause resp distress
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
laryngotracheobronchitis (aka croup)
inflamm and edema of the subglottic larynx, trachea and bronchi
two forms of croup
viral and spasmodic
what is the most common cause of stridor
viral croup
age of croup
3 months to 3 years; male to female ratio is 2:1
season for croup
late fall and winter
most common cause of viral croup
parainfluenza; but can also be caused by RSV, rhinovirus, adeno , influenza A and B, and mycoplasma pneumoniae
cause of spasmodic croup
hypersensitivity reaction
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
what does xray show for croup?
“steeple sign” of subglottic narrowing
treatment for spasmodic croup
resolves without treatment
management of viral croup
supportive care (col mist and fluids), systemic corticosteroids such as IM dexamethasone, nebulized budesonide, or oral corticosteroids
children with resp distress from croup get treated how?
should be hospitalized; racemic epinephrine aerosols, which vasoconstrict subglottic tissue
bacterial tracheitis
uncommon, but can be a cause of stridor
causes of bacterial tracheitis
staph aureus, strep, HIB
clinical features of bacterial tracheitis
abrupt onset; high fever, and mucous and pus in the trachea
management of bacterial tracheitis
anti-staph agent and airway support
bronchiolitis
inflamm bronchiolar obstruction
most common cause of bronchiolitis
RSV
less common causes of bronchiolitis
parainflluenza, adeno, rhino, mycoplasma pneumoniae
bronchiolitis predominantly affects what age
kids less than 2 yo
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
cxr for bronchiolitis
hyperinflation with air trapping; patchy infiltrates and atelectasis
will babies have recurrent wheezing after they recover from bronchiolitis?
yes
when do babies with bronchiolitis usually recover by?
2 weeks
what is a complication of bronchiolitis?
apnea
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
