Infant/Child Path 1 Flashcards
What deficiency do we associate with neural tube defects?
neural tubes (just kidding)
Folate deficiency
What leads to anencephaly? What does it look like?
anencephaly- anterior end of the tube is not closed; calvarium and forebrain absent and orbits shallow
What leads to encephalocele?
encephalocele- malformed CNS tissue extends through a defect in the cranium.
Describe what causes spina bifida and the three subtypes
spina bifida- caudal region of the neural tube not closed = vertebral malformation
spina bifida occulta- asymptomatic defect in the vertebral arches; skin intact
meningocele- meningeal extrusion through a vertebral arch defect
Meningomyelocele- herniation of CNS tissue through the incompletely fused vertebral arches= motor and sensory deficits
Amniotic AND maternal serum AFP levels are up. What could it be?
- anencephaly
- encephalocele
- spina bifida
Multiple gestation and Bicornate uterus are examples of what type of issue?
Deformations.
extrinsic disturbances due to compression
Prematurity is specifically defined as:
Delivery before 37 weeks
Risk factors for prematurity
risk factors: Preterm rupture of placental membranes (PPROM)2* ascending infection → chorioamnionitis & funisitis, intrauterine infection
Uterine, cervical, placental structural abnormalities
Multiple gestation
What complications do we see with prematurity?
Sepsis, developmental delay, brain damage and hydrocephalus 2* intraventricular hemorrhage/Germinal matrix hemorrhage (cannot regulate cerebral blood flow and lack glial support)
Sometimes, especially in premature infants, we see fetal growth restrictions causing the fetus to be small for its gestational age.
What risk factors are associated with this, both from the fetus itself and those from the mother
Fetus Issues: Chromosomal disorders, Congenital anomalies, infections (TORCH)
Maternal: Vascular diseases (preeclampsia), Inherited thrombophilias (factor V leiden), avoidable teratogens (narcotic, alcohol, smoking, drugs, malnutrition)
Malnutrition from a placental defect would also cause smaller babies
What is the third most common cause of death in neonates?
Necrotizing enterocolitis caused by necrosis and inflammation in bowel.
What symptoms do we see with necrotizing enterocolitis?
Bloody stools, abdominal distension, circulatory collapse
What does necrotizing enterocolitis look like on X-Ray and grossly?
X-ray: gas in intestinal wall, dilated loops of bowel
gross: mucosal/transmural coagulative necrosis, ulceration, bacterial colonization, submucosal gas bubbles (pneumatosis intestinalis)
Treatment for necrotizing enterocolitis?
Tx: stop feeding, NG tube (decompress abd), IV fluids, remove bowel
What complications do we see down the road from necrotizing enterocolitis?
Complications: sepsis, shock, DIC, short-gut syndrome, malabsorption, strictures, obstruction
What leads to neonatal respiratory distress syndrome (RDS) and who is at risk?
surfactant deficiency= increased surface tension= alveolar collapse= R–>L shunt
patho: surfactant (phosphatidylcholine or lecithin) made by type II pneumocytes; dec surface tension and prevents collapse of alveolar air sacs in expiration.
Lack of surfactant= collapse of air sacs and formation of hyaline membranes.
risk: white males
What lab values do we use to measure if a neonate has RDS?
Amniotic fluid lecithin: sphingomyelin (L:S) ratio is used to screen for lung maturity.
Phosphatidylcholine (lecithin) levels ↑ as surfactant is produced; sphingomyelin remains constant.
Ratio > 2 indicates adequate surfactant production.
Besides prematurity, what can lead to RDS and why?
C-section delivery- d/t lack of stress-induced steroids; steroids ↑ synthesis of surfactant.
Maternal diabetes- Insulin dec surfactant production.
X-Ray of RDS looks like? Grossly?
X-ray: Diffuse granularity of the lung (‘ground-glass’ appearance)
Gross: lungs are firm, deep red, “liver-like”
If I am struggling to breathe/ventilate, what will my ABG look like?
labs: ABG= hypoxemia and hypercapnia
Histo of RDS?
Histo: focal atelectasis & hemorrhage w/dilated terminal & respiratory bronchioles lined by pink, smooth hyaline membranes, fibrin
What can RDS lead to? Third one is weird, but linked to the second one…
can lead to: Hypoxemia= ↑risk for persistence of PDA and necrotizing enterocolitis.
Supplemental oxygen= ↑ risk for free radical injury (leading to number 3…)
Retinal injury= blindness and also, lung damage=bronchopulmonary dysplasia
How does we treat the RDS
tx: surfactant replacement therapy, O2, support; prophylaxis: steroids
What causes Transient tachypnea of the newborn (TTN)?
abnormal clearance of lung fluid from the alveolar space
patho: not reabsorbed
Who is at risk for TTN?
risk: short labor or c-section, maternal diabetes and asthma
Discuss the chest x-ray for TTN
chest X-ray: “wet” lung fields; hyperinflated lungs with shaggy heart border and clear periphery
typically resolves within 24-48 hours
What causes persistent pulmonary hypertension (PPHN)?
high pulmonary vascular tone
patho: pulmonary vascular resistance does not appropriately decrease after birth→ blood shunted right–> left across the foramen ovale and ductus arteriosus; Blood bypasses the lungs= deoxygenated blood returning from the body returns to the arterial system without being oxygenated.
What can lead to PPHN?
meconium aspiration, pneumonia, birth asphyxia, diaphragmatic hernia (one lung flat), premature ductus arteriosus/foramen ovale closure (maternal NSAIDs), polycythemia, sepsis (constriction 2* COX/PE)
How will PPHN present to us and how do we diagnose it?
sx: tachypnea, severe cyanosis, retractions, hypoxia; O2 sat lower in LE vs UE
dx: not responsive to 100% O2 challenge; echo= elevated pressure in pulmonary artery
PPHN treatment
tx: O2, NO to dilate pulmonary vasculature, ECHMO
In regards to intrauterine meconium aspiration, what does it lead to and what are complications of it?
respiratory distress after delivery through meconium+amniotic fluid
complications: inflamm pneumonitis, bronchial obstruction
In regards to intrauterine meconium aspiration, how will it present and how do we diagnose it?
sx: rales/rhonchi, O2 desaturation, asphyxia, green/brown muconium staining on baby
dx: chest xray= hyperinflation + atelectasis
Treating intrauterine meconium aspiration
tx: intubate and suction, abx, O2
What is the risk factor for a patent ductus arteriosus and what leads to this?
failure of the ductus arteriosus to close shortly after birth
risk: premature infants
patho: as pulmonary vascular resistance drops after birth, the PDA allows blood to pass from aorta to the pulmonary artery (L–>R shunt); additional fluid returning to the lungs increases lung pressure and neonate has difficulty inflating lungs
How will PDA present and how do we diagnose it?
sx: increased blood flow =pulmonary edema and congestive heart failure, murmurs, thrill
dx: US/echo and clinical sx
Whta bacteria/viruses can cause transcervical (ascending) infections transferred to the baby in the birth canal?
Most bacteria: Group B streptococcus, Ureaplasma urealyticum, E. coli, Mycoplasma hominis, Streptococcus viridans, Gardnerella vaginalis
few viral: (HSV2) Herpesvirus- encephalitis, vesicular rash
What is chorioamniotis and what causes it?
inflammation of the fetal membranes (amnion and chorion)
cause: bacterial infection ascending into the uterus from the vagina
Who is at risk for a intra-amniotic infection (IAI) and how does it present?
at risk: prolonged labor
sx: maternal fever, uterine tenderness, maternal/fetal tachycardia, foul-smelling amniotic fluid
What will intra-amniotic infections look like grossly/histologically?
gross: Greenish opaque membranes
histo: acute inflamm
What is villitis/intervillositis and what does it look like histologically?
What can it lead to?
histo: villous inflammation, PMNs/lymphocytes, fibrosis or edema
can lead to: miscarriage, intrauterine growth restriction
The next section of cards is better explained in the microdecks, but for review, I’ve rehashed the pertinents here.
Also, Sketchy Micro is AWESOME. Use it and you will get all of the micro answers correct.
How does toxoplasmosis present?
Sx: fever, IUGR, microcephaly, seizure, hearing loss, maculopapular rash, jaundice, hepatosplenomegaly, anemia, and lymphadenopathy
First Trimester – often results in death
Second Trimester – classic triad: Hydrocephalus, Intracranial calcifications, chorioretinitis
Third Trimester – often asymptomatic at birth
How do we diagnose and treat toxoplasmosis?
Dx: placenta, serum, or CSF: PCR & IgM titer (IgG will be elevated if mother is infected regardless of transmission)
Tx:
Mom infected - Spiramycin
Baby infected symptomatic - Pyrimethamine and spiramycin and sulfadinazine - 12 months
Baby no symptoms - Same as above, but not 12 months (case by case)
Can also use glucocorticoids and folic acid
Discuss the early and late symptoms of Syphillis
Early Sx~1month: Maculopapular rash, “snuffles,” lymphadenopathy, hepatomegaly, thrombocytopenia, anemia, meningitis, chorioretinitis, osteochondritis, failure to thrive
Late sx~2yrs: Hutchinson Teeth, Mulberry Molars, Perforated hard palate, Rhagades (cracks or fissures in the skin around the mouth), Saber Shins, Sensorineural hearing loss (CN VIII), Saddle Nose
How do we treat Syphillis?
PCN
Symptoms of Rubella
Sx: “Blueberry Muffin Baby” (rash due to extramedullary hematopoiesis), Cataracts , “Salt and Pepper” retinopathy, Radiolucent bone disease (long bones), IUGR, glaucoma, hearing loss, pulmonic stenosis, patent ductus arteriosus (CHD), lymphadenopathy, jaundice, hepatosplenomegaly, thrombocytopenia, interstitial pneumonitis, diabetes mellitus
Cytomegalovirus symptoms
sx: most infants asymptomatic at birth; IUGR, developmental delay, microcephaly, sensorineural hearing loss, retinitis, jaundice, hepatosplenomegaly, thrombocytopenia, hypotonia, lethargy, poor suck, Periventricular calcifications
Treatment for CMV?
Gancyclovir - Stops further hearing loss
Discuss the SEM presentation of HSV
SEM disease (Localized to skin, eyes, and mucosal)-Vesicular lesions on an erythematous base, Keratoconjunctivitis, cataracts, chorioretinitis, Ulcerative lesions in mouth/palate/tongue
Discuss the CNS presentation of HSV
CNS disease: Seizure, lethargy, irritability, tremor, poor feeding, temperature instability, full anterior fontanelle
Discuss the disseminated disease symptoms of HSV
Disseminated disease: Multiple organ involvement (CNS, skin, eye, mouth, lung, liver, adrenal glands), appear septic (fever/hypothermia, apnea, irritability, lethargy, respiratory distress), Hepatitis, ascites, direct hyperbilirubinemia, neutropenia, disseminated intravascular coagulation, pneumonia, hemorrhagic pneumonitis, necrotizing enterocolitis, meningoencephalitis, skin vesicles
How do we treat HSV?
Acyclovir
How does Sepsis present in neonates and what causes it?
Early (first 7 days) or late (up to 3mo) onset
Symptoms: pneumonia, sepsis, meningitis
cause: Most commonly GBS, listeria & candida