pediatrics modern day analysis

8/18/2019 Pediatrics Modern Day Analysis

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2/144

The Newborn


3/144

APGA

R• Plse of !"#$ acrocyanotic$ grimaces

to stimlation$ moving all extremitiesand crying%

• Score& '%

()ts for )lse$ ! for color$ ! forirritability$ ( fortone and ( for res)iration

• *hat does the APGAR tell yo&


4/144

General info abot how the newborn tolerated labor+!min, andthe newborn-s res)onse to resscitation +.min,

• *hat does the APGAR not tell yo*hat to do next +does not gide thera)y,How the baby will trn ot +does N/T )redictnerologic otcome,


5/144

And on )hysical exam yo

0nd1• *hen assessing 2oro on an 3GA

newborn$ the right arm remainsextended

and medially rotated%

Erb-Duchenne 4.546%+7lm)8e is 4954': T!,

Refer if not betterby "56mo fornero)lasty

• *hen )al)ating the clavicles

on a 3GAnewborn$ yo feel cre)itsanddiscontinity on the left%

Clavicular Fracture.

*ill form a calls in!w8% No tx needed%4anse 0gre of ' s)lint%


6/144

Caputsuccedaneum

;Edema%4rossesstre

lines%<

Cephalo-hematoma

;=lctance%>oesn-tcross

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2ongolian S)ots Nevs

Sim)lex+Salmon Patch,

2ilia

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Erythema toxicmStrawberry

Hemangioma

NeonatalAcne


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ww w %d er m is% net @b il d er @4 > #D9@..#)x@ img # #9 % C)g

Nevs Sebaceos

>escribed as ;an area of

alo)ecia with orangecolored nodlar s8in


10/144

>escribed as ;thic8$yellow@whiteoily scale on an inFammatory

base


11/144

Neonatal Screen

•

Two disorders screened for in everystate becase they are disastros if notcaght early +and ha))en to be acontraindication to breast feeding1,

Phenylketonuria%• >e0cient Phe

hydrolxalase%• Sxs 2R$ vomiting$

athetosis$ seires$develo)mental delayover!st few mos

• Signs fair hair$eyes$

s8in$ msty smell%• 3ow Phe diet%


12/144

Galactosemia%• >e0cient G!)5ridyl5

transferase% G!)

accm todamage 8idney$ liver$brain%

• Sxs 2R direct

hy)erbili I

Candice$ Jglc$cataracts$seires%

• Predis)osed to E% colise)sis%

• No lactose )or vida%


13/144

A Yellow Kaby• " days old$ bili L !#$

direct is#%.% Eating I )oo)ingwell%

• 9 days old$ bili L !($

direct is#%.% dry mcosmembranes$not gaining weight%

• ! days old$ bili L !($direct is #%.% Kabyregained birth weight$otherwise healthy%

• ! day old$ bili L !$

direct is#%.% Are yo worried&


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Physiologic Jaundice%Gone by .th

>/3%

3iver conCgation not yetmatre%

Breast eeding Jaundice%Jfeeding dehydration retain meconim I re5

absorb deconCgated bili%

Breast milk Jaundice%Kreast mil8 hasglcronidase and de5

conC bili%

Pathologic Jaundice on !st

>/3$ bili

M!($ d5bili M($ rate of riseM.@day%

– Next besttest&

4oombs

– f

)ositive& – fnegative&

2eans Rh or AK/ incom)atability

2eans twin@twin or mom@fets transfsion$ >2$s)herocytosis$ G6)5>H de0ciency$ etc%

• 9 days old% >ar8 rine$

)ale stool% Kili L !($

dbili is '% 3=Ts also

elevated%


15/144

Biliary atresia% Kiledcts cannot drain bile%

4ases liver failre%Need srgery%

• /ther cases ofdirecthy)erbilirbinemia&

!l"ays r#o sepsis$

Galactosemia$ hy)othyroid$choledochal cyst$ 4=

• Random inheritedcases of indirecthy)erbili& +(,

• Random inheritedcases

of direct hy)erbili+(,

• *hy do we care abot

hy)erbilirbinemia&

• *hat is the treatment&


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Gilbert. Jglcoronyltransferase levelCrigler-%a&&ar. +ty)e!,

total de0ciency

Dubin Johnson. blac8liver%'otor. No blac8 liver%

ndirect bili can cross KKK$de)osit in KG and brainstemnclei and case 8ernicters%

+es) if bili is M(#,Photothera)y ionies the ncoCbili so it can be excreted%>oble volme exchangetransfsion if that doesn-t wor8%

Res)iratory>isorders

Kaby is born w@ res)iratorydistress$ sca)hoid abdomenI this 4OR%

>ia)hrag5

matichernia

• Kiggestconcern& Plmonary hy)o)lasia


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e m edici ne %m edsca )e %co m

