MIDTERM

Question 1

lead screening

Answer 1

-CDC recommends blood lead screening at 12 + 24 months
-screen in temper tantrum
-Lead intoxication (plumbism) can cause developmental and behavioral abnormalities
-RF: older homes w/ cracked lead-based paint, industrial exposure, use of foreign remedies, and use of pottery with lead paint glaze
-affects the CNS

-vague sx:
-Early: Weakness, irritability, wt loss, vomiting, personality changes, ataxia, constipation, h/a, and colicky abdominal pain
-Late: Developmental delays, convulsions, and coma assoc with increased ICP

-If lead +, investigation if:
-levels of 20 mcg/dL on 1x test OR
-persistent 15 mcg/dL over 3mo (decontamination techniques used once source identified)

-Tx:
-Succimer- oral chelator recommended in asymptomatic with levels > 45 mcg/dL
-IM dimercaprol/BAL and IV calcium sodium edetate in symptomatic with encephalopathy or levels > 70 mcg/dL

Question 2

sleep safety

Answer 2

-Sudden infant death syndrome (SIDS)- sudden death of infant < 1yo that is unexplained after thorough case investigation
-Sudden unexpected infant death syndrome (SUID)- sudden and unexpected infant death (explained or unexplained) –> preferred term
-Includes deaths due to infection, ingestions, metabolic diseases, cardiac arrhythmias, and trauma
-peaks between 2-4 months
-Most occur at night
-RF: Socioeconomically disadvantaged (continued prone positioning, sharing beds), preterm, low birth wt, recent infection, young maternal age, high maternal parity, maternal tobacco or drug use, and crowded living conditions
-MC findings in SUID: Intrathoracic petechiae, mild inflammation/congestion of respiratory tract, findings consistent with chronic hypoxia prior to death

-Tx + prevention:
-Back to Sleep initiative -> 60% decline in SIDS since 1990
-Supine positioning
-Sleep in parents’ room, but on a separate surface for 1st 6mo
-Remove soft objects/loose bedding, stuffed animals, or wedge positioners
-Breastfeeding is recommended
-Pacifier at naptime and bedtime
-no smoking during pregnancy and after birth
-Avoid car seats, swings, and baby slings for sleep
-Avoid use of adult beds and bed rails (increases risk of suffocation)

Question 3

colic

Answer 3

-Episodes of uncontrollable crying in healthy infant
-Paroxysmal, characterized by facial grimacing, leg flexion, and passing flatus

-Wessel rule of 3s:
-Crying for > 3 hours/day
-At least 3 days/week
-> 3 weeks

-Etiology: Unknown
-Cow’s milk intolerance
-Change in fecal flora
-Increase in serotonin secretion
-Poor feeding technique
-Maternal smoking may be assoc

-Tx:
-Dr. Harvey Karp’s “5 Ss”:
-Swaddling
-Side/stomach holding
-Soothing noises
-Swinging/slow rhythmic movement
-Sucking on pacifier

-Educating on hunger cues, avoid excessive caffeine and alcohol in nursing mothers, ensuring adequate bottle/nipple flow, and cautioning overfeeding
-Effectiveness of dietary changes, herbal supplements, and/or meds very limited
-No evidence of long-term effects for patient or parents
-Most serious complication -> nonaccidental trauma

Question 4

teething

Answer 4

-can mimic ear infection
-Remedies for pain:
-OTC teething gels or liquids that contain benzocaine
-Systemic analgesics
-Chewing on teething object (distraction)

-Eruption Cysts: Localized, red/purple, round, raised, and smooth lesion, resolved with eruption of tooth (NORMAL)
-All children 1yo+ should have dental exam by dentist at least annually and cleaning q 6mo
-Preventative: Brushing, flossing, concentrated fluoride topical tx (dental varnish) and acrylic sealants on molars
-Recommended PCPs apply dental fluoride varnish to infants and children q 3-6mo between 9mo-5yo
-Fluoridation of water or fluoride supplements if no fluoridation are important in prevention of caries

Question 5

car seat safety

Answer 5

-infants + toddlers (<2yo)- rear facing
-toddlers + preschool (2-6)- forward facing/harness
-school age (6-8)- booster

-4ft 9in or 8-12yo -> seat belts

Question 6

tanner stages (female)

Answer 6

-growth spirt is 1yr after thelarche (tanner stage 3)
-menarche at tanner stage 4

-Stage 1- elevation of papilla only
-Stage 2- breast bud -> elevation of papilla and breast
-Stage 3- increase breast + areola
-Stage 4- increase areola + papilla
-Stage 5- areola recession

-Stage 1- none
-Stage 2- straight, along labia
-Stage 3- dark, course, curly
-Stage 4- adult, no thighs
-Stage 5- adult, thighs

Question 7

anorexia

Answer 7

-1.5% in teenage girls
-F:M- 20:1, familial pattern

-Dx Criteria:
-Restriction of energy intake relative to requirements leading to low wt for age, sex, physical health, and developmental trajectory
-Strong fear of gaining wt or becoming fat, even though underwt
-Disturbance in body wt or shape is experienced, undue influence of body wt or shape on self-evaluation, or denial of seriousness of low body wt

-1st event - behavioral change in eating or exercise
-oversized or excessively tight clothing, fine hair on face and trunk, rough/scaly skin, bradycardia, hypothermia, decreased BMI, erosion of tooth enamel (if emetic episodes with the binge-purge type versus restricting type), and acrocyanosis of hand/feet

-Tx and Prognosis:
-Multi-disciplinary approach with family-based therapy
-voluntary freeding of regular foods, nutritional formula orally, or NG tube
-When 80% of normal body wt -> pt given freedom to gain wt at personal pace
-3-5% mortality, development of bulimic sx (30%), and persistent anorexia nervosa syndrome (20%)

Question 8

bulimia

Answer 8

-5% in female college students (overwt, hx of dieting)
-F:M ratio 10:1

-Dx Criteria:
-Recurrent episodes of binge-eating, characterized by both:
-Eating a larger amount of food than most people would eat in a discrete period of time
-lack of control during episode

-Recurrent inappropriate compensatory behavior to prevent wt gain (vomiting; laxatives, diuretics, excessive exercise; fasting)
-Binge eating and behaviors occur at least 1x week for 3mo

-Complications: Metabolic disturbances from excessive vomiting

-Tx and Prognosis:
-Nutritional, educational, and self-monitoring techniques to increase awareness of maladaptive behavior, followed by efforts to change the eating behavior
-May respond to anti-depressants
-Attempted suicide and completed suicide (5%)

Question 9

HEADDSS

Answer 9

-Home
-Education
-Alcohol
-Drugs
-Diet
-Sex
-Suicide

-15-17yo is high risk behavior

Question 10

birth injuries

Answer 10

-Caput Succedaneum:
-Diffuse, edematous, dark swelling of soft tissue of scalp
-boggy
-extends across midline and suture lines
-Often following prolonged labor
-over the periosteum -> free flowing

-Cephalohematoma:
-Subperiosteal hemorrhage
-does not cross suture lines
-May organize, calcify, and form a central depression

-tx-
-observe- they will absorb

Question 11

birth injury: brachial plexus

Answer 11

-Phrenic nerve palsy: C3-5- diaphragmatic paralysis/respiratory distress

-Erb-Duchenne paralysis: C5-6 injury
-Cant abduct arm at shoulder, externally rotate arm, or supinate forearm

-Klumpke paralysis: C7-C8, T1–
-Paralyzed hand with ipsilateral Horner syndrome, claw hand

-Tx: Supportive, active/passive ROM exercises, nerve grafting

Question 12

jaundice

Answer 12

-1st day: PATHOLOGIC -> can be unconjugated or conjugated (MC)
-usually hemolysis, internal hemorrhage, or infection

-2-3 days after birth- PHYSIOLOGIC (dx of exclusion) -> Increased RBC mass, shortened RBC lifespan, hepatic immaturity
-indirect, unconjugated
-Peak, indirect bilirubin level of < 12 mg/dL on day 3 of life

-> 2wks: PATHOLOGIC -> direct hyperbilirubinemia
-Physical signs: Observed when bilirubin reaches 5-10 mg/dL

-Labs: Total bilirubin (possible breakdown), blood typing, Coombs test, CBC, blood smear, and reticulocyte count

-Breast-feeding jaundice (1st few days) -> decreased fluid intake
-Breast Milk Jaundice (1wk-2wk) -> milk may contain inhibitor of bilirubin conjugation or may increase enterohepatic recirculation of bilirubin

-kernicterus spectrum disorder (bilirubin encephalopathy):
-Earliest sx (4 days of life): Lethargy, hypotonia, irritability, poor Moro response, poor feeding
-Late sx: Bulging fontanel, pulmonary hemorrhage, fever, hypertonicity, paralysis of upward gaze, seizures

-Crigler-Najjar Syndrome: Serious, rare, autosomal recessive, permanent deficiency of glucoronosyl transferase (conjugation enzyme)

-Gilbert Disease: Mutation of glucoronosyl transferase

Question 13

jaundice tx

Answer 13

-INDIRECT HYPERBILIRUBINEMIA:
-Phototherapy: Blue/white lights reduce bilirubin

-Exchange transfusion
-Level of 20 mg/dL of indirect-reacting bilirubin (infants > 2000 grams)
-Umbilical venous catheter placed in IVC/umbilical vein/portal system
-immediate exchange transfusion if kernicterus spectrum disorder (bilirubin encephalopathy) -> >25 mg/dL

-DIRECT/CONJUGATED:
-always pathologic
-treat the cause -> does NOT respond to phototherapy or exchange transfusion

Question 14

respiratory distress syndrome (hyaline membrane disease)

Answer 14

-after onset of breathing
-assoc with insufficiency of surfactant -> prevents atelectasis
-Surfactant contributes to lung recoil
-ALWAYS THINK THIS FIRST WHEN THERE IS RESPIRATORY DISTRESS

-RF: Prematurity, low GA, hx of preterms with RDS, maternal DM, hypothermia, fetal distress, asphyxia, males, 2nd born twin, C-section
-Initial sx: Cyanosis, tachypnea, nasal flaring, intercostal/sternal retractions, and GRUNTING (BAD); over 72 hrs - increased distress, hypoxemia

-CXR: Ground-glass haze surrounding air-filled bronchi or white-out (severe)
-Uncomplicated cases -> spontaneous improvement –> diuresis and marked improvement of edema
-Severe cases (edema, apnea, respiratory failure) –> assisted ventilation

Question 15

respiratory distress syndrome (Hyaline Membrane disease): complications

Answer 15

-PDA
-pulmonary air leaks from ventilation -> distention -> rupture -> interstitial emphysema -> PTX!
-retinopathy of prematurity (retrolental fibroplasia)- MCC blinding in VLBW -> vasoconstriction -> vaso-obliteration

-bronchopulmonary dysplasia (chronic lung ds):
-due to prolonged ventilation and O2 therapy
-failure to improve >2wks, continued need for ventilation/O2 @ 36wks
-hypercapnia with compensatory metabolic alkalosis, pulmonary HTN, poor growth, R-sided HF
-CXR: Lung opacification -> cyst development = Sponge-like appearance

-Tx:
-ventilation for several months
-Tracheotomy may be indicated (prevent subglottic stenosis)
-Dexamethasone

Question 16

PE of newborn neck

Answer 16

-Short and symmetric
-Midline clefts (thyroglossal duct cysts), lateral masses (branchial clefts), cystic hygromas, hemangiomas

-Neonatal torticollis!!: Shortening of SCM muscle with fibrous tumor over muscle produces head tilt/abnormal facies -> goes away with PT usually
-vision loss in eye thats not working as much

-Edema/webbing of neck suggest Turner syndrome
-Palpate clavicles for fractures

Question 17

congenital toxoplasmosis

Answer 17

-TRIAD:
-1. Hydrocephalus
-2. chorioretinitis
-3. diffuse intracerebral/cortical calcifications

-small, early-onset jaundice, hepatosplenomegaly, and generalized maculopapular rash
-seizures are common

-Dx- Serologic testing

-Tx- Pyrimethamine (supplement with folic acid) + sulfadiazine, up to 1 year

Question 18

congenital rubella

Answer 18

-TRIAD:
-B/L cataracts
-sensorineural hearing loss
-cardiac defects- PDA, pulmonary artery stenosis

-eyes: Cataracts, retinopathy, glaucoma
-Neurologic: Behavioral disorders, meningoencephalitis, developmental delay

-Dx: Serologic testing; isolation from blood, urine, CSF, throat

Question 19

congenital CMV

Answer 19

-MC congenital infection and leading cause of sensorineural hearing loss, intellectual disability, retinal disease, and cerebral palsy

-Microcephaly, thrombocytopenia, hepatosplenomegaly, hepatitis, intracranial calcifications, chorioretinitis, and hearing abnormalities

-Dx: Detection of virus in urine or saliva

-Tx: Ganciclovir has shown positive effect on hearing loss

Question 20

congenital herpes simplex

Answer 20

-Sx develop 5-10 days of life: Disseminated infection (liver, lungs, possibly CNS)

-Dx: Sampling from skin vesicle, NP, eyes, urine, blood, CSF, stool, rectum (PCR)

-Treatment: Acyclovir (parenteral)

Question 21

congenital syphilis

Answer 21

-Hepatosplenomegaly, snuffles, lymphadenopathy, mucocutaneous lesions, osteochondritis, rash, hemolytic anemia, and thrombocytopenia

-Later sx in untreated:
-!Hutchinson triad! - Interstitial keratitis, CN VIII deafness, Hutchinson teeth
-Bowing of the shins, frontal bossing, mulberry molars, saddle nose, rhagades (skin cracks), and Clutton joints

-CXR- >90% abnormal long bones consistent with osteochondritis/perichondritis

-Dx:
-Dark-field exam of direct fluorescent antibody staining of organisms obtained by scraping of a skin or mucous membrane lesion
-Serologic testing (VDRL/RPR)
-CSF should be examined as well
-Increased WBC count + protein concentration suggest neurosyphilis
-+ CSF VDRL is dx

-Tx:
-Penicillin (parenteral) x 10-14 days
-repeat titers at 3, 6, 12, months (neurosyphilis q 6 months x 3 years)

Question 22

congenital infection: zika, G/C, herpes

Answer 22

-HERPES:
-Sx- 5-10 days of life: Disseminated infection (liver, lungs, possibly CNS)
-Dx: Sampling from skin vesicle, NP, eyes, urine, blood, CSF, stool, rectum (PCR)
-TX: Acyclovir (parenteral)

-ZIKA:
-arthropod-borne via mosquitos
-transmitted to fetal brain -> craniofacial abnormalities, pulmonary hypoplasia, contractures
-microcephaly, cerebellar hypoplasia, ventriculomegaly, lessencephaly

-GONORRHEA:
-MC infection of eyes- ophthalmia neonatorum (1st 5 days)
-hyperacute onset of mucopurulent conjunctivitis
-TX: IM ceftriaxone x 1

-CHLAMYDIA:
-MC site is nasopharynx; conjunctivitis, PNA
-conjunctivitis- 1-2wks, mucopurulent
-Dx- culture discharge, Giemsa staining of conjunctival scraping is dx, PCR
-Tx- Azithromycin (PO) x 3days or erythromycin x 14 days

Question 23

down syndrome

Answer 23

-1:700 newborns
-facial features and generalized hypotonia

-Newborn: feeding problems, constipation, prolonged physiologic jaundice, and transient blood count abnormalities
-Childhood: thyroid dysfunction, visual issues, hearing loss, OSA, celiac disease, and atlanto-occiptal instability
-Increased incidence of transient myeloproliferative disorder and leukemia!!

-Facies: Upslanting palpebral fissures, flat nasal bridge, epicanthal folds, midface hypoplasia, flattened occiput
-Minor limb abnormalities
-Generalized HYPOTONIA!!
-up to 50% have CHD (septal defects)- VSD
-GI: Esophageal/duodenal atresias

Question 24

sex chromosome: turner syndrome (monosomy x)

Answer 24

-1:2500 females
-95% are miscarried
-Caused by missing X chromosome or structurally abnormal X chromosome (only a single functional copy of X chromosome) in females

-Short, webbed neck; edema of hands/feet, triangular facies
-Older females: Short stature, shield chest with wide-set nipples, mixed conductive/SN hearing loss, horseshoe kidneys, streak ovaries, amenorrhea, absence of development of secondary sex characteristics, infertility
-Cardiac anomalies: Coarctation of the aorta, bicuspid aortic valve (newborn), aortic root dilatation (adults)
-Learning disabilities common, secondary to difficulties in perceptual motor integration

-Tx:
-Hormonal therapy (GH, estrogen/progesterone)
-surgical intervention (cardiac anomalies)
-speech therapy/academic support

Question 25

klinefelter syndrome (XXY)

Answer 25

-1:1000 in males -rarely causes spontaneous abortions -After puberty: Small testes, gynecomastia, diminished facial/body hair, tall/eunuchoid build, decreased muscle mass -IQ borderline-normal -Extra X chromosome > decreased testicular growth > low testosterone > delayed/absent/incomplete puberty, azoospermia, and infertility -Tx: Testosterone replacement

Question 26

measles (rubeola)

Answer 26

-Highly contagious (droplet and airborne transmission) -caused by single-stranded RNA paramyxovirus -4 phases: Incubation (8-12 days from exposure to onset), prodromal (catarrhal), exanthematous (rash), and recovery -3 day prodromal period: Cough, coryza, conjunctivitis and pathognomonic Koplik spots! (gray-white, sand-grain-sized dots on buccal mucosa, opposite lower molars) -Conjunctiva with possible Stimson line! (transverse line of inflammation along the eyelid margin) -Exanthematous phase: Sx + FEVER -Macular rash, spreading from HEAD TO TOE over 24 hours; rash fades in the same pattern -Generalized lymphadenopathy (cervical nodes most prominent) -AOM, PNA, and diarrhea common in infants -Dx: -Serologic testing for IgM antibodies (1-2 days into rash, persist for 1-2 mo) -+/- genetic testing -> RT-PCR -Tx: -Supportive care– fluids, antipyretics -routine vitamin A x 2 days to children with acute measles

Question 27

rubella (german/3-day measles)

Answer 27

-Caused by single-stranded RNA togavirus, humans only host -Incubation 16-18 days, mild catarrhal symptoms -Retroauricular, posterior cervical, and posterior occipital lymphadenopathy with erythematous, maculopapular, discrete rash -Rash spreads from head to toe, lasts for 3 days -Rose-colored spots (FORCHHEIMER SPOTS) on soft palate may appear before rash -Other sx: Pharyngitis, conjunctivitis, anorexia, headache, malaise, LOW GRADE fever, polyarthritis, parasthesias, tendonitis -Dx: -Serologic testing for IgM antibodies (+ 5 days after onset) or by 4-fold or greater increase in specific IgG antibodies in acute/convalescent sera -Treatment: Supportive

Question 28

erythema infectiosum (5th ds)

Answer 28

-Caused by single-stranded DNA virus, parvovirus B19!! -Viral affinity for RBC progenitor cells -> aplastic crisis in pts with hemolytic anemias (SCD, spherocytosis, and thalassemia)!!! -Incubation 4-14 days -Begins with mild illness -> fever, malaise, myalgias, and headache -> rash 7-10 days later -3 stages of rash: -1. Initially: “Slapped cheek” rash with circumoral pallor -2. 1-4 days later: Erythematous, symmetric, maculopapular, truncal rash -3. Central clearing of rash takes place, distinct LACY!, RETICULATED! rash -Rash may be pruritic, does not desquamate -possible myalgia, significant athralgias/ARTHRITIS, headache, pharyngitis, coryza, and GI upset -May cause hepatitis, myocarditis, and papular-purpuric gloves and socks syndrome -Transient aplastic crisis (SCD): Fever, lethargy, malaise, pallor, headache, GI symptoms, respiratory symptoms -Extremely low reticulocyte count, low hemoglobin, transient neutropenia/thrombocytopenia -Dx: -Hematologic abnormalities: Reticulocytopenia x 7-10 days, mild anemia, thrombocytopenia, lymphopenia, and neutropenia -Detected by PCR and electron microscopy of erythroid precursors in bone marrow -Serologic testing (IgM antibodies) is diagnostic (detects infection within prior 2-4 months) -Tx: Supportive care, transfusion (aplastic crisis), IVIG for immunocompromised

Question 29

roseola infantum (exanthem subitum, 6th disease)

Answer 29

-Caused by double-stranded DNA virus, human herpesvirus type 6 (HHV-6) in most cases (HHV-7 in 10-30% of cases) -Major cause of acute febrile illnesses in infants -up to 20% of ER visits for 6-18mo -HIGH FEVER!!! (> 40C) with abrupt onset, lasts 3-5 days -> maculopapular, rose-colored rash (lasts 1-3 days) -URI symptoms, erythematous TMs, and cough -Dx: PCR for detection of HHV-6 in blood (does not differentiate latent, reactivation, or primary infections) -Tx: Supportive

Question 30

varicella zoster

Answer 30

-Caused by double-stranded DNA virus, varicella-zoster virus (VZV) -direct contact, droplet, and air -Incubation 14-16 days -Prodromal sx- fever, malaise, and anorexia may precede rash by 1 day -Rash progression: -Small red papules -> nonumbilicated, oval, tear-drop-like vesicles on an erythematous base -> vesicles ulcerate, crust -> heal -New crops appear for 3-4 days -begins on trunk -> head, face, and extremities (rare) -All forms of lesions are present at same time -Marked pruritis -Pre-eruption phase: Intense, localized, burning pain and tenderness (acute neuritis) along a dermatome, accompanied by malaise and fever -Rash progression: -Several days later, eruption of papules -> vesiculation (in dermatomal distribution/unilateral) -> crusting/healing -Thoracic and lumbar MC -CN V: Corneal/intraoral lesions -CN VII: Ramsay Hunt Syndrome –facial paralysis and ear canal vesicles -Postherpetic neuralgia: Pain persisting > 1 month is uncommon -Dx- PCR of vesicular fluid is method of choice -Tx: -Varicella- Anti-pyretics, cool baths, and careful hygiene -Acyclovir (all age groups), valacyclovir (> 2yo) -NOT recommended in otherwise healthy children ->Early (within 24hrs of rash) in immunocompromised -> effective in preventing PNA, encephalitis, and death -Zoster: -Acyclovir, valacyclovir, famciclovir (adults)

Question 31

coxsackie (hand-foot-mouth ds)

Answer 31

-Caused by coxsackieviruses, especially types A5, A10, and A16 -Mild fever, sore throat, and malaise -Rash: -Vesicles/red papules found on pharyngeal pillars, tongue, oral mucosa, hands (palms), and feet (soles) -Lesions may last 1-2 weeks -soft palate tiny red spots -nails are peeling 1-2 months after -swab for strep jic -Treatment: Supportive

Question 32

impetigo

Answer 32

-Erosions covered by honey-colored crusts -Staphy and group A streptococci -Tx: Topical (mupirocin, polymyxin, gentamycin, erythromycin) and/or PO -> if widespread (B-lactamase- resistant PCN, cephalosporins, clindamycin, amoxicillin-clavulanate) x 7-10 days -Bullous impetigo: Border filled with clear fluid; tx with PO abx x 7-10 days -Ecthyma: Firm, dry crust, surrounded by erythema that exudes purulent material (deeper form of impetigo affecting the superficial dermis); tx with systemic PCN

Question 33

cellulitis

Answer 33

-Erythematous, hot, tender, ill-defined, edematous plaques accompanied by regional lymphadenopathy -Invasion of microorganisms into lower dermis/beyond -MCC- GABHS (Group A beta-hemolytic streptococcal) and coagulase-positive staphylococci -Tx- systemic antibiotic

Question 34

folliculitis

Answer 34

-Pustule at follicular opening -MC- Staph and strep -Tx: Warm, wet compresses x 24 hrs, topical keratolytics, topical/PO antibiotics

Question 35

abscess

Answer 35

-Erythematous, firm, acutely tender nodule with ill-defined borders -MC- Staph -paronychia -Tx: I&D alone/with adjuvant antibiotics -bactrim

Question 36

bacterial conjunctivitis

Answer 36

-MCC: Haemophilus species, S. pneumoniae, M. catarrhalis, and S. aureus -Injected conjunctiva with significant discharge (purulent), typically unilateral > bilateral -very contagious -Tx: -Topical antibiotics: Polymyxin/trimethoprim sulfate or fluoroquinolones -Systemic therapy for conjunctivitis associated with C. trachomatis, N. gonorrhoeae and N. meningitidis

Question 37

viral conjunctivitis

Answer 37

-MCC is adenovirus -Injected conjunctiva with watery discharge -typically unilateral > bilateral -Enlarged preauricular lymphadenopathy can be present -red w/ no purulent discharge -Tx: -Supportive -Contagious 10-21 days from day of onset or as long as the eyes are red/tearing persists -Herpes conjunctivitis: Topical versus PO antivirals -when can you go back to school -> until you stop seeing discharge and at least 24hrs on the antibiotic

Question 38

allergic conjunctivitis

Answer 38

-Itchy, watery eyes with injected conjunctiva, usually bilateral! -Allergic shiners may be present -> nasal congestion causes venous congestion -may be a hx of asthma or other allergies -chemosis if severe- bubbling, swelling of conjunctiva -Tx: -Topical solutions that combine antihistamine and mast cell stabilizer -Systemic anti-histamines -Limitation of exposure to allergen -if not old enough for drops -> cool packs

Question 39

corneal abrasion

Answer 39

-Sudden, severe eye pain -Decreased vision, tearing, conjunctival injection, poor cooperation with ocular exam -Dx: -Fluorescein dye > illumination with Wood lamp -Evert upper/lower eyelids to evaluate for FBs -Tx: -Ophthalmic ointment, follow-up until healing complete -preventative antibiotics -refer to specialist 2-3 days later to make sure healing -if the pt cant tolerate drops for staining -> empiric tx and f/u with specialist who can sedate and view the eye

Question 40

blunt ocular trauma

Answer 40

-Blunt trauma can lead to orbital fx, retrobulbar hemorrhage can lead to orbital compartment syndrome -> permanent vision loss -Orbital blowout fx: Diplopia, pain with eye movements, restriction of EOM -CT scan! useful in dx extent of injuries -Orbital compartment syndrome: Severe eyelid edema and proptosis (can progress to this) -Neuroimaging will show retrobulbar hemorrhage and proptosis -Tx: -OBF: Nonurgent repair to prevent enophthalmos, advise not to blow nose (orbital emphysema/proptosis) -OCS: Emergent lateral eyelid canthotomy and cantholysis to decompress orbit

Question 41

eye foreign body

Answer 41

-discomfort, tearing, red eye -Pain with blinking suggests FB trapped under eyelid/corneal surface -every time you blink can be scratching the cornea -Tx: -Eyelid eversion! -Removal with irrigation or cotton applicator -Referral to specialist for failed attempt/corneal FB -Topical antibiotics typically prescribed for several days following FB removal -corneal FB / rust ring -> specialist

Question 42

pre-septal vs orbital cellulitis

Answer 42

-PRE-SEPTAL: -from local exogenous source (abrasion/insect bite), other infections (hordeolum, dacryocystitis), or after hematogenous spread -May progress to orbital cellulitis -Red, swollen eyelids; pain; mild fever -NORMAL- Vision, eye movements, and eye -TX: PO antibiotics -ORBITAL: -Serious complications: Subperiosteal abscess, meningitis, septicemia, and optic neuropathy -MC- from paranasal sinus infection -Proptosis!, eye movement restriction, decreased vision -Eye red and chemotic -decrease visual acuity -CT w/ contrast- establish extent and eval for subperiosteal abscess -Complications: Permanent vision loss (compressive optic neuropathy), corneal exposure/drying/scarring (proptosis), cavernous sinus thrombosis, intracranial extension, blindness, death -Tx: -Initially, broad-spectrum antibiotics -Surgical drainage of abscesses/sinuses

Question 43

acute otitis media

Answer 43

-MC reason for antibiotics -S. pneumoniae > H. influenzae > M. catarrhalis > S. pyogenes -2 critical findings: Bulging TM AND a MEE -Otoscopic findings: Bulging TM, impaired visibility of ossicular landmarks, yellow or white effusion, opacified/inflamed TM, squamous exudate/bullae on TM -Season (winter > summer) -Ages 1-3 -Eustachian tube dysfunction -Infants and young children more prone due to shorter, more compliant, horizontal ET -Trisomy 21, cleft palate risk factors -RF: Bacterial colonization of nasopharynx: S. pneumoniae, H. influenzae, M. catarrhalis -Smoke exposure, poor immune, daycare, genetics -Bottle feeding: aspiration into middle ear -Tx: -Pain control (1-3 days before antibiotic relieves pain) -Wait and see approach: -Observe episode w/o tx -Option for healthy children (> 2 years) with mild-moderate OM and no underlying conditions -Decision with parents to begin antibiotics if worsening or lack of improvement within 48-72 hrs -Antibiotic therapy: (know this) -1st line: High dose amoxicillin (80-90 mg/kg divided BID) -Augmentin if child has had amoxicillin within last 30 days or clinically failing x 48-72 hours on amoxicillin -Cephalosporins (cefuroxime, cefpodoxime, cefdinir) for those with rash to PCN – risk of cross-sensitivity is less than 0.1% -Macrolide (azithromycin) only if history of type 1 hypersensitivity to PCN -> Resistance of S. pneumoniae and H. influenzae (macrolide efflux pump) -Second-line antibiotics indicated when child experiences symptomatic infection within 1 month of finishing amoxicillin

Question 44

acute otitis externa

Answer 44

-Cellulitis of soft tissues of external auditory canal -> extend to surrounding structures -> pinna, tragus, and lymph nodes -Humidity, moisture, heat known to contribute -Trauma (breaks skin-cerumen barrier) -> first step in infection -> swab use, earbuds, scratching, ear plugs -MC organisms: S. aureus, S. epidermidis, P. aeruginosa -fungal in 2-10% -Acute onset of pain, aural fullness, decreased hearing, and pruritis (peak within 3 days) -touching tragus/pinna -> considerable pain -Discharge may be clear/purulent -EAC narrowed/swollen (may be difficult to visualize entire TM) -Complications: -Cellulitis of neck and face -Malignant OE: Spread of infection to skull base with resultant osteomyelitis -Tx: -Pain control -removal of debris from canal -topical antimicrobial therapy -avoidance of causative factors -1st line- Fluoroquinolone ear drops; combo with a steroid may be necessary (ciprofloxacin/dexamethasone) -> Ensure no TM perforation before STEROIDS! -PO antibiotics for any invasive infection signs (fever, cellulitis, cervical lymphadenopathy)

Question 45

middle ear effusion

Answer 45

-fluid in middle ear w/o signs or sx of acute inflammation -TM may be opacified/thickened, fluid can be clear/amber/or opaque, typically in neutral/retracted position -Pneumatic otoscopy and/or tympanometry for confirmation -Management: -Abx, anti-histamines, and steroids NOT shown to be useful in tx -Audiology eval after 3mo of continuous B/L effusion -Hearing loss/speech delay -> referral to ENT for possible T-tube placement -Uncomplicated -> observed 3mo with f/u q 3-6mo to eval for effusion clearance -T-tube indications: -Hearing loss > 40 dB, TM retraction pockets, ossicular erosion, adhesive atelectasis, and cholesteatoma

Question 46

tympanic membrane perforation

Answer 46

-AOM may result in rupture of TM -Discharge from ear seen with rapid relief of pain -Tx: -Ototopical antibiotics x 10-14 days with ENT f/u for exam/hearing eval -Failure to heal may require surgery (typically deferred until age 7, when ET has reached adult orientation)

Question 47

mastoiditis

Answer 47

-Infection from middle ear spreads to mastoid of temporal bone -Inflammation of periosteum to bony destruction of mastoid air cells with abscess development -any age, most < 2yo -Clinical dx! -Postauricular pain, fever, and outwardly displaced pinna -Exam: Mastoid appears indurated, red, swollen, fluctuant with severe tenderness (earliest sx) -AOM -> almost always present -Imaging Studies: -CT scan -> determine extent -Progressed ds: Coalescence of mastoid air cells with bone destruction -Complications: -Meningitis (high fever, stiff neck, severe headache) -Brain abscess (fever, headache, changes in sensorium) -Facial palsy, sigmoid sinus thrombosis, epidural abscess, cavernous sinus thrombosis, thrombophlebitis

Question 48

ear foreign bodies/cerumen impaction

Answer 48

-removed if easily visualized and with appropriate instrumentation -ENT referral for large FBs, rounded/globular objects, deep objects -Vegetable matter -> NEVER irrigated (may swell) -Button-battery -> emergent referral -Cerumen impactions removed if symptomatic and obstructing visualization of TM

Question 49

acute pharyngotonsillitis: viral pharyngitis: Infectious Mononucleosis (Epstein-Barr Virus)

Answer 49

-Infectious Mononucleosis (Epstein-Barr Virus) -MC > 5yo -Exudative tonsillitis!! -generalized cervical adenitis -fever -Dx: -palpable spleen or axillary lymphadenopathy increases likelihood of dx -> 10% atypical lymphocytes on peripheral blood smear or + Monospot -> supports dx (though false neg in kids < 5yo) -!!Epstein-Barr virus serology showing elevated IgM antibody is definitive -Tx: -Supportive -avoid contact sports -4-6-week f/u (LFTs) -Amoxicillin is contraindicated –> precipitates rash

Question 50

acute pharyngotonsillitis: bacterial pharyngitis

Answer 50

-20-30% w/ pharyngitis have group A streptococcal (GAS) -Other causes: Mycoplasma pneumoniae, Chlamydia pneumoniae, groups C/G streptococci, and Arcanobacterium hemolyticum -MC 5-15 yo -> winter/early spring -Sudden onset sore throat, fever, tender cervical adenopathy, palatal petechiae!, beefy-red uvula, and a tonsillar exudate -Headache, stomachache, nausea/vomiting -Scarlet fever!: 1-2 days into sx -> sandpapery rash (diffuse, finely papular, erythematous, blanchable) with strawberry tongue -Modified Centor Score -Def dx with throat culture or rapid antigen test -> RAT specific but only 85%-95% sensitive -KNOW THE CHART

Question 51

Acute pharyngotonsillitis: diptheria

Answer 51

-Acute infection of URT or skin -caused by exotoxin-producing Corynebacterium diphtheriae -Gram-positive, club-shaped rods on gram stain -Toxin absorbed into mucous membranes -> destruction of epithelium -> inflammatory response -> embedded in fibrin with WBCs/RBCs -> grayish pseudomembrane forms over tonsil, pharynx, larynx -Pharyngeal diphtheria: -Mild sore throat, moderate fever, and malaise -> rapid decompensation -Pharyngeal membrane forms (tenacious, gray, border of erythema/edema) -> may spread to nasopharynx or trachea -> obstruction -Cervical lymph node swelling -> “bull neck” -Dx: Culture of C. diphtheriae from infected tissue(s) -Tx: -Report to CDC to acquire anti-toxin (give within 48 hrs of sx) -Antibiotics (erythromycin IM/PO or procaine PCN G IM, q12hrs) x 14 days -Vaccination during convalescence, observation in hospital setting 10-14 days (isolate for at least 1-7 days, until respiratory secretions are noncontagious)

Question 52

strep complications (untreated)

Answer 52

-Acute rheumatic fever -Glomerulonephritis -Suppurative complications: Cervical adenitis, peritonsillar abscess, AOM, cellulitis, septicemia -Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus (PANDAS) -Sudden onset of OCD and/or tics (or worsening of such symptoms) following a strep infection

Question 53

croup

Answer 53

-MCC- parainfluenza virus serotypes!!!!!!! -6mo – 5yo -> fall and early winter -Edema in subglottic space -> signs of upper airway obstruction (inflammation of entire airway is typically present) -Prodrome of URT sx, followed by barking cough, laryngitis, and stridor -Mild: Stridor only with agitation -Worsening obstruction: Stridor at rest, retractions, air hunger, cyanosis, hypoxemia -Imaging: Not indicated (maybe in atypical presentations to r/o other causes) -Tx: -!!!Mild- Supportive, hydration; with or w/o dexamethasone -expose child to cold moist air -Dexamethasone: 0.15-0.6 mg/kg PO/IV x 1 dose -Adjuvant tx: Prednisolone (1 mg/kg) QD x 3 days and/or inhaled budesonide -!!!!Severe/stridor @ rest: Nebulized racemic epinephrine (diluted in sterile saline!) -give dexamethasone too (prevents rebound) -Sx resolution within 3-4 hrs of glucocorticoids/nebulized epinephrine -> safely discharged w/o fear of rebound -If recurrent nebulized epinephrine tx required -> admission for observation, supportive care, and repeat steroid dosing/neb tx PRN -Impending respiratory failure -> intubation with ET tube, with extubation within 2-3 days

Question 54

epiglottitis

Answer 54

-MC- Haemophilus influenzae!!!!!!!; streptococcal species in immunized pts -Sudden onset of high fever, dysphagia, drooling, muffled voice, inspiratory retractions, cyanosis, and soft stridor -Pts sit in “sniffing dog” position (neck hyperextended, chin stretched forward) -can progress to total airway obstruction -> respiratory arrest -Def dx: Direct inspection of epiglottis (OR during intubation) -> cherry-red and swollen epiglottis -!!!Imaging: Lateral neck radiograph -> “thumbprint” sign caused by swollen epiglottis -Tx: -ET intubation to establish airway -Blood and epiglottis cultures performed -IV antibiotics (ceftriaxone or equivalent cephalosporin) x 2-3 days, followed by PO abx x 10 days -Extubation within 1-2 days, when reduction in size of epiglottis is evident

Question 55

pertussis

Answer 55

-Caused by Bordetella pertussis!!!!!!(only infects humans, transmitted via coughing) -Incubation 7-10 days -> most contagious 1st 2 wks of cough -Peaks among <6mo of age (too young for complete immunization + most likely to have severe complications) -Catarrhal stage: 1-2 weeks -Non-specific signs (low-grade fever, nasal congestion) -Paroxysmal stage: 3-4 weeks -Coughing occurs in paroxysms during expiration (to dislodge plugs of necrotic bronchial epithelial tissues and thick mucus) -Forceful inhalation against narrowed glottis that follows = “whoop” -Convalescent stage: 1-2 weeks -Gradual resolution of sx -Dx: -Isolation of B. pertussis or PCR -Culture from NP swabs or aspirates -Lymphocytosis in 75-85% but not dx -Imaging: Segmental lung atelectasis, perihilar infiltrates -Tx: -Macrolides (azithromycin, clarithromycin, erythromycin)! -Complications/Prognosis: -MC is PNA (primary or secondary) -Other complications: Atelectasis, free air (from paroxysms), epistaxis, hernias, retinal/subconjunctival hemorrhage, OM, sinusitis

Question 56

asthma

Answer 56

-80% develop sx < 5yo -40% who have wheezing w/ viral infections in 1st few years -> continuing asthma -Strongest predisposing factor is atopy (personal or familial) -Shedding of airway epithelium, edema, mucus plug formation, mast cell activation, and collagen deposition beneath basement membrane -Air wall remodeling/irreversible changes -Dx- clinical -Auscultation: Prolonged expiratory phase with wheezing -Increased severity: High-pitched wheeze with diminished breath sounds -Severe obstruction: Nothing -Flaring of nostrils, intercostal and suprasternal retractions, use of accessory muscles -Cyanosis -Tachycardia/pulsus paradoxus -Agitation/lethargy may be signs of impending respiratory failure

Question 57

acyanotic vs cyanotic defects

Answer 57

-left to right shunts- acyanotic -right to left shunts- cyanotic

Question 58

VSD

Answer 58

-MC -Small VSD: asymptomatic; high-frequency, loud murmur -Moderate-large- HF -Auscultation: Pansystolic murmur at LLSB -Large shunts increase flow over mitral valve causing a mid-diastolic murmur at apex -Splitting of S2 and intensity of P2 depend on PA pressure -Imaging: -ECG: LAE/LVH -CXR: Cardiomegaly, LVH, increase PA size/blood flow -PHTN -> RVH -Echo: Location/size of defect + hemodynamic information (magnitude of shunt and R/PA pressure) -Tx: -1/3rd close spontaneously -Small: Close spontaneously; if not -> surgical closure -Initial for moderate-severe: Diuretics + digoxin/afterload reducers -> if still poor growth/PHTN -> surgical closure (closure device)

Question 59

ASD

Answer 59

-asymptomatic, even if large -RV impulse at LLSB palpable -Auscultation: Soft (grade I/II) systolic ejection murmur in region of RV outflow tract and a fixed split S2 (due to overload of RV with prolonged ejection into pulmonary circuit) -Larger shunt: Mid-diastolic murmur at LLSB from increased volume across tricuspid valve -Imaging: -ECG- RV enlargement -CXR- cardiomegaly, RAE, prominent pulmonary artery -Echo- location/size and magnitude of shunt -Tx: Significant shunt still at 3yo -> closure (cath lab – closure device)

Question 60

patent ductus arteriosus

Answer 60

-Small- asymptomatic -moderate-large- HF -Widened pulse pressure -Auscultation: Continuous, machine-like murmur at L infraclavicular area, radiating along pulmonary arteries -Larger shunts: Increased flow across MV -> mid-diastolic murmur at apex and hyperdynamic precordium -Splitting of S2/intensity of P2 depend on PA pressure -Imaging: -ECG- LVH -> RVH if PHTN present -CXR- full pulmonary artery silhouette and increased pulmonary vascularity -Echo- size/anatomy of PDA and magnitude of shunt and PA pressure -Tx: -Moderate-large/symptomatic: Indomethacin!!!!!!, diuretics, eventual closure (cath lab with coil embolization or PDA closure device)

Question 61

tetralogy of fallot

Answer 61

-MC cyanotic -4 structural defects: -1. VSD -2. Overriding aorta (over VSD) -3. Pulmonary stenosis -4. RVH -Pulmonary stenosis murmur -> initial finding -R-L shunting increases -Single S2 and right ventricular impulse at L sternal border -Hypoxic/Tet spells -Infant: Restless, agitated, inconsolable crying -Toddler: Squatting -> increase venous return -Hyperpnea with gradually increasing cyanosis -blue mucous membranes -> BAD -Severe spells: Prolonged unconsciousness and convulsions, hemiparesis, or death -Imaging: -ECG: RAD, RVH -CXR: Boot-shaped heart (small main pulmonary artery and upturned apex from RVH) -Echo: Anatomy of pulmonary stenosis, coronary anomalies -Tx: -Progressive pulmonary stenosis/cyanosis -Hypoxic spells: Oxygen, knee-chest position, ketamine/phenylephrine (increased SVR) -Indication for surgical repair -Complete surgical repair with VSD closure and removal/patching of pulmonary stenosis

Question 62

transposition of the great arteries

Answer 62

-Cyanosis, tachypnea, single S2 -Imaging: -ECG: RAD/RVH -CXR: Increased pulmonary vascularity, “egg on a string” cardiac shadow (narrow superior mediastinum) -Echo: transposition, sites/amounts of mixing, assoc lesions -Tx: -Prostaglandin E1 -> maintain patency/relax pulmonary vasculature -Balloon atrial septostomy -Arterial switch (complete surgical repair)

Question 63

tricuspid atresia

Answer 63

-Severely cyanotic, single S2 -Imaging: -ECG: LVH -CXR: Normal/slightly enlarged cardiac silhouette with decreased pulmonary blood flow -Echo: Anatomy, lesions, source of pulmonary blood flow -Tx: -Prostaglandin E1 (maintains pulmonary blood flow until surgery) -Surgery in stages: (dont need to know) -Blalock-Taussig procedure -Glenn procedure -Fontan procedure

Question 64

truncus arteriosus

Answer 64

-HF as pulmonary vascular resistance decreases -Tachypnea, difficulty feeding, poor growth -Bounding peripheral pulses -Auscultation: Possible systolic murmur with click at L sternal border, single S2 (single valve) -Imaging: -ECG/CXR: LVH + RVH, cardiomegaly, increased pulmonary vasculature -Echo: Anatomy, truncal valve function, and origin of PAs -Tx: -Medical management: Anti-congestive medications -Surgical repair: VSD closure with placement of conduit between RV and PAs

Question 65

total anomalous pulmonary venous return

Answer 65

-Most important determinant is presence or absence of obstruction to pulmonary venous drainage -pulmonary veins empty into RA instead of LA -Absence of obstruction: -Minimal cyanosis/asymptomatic -Hyperactive RV impulse, widely split S2 (increased RV volume) and a systolic ejection murmur at LUSB -Mid-diastolic murmur at LLSB from increased flow across the tricuspid valve -Presence of obstruction: -Cyanosis, marked dyspnea/tachypnea, and signs of R sided HF -Imaging: -Absence of obstruction: -ECG: RV overload -CXR: Cardiomegaly with increased pulmonary blood flow -Presence of obstruction: -ECG: RAD/RVH (from increased pulmonary congestion) -CXR: Heart normal/mildly enlarged with varying degrees of pulmonary edema -Echo: Extent of volume overloaded R side of heart, R-L atrial level shunting, and common pulmonary vein site of drainage/degree of obstruction -Tx: -Surgery: Common pulmonary vein opened into the LA, ligation of any vein/channel that had been draining the common vein

Question 66

hypoplastic left heart syndrome

Answer 66

-Ductus arteriosus constriction -> S&S of HF from excessive pulmonary blood flow/obstruction of systemic blood flow -Diffusely weak/absent pulses, usually no heart murmur, S2 single/loud -Low CO -> grayish color to cool, mottled skin -Imaging: -ECG: RVH with decreased LV forces -CXR: Cardiomegaly (R-sided enlargement) and pulmonary venous congestion/edema -Echo: Small L heart, degree of mitral/aortic valve stenosis, hypoplastic ascending aorta, adequacy of L-R atrial flow and R-L ductal flow -Tx: -Medical: PGE1, correction of acidosis, BP support as needed -Surgery is staged (newborn, 4-6 months, 2-3 years) -Heart transplant for failed surgical palliation or if systemic RV fails

Question 67

coarctation of the aorta

Answer 67

-Infants: -Hypoplastic aortic arch, abnormal aortic valves, and VSDs -Sx develop when ductus closes (2 wks) -Poor feeding, respiratory distress, and shock possibly sooner -Femoral pulses are weaker compared to R radial pulse -BP in LE < UE -Older children: -Leg discomfort with exercise, headache, epistaxis -Decreased/absent LE pulses, HTN of UE -Auscultation: L interscapular area of back, continuous (if collateral vessels have formed) -Systolic ejection murmur with click if abnormal aortic valve (50% of time) is present -Imaging -Infants: -ECG/CXR: RVH, marked cardiomegaly, pulmonary edema -Older children: -ECG/CXR: LVH, mildly enlarged heart; rib notching (from collateral vessels) -Echo: Site/degree of coarctation, presence of LVH, aortic valve morphology/function -Tx: -Infant with cardiac decompensation: IV prostaglandin (chemically opens ductus – closure would otherwise worsen coarctation), inotropic agents, diuretics, supportive care -Balloon angioplasty < surgical repair

Question 68

rheumatic fever

Answer 68

-Group A B-hemolytic streptococcal infection -Carditis -Pancardiac inflammation -Mitral valve MC affected -> insufficiency -Aortic valve 2nd MC affected -Early decrescendo diastolic murmur consistent with aortic insufficiency -Polyarthritis (80% of pts) -Large joints MC -> typically migratory -Joint swelling/limitation of movement -Sydenham Chorea -emotional lability, ataxia/slurring of speech, Muscular weakness -up to 3mo, may not be apparent for months to years after acute episode of rheumatic fever -Erythema Marginatum: Macular, serpiginous, erythematous rash with sharply demarcated border on trunk/extremities -Subcutaneous Nodules (severe cases) -Occur over joints, scalp, and spinal column -Few mm – 2 cm in diameter, nontender, free mobile -Jones- 2 major or 1 major + 2 minor PLUS evidence of streptococcal injection -Acute Treatment: -Anti-Infective tx: -Benzathine PCN: Single IM injection -Alternative: PCN V or amoxicillin -Allergic to PCN? Narrow-spectrum cephalosporins, clindamycin, azithromycin, or clarithromycin -Anti-inflammatories: -Aspirin -Tx for HF as indicated, activity limitations -Tx after acute episode: -Prevention -Regular, long-acting IM injections of benzathine PCN q 4 weeks x 5-10 years of therapy (or d/c at 21yo whichever is longer) -Alternatives: PCN V, sulfadiazine, erythromycin less effective -Residual Valvular Damage: Replacement, if indicated

Question 69

endocardial cushion / AV septal defect

Answer 69

-May be complete, partial, or transitional -Complete: ASD, posterior/inlet VSD, common AV valve -Partial/transitional: 2, separate AV valves -May have AV valve insufficiency at either level -common with downs syndrome -Sx of HF by 6-8 wks of life (decreased PVR) -PHTN present due to increased pulmonary blood flow, and pulmonary vascular obstructive ds may develop early -Murmurs vary -Imaging: -ECG: LAD and combined atrial enlargement/ventricular hypertrophy -CXR: Cardiomegaly with enlargement of all chambers and presence of increased vascularity -Echo is dx

Question 70

hypertension

Answer 70

-start checking at 3yo -Treatment (primary HTN) -1st line: Lifestyle changes with diet and exercise!!!!!!!!!!!!!! -Anti-HTN agents: ACEIs, ARBs, CCBs, diuretics -Maximizing monotherapy prior to introducing a second agent remains official guideline

Question 71

syncope

Answer 71

-Hx: -Age, time of day, state of hydration/nutrition, environmental conditions, activity/body position, frequency/duration of episodes, aura/prodrome/symptoms prior to episode -Additional: Meds, hx of viral illness, medical hx (neuro disorders, TBI, neurosurgical interventions), family hx (cardiac, exertional, sudden death in children, seizures), witnesses -NO loss of urine, NO AMS -Physical: -General: Hydration, nutritional status, thyroid disease manifestations -Orthostatic VS -Decrease in systolic BP of 20 mmHg or diastolic of 10 mmHg after 3 minutes of standing when compared to BP in supine or sitting position -Pulse strength, rate, and differences between U/L extremities -Heart murmurs > echocardiogram -Phenotype of inherited genetic disorders -ECG: HR, QT interval, T-wave abnormalities, ventricular arrythmias, AV conduction disturbances, or Brugada syndrome (predisposes to VT) -Tx: -adequate intravascular volume -Clear urine 5x daily -Increase salt -Leg pumping, leg crossing, and squatting with presyncopal sx -Regular aerobic exercise -Meds (limited studies)

Question 72

iron deficiency anemia

Answer 72

-ID- Insufficient iron to maintain normal functions such that iron stores are reduced -IDA- Hmg > 2 SDs below normal for age and gender, 2ndary to ID -Normal term infants born with sufficient iron to prevent ID x 4mo -Premature, LBW, neonatal anemia, perinatal blood loss, hemorrhage –> reduce iron stores -Breast milk is low in iron relative to cow’s milk and fortified formulas -Exclusively breast-fed -> 1 mg/kg/day of supplemental iron x 6mo -Pallor, fatigue, irritability -Hx of pica is common -Assoc with increased lead absorption – neurotoxicity -screening for anemia @ 12mo with hmg concentration and review of RF -RF: Low socioeconomic status, premature/LBW, exclusively breast-fed > 4 mo w/o iron supp, lead exposure, weaning to whole milk or complementary foods low in iron, feeding/growth problems, inadequate nutrition -Hmg < 11 = high-risk ID –> iron eval (iron saturation, ferritin, CRP, reticulocyte hemoglobin concentration) -Tx: -Hmg 10-11 at screening: Closely monitored/empirical tx with iron supplementation and a re-check in 1 mo -If ID/IDA: PO iron 3 mg/kg/day x 3 mo -supplementation -> increased reticulocyte hmg x 48 hrs; maximal between 5-7 days -Rise in hmg of 1 or more g/dL after 1mo is a good response -Therapy continued x 3 months to replenish stores of iron -Parenteral iron for those with CKD and erythrocyte stimulants maybe celiac or IBD as well

Question 73

idiopathic thrombocytopenic purpura (ITP)

Answer 73

-MC bleeding disorder of kids -Often after viruses (rubella, varicella, measles, parvovirus, influenza, EBV, or HIV) -Thrombocytopenia from clearance of circulating IgM/IgG-coated platelets by reticuloendothelial system -Most pts recover spontaneously x several mo -Acute petechiae and ecchymosis -Epistaxis and gingival bleeding are common -Dx Blood: -Platelets markedly reduced (< 50,000 and often < 10,000) -Large platelets on blood smear (accelerated new platelet production) -WBC/diff and Hb usually normal -PT and aPTT normal -Bone marrow: Megakaryocyte hyperplasia with normal erythroid and myeloid cellularity -Complications: -Severe hemorrhage and bleeding -RF: Platelets < 10,000/uL and mean platelet volume < 8 fL -Tx: -Observation in absence of bleeding, regardless of platelet count -Avoid NSAIDs, physical activities -Corticosteroids -Severe acute bleeding- intravenous immunoglobulin (IVIG) is tx of choice (alt or adjunct to corticosteroids) -Anti-Rh(D) IG: Only effective for Rh (+) pts -Splenectomy: -Considered if persists x 12mo and failure of preferred/alt 2nd-line tx -Thrombopoietin receptor agonists (eltrombopag, romiplostim); FDA-approved for > 12mo -Typically postponed to > 5yo if possible -Complete response in 70%, partial in 20% -Prognosis: -80% remission -Tx with combination steroid and IVIG better remission rates at 12 and 24mo than either alone

Question 74

immunoglobulin A vasculitis/Henoch Schonlein Purpura (HSP)

Answer 74

-MC type of small vessel vasculitis in kids -URI precedes dx in 2/3 -Antigens from GABHS, viruses, drugs, foods, and insect bites -Small vessels of skin, GI tract, and kidneys MC affected -Skin: Urticarial -> maculopapular -> symmetrical, palpable, purpuric rash on legs, buttocks, and elbows -Migratory polyarthralgia or polyarthritis (ankles/knees) -Intermittent, sharp abdominal pain (50%) -Renal involvement in 2nd-3rd weeks with nephritic or nephrotic picture, often high BP (25-50%) -Intussusception, hemorrhage/edema of small intestine, testicular torsion, neurologic symptoms also assoc -Dx: -Platelets normal/elevated, -hemostasis and platelet function are normal -UA with hematuria, sometimes proteinuria -Stool with occult blood possible -ASO titer elevated and throat culture + for group A B-hemolytic streptococci -Serum IgA may be elevated -Tx/Prognosis: -Supportive, NSAIDs, corticosteroids -If ASO titer elevated or + strep culture –> PCN -Good prognosis: Recurrence of symptoms in 25-50%, progressive renal failure in < 5%

Question 75

Acute lymphoblastic Leukemia (ALL)

Answer 75

-MC malignancy of kids (25% of cancer dx < 15yo) -Peak age- 4yo; 85% dx 2-10yo -Trisomy 21– 10-20x risk -Uncontrolled proliferation of immature lymphocytes -> 25% malignant hematopoietic cells (blasts) in bone marrow aspirate -> 70% w/ aggressive combo chemotherapy and early pre-symptomatic tx to CNS are cured -decreased production of RBCs, WBCs, or platelets, and leukemic infiltration of extramedullary sites -Intermittent fevers, bruising/pallor, bone pain (pelvis, back, legs) -PE- normal to highly abnormal -Pallor, petechiae, purpura -Hepatomegaly/splenomegaly (> 60%) -Lymphadenopathy, testicular enlargement, SVC syndrome (adenopathy), tachypnea/orthopnea/respiratory distress (mediastinal mass), CN palsies (leukemic infiltration), leukemic infiltration/hemorrhages of optic fundi, flow murmur/tachycardia (anemia) -Labs: -CBC w/ diff- most useful -decrease in at least 1 cell type (neutropenia, thrombocytopenia, or anemia) - 95% -WBC low/normal (50%), with neutropenia (< 1000) -Peripheral smear: RBC abnormalities (teardrop shapes) -Uric acid/LDH elevated as a result of cell breakdown -Bone marrow bx: Infiltration of leukemic blasts replacing normal marrow -Imaging: -CXR: Mediastinal widening/mass, tracheal compression due to lymphadenopathy or thymic infiltration -AUS: Kidney enlargement, intra-abdominal adenopathy -Plain films of long bones/spine: Demineralization, periosteal elevation, growth arrest lines, compression of vertebral bodies -Tx: -Induction (1st month)– > 95% remission -Consolidation -Intensification -Maintenance -Tumor lysis syndrome- anticipate -Hyperkalemia, hyperuricemia, and hyperphosphotemia -Maintaining urine output with IV fluids + PO allopurinol -Hyperleukocytosis (with hyperviscosity and respiratory distress, AMS): Leukopheresis -During tx, fever/neutropenia requires prompt assessment -> blood cx/tx with empiric broad-spectrum abx -Prophylaxis for PCP (trimethoprim-sulfamethaxzole) -Prognosis- Most important features: WBC count and age -1-9yo w/ WBC count < 50,000– survival rate > 90% range -Ages > 10 - ~ 88%

Question 76

acute myeloid leukemia (AML)

Answer 76

-25% of all leukemias kids -> but 1/3 of deaths -RF: Congenital, acquired (ionizing radiation, cytotoxic CTX agents, benzenes) -Aggressive induction therapy -> 75-85% remission -Anemia (44%), thrombocytopenia (33%), and neutropenia (69%) -Hyperleukocytosis: Venous stasis, sludging of blasts in small vessels (hypoxia, hemorrhage, infarction) -CNS leukemia present in 5-15% of pts at dx (higher than ALL) -auer rods

Question 77

hodgkin lymphoma

Answer 77

-Better response to tx than adults -5-10 year survival rate of > 90% -15% in children < 16yo (3% < 5yo) -M>F -Painless cervical lymphadenopathy -Firm, rubbery texture; not fixed to surrounding tissues; variable growth rate -Bx: No identifiable infection in region by enlarged node, > 2 cm, supraclavicular/abnormal CXR, increase in size after 2wks or failing to resolve within 4-8wks -Constitutional symptoms (33%): Fever, wt loss of 10% in prior 6mo, drenching night sweats (Ann Arbor staging) -Asymptomatic mediastinal disease (adenopathy, mass) in 50%: Sx with compression of vital structures -Labs: CBC usually normal (although anemia, neutrophilia, eosinophilia, and thrombocytosis may be present), ESR and other acute phase reactants often elevated -Staging: -Ann Arbor classification (determines tx and prognosis) -CT of neck, chest, abdomen, and pelvis, as well as PET scan (with or w/o bone marrow bx) -Pathologic Findings: -Reed-Sternberg cell (germinal-center B cells with malignant transformation) -20% in developed countries are + for EBV -Tx/Prognosis: -Based on stage, presence of B symptoms, tumor bulk, and # of involved nodal regions -CTX and/or immunotherapy alone – less often by radiation therapy -5-year survival rate- 90-95% in Stages I and II (slightly lower in Stages III and IV) -2/3rds of all relapses within 2 years after dx, relapse rarely beyond 4 years

Question 78

non-hodgkin lymphoma

Answer 78

-5-10% of malignancies in < 15yo -Incidence increases with age -3:1 male predominance -in Africa ~50% due to EBV and assoc w/ Burkitt lymphoma -congenital or acquired immune deficiencies -> 100-10K x greater risk -rapidly proliferating, high-grade, diffuse malignancies -> very responsive to tx though -Arise at any site of lymphoid tissue as well as extra-lymphatic sites (bone, bone marrow, CNS, skin, and testes) -S&S determined by location of lesions and degree of dissemination -Dx: -Bx of involved tissue with histology immunophenotyping, and cytogenetic studies -CBC, LFTs, and biochemical profile -> Elevated LDH reflects tumor burden -CXR, CT scan- neck/abdomen/pelvis, PET scan -Bone marrow and CSF exams -Tx/Prognosis -Management of life-threatening problems at presentation is critical -> Acute TLS, SVC syndrome, airway compromise, cardiac tamponade -Systemic CTX -Intensive intrathecal CTX for CNS prophylaxis x 3-9mo -LL: Tx protocols for ALL x 2 years -BL/BLL, LCBL, ALCL: Alkylating agents/methotrexate -Prognosis based on extent of ds at dx -90% with localized disease – long-term, disease-free survival -Disease on both sides of diaphragm, CNS involvement, or BM involvement – 70-80% failure-free survival rate -Relapses early – may have chance for cure with HSCT

Question 79

brain tumors

Answer 79

-MC solid tumors of childhood -misdx or dx late -Infratentorial tumors (<2): -Sx: Vomiting, unsteadiness, lethargy, irritability -Signs: Macrocephaly, ataxia, hyperreflexia, cranial nerve palsies -Supratentorial tumors (older kids): -Headache, visual symptoms, seizures, and focal neurologic deficits (personality changes) -Cerebellar/posterior fossa tumors: Morning vomiting -Brainstem tumors: Facial and extraocular muscle palsies, ataxia, hemiparesis, hydrocephalus -Dx: -MRI- study of choice (with and w/o contrast) -Imaging of entire neuraxis and CSF cytologic exam for medulloblastoma, ependymoma, and pineal region tumors -MRI of spine for midline tumors of 4th ventricle/cerebellum -Lumbar CSF >>>> ventricular CSF -Biomarkers (hcg and a-fetoprotein) helpful in dx and f/u -2 categories (based on cell origin): -1. Glial tumors: Astrocytomas, ependymomas -2. Embryonal tumors: Medulloblastomas, atypical teratoid/rhabdoid (AT/RT) tumors -Astrocytoma !!! -MC brain tumor of childhood -Low-grade may be curable by complete surgical excision alone -CTX effective alone in 40-50% of low-grade cases (multiple courses) -Tx: -Supportive care: Dexamethasone, anti-convulsants (levetiracetam) -Specific therapy -Maximal safe surgical resection is preferred initial approach -Radiation use varies -CTX effective in tx low-grade and malignant astrocytomas and medulloblastomas -Older children with malignant gliomas: Combination of above -Prognosis: -Low-grade astrocytomas: 5 and 10-year survival rates 60-90% -Low-stage medulloblastoma: 10-year survival rate ~ 40-60% -High-risk: 5-year survival rate ~ 25-40% -Glioblastoma: Cure rates poor (survival rates < 10%)

Question 80

neuroblastoma

Answer 80

-from neural crest tissue of sympathetic ganglia or adrenal medulla -Small cells with scant cytoplasm and hyperchromatic nuclei (rosette pattern) -> differentiate from other “small, round, blue cell” malignancies -MC solid neoplasm outside CNS -50% dx < 2yo and 90% < 5yo -Constitutional: Fever, wt loss, irritability -PE: Firm, fixed, irregularly shaped, midline abdominal mass -wherever there is sympathetic nervous tissue (location determines varied exam findings) -Common metastatic sites: Bone, bone marrow, lymph nodes, liver, and subcutaneous tissue -Skull/retrobulbar area: Periorbital ecchymosis, proptosis -Liver: Massive hepatomegaly -Skin: Bluish/purplish subcutaneous nodules (“blueberry muffin baby”) -Paraneoplastic: Opsoclonus-myoclonus ataxia (“dancing eyes/dancing feet”) -Rapid, chaotic eye movements, myoclonic jerking of limbs and trunk, ataxia, and behavioral disturbances -Labs: Anemia (60%), urinary catecholamines (VMA/HVA) elevated (90%) -Imaging: -X-rays- stippled calcifications -CT- extent of primary tumor, effects on surrounding structures, and presence of metastatic ds -MRI- spinal cord involvement -PET- bone metastases -Staging: -International Neuroblastoma Staging System/Risk Group -Bx of tumor and bone marrow -Tumors classified as favorable or unfavorable based on histologic characteristics and pt of dx (younger age -> more favorable dx) -Tx based on classification -Low-risk: Surgical resection of >50% of tumor is usually sufficient for cure; survival rates 98% -Intermediate-risk: Surgery + CTX; survival rates 90-95% -High-risk: Intensive, multimodal therapy including CTX, surgery, HSCT, irradiation, biologic therapy, and immunotherapy; 5-year overall survival rate 73%

Question 81

nephroblastoma (wilms tumor)

Answer 81

-2nd MC abdominal tumor in kids (after neuroblastoma -sporadic -MC between 2-5yo, unusual after 6 yo -Increasing size of abdomen or an asymptomatic abdominal mass -Mass is smooth, firm, well-demarcated, rarely crossing midline -Labs- CBC normal, possible anemia; UA blood/leukocytes -Imaging/Staging: -US/CT- mass -> Liver, chest for metastases -IVC evaluated via Doppler flow -Staging at time of surgery -Tx: -Surgical exploration of abdomen -Following excision, stage assigned, -> defines tx (CTX) -Higher stages require irradiation to tumor bed/sites of metastases -Overall cure rate of 90% (varies based on stage)

Question 82

osteosarcoma

Answer 82

-relationship between rapid bone growth and malignant transformation -Metaphyses of long bones primarily affected -Distal femur > proximal tibia > proximal humerus > mid-proximal femur -Pain with or w/o assoc soft tissue mass; systemic sx rare -Labs: Possible elevation in serum alk phosphatase or LDH levels -Imaging: -Permeative (“moth-eaten”) destruction of normal bony trabecular pattern with indistinct margins -Periosteal new bone formation and lifting of bony cortex may create a Codman triangle -Soft tissue mass + calcifications in radial/sunburst pattern -MRI > CT scan in defining primary tumor -CT scan chest/bone scan for metastases -MC sites of metastases: Lungs and other bony sites -Bx of tissue for def dx -Tx: -Surgery + CTX (prior to definitive surgery) -Resection of tumor -Postsurgical CTX x 1 year -> Highly radioresistant -Localized ds – 70-75% long term survival rate

Question 83

ewing sarcoma

Answer 83

-2nd decade of life -white males -Small, round, blue cell malignancy -Pain with or w/o swelling and erythema -Fevers, wt loss -Elevated LDH possible -Imaging/Staging: -Similar to osteosarcoma, but involves diaphyses of long bones -MRI of primary lesion -CT scan for staging (chest) -> MC sites of metastases: Lung, bone, BM -Bone scan, BM bx also essential -Further electron microscopy, immunocytochemistry, and/or cytogenetic analysis may be necessary -Tx: -CTX followed by surgery, radiation -CTX continued x 6mo following local control -Localized ds – 70-75% long-term survival rate

Question 84

rhabdomyosarcoma

Answer 84

-MC soft tissue sarcoma of childhood (10%) -Peak- 2-5yo, M > F, -70% dx < 10yo -anywhere in body -Classified into subtypes based on features: embryonal RMS and alveolar RMS -Embryonal RMS (70%): GU tract, head/neck; young children -Alveolar RMS: Trunk and extremities; older children/adolescents -Sx- Vary based on site affected -Staging: -CT and/or MRI scan -CT of chest as well – Lungs MC site of metastasis -Bone scan and bone marrow bx -Tx: (dont need to know) -Tumor excised if feasible (CTX prior if not) -Radiation for localized tumor control -CTX for all pts following resection -Low-risk – FFS 90%, intermediate-risk – FFS 60-70%, high-risk – FFS < 20%

Answer 85

-MRI is best imaging for osteosarcoma -tanner stage? -rheumatic fever