Peds Flashcards

Question

lymphadenitis

Answer 1

acute unilateral lymphadenitis - usually bacterial - 1) S aureus, 2) GAS - children will be non-toxic tularemia - can present with acute unilateral cervical LAD - affected children have systemic sxs - contact with animals in kids with perio-odontal disease - acute unilateral lymphadenitis due to peptostreptococcus non-tb mycobacteria - unilateral subacute-chronic LAD - overlying skin thins and becomes violaceous EBV - bilateral subacute-chronic LAD - plus fever, exudative pharyngitis, HSM (and transient hepatitis), atypical lymphocytosis - dx - positive heterophile antibody (monospot, 25% false negative during first week of illness) - complication - acute airway obstruction due to enlarging tonsils (give corticosteroids)

Answer 2

croup (laryngotracheitis) - parainfluenza - age 6 mo - 3 yrs, low O2 sats - upper airway obstruction - stridor (inspiratory, becomes inspiratory and expiratory in very severe cases), hoarse voice - steeple sign (subglottic edema) - mild - humidified air and corticosteroids x1 - severe - give dexa and racemic epi - to reduce edema bronchiolitis - RSV (in older kids, RSV is a self-limiting URI) - <2yrs - runny nose, wheezing, and coughing, RDS - tx - supportive care - prevention - palivizumab for infants who are <29 premie, chronic lung disease of prematurity, hemodynamically significant congenital heart disease - complications - apnea, respiratory failure, recurrent wheezing throughout childhood, associated with increased risk of otitis media diphtheria - grey-colored pseudomembrane ****************************************** when would you need to secure an airway - 1) intubate, 2) trach: epiglottitis - H flu - unvaccinated children - high fever, sore throat, dysphagia, tripod - thumb sign - swollen epiglottis retropharyngeal abscess - high fever, muffled voice, limited neck rotation (due to pain) - widened prevertebral space

Answer 3

ID, seizures, visual impairment due to capillary-venous malformations port-wine stain in trigeminal distribution

Answer 4

risk factors - prematurity, low birth weight, reduced mesenteric perfusion (hypotension, congenital HD), enteral feeds (formula > breast milk) - formula and milk are substrates for bacterial proliferation --> when milk is not absorbed --> bacterial fermentation, inflammation, translocation of bacteria and gas into bowel wall - TI and colon are most commonly affected bloody stools, **abd distention** xray - pneumatosis intestinalis (pathognomonic), portal venous gas, pneumoperitoneum labs - metabolic acidosis (lactate), leukocytosis tx - supportive care (bowel rest, IVFs) - BS IV abx - +/- surgery complications - septic shock, intestinal strictures, short bowel syndrome, death **************************************** vs milk protein enterocolitis - bloody stools, which will occur in a healthy infant 2-8 wks after sensitization to milk protein

Answer 5

aka pinworm perianal pruritis - at NIGHT - vulvovaginitis eggs on tape test tx - albendazole or pyrantel pamoate for pt and all household contacts

Answer 6

hair-pulling disorder (has nothing to do with eating the hair) - pts attempt to stop (but are unable to) - rule out medical and psych conditions first (body dysmorphic disorder) tx - CBT (habit reversal training) trichophagia - swallowing of hair

Answer 7

ectopic - 1) urine hcg 2) transvaginal US - risk factors are tubal damage - prior ectopic, hx of PID, prior tubal ovarian torsion - sudden-onset ruptured ovarian cyst - sudden onset PID

Answer 8

risk factor - poor hygiene, humidity handwashing for prevention - contagious infection non-bullous: S aureus, GAS (S pyogenes) - honey-crusted lesions (non-pruritic) - pts with underlying skin problems (eczema, abrasion) are at increased risk bullous - S aureus - rapidly enlarging flaccid bullae - scale at ruptured lesions both: topical abx (mupirocin) for limited disease, keflex for extensive skin involvement complications - PSGN

Answer 9

small erythematous patch --> red, indurated, tense, shiny plaque - raised, sharply demarcated margin overlying skin streaking and regional LAD - indicates lymphatic involvment

Answer 10

B1 - beriberi (peripheral neuropathy, heart failure), Wernicke-Korsakoff syndrome pellagra/B3 deficiency - diarrhea, dermatitis (in skin exposed areas, looks like a sunburn), dementia (mental status changes...poor concentration, irritability), death - can also have glossitis - common in developing countries (diet is primary of cereal/corn), IBD riboflavin/B2 - seborrheic dermatitis (often affecting genitals), pharyngitis, edema/erythema of mouth - normochromic anemia pyridoxine/B6 - *irritability, depression*, stomatitis, dermaitis - can also cause elevated homocysteine (known atherosclerotic risk factor) B12 - macrocytic anemia, p. neuropathy vitamin D - risk factors are increased skin pigmentation, exclusive breastfeeding - ping-pong ball skull, delayed fontanel closured, frontal bossing, rachitic rosary, wrist widening (long-bone joints), genu varum - labs - ca and phos close to normal, alk phos very elevated, PTH elevated, vitamin D down

Answer 11

erythema chronicum migrans = target lesion - and nonspecific constitutional sxs in wks-mo - multiple erythem amigrains - bells palsy (unilateral or bilateral) - meningitis - carditis (AV block) - migratory arthralgias late - mo-yrs - arthritis (will have leukocytosis and neutrophils), encephalitis, p. neuropathy confirm dx with ELISA and Western blot tx - doxcycline is often used (because it is effective in treating a coexisting infection that is carried by the same tick) - BUT doxy is contraindicated in pts <8 and pregnant women --> give *oral amox* instead

Answer 12

1) RVOT (pulm stenosis or atresia) 2) RVH 3) overriding aorta 4) VSD crescendo-decrescendo SEM and single heart sound (because pulm S2 is inaudible) - murmur becomes louder, cyanosis improves during squatting knee-chest - increases SVR - inhaled O2 - stimulates pulmonary vasodilation, decrease PVR and systemic vasoconstriction - IVFs improve RV filling and pulmonary flow Digeorge and DS

Answer 13

XR clinical features - prolonged bleeding after mild trauma - joint, muscle, GI, GU bleeding lab findings - prolonged PTT - normal plts, bleeding time, PT - decreased or absent factor 8 (A) or 9 (B) tx - administration of missing factor - desmopressin for mild hemophilia A complications/consequences - hemophilic arthropathy - late complication, due to Fe/hemosideran deposition --> synovitis and *fibrosis* of joint - -> px as chronic worsening joint pain and swelling, limited ROM

Answer 14

proliferation of metanephric blastema most common renal malignancy in childhood - 4th most common childhood cancer - usu sporadic - can be syndromic - WAGR (Wilms, aniridia, GU abnormalities, retardation), Beckwith-Wiedemann syndrome, Denys-Drash syndrome smooth firm abd mass that does not cross midline (vs neuroblastoma, will does cross the midline, and will have sxs) - lungs are the most common site for mets dx - abd US --> constrast CT tx - tumor excision or nephrectomy, chemo, +/- rads - 5 yr survival rate is 90%

Answer 15

HIGHLY contagious, respiratory droplets 1-2 wks - mild cough, rhinitis 2-6 wks - cough with whoop (post-tussive emesis) wks-mo - symptoms resolve gradually dx - pertussis culture or PCR, lymphocyte-predominant leukocytosis tx - macrolides (to reduce disease duration or transmission, depending on when in disease you give it) - ppx for close contracts with a macrolide (regardless of immunization status) avoid OTC antitussives - because of lack of proven efficacy and risk of tox in kids < 6yrs prevention - vaccine - DTaP given during pregnancy and early childhood (5 doses spread out) - Tdap booster in adolescence - vaccines dont provide lifelong immunity subQ emphysema in kids - severe cough --> high intraalveolar pressure --> air leaks, *pneumothorax* --> get CXR

Answer 16

type 1 collagen gene defect - skin, sclera, bone, tendon, ligament disorders (+hearing loss) note Marfans (AD) is a fibrillin-1 gene defect - aortic root dilation (diastolic murmur) - vs homocystinuria - ID... - vs Ehlers Danlos - not tall

Answer 17

graded by dilation 1 - nondilated ureter 2 - into pelvis and calcyes without dilation 3 - mild-mod dilation of ureter, pelvis, and calcyces 4 - moderate 5 - gross dilation of ureter, pelvices, and calyces, loss of papillary impressions consequence - renal scarring dx - voiding cystogram (VCUG) - kids with first UTI at age 2-24 mo --> renal and bladder US - get VCUGs in kids with recurrent UTIs

Answer 18

viral prodome annular pink herald patch on trunk --> oval lesions in Christmas tree pattern (along lines of tension) - pruritus tx - reassurance (spont resolution), antihistamines for pruritus

Answer 19

erythema multiforme - infection, medication eczema (atopic dermatitis) - very itchy - nummular eczema - infants - very itchy, on trunk, cheeks, and scalp - child/adult - lichenified plaques in flexural creases - tx - emollient and steroid ointment - complications - eczema herpitcium (superinfection), cellulitis/absces erythema marginatum - ring-like rash, comes and goes - acute rheumatic fever (JONES criteria) tinea - central clearing with raised borders - Dr. Kalan: ringworm vs eczema - ringworm will have central clearing, tx with clomitrazole - ringworm is a fungal infection erythroderma - exfoliative dermatitis, >90% of body scabies - pruritic papules folds/moist spaces chickenpox - several stages of lesions

Answer 20

multiple motor and vocal tics, onset < 18 yo, lasts for 1 yr tx - **antipsychotics** (second gen like risperidone are used due to fewer side effects) - a2 adrenergic agonists (for mild disease, clonidine, guanfacine) - behavioral therapy - habit reversal training, most effective note - tourettes is often comorbid with adhd and ocd but they are not the same and are not treated with the same meds

Answer 21

failure to thrive - kids will start on the growth curve and then fall off respiratory (due to defective mucociliary clearance of secretions) - obstructive lung disease --> bronchietasis - recurrent pneumonia - chronic rhinosinusitis and nose bleeds (vitamin K deficiency) - orgs: s aureus is the most common pathogenic org in young children (<20 yo), above age 20 pseudomonas dominates - -> give cefepime (MSSA, pseudomonas), vanc (MRSA), IV if severe pneumonia - -> amox does NOT cover S aureus (covers S pneumo) - -> azithro covers atypicals - -> cipro covers pseudomonas GI - meconium ileus, distal intestinal obstruction syndrome - pancreatic disease (due to duct obstruction) - exocrine pancreatic insufficiency, CF-related diabetes (- pancreatitis is not on this list) - biliary cirrhosis reproductive - infertility (95% of men, due to congenital absence of vas deferens) MSK - osteopenia, kyphoscoliosis, digital clubbing ********************************************************* CF v.s. Kartageners: both have chronic sinopulmonary infections and nasal polyps (intranasal glucocorticoids, surgical resection) primary ciliary dyskinesia - situs inversus, infertility, normal growth CF - pancreas (ADEK def, poor growth), infertility, FTT

Answer 22

opiates - heroin and methadone - increased risk of IUGR, macrocephaly, SIDs, neonatal abstinence syndrome (NAS) - NAS - irritability, high-pitched cry, poor sleeping, tremors, sweating, tachy (withdrawal symptoms) - -> can present a few days after birth (up to a mo after birth) cocaine - jitteriness, excessive sucking, hyperactive Moro - but sxs are not as severe as opiates

Answer 23

SPF 15-30 15-30 min before sun exposure - reapply every 2 hrs - sunscreens above SPF 50 dont provide any increased UV protection - avoid sunscreen in infants < 6 mo (because of thinner skin and high SA/BW ratio) mild-moderate tx - cool compresses, calamine lotion, aloe, NSAIDs severe - hospitalize, IVFs and analgesia, wound care random note - dark fabrics offer greater UV protection because the rays cant penetrate the fabric as well

Answer 24

blood, sex, vertical transmission key thing to look for - HBeAg indicates infectivity - if pos - 95% infection risk - if neg - 20% infection risk for newborns with moms who have active hep B - give HBIG (for temp protection) then HBVax 90% of hep B in the newborn will progress to chronic hepatitis

Answer 25

Malassezia globosa - exposure to hot and humid weather hypopigmented, hyperpigmented lesions - surrounding skin will sunburn but spots wont sunburn - may have fine scale/pruritus dx - KOH prep will show spaghetti and meatballs pattern tx - topical ketoconazole, terbinafine, or selenium sulfide ************************************* mycosis fungoides - also a hypopigmented rash - may be an initial px of cutaneous T cell lymphoma vitiligo - lesions are asymptomatic

Answer 26

premature ovarian failure - ovarian dysgenesis --> low estrogen and progesterone --> absent thelarche, abnormal feedback to pituitary - elevated FSH and LH - GH will be normal - short stature is a result of loss of genes from X chromosome - consequence of estrogen deficiency --> decreased bone mineral density - give these girls estrogen replacement therapy - gonadal dysgenesis is associated with 15-30% of malignancy - close surveillance and gonadectomy coarctation of aorta, bicuspid aortic valve - echo --> thoracic MRI horseshoe kidney congenital (nonpitting) lymphedema - abnormal development of the lymphatic network --> cystic hygroma

Answer 27

airborne (negative pressure room, N95 mask) cough, coryza, conjunctivitis, fever, Koplik spots - NEXT maculopapular exanthem - spares palms/soles (fever still concurrent) dx - PCR - anti-measles IgM and IgG tx - supportive, vitamin A for hospitalized pts (vitamin A def is assoc with increased morbidity with measles infection) complications - otitis media, pneumonia, encephalomyelitis (wks), subacute sclerosing panencephalitis (yrs) MMR vaccine (x2 doses at ages 1 and 4) - vaccine-strain (live attenuated) - can cause a mild self-limiting case of measles within 1-3 wks - avoid contact with immunocompromised (no need for airborne precautions) * ******************************************* v. s roseola - rash appears as fever subsides rubella = 3d measles, lower fever, (no arthritis)

Answer 28

unimmunized children fever, parotitis (bilateral, facial swelling) self-limited - but can lead to aseptic meningitis, orchitis

Answer 29

infectious - acute, constant, painful, elevated WBC/plts and inflammatory markers inflammatory/rheum - subacute-chronic = systemic-onset juvenile idiopathic arthritis - worse in morning + morning fevers, rash - able to bear weight, common in multiple joints - WBC/plts and inflammatory elevated, low RBC (anemia of chronic disease), thrombocytosis, hypergammaglobulinemia, hyperferritinemia (acute phase reactant) - uveitis is a common complication - tx with NSAIDs, glucocorticoids, biologics reactive arthritis - few weeks after an enteric (campy, shigella) or chlamydial infection - asymmetric inflammatory arthritis, urethritis, conjunctivitis, uveitis neoplastic (ALL)- subacute-chronic - worse at evening/night - low WBC/plts ******************************************************** non-inflammatory - OA - clear, low WBC count inflammatory - crystals, RA - translucent, opaque - corticosteroid shot septic joint - opaque fluid, 50-150K WBC, majority PMNs - get BCs and aspirate joint - most common orgs are s aureus and strep --> IV vanc for empiric tx and surgical drainage (delayed of debridement for even 4-6 hrs can lead to femoral head necrosis) - gram stains can be falsely negative if pt has been pretreated with abx - birth - 3mo - staph, GBS, gram negative bacilli (anti-staph + gent or cefotaxime) - older than 3 mo - staph, GAS, S pneumo (naf, clinda, cefazolin, vanc)

Answer 30

aplastic anemia - causes - drugs (NSAIDs), toxic chemicals (glue), idiopathic, viral infections (HIV, EBV), immune disorders, thymoma, fanconi anemia (chromosomal breaks, DNA repair genes) - bone marrow bx essential for dx - will show decreased cell lines (all) and fatty infiltration of the marrow Fanconi's anemia - **pan**cytopenia, congenital abnormalities - px at 4-12 yrs of age - short stature, microcephaly, abnormal thumbs, hypogonadism - skin - abnormal pigmentation/spots - strabismus, low-set ears, middle ear abnormalities - definitive tx is bone marrow transplant Diamond-Blackfan anemia - congenital pure red cell aplasia - presents in first 3 mo of life with pallor and poor feeding - anemia with low retics - congenital anomalies transient erythroblastopenia - acquired red cell aplasia that occurs in healthy children leukemia - pancytopenia because of crowding out of normal bone marrow - leukocytosis - ALL- predominant type of leukemia from ages 2-10yrs - -> infections, LAD, HSM (due to extramedullary leukemic infiltration) - -> dx - >25% blasts in bone marrow, PAS positive, TdT positive (on pre-lymphoblasts) - -> imatinib - tyrosine kinase

Answer 31

firm palpable "mass" = SCM - flattening of head (positional plagiocephaly) - common consequence risk factors - crowding in uterus, positioning issues (developmental dysplasia of hip, metatarsus adductus, clubfoot) tx - tummy time, passive stretching, physical therapy

Answer 32

XR anosmia/hyposmia no GnRH secretion female - no breast development, primary amenorrhea, no body hair male - no body hair, small phalus and testicles tx - hormone treatment

Answer 33

inattentive or hyperactive sxs > 6 mo - kids can get in trouble, lose friends due to intrusive behavior (rejection, teasing) - several sxs present before age 12 - sxs in 2 settings tx - stimulants - methylphenidate, amphetamines - nonstimulants - atomoxetine, a2 adrenergic agonists - behavioral therapy

Answer 34

administration of multiple vaccines at one visit is safe - UNLESS it is a live attenuated vaccine - administer those 4 wks apart (due to possible interference by immune response) give vaccines based on chronological age (vaccination of premies is safe) hepB= birth and 2 mo 2-4-6 mo - rotavirus, DTaP, H flu, pneumococcal, inactivated polio 1 yr - MMR, varicella meninogococcal - 11 and 16 yo ******************************************************** rotavirus - 2-8 mo, live attenuated - rotavirus - most common cause of gastroenteritis (watery osmotic diarrhea) in infants and young children - fecal-oral - contraindications - ...hx of intussusception, hx of uncorrected congenital malformation of GI tract (Meckels diverticulum), SCID

Answer 35

umbilical hernia - age 5 gastrochisis - RIGHT of cord insertion (bowels) - bowel is exposed to amniotic fluid --> causes inflammation and edema --> increased risk of complications and dysmotility - immediate surgery after birth - NG tube and abx prior to surgery - note - due to a vascular defect omphalocele - midline defect (multiple organs) - immediate surgery after birth

Answer 36

in kids older than 3 (due to anatomy/micro) strep pharyngitis + arthritis --> 2-4 wks later --> acute rheumatic fever (JONES) - joints - *migratory* arthritis (supportive care) - O - carditis - nodules - subQ - erythema marginatum - pink rash with sharp edges - sydenham chorea - minor criteria late sequelae - mitral regurg and stenosis *************************************** ppx for ALL (duration varies) - IM penicillin G q4wks - goal is to prevent recurrent GAS pharyngitis heart disease = mitral stenosis (loud first heart sound, mid-diastolic rumble) rheumatic fever without carditis - 5 yrs or until 21 yo (whichever is longer) rheumatic fever with carditis but no residual heart/valvular disease clinically or by echo - 10 yrs or until 21 yo (whichever is longer) rheumatic fever with carditis and persistent heart or valvular disease - 10 yrs or until 40 yo old (which ever is longer) *********************************** for symptomatic mitral stenosis - preload reduction by diuretics or nitrates - can cause afib - bblockers, digoxin, warfarin --> mitral stenosis --> atrial dilation, afib --> chronic pulm HTN

Answer 37

coxsackie, adenovrius - direct viral injury, autoimmune inflammation --> myocyte necrosis px - viral prodrome and signs of HF - worsening dyspnea/respiratory distress, syncope, tachy, N&V, *hepatomegaly*, holosystolic murmur (dilated CM, functional mitral regurg) dx - CXR - cardiomegaly, pulm edema - echo - decreased EF, diffuse hypokinesis - send viral studies endomyocardial bx is gold std - for dx - inflammatory infiltrate with myocyte necrosis tx - diuretics, inotropes - send to ICU - monitor for fatal arrhythmias and shock mortality is high, morbidity is cardiomyopathy/chronic heart failure

Answer 38

fever for **5 d** - cervical LAD - rash - bilateral nonexudative conjunctivitis - mucositis - swelling/erythema of palms/soles disease itself is self-limited BUT can result in coronary artery aneurysms - so get echo at time of dx (and at 2-6-8 wks after) give single high dose of IVIG (within first 10d of presentation) - and ASA ************************************* Reyes syndrome: ASA after flu or varicella infection - ASA is a mitochondrial toxin px - vomiting, abnormal behavior --> seizures and lethargy features - acute liver failure, encephalopathy (kids will be in a coma, require intubation) - elevated intracranial pressure is a major cause of death histology - microvesicular steatosis tx - *supportive*

Answer 39

cafe au lait - McCune-Albright, NF congenital dermal melanocytosis (Mongolian spots) - blue-gray - more common in Asians and AAs - will spontaneously fade in first decade of life congenital melanocytic *nevus* - hairy, benign melanocyte proliferation - transformation to melanoma increases with size - large lesions are removed surgically nevus simplex and nevuxs flammeus (port-wine) - flat, blanchable, vascular birthmarks strawberry/superficial hemangioma - complications are rare - but give b-blockers (- cherry - adults)

Answer 40

will present after 6 mo - when fetal Hgb disappears baseline anemia - normocytic and normochromic - but can get co-existing folate, other vitamin deficiencies because bone marrow tries too make too many blood cells - if folate def - macrocytic anemia and LOW retic count acute severe anemia (hyperhemolytic crisis, nl retics) - aplastic crisis - secondary to parvovirus B19 infection, decreased retics - splenic sequesteration (occurs in younger pts) - splenic vaso-oclusion (rapidly enlarging spleen) --> autosplenectomy (usu by age 5) - -> retics will be increased - can lead to shock - because a large portion of the total blood volume is trapped in the spleen vaso-occlusive crisis - dactylitis (sausage fingers) - acute pain crises - hydration, analgesia, +/- transfusion - prolonged priapism avascular necrosis of femoral heads - *progressive* hip pain on weight-bearing osteomyelitis - 1) Salmonella, 2) S aureus - give anti-staph and ceftriaxone pts with SCD are at increased risk for stroke Howell-jolly bodies - nuclear remnant of RBCs (that spleen will normally remove) maintenance - vaccination - S pneumo, H flu, N meningitidis (if vaccinated kids present with sepsis - most likely will be S pneumo (non-vaccine serotypes)) - **penicillin until age 5** - bid - folic acid supplementation - hydroxyurea for pts with recurrent pain crises (increases fetal Hgb, side effect is myelosuppression, macrocytosis)

Answer 41

self-mutilating behaviors - biting fingers and tongue, dystonia, writhing movements, delayed motor development, nephrolithiasis, gout XR - deficiency of hypoxanthine-guanine phosphoribosyl transferase (HPRT) - involved in purine metabolism...increased levels of uric acid allopurinol to reduce uric acid levels

Answer 42

hct > 65% causes - increased EPO from intrauterine hypoxia - mat DM or pre-eclamp (due to poor placental gas exchange), HTN, smoking, IIGR - erythrocyte transfusion - delayed cord clamping (excess transfer of placental blood), twin-twin tranfusion - genetic/metabolic disease - hypo/hyperthyroid, genetic trisomy px - most are asx, ruddy skin, RDS, cyanosis, apnea - hypoglycemia (increased uptake by RBCs), hyperbili - **increased blood viscosity impairs blood flow to various organs** tx - IVFs, glucose, partial exchange transfusion note - dehydration can increase Hct but it is very unusual in term neonates in the first 2d of life

Answer 43

common cause of respiratory distress CXR - increased pulmonary vascular markings/fluid in fissures - lungs have a little bit of fluid left in them (c-section baby, doesnt get the "squeeze") will resolve in 24hrs

Answer 44

recurrent sinopulmonary infections with *encapsulated bacteria* all have normal B cells except x-linked agammaglobulinemia WHEN WOULD YOU GIVE IVIG: x-linked agammaglobulinemia - absence of lymphoid tissue on exam - small tonsils, LNs - low B cells and immunoglobins, normal T cell concentration, no response to vaccinations - tx - IVIG replacement therapy, ppx abx if severe CVID - ADA deficiency (mainly affects B cells, T cell immunity normal) - severe viral, fungal, and bacterial infections - respiratory and GI (giardia) infections, AI disease, chronic lung disease - failure to thrive - long-term IVIG (these pts have very low Ig levels, show no response to vaccination) T CELL PROBLEMS: SCID - severe T cell deficiency --> leads to B cell dysfunction as well (CD3 T cells, CD19 B cells) - recurrent viral, fungal, and opportunistic infections - failure to thrive - diarrhea - tx - early stem cell transplant hyper-IgM syndrome - XL, defective CD40 ligand - CD40 ligand on T cells bind to CD40 receptor on B cells - responsible for class switching (IgM --> IgG, etc.) - tx - abx ppx, interval administration of IVIG IG DEFICIENCY: (selective) IgA deficiency - most common primary immune deficiency - usu asx, recurrent sinopulm and GI infections - associated with autoimmune disease and atopy - anaphylaxis during tranfusions - tx - supportive care, medical alert bracelet IgG subclass deficiency OTHER: complement deficiency - encapsulated (S pneumo, N menin) - CH50 assay CGD - impaired oxidative burst --> chronic granulomatous disease, catalase pos orgs (S aureus, Serratia, Aspergillus) - infected LN - numerous organism filled neutrophils - dihydrorhodamine test, nitroblue test - need lifelong antimicrobial ppx - IFN gamma (immunomodulator) helps HIV infection should be excluded ************************************* transient hypogammaglobulinema of infancy - low serum IgG levels due to cessation of breast feeding - counts will return to normal by age 12 mo

Answer 45

DS: complete AV septal defect is the most heart defect - failure of endocardial cushions to merge - volume overload and excessive pulmonary blood flow - pt will present as diaphoretic/dyspneic during feeds - loud S2 due to pulmonary HTN, SEM due to ASD, holosystolic murmur of VSD atlantoaxial instability (10-15%, but only 2% are symptomatic) --> compression of SC --> behavioral changes, torticollis, urinary incontinence, dizziness/vertigo, UMN sxs - dx by c-spine radiographs - tx by fusion of C1-C2 ******************************************************** trisomy 18 - micrognathia, prominent occiput, low set ears, heart defects, renal defects, limited hip abduction - clenched hands and overlapping fingers, rocker bottom feet - die in first year of life trisomy 13 - cutis aplasia (no epidermis over skull) - midline defects - holoprosencephaly, omphalocele VSDs are common

Answer 46

RFs - prematurity, lead exposure, milk-based diet or limited diet - note - children should not be started on cow's milk until 1 yo (cows milk is low in Fe and can lead to occult intestinal blood loss) anemia of prematurity - impaired EPO production, short RBC life span, repeated blood draws - usu asx, sometimes tachy, apneic, poor weight gain - normocytic, normochromic RBCs - tx - minimize blood draws, Fe supplementation, transfusions (but this will further suppress EPO levels and delay recovery) dx - universal screening Hgb at age 1 (children will not present with classic symptoms) *tx - empiric trial of Fe supplementation*

Answer 47

6 mo - 3yrs lead point - Meckels, Henoch-Schonlein purpura, celiacs, intestinal tumor, polyps tx - *air* or saline enema (barium enema is no longer used because it can leak and cause peritonitis), surgery for removal of lead point - air enema is less messy

Answer 48

disruptive mood dysregulation disorder - severe, pervasive irritability and temper tantrums ODD - tempermental, hostile, angry, defiant towards authority

Answer 49

RDS - incidence is inversely proportional to gestation age - risk factors - *prematurity*, male sex, perinatal asphyxia, maternal diabetes (hyperinsulinemia affects surfactant production), c-section without labor - CXR - diffuse reticulogranular pattern, air bronchograms - antenatal treatment with corticosteroids, postnatal treatment with surfactant PDA - continuous flow murmur - kids will be healthy - mildly accentuated peripheral pulses bronchopulmonary dysplasia intraventricular hemorrhage - due to capillary fragility and immature autoregulation of cerebral blood flow - need screening head ultrasounds NEC retinopathy of prematurity antenatal corticosteroids - reduce IVH, improves overall mortality

Answer 50

initially well appearing, development of jaundice over 1-8 weeks - **jaundice and hepatomegaly** - acholic stools - conjugated hyperbili, mild elevation in transaminases dx - ultrasound will show absent/normal gallbladder - HIDA - failure of tracer excretion - liver bx - expanded portal tracts with bile duct obstruction - intra-operative cholangiogram (gold std) - biliary obstruction tx - hepatoportenterostomy (Kasai procedure, temporizing) - liver transplant - without tx - the liver will become inflamed (hepatomegaly, hepatitis) and eventually fibrose conjugated (direct) hyperbili is ALWAYS pathologic other reasons for jaundice in infants - physiologic jaundice - within the first 24hrs, indirect hyperbili - breast milk jaundice - second week of life, indirect hyperbili - Dubin Johnson - AR, conjugated hyperbilirubinemia, typically individuals are asx except for mild scleral icterus - Crigler-Najjar and Gilbert - UDP-glucuronyl transferase deficiency/absence - erythroblastosis fetalis (alloimmune hemolytic disease) - indirect hyperbili, pos Coombs (tests for antibodies)

Answer 51

Bartonella henselae, gram neg bacilli papule at scratch/bite site (but most pts wont remember the initial bite), tender regional adenopathy, fever of unknown origin tx - self-limiting (1-2 mo), azithromycin

Answer 52

findings of all congenital infection - IUGR, HSM, jaundice, blueberry muffin spots (extramedullary hematopoesis) toxo (cat litter) - macrocephaly, chorioretinitis, diffuse intracranial calcifications CMV - chorioretinitis, periventricular calcifications rubella - 1) cataracts, 2) sensorineural hearing loss, 3) PDA - dx rubella IgM, PCR - prevention - vaccine for mom - in older children - fever and cephalocaudal spread of maculopapular rash (spares palms and soles) + LAD - in adults - may be asx or present with fever, rash, and arthralgias/arthritis varicella - limb hypoplasia, cataracts, scarring syphilis - hepatomegaly, nasal discharge, osteoarticular destruction, maculopapular rash (desquamating or bullous rash), abnormal long-bone radiographs - prenatal dx (mom's serologies) and penicillin tx can prevent the majority of cases HSV - acquired perinatally - vertical transmission is rare - brain destruction, seizures, vesicular lesions

Answer 53

due to withdrawal of estrogen - presents in neonatal period, lasts < 1wk, exam nl - effect of maternal hormones may also lead to temp breast bud and ext genitalia engorgement during first mo of life v.s. trauma - fall, sexual abuse, genital exam will show lac/abrasion vaginal foreign body - TP, foul smelling discharge - irrigation with warmed fluids, swab for removal rhabdomyosarcoma - rare, presents < 3yo, protruding vaginal nodules

Answer 54

most common cause of short stature and pubertal delay in adolescents - normal birth wt/ht --> between 6 mo and 3yrs, child drops percentiles, but tracks normally on curve puberty and growth spurt are delayed - but WILL occur - bone age will be delayed compared to chronological age

Answer 55

pain with neck *flexion* BACTERIAL less than 3 mo - *GBS*, ecoli, listeria, HSV - listeria - meningitis, granulomas? - 3 mo - 10yrs - s pneumo, N meningitidis - 11yrs - N meningitidis listeria - mom will have flu-like sxs too meningococcal disease - petechial rash - give ceftriaxone and vanc - Waterhouse-Friderichsen syndrome = shock and skin rash (large purpuric lesions on the flanks), 100% mortality kids >1mo - CBC, BCs, LP (no need for CT beforehand because infants have open fontanelles so herniation is rare) - IV vanc and ceftriaxone (most common bugs are Strep pneumo and N meningitidis) - dexa for H flu type B (reduces associated sensorineural hearing loss) ************************************ viral - echovirus, coxsackie, normal glucose - supportive tx tb meningits - like bacterial, but glucose is very low (<10) GB - very high protein

Answer 56

GERD - very common (usu resolves by 1 yr) - physiologic - asx, happy spitter, give reassurance and positioning therapy (frequent small volume feeds, hold infant upright after feeds, prone when awake) - due to short esophagus and incompetent LES - pathologic - failure to thrive, irritability, Sandifer syndrome (arching back, twisting neck) - -> thickened feeds, antacid therapy - -> esophageal pH probe and EGD (if severe) milk protein allergy - regurg/vomiting, eczema, bloody stools - eliminate dairy and soy protein from diet - switch to hydrolyzed formula - will resolve by age 1

Answer 57

type 1: impaired glycogen to glucose conversion - hypoglycemia (seizures), lactic acidosis (build up in liver), hepatomegaly - doll like features

Answer 58

most common in AA children transmission by direct contact or fomite (dermatophyte infection) scaly erythematous patch with hair loss +/- tender postauricular LAD tx - **oral** griseofulvin or terbinafine (and treat household contacts with selenium sulfide or ketoconazole shampoo) - oral griseofulvin for scalp and nail tinea v.s. trichtillomania - strands of varying length

Answer 59

unrelated to growth features - LE, bilateral, night pain - normal physical exam and activity parental education, reassurance, supportive care

Answer 60

optic chiasm compression pituitary stalk compression - endocrinopathies (GH deficiency, DI) suprasellar calcified mass on imaging (v.s pituitary adenoma or Rathke cleft cysts, which would not have a calcification)

Answer 61

most common cause is thyroid dysgenesis - aplasia, hypoplasia, ectopic gland initially infants appear normal because of presence of maternal hormones - newborn screening with T4 and TSH levels is mandated for all newborns - tx with levothyroxine eventually - large anterior fontanelle, lethargy, feeding difficulties, macroglossia

Answer 62

botulism - DESCENDING - honey or spores (CA, PA, UT) - sluggish pupillary reaction - give botulism Ig - vs tetanus - which is a baby with spasms and hypertonicity (supportive care, abx, tetanus Ig) Werdnig-Hoffman syndrome - AR, degeneration of anterior horn cells and CN motor nuclei - lower > upper GB - ascending flaccid paralysis

Answer 63

rule of 2s: 2% prevalence, 2:1 M:F, 2% symptomatic at age 2, located within 2 FEET of ileocecal valve due to incomplete obliteration of vitelline duct px - painless hematochezia, intussusception, intestinal obstruction, volvulus dx - tch-99m pertechnetate scan surgery for symptomatic pts ddx for hematochezia in early toddlerhood - hemorrhoids, infectious colitis, intussception, Meckels, IBD

Answer 64

symptoms often preceded by mild URI IgA mediated leukoclastic vasculitis - (1) palpable purpura and joint pain, abd pain/intussusception (due to intestinal edema), renal disease similar to IgA nephropathy lab findings - hematuria, RBC casts, proteinuria tx - supportive (hydration, NSAIDs) - hospitalization and systemic glucocorticoids for severe sxs

Answer 65

early death Duchenne - XR, test by gene testing - onset 2-3 yo - progressive weakness, Gower maneuver (walks up legs to stand), calf pseudohypertrophy - comorbidities - scoliosis, cardiomyopathy - wheelchair dependent by adolescence, death by 20-30 from respiratory/heart failure Becker - XR - onset age 5-15 - comorbidities - cardiomyopathy (death at age 40-50 due to heart failure) myotonic - AD, CTG repeat - onset at 12-30 yo - facial weakness, hand grip, myotonia, dysphagia - delayed muscle contraction - comorbidities - arrhythmias, cataracts, balding, testicular atrophy/infertility - death from respiratory or heart failure elevated CPK and aldolase

Answer 66

anti-DS DNA abx anemia due to SLE

Answer 67

nursemaids elbow: radial head subluxation - response to reduction is diagnostic of this condition Panner disease - osteochondrosis of capitellum - occurs in active athlete, pitchers - chronic dull pain, crepitation, loss of pronation and supination

Answer 68

osteosarcoma (most common primary bone tumor) - metaphysis of long bones - sunburst pattern and periosteal elevation (Codmans triangle) Ewing - osteolytic lesions and onion skinning (periosteal reaction) - sarcoma mets to lungs (and LNs) - pre-op chemorads --> surgery osteoid osteoma - cortical lesion, central nidus of lucency, pain relieved by NSAIDs chronic osteomyelitis - central lytic bone defect with surrounding sclerosis (Brodie's abscess) - acute osteomyelitis - S aureus is the most common org Langerhans histiocytosis - single lytic lesion - benign, resolve spontaneously, conservative treatment

Answer 69

mosquito periodic febrile paroxysms - nonspecific malaise, headache, N&V, abd pain, diarrhea, myalgia, pallor, jaundice, petechiae, HSM - parasites cycle from liver to bloodstream - can have worse complications - cerebral malaria (seizures, delirium, coma) dx - blood smears protection - hemoglobinopathies, partial immunity from previous malarial illness preventative drugs and safe quarters

Answer 70

red flags - sparing of flexor surfaces (dunking injury)...

Answer 71

RMSF - febrile prodrome, erthematous macular rash that spreads inward erythema infectiosum - Parvo B19, slapped cheek (can progress to reticular rash on entire body)

Answer 72

risk factors - initiation of solid food and cows milk, toilet training, school entry encopresis can occur - rectum dilates --> increase rectal pressure --> internal anal sphincter relaxes in response - enuresis can also occur complications - anal fissures, hemorrhoids, enuresis/UTIs tx - dietary fiber and water, limit cow's milk intake, laxatives, suppositories and enemas - sit on the toilet after each meal

Answer 73

stridor = upper airway symptom laryngomalacia (4-8 mo) - inspiratory stridor, worse when supine (better when prone) - supraglottic structures that collapse during inspiration laryngomalacia - confirm dx by laryngoscopy (only for moderate/severe cases) - reassurance (supraglottoplasty for severe sxs) - spontaneous resolution of stridor occurs in most cases - majority of kids will have GERD sxs - treat with positioning and PPIs vascular ring - biphasic stridor, improves with neck extension - up to 50% of kids will have a cardiac anomaly - *barium swallow* --> MRI angio

Answer 74

type 1 = immediate - IgE mediated (mast cells) - anaphylaxis, urticaria - allergic sxs affecting more than 1 organ system or sudden hypotension - IM epi - b2 (bronchial smooth muscle relaxation), a1 (vasoconstriction, decreased edema, raised blood pressure) - for bee stings - refer to allergist for venom immunotherapy (can reduce repeat anaphylaxis risk significantly) type 2 = cytotoxic - IgG and IgM - autoimmune hemolytic anemia, goodpasture syndrome type 3 = immune complex - serum sickness, PSGN, lupus nephritis - serum sickness - fever, *hives*, joint pain, occurs 1-2 wks after administration of b-lactams or bactrim type 4 = delayed - T-cell and macrophage-mediated - contact dermatitis - pruritic vesicles - PPD - tx - avoidance of allergen, topical/corticosteroids

Answer 75

recurrent skin and mucosal bacterial infection (omphalitis, periodonitis) - no pus, poor wound healing - ulceration and necrosis delayed umbilical cord separation > 21d marked peripheral leukocytosis with neutrophilia

Answer 76

platelets disorders - present with easy/prolonged *mucosal bleeding*, ecchymoses, petechiae vWF def is the most common inherited bleeding disorder - prolonged bleeding time, peri-op, heavy menses - prolonged aPTT due to decreased factor 8 activity Bernard-Soulier syndrome - abnormal plt function testing TCP - ITP, leukemia - ITP - isolated TCP after viral infection Wiskott-Aldrich syndrome - XR, WAS protein gene - px in infancy - recurrent sinopulmonary infections, eczema, and TCP (easy bleeding) - impaired cytoskeleton changes in leukocytes (so recurrent infections), plts - tx - stem cell transplant

Answer 77

starry sky, aggressive tumor but respond well to high dose chemo

Answer 78

hereditary spherocytosis - AD, northern european descent, ankyrin and spectrin deficiency - hemolytic anemia, jaundice, splenomegaly - increased MCHC - osmotic fragility test - tx - folic acid supplementation, blood transfusions, splenectomy - complications - pigment gallstones, aplastic crises from B19 infection - with splenectomy - life-long risk for S pneumo sepsis G6PD deficiency - Hgb becomes oxidized and precipitates with oxidant stress --> Heinz bodies and bite cells - acute hemolytic anemia

Answer 79

acute otitis media - middle effusion + acute eardrum inflammation - often follows a URI - nasal congestion contributes to Eustachian tube inflammation... - decreased mobility and bulging of tympanic membrane - orgs - H flu, Moraxella, Strep pneumo - give po amox (second line is augmentin) 10d course otitis media with effusion - middle ear effusion WITHOUT acute inflammation chronic suppurative otitis media - hearing loss, tympanic membrane *perf*, otorrhea for > 6wks - no fever - can give otic drops bullous myringitis - serous liquid-filled blisters on tympanic membrane cholesteatoma - congenital or acquired (due to chronic middle ear disease) - think in a pt who has persistent drainage despite abx - appearance - granulation tissue, skin debris (v. s. otosclerosis - would NOT have drainage) - complications include - hearing loss cerum impaction hemotympanum otitis externa - pain with tragal traction, erythematous swollen external auditory canal, otorrhea - Pseudomonas, S aureus - FQ (+/- topical glucocorticoid) ************************************* otitis - mastoiditis - temporal brain abscess (ring enhancing lesion) - get brain CT (can examine mastoid too)

Answer 80

neonatal (<28d) sepsis should be high on the differential - for kids with decreased activity or poor feeding - signs that are present in older kids or adults will not be present temp instability - fever or hypothermia - poor feeding, jaundice, AMS - abnormal WBC count - high or low - left shift - bandemia dx - blood, urine, and CSF culture tx - parenteral abx therapy = amp and gent

Answer 81

in infants and toddlers - dx and tx promptly because they usu involve the kidneys - symptoms are non-specific or vague - fever, fussiness, DECREASED urine output - fever > 39 in any child < age 3 - prompt eval for UTI - for kids who dont wear a diaper - midstream clean cath - young kids - straight cath in older kids/females - E coli - urethra and bladder are normal sterile - UTI occurs when bacteria around vaginal introitus ascend the urethra urine dipstick - pos in 10% of school-aged kids - proteinuria can be transient (stress, fever, exercise, volume depletion), orthostatic, persistent - get 2 additional specimens to confirm refer to peds nephrologist otherwise

Answer 82

Fe tabs will appear radio-opaque (and they are the only tabs that are radio-opaque) Fe poisoning --> free radical production... - GI upset + toxic - abdominal pain, hematemesis (gastritis), hypovolemic shock, metabolic acidosis - in 2 d - hepatic necrosis - in 2-8wks - pyloric stensois (due to gastric scarring) tx - deferoxamine is antidote - plus whole bowel irrigation and supportive care **************************************** acetaminophen - asx for 24hrs ASA - tinnitus, fever, resp alkalosis and metabolic acidosis Pb - chronic, irritability, poor appetite, headaches, abd pain, anemia - tx - calcium EDTA is lead chelator - screening is not recommended in children with no risk factors

Answer 83

benign = provide reassurance - early or mid-systolic, low intensity - **decreases on standing and valsalva (so with decreased preload)** - Stills murmur - low-pitched, musical at LLSB - pulm flow murmur - high-pitched at LUSB pathologic - diaphoresis and fatigue with feeding/exercise..., pos FHx - harsh, holosytolic, diastolic, high intensity - increases with standing and valsalva - ASD = loud, fixed split or single S2 - decreased/absent femoral pulses - work-up - EKG (LVH), echo, cards referral VSD - harsh, holosystolic murmur at LLSB - small VSDs close spont in 75% by age of 2 (but you still need to work this up) Holter monitor - to detect arrhythmias

Answer 84

thickening of tunica media of aortic arch near ductus HTN in upper extremities, poor perfusion to lower extremities - heart failure - irritability, poor feeding/diaphoresis, cardiogenic shock (infants) - SEM at left interscapular area - palpable pulsations of intercostal vessels (adults) infants become dependent on flow through the ductus - begin to have problems when ductus closes at day 3 - side note - PGs are administered to keep ductus open tx - surgery

Answer 85

PW - paternal deletion - hyperphagia, hypogonadism, dysmorphic facies - complications due to obesity - sleep apnea, type 2 DM, gastric distention/rupture, death by choking Angelman - maternal deletion - happy puppet, ID

Answer 86

UTI in kids <2 yo - abx for 1-2 weeks infants and children < 24 mo with first febrile UTI recurrent febrile UTIs UTIs in child of any age with FHx of renal/urologic disease, HTN, or poor growth children who dont respond to abx

Answer 87

wheezing - lower airway disease - trach will NOT help, have to intubate 1) bronchodilators (inhaled, IV), corticosteroids 2) BiPaP respiratory failure in asthma - extreme fatigue, AMS, absent/minimal wheezing (poor air entry), cyanosis, chest wall retractions lab findings - low PaO2 and high pCO2 (cant get air in or out)

Answer 88

DKA - infection/stress - -> polyuria, decreased level of consciousness, diffuse abd pain, metabolic acidosis - catecholamines --> glucagon --> hyperglycemia, ketonemia, osmotic diuresis (accelerates renal K loss) - low total body K

Answer 89

hx - exposure to oxidizing substances (dapsone, nitrites, local/topical anesthetic) methHgb - ferric site has decreased O2 affinity, remaining 3 sites have INCREASED O2 affinity --> wont release O2 to tissues - O2 supplementation wont change pulse ox reading cyanosis, low pulse ox, dark chocolate blood lab findings - saturation gap - >5% difference between pulse ox and ABG - normal PaO2 - co-oximetry - will measure different types of Hgb, will tell of elevated methHgb tx - methylene blue

Answer 90

blue-green discoloration of blood and mucocutaneous surfaces

Answer 91

minimal change disease: edema, fatigue, abdominal pain, proteinuria light microscopy - NORMAL - electron microscopy will show effacement of foot processes - most common peds nephrotic syndrome - so bx is not required for kids under age 10 with this px tx - corticosteroid therapy will result in complete remission ********************************************** Alport disease - defect of type 4 collagen - hematuria and sensorineural deafness - thinned and thickened capillary loops, split GBM

Answer 92

initial insult from Shiga toxin - Ecoli O157:H7 - bloody diarrhea in a few days - vascular damage and microthrombi formation * = triad is hemolytic anemia, TCP, and AKI* - fatigue, pallor, bruising, petechiae, edema lab findings - hemolytic anemia (shistocytes, elevated bili) - TCP - AKI - elevated BUN/Cr tx - supportive - fluid and electrolyte management - blood transfusions - dialysis - if kidney injury becomes severe

Answer 93

both have cherry red macula and regression of motor milestones (+ hypotonia, feeding difficulties) Tay SAX - heXosaminidase deficiency - hyperreflexia Niemann picks his nose - sphingomyelinase deficiency - hSM, protuberant abdomenm - areflexia - no treatment, supportive care, high early mortality

Answer 94

S pyogenes fever, pharyngitis (exudative), *sandpaper-like erythematous rash (worst at skin folds)* - strawberry tongue - tender anterior cervical nodes, dx - rapid strep test and throat culture tx - amox ************************************** Staph scalded skin syndrome - fever, blisters (axillae, groin), epidermal shedding - antistaph, wound care TEN - would expect mucous membrane involvement, > 30% BSA

Answer 95

SSRI and CBT (exposure and response prevention) | - clomipramine is second-line

Answer 96

preseptal cellulitis - eyelid erythema, swelling, chemosis - oral abx - clinda orbital cellulitis (occurs due to contiguous spread) - pain with EOM, proptosis (orbital fat involvement), and or ophthalmoplegia with diplopia - IV abx and surgery retinitis pigmentosa - bilateral tunnel vision --> binocular blindness

Answer 97

effects of anovulatory cycles on endometrium - anovulatory cycles occur due to immature HPA-O axis - persistent endometrial proliferation, necrosis, heavy bleeding when ovulation does occur - pt will come in with heavy menses: - -> stable pts - give high dose OCPs - stabilizes endometrium - -> unstable pt - D&C, transfusion

Answer 98

tx - reassurance, gentle handling, analgesia (but not with ASA), splint/sling

Answer 99

formerly Churg-Strauss chronic rhinosinusitis and nasal polyps, asthma, prominent eosinophilia

Answer 100

normally visible on CXR in kids < 3 yo - should atrophy by puberty sail sign on R side of heart shadow responsible for lymphocyte production and maturation

Answer 101

airborne transmission vaccine - doses at age 1 and 4 post-exposure ppx - for kids > 1 only - hx of immunity - yes --> obs - -> no --> immunocompetent, give varicella vaccine; immunocompromized, give VZIG (give vaccine or Ig, NOT both)

Answer 102

primary amenorrhea: no menses by age 15 pelvic exam/US (evaluate organs) - uterus present --> serum FSH --> increased, get karyotype (or other tests, get 17-hydroxyprogesterone level) - -> decreased, get cranial MRI - uterus absent - karyotype serum, testosterone - -> 46XX, normal female testosterone levels --> abnormal Mullerian development - -> 46 XY, normal male testosterone --> AIS *********************************************************** complete androgen insensitivity syndrome - 46 XY - AMH --> internal male genitalia - breasts (testosterone is converted to estrogen) and cryptorchid testes - no axillary/pubic hair - insensitivity to androgens - keep gonads until puberty finishes (for hormones) - remove after puberty due to small risk of cancer 5a-reductase deficiency - undermasculinized until puberty --> when they experience masculinization Mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) - 46XX - normal ovaries, no uterus transverse vaginal septum - normal uterus and ovaries - abnormal vagina - 46XX imperforate hymen - cyclic abdominal pain Turners

Answer 103

1kg of weight loss = 1 L of fluid loss - but difficult to obtain accurate well weight - mild dehydration - hx, no clinical sxs, oral rehydration - moderate - sxs, IVFs (NS) - severe - hypovolemic shock sxs, limited urine output

Answer 104

acquired - hypocalcemia, hypoK, hypoMg - med-induced- abx, psychotropics, opioids, antiemetics, antiarrhythmics inherited - Jervell and Lange-Nielsen (congenital deafness), Romano-Ward tx - propanolol and pacemaker

Answer 105

6 mo - 2yrs - normal crying followed by breath-holding --> cyanosis and LOC - quick return to baseline minor trauma followed by breath-holding --> pallor, diaphoresis, and LOC - confusion and sleepiness for few minutes after

Answer 106

age < 30 provide reassurance in an adolescent

Answer 107

get lumbosacral US

Answer 108

rapid onset: - edema of *face*, limbs, genitalia - laryngeal edema - can be life-threatening - edema of intestines - NO urticaria C1 inhibitor deficiency - elevated levels of bradykinin --> edema - C1q levels will be normal (in hereditary forms, low in acquired forms) episodes usu occur after infection, dental procedure, or trauma

Answer 109

bat. .. (mammals) - incubation period - wks-mo encephalitic - hydrophobia, aerophobia --> pharyngeal spasm and spastic paralysis - agitation paralytic - ascending flaccid paralysis poor prognosis - almost all pts die in 1-3 mo post-exposure prophylaxis - Ig and vaccine

Answer 110

3mo - 5yrs

Answer 111

metatarsus adductus - flexible positioning (question will say ankle movements appear normal) - usu bilateral - reassurance - will correct spontaneously clubfoot - rigid positioning - serial casting, surgery

Answer 112

viral (adeno), bacterial - 1-2 wks allergic - bilateral, watery, ocular prurits - due to IgE-mediated HSR v.s. corneal abrasion - painful, photophobia

Answer 113

refusal to speak in social situation - > 1mo - this is NOT normal shyness - tx - CBT, graded exposure, family therapy, SSRIs social (pragmatic) communication disorder - difficulties with verbals and non-verbals (eye contact, body language)

Answer 114

testicles should descend by age 6 mo - otherwise surgery

Answer 115

heart sounds louder on one side, no breath sounds on affected side polyhydraminos (due to esophageal compression), concave abdomen, barrel-shaped chest pulm hypoplasia and pulm hypotension 1) emergency intubation 2) NG/OG