• Kesttreatment&

f dx )renatally$ )landelivery atL )lace w@ E42/% 3et

lngsmatre "5 days then dosrg

Kaby is born w@res)iratory distress

w@ excess drooling%

TE5 =istla

• Kest diagnostictest&

Place feeding tbe$ ta8e xray$ see it coiledin thorax

• *hat else do yo

loo8 for&

! wee8 old babybecomes

A4TER associated anomalies5vertebral$ anal atresia$ cardiac$

radial and renal%

cyanotic when feedingbt )in8s ) when crying%

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4hoanal Atresia

• *hat else do yoloo8 for&

4HARGE associated anomalies5coloboma$

heart defects$ retarded growth$ GQ anomalies $ Ear anomalies anddeafness

"( w8 )remie hasdys)nea$RR of '# w@ nasal

Faring%

R>S

Prenataldx&Patho)hy

s&

3@S($ give antenatal betamethasone

Srfactant def$ can-t 8ee) alveoli o)en%

Tx&

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ing Re s o rce s@ R a diolo g y @3 n gP a re n c h y m a%h tm R> S

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"' w8 3GA infant born by4@S to an A(G>2 hasdys)nea@grnting

TTN

Patho)hys&Prognosis&

3ng Fid not seeed ot$ retained

Qsally minimal /(needed% Self5 resolves inhors to days%

! w8 AGA infant wasborn after R/2yielded greenish5brown Fid%

2econimas)irationsyndro

me

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Next bestste)&4om)licatio

ns&

ntbate I sction beforestimlation

Plmonary artery HTN$)nemonitis

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G

disorders

Gastroschisis

• >efect lateral +sallyR, of the midline$ nosac%

will see highmaternal A=P

– Assoc w@ other disorders& Notsally%

bms% bro wn% e d

–

4om)lications&2ay be atretic ornecrotic reremoval% Short gt

syndrome

/m)haloce

le

• >efect in themidline%

4overed by sac%

– Assoc w@ otherdisorders&

• >efect inthemidline% No

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bowel )resent% Assoc w@ Edwards IPata BeckwithWiedemann Syndrome

big baby w@ bigtonge$ Jglc$ ear )its

b m s %bro w n %ed

QmbilicalHernia

– Assoc w@ other disorders& Assoc w@ congenital hy)o5

–

Treatment&

thyroidism% +also big

tonge,im a g e s% s ite !#!% co m @6! 9! ! B c omB)ictre#69%C)g

Re)air not needed nless )ersists )ast age ( or "%

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A vomiting baby• w8 old infant w@ non5

bileos vomitingand)al)able ;olive<

Pyloric (tenosis

– 2etabolic com)lications& Hy)ochloremic$ metabolic

al8alosis –

Tx&mmediate srg referral for myotomy

• (w8 old infant w@

bileos vomiting% The )regnancy wascom)licated by)oly5 hydramnios%

)ntestinal !tresia

/r Annlar Pancreas

–

Assocw@&


23/144

>ownSyndrome +es)dodenal,

3 e a r n ing r a diolo g y %co m

•

! w8 old baby w@bileos vomiting$draws ) his legs$ hasabd distension%

*alrotation and volvulus3add-s bands can 8in8 thedodenm

–

Patho)hys&>oesn-t rotate (9# ccw arond S2A

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24/144

Poo)ing Problems• A " day old newborn

hasstill not )assedmeconim% – >>O& +name (,

•

A . day old former""

*econium ileus5 consider 4= if:=H

gastrograf0n enema is dxI tx

+irschsprung,s5 >RE ex)osion of )oo% bx showing noganglia is gold standard

wee8er develo)sbloody diarrhea –

*hat do yo see onxray&

%ecrotiing Enterocolitis

Pnemocystis intestinalis +air in thewall,

–

Treatment&NP/$ TPN +if nec,$ antibiotics and resection ofnecrotic bowel

– Ris8

factors&


25/144

Prematre gt$introdction offeeds$ formla%

•

A (mo old baby hascolic8y abd )ain andcrrent Celly stool w@ asasage sha)endmass in the RQU%

)ntussusceptionKarim enema is dx and tx


26/144

GQ disorders• Newborn male with no

)al)abletestes%

CryptorchidismAssoc w@ )rne bellysyndrome

– *here are theysally&

nginal canal

– Next besttest&

Qltrasond if not )al)able

– *hen to dosrgery&

f not descended by !yr to avoidsterility@cancer

• Newborn male withrethral o)ening on theventral srface%

+ypospadias

– *hat do yo N/T

do&

4ircmciseV =ores8in is sed inevental re)air%


27/144

• Newborn child withambigos genitalia% /nemonth later has vomiting I

JNa W7 and acidosis%

Congenital !drenal+yperplasia


28/144

– 244ase&

(! Hydroxylase de0ciency% +atosomalrecessive,

– >efinitive

test&

!95/H )rogesterone before and after

A4TH bols –

Tx&Hydrocortisone and Fdrocortisone +W doses intimes of stress,


29/144

nfants of >iabetic2others

• 2others with )re5existing diabetes +es) ty)e !, – 4ontrol glc in the !st trimester I ta8e mg folate@day

– Placental insfficiency@QGR$ 4ongenital heart d$ NT>$4adal

regression syndrome$ Small left colon syndrome

• 2others with gestational diabetes – G!%4om)lications&

– +ypoglycemia%*hy&

Wris8 of birth trama +clavicle$ Erbs,$ 4@SI TTN

2aternal hy)erglycemia

fetalhy)erinslinemia

•

4om)lications&

Neonatal seire +always chec8 glcV,


30/144

•

Treatment&

=eed freently if #% