Pediatrics Flashcards

viral induced inflammation of small, distal airways; bronchiolar obstruction from sloughed epithelial cells, leukocytes and mucus; RSV most common cause

Age <2; antecedent nasal congestion, rhinorrhea, cough, wheezing and crackles, tachypnea, retractions (peak severity days 3-5)

bilateral hyperinflation, increased interstitial markings and peribronchial cuffing

supportive

apnea (<2 months old), resp failure

nirsevimab for all children under 8 months old

a congenital defect that typically presents within the first 24 hrs of life w/ cyanosis and a single loud S2

maternal hyperglycemia leading to fetal hyperglycemia and hyperinsulinemia; increased glycogen and fat deposition in interventricular septum leading to LVOT obstruction

often asymptomatic but may have resp distress and/or hypotension; systolic ejection murmur

CXR: cardiomegaly

ECHO: increased thickness of interventricular septum and decreased LV chamber size

IV fluids and beta blockers to increase LV blood volume

spontaneous regression age 1

a type of conversion disorder characterized by an apparent loss of consciousness w/o impaired cerebral perfusion. Prolonged duration of unconsciousness, absence of objective physical findings, and high frequency of episodes are common.

They'll say stuff like "I felt my head throbbing after I hit the floor." or "I felt weak before I passed out"

an emotionally triggered, sudden loss of muscle control, can mimic syncope; however, it typically occurs in pts w/ narcolepsy, who usually have other symptoms such as excessive daytime sleepiness. The episodes are brief.

macrolides and fluoroquinolones, odansetron and them, azoles (fluconazole), antipsychotics, TCAs, SSRIs, some opioids (methadone, oxycodone), quinidine (class Ia antiarrythmics), dofetilide, sotalol (class III antiarrythmics), electrolytes abnormalities (decreased Mg, K, and Ca)

Romano-Ward syndrome, Jervell and Lang-Nielsen syndrome

hypercyanotic tet spells due to RVOT obstruction and right to left shunting in the setting of exertion of agitation. Squatting increases afterload, which increases blood flow across the RVOT, which in turn improves cyanosis

an acyanotic lesion that causes a wide and fixed splitting of S2 and a systolic ejection murmur due to increased blood flow across the pulmonic valve

drainage of some but not all pulmonary veins into the right sided circulation typically demonstrating a step-up in oxygen saturation from the inferior vena cava to the right atrium. Other clinical findings include right-sided cardiac enlargement and signs of pulmonary HTN (exertional dyspnea)

characterized by micrognathia, microcephaly, rocker bottom feet, overlapping fingers, and absent palmar creases. Ventricular septal defect in common in these pts w/ a holosystolic murmur

increased tissue oxygen extraction

vasomotor instability, cold exposure, low cardiac output, venous obstruction

finger, toe, perioral cyanosis, cool extremities, does not improve with oxygen

decreased systemic O2 sat

pulm disease, cyanotic heart disease

diffuse and/or mucosal cyanosis, warm extremities, may improve w/ O2

infants: poor weight gain, resp distress, difficult feeding

older children: exertional fatigue, chest pain, syncope

fam Hx of SCD of CHD

holosystolic or diastolic, harsh, grade at least 3 intensity, intensity persists w/ standing and valsalva maneuver

central cyanosis, loud fixed, or single S2, weak femoral pulses, hepatomegaly

EKG and ECHO

asymptomatic, normal growth, no significant family history

early or midsystolic, musical or vibratory, grade 1-2 intensity, decreases or disappears w/ standing and valsalva maneuver

normal vitals, normal S1 and S2, symmetric pulses

reassurance

abnormal persistent of increased fetal pulm vascular resistance, right to left shunting across ductus arteriosus

lung hypoplasia (congenital diaphragmatic hernia), meconium aspiration syndrome, infection (neonatal pneumonia)

decreased postductal relative to preductal O2 sat, resp distress and cyanosis, prominent S2

O2 and ventilation, inhaled nitric oxide (pulm dilator)

tuberculosis, varicella, SARS, measles

VRE, MRSA, E coli O157:H7, scabies, RSV, C. diff

mycoplasma pneumonia, influenza, Hib, neisseria meningitidis, adenovirus

galatocerebridase deficiency, AR, presents early in infancy w/ developmental regression, hypotonia, and areflexia

lysosomal hydrolase deficiency, presents at age 6 months to 2 yrs w/ coarse facial features, inguinal or umbilical hernias, corneal clouding, and hepatospenomegaly

assess pulm function w/ spirometry. FVC and negative inspiratory force monitor resp muscle strength and serial pulm function testing should be performed given the potential for rapid progression of disease

WBC: 0-5

Glucose: 40-70

Protein: 45- 1,000

WBC: 10-500

Glucose: 40-70

Protein: <150

WBC: 100-500

Glucose: <45

Protein: 100-500

WBC: >1000

Glucose: <40

Protein: >250

chorioretinitis, obstructive hydrocephalus, parenchymal calcifications

osteoarticular destruction (Hutchinson teeth, saddle nose), hepatosplenomegaly, jaundice, rhinitis, and rash

cataracts, sensorineural hearing loss, PDA

sensorineural hearing loss, hydrocephalus ex vacuo, and periventricular calcifications

acute vision loss, floaters, and flashes of light. Funduscopic exam shows a retinal tear and/or elevation

painless loss of peripheral vision and presents in late adulthood. Funduscopic exam shows optic nerve cupping

xerophthalmia, characterized by excessive dryness of the cornea and conjunctivae, and night blindness

genetic mutation causing loss of photoreceptors, progressive retinal degeneration, symptom onset from age 10 through adulthood

night blindness, progressive visual field loss (midperiphery), decreased visual acuity (late finding)

retinal vessel attenuation, optic disc pallor, abnormal retinal pigmentation

most are legally blind by age 40

asymptomatic at birth; those with symptoms typically have rhintis (snuffles), hepatomegaly, and a maculopapular rash

fetal renal failure and associated oligohydramnios that results in pulm hypoplasia, growth restriction, and limb defects (Potter sequence)

titrated to the lowest dose for seizure control prior to conception and started on high dose (4mg) folic acid supplementation

microcephaly, cleft lip and palate, cardiac defects, hypoplasia of nails and phalanges, wide anterior fontanelle, due to in utero exposure to an antiepileptic (phenytoin, carbamazepine, valproate)

involves the 5th, 6th, and sometimes 7th cranial nerves

observation and physical therapy b/c up to 80% of pts have spontaneous recovery within 3 months

mucocutaneous vesicles, keratoconjunctivitis

seizures, fever, lethargy, temporal lobe hemorrhage/edema

sepsis, hepatitis, pneumonia

viral surface cultures: HSV PCR (blood, CSF)

acyclovir

underdeveloped and are at risk for hypothermia. Skin to skin contact prevents heat loss. You could also swaddle w/ warm blankets and place under a radiant warmer.

postpone for an hour

2nd/3rd trimester

uteroplacental insufficiency, maternal malnutrition

head-sparing growth lag

monitor/treat complications (hypoglycemia, hypothermia, polycythemia and hypocalcemia)

1st trimester

chromosomal abnormalities, congenital infection

global growth lag

monitor or treat complications (hypoglycemia, hypothermia, polycythemia and hypocalcemia)

hypoglycemia, polycythemia (low iron), hypocalcemia and hypomagnesemia, hyperbilirubinemia, congenital anomalies, macrosomia (w/ brachial plexus injury or clavicle fracture), resp distress syndrome, hypertrophic cardiomyopathy

testicular cancer (dysgerminoma, gonadoblastoma), due in part to an increase in intraabdominal temperature that causes abnormal spermatogenesis and aberrant germ cell differentiation

x-linked mutation in androgen receptor

gender identity/assignment counseling: gonadectomy (malignancy prevention)

upper outer quadrant of the breast after menarche

absence of menarche in a girl at least 13 years old w/ no secondary sexual characteristics, or a girl at least 15 years old w/ secondary sexual characteristics. If pelvic US shows uterus, test FSH levels and that'll give you your answer

topical estrogen

inflammation of the glans penis

inflammation of the glans penis and foreskin

irritation, infection, trauma

KOH microscopy for suspected candida infection

STI screen if urethral discharge present

foreskin hygiene, sitz baths, topical treatment, diabetes 2 screening for candida infection w/o risk factors

fluid collection within the tunica vaginalis, which surrounds the testis. Should spontaneously resolve by 1 y/o and can safely be monitored during that period

prematurity, small for gestational age

empty, poorly rugated scrotum on affected side(s) +/- inguinal fullness/mass

orchiopexy and 6-12 months

inguinal hernia, testicular torsion, infertility, testicular cancer

evaluate 17-hydroxyprogesterone level and electrolytes. Perform karyotype and pelvic US

urologic evaluation b/c most cases require surgical correction. Circumcision is deferred until after evaluation b/c the foreskin may be required for hypospadias repair

failure of urethral folds to fuse

ventrally displaced urethral meatus, dorsal hooded foreskin, +/- underdeveloped penis and glans, +/- penile curvature (chordee)

+/- karyotype, pelvic US if severe

acute otitis media most commonly due to strep pneumo

IV antibiotics, drain via tympanostomy or mastoidectomy

headache, seizure, in addition to focal neurological deficits

urgent MRI/MR angio to confirm Dx and determine eligibility for potenital reperfusion therapies

autoimmune complication that can occur months after GAS infection

molecular mimicry btw anti-GAS antibodies and neuronal antigens in basal ganglia

involuntary jerky movements, hypotonia, emotional lability, obsessive complusive behaviors, +/- sx of acute rheumatic fever

GAS test w/ throat culture, ASO and Anti-DNAse B titers, ECHO and EKG

abx and antidopaminergics (haloperidol)

displacement of the cerebellar tonsils through the foramen magnum

syringomyelia

although frequently asymptomatic, presentation may occur in adolescence/adulthood w/ occipital headache exacerbated by activity and valsalva maneuvers

herniation of cerebellum through foramen magnum

obstruction of cerebrospinal fluid flow through 4th ventricle, lateral ventricular dilation, myelomeningocele

walks up stairs w/ alternating feet, rides tricycle

dresses w/ help, uses fork, copies circle

says at least 3-word sentences, speech 75% intelligible

knows age/sex, plays w/ other children

jumps

removes clothes, turns pages

says at least 50 words

follows 2-step command, knows 1 color

runs, kicks ball, walks up stairs

uses spoon

says 2-word phrases

knows 2 body parts, parallel play

walks easily, climbs on/off chair

scribbles, feeds w/ fingers

says at least words in addition to mama and dada

follows 1-step command w/o gesture, imitates

takes a few steps

stacks 2 blocks

says 1 or 2 words plus mama and dada

follows 1-step command w/ gesture, points to get something, shows affection

pulls to stand, cruises

pincer grasp

says mama and dada, understands no

plays pat-a-cake, looks for hidden object

optic neuropathy +/- increased IOP

impaired drainage of intraocular fluid, primary anatomic abnormaility (angle dysgenesis), Sturge-Weber syndrome, tumor, trauma, infection involving the angle, corticosteroid induced

tearing, photophobia, blepharospasm, enlarged cornea or globe, optic nerve cupping, increased IOP on tonometry

surgery +/- pressure-reducing eye-drops

X-linked dominant, remember they exhibit self-injurious behavior like hand-biting

neuro abnormalities like hypotonia and developmental delay, dysmorphic features, rapidly decreasing head circumference percentiles (ie curve crossing multiple major percentiles)

substance use (cigs, alc, drugs)

prematurity or low birth weight, sleep environment (prone/side-sleep position, soft sleep surface, loose bedding, bed-sharing), smoke exposure

Duchenne muscular dystrophy

greater incidence in girls, onset age 6-18 months

initially normal development followed by loss of speech, loss of purposeful hand use, and gait abnormalities

microcephaly, seizures, breathing abnormalities, sleep disturbance, autistic features

MECP2 gene mutations

deceleration of brain growth

middle aged life expectancy, decreased mobility, seizures, resp difficulties

B- hexosaminidase A deficiency

AR, Ashkenazi Jews, onset 2-6 months old

loss of motor milestones: hypotonia, feeding difficulties, cherry-red macula, hyperreflexia

sphingomyelinase deficiency

AR, Ashekensazi Jews, onset 2-6 months

loss of motor milestones, hypotonia, feeding difficulties, cheery-red macula, hepatospenomegaly, areflexia, fatal by age 3

btw periosteum and gala aponeurotica

can expand over days, soft, fluctuant, diffuse, crosses sutures, +/- overlying bruising

can cause life-threatening blood loss

subperiosteal (btw skull and periosteum)

present hours after birth, firm, nonfluctuant, does not cross sutures, overlying skin is normal, forceps or vacuum assisted delivery increases risk

increased hyperbilirubinemia risk, resorbs within a month

subcutaneous

present at birth, soft and boggy, crosses sutures, overlying skin is normal

self-resolves in days

tuberous sclerosus complex

AR mutation of ATP7B --> hepatic copper accumulation --> leak rom damaged hepatocytes --> deposits in tissues (basal ganglia, cornea)

Hepatic: acute liver failure, chronic hepatitis, cirrhosis

Neuro: parkinsonism, gait disturbance, dysarthria

Psych: depression, personality changes, psychosis

decreased ceruloplasmin and increased urinary copper excretion, kayser-fleischer rings on slit lamp exam, increased copper on liver biopsy

chelators (D-penicillamine, trientine), zinc (interferes w/ copper absorption)

AR disorder where defective DNA repair results in cerebellar ataxia, oculocutaneous telangiectasias, and recurrent sinopuml infection. Lymphoid tissue is often small/nonpalpable due to lymphopenia

immature lower esophageal sphincter

spit up, normal weight gain, no pain/back-arching

upright positioning after feeds, burping during feeds, frequent, small volume feeds, vitamin D, supplements

omphalocele

presents w/ history of decreased intake or increased fluid loss w/ minimal or no clinical symptoms

presents w/ decreased skin turgor, dry mucous membranes, tachycardia, irritability, a delayed capillary refill (2-3 seconds) and decreased urine output

presents w/ cool, clammy skin, delayed capillary refill, (>3 seconds), cracked lips, dry mucous membranes, sunken eyes, sunken fontanelle (if still present), tachycardia, lethargy, and minimal or no urine output. Pts can present w/ hypotension and signs of shock

Th2-mediated inflammatory response triggered by food antigen exposure, comorbid atopy (asthma, eczema, food allergy, allergic rhinitis) common

feeding difficulties (solid food refusal), weight loss

abdominal pain, vomiting

dysphagia, heartburn, food impaction

endoscopy and esophageal biopsy (eosinophils: at least 15/hpf), exclusion of alternate Dx (achalasia, infection)

elimination from diet, PPIs, topical glucocorticoids, feeding therapy

typically presents in neonates w/ decreased stooling, increased rectal tone, and signs of intestinal obstruction (bilious emesis, abdominal distention, dilated bowel loops). Contrast enema is performed to identify the level of obstruction

ingestion of contaminated/undercooked beef or contact w/ farm animals, invasion of intestinal epithelial cells, production of toxin

watery to bloody diarrhea within 3 days, NO high fever

multiplex stool PCR testing, stool toxin assay, stool culture

supportive care (aggressive fluids), avoidance of antibiotics and antidiarrheals

HUS (may develop 1-2 weeks after diarrhea onset)

most commonly transmitted via undercooked poultry

fever, abdominal pain, diarrhea (mucoid +/- blood), pseudoappendicitis (RLQ pain due to acute ileocecitis)

supportive care (sx usually self-limited <7 days)

abx only in severe of high-risk cases

Guillan Barre syndrome, reactive arthritis

inspissated stool causes obstruction at terminal ileum, strong association w/ CF

failure to pass meconium within 24hrs of birth, abdominal distention, no stool in rectal vault, +/- bilious emesis

X-ray: dilated loops of small bowel

Contrast enema: microcolon

dx eval for CF via sweat test

hyperosmolar enema, +/- surgical management

9 months

discontinuation of enteral feeds, nasogastric decompression, blood cultures, and empiric abx, IV fluid repletion

serial CBC and electrolytes, serial abdominal exams and imaging

bowel perf, clinical deterioration, despite medical management (suggestive of bowel necrosis)

discontinue enteral feeds, NG tube decompression, and surgical repair

personal or family hx of migraines, episodes often have identifiable trigger (infection, stress)

stereotypical vomiting episodes: acute onset of nausea, abdominal pain/headache, vomiting, self-limited, lasting 1-2 days

btw episodes: usually asymptomatic, often regular intervals (2-4 weeks)

group B strep, E. coli, listeria (age <7 days)

temp instability (fever, hypothermia), CNS signs (lethargy, irritability, apnea), poor feeding, resp distress (tachypnea, grunting), jaundice

inflammatory markers (CRP, ANC, procalcitonin), blood, urine, and CSF cultures

parenteral abx (ampicillin and gentamicin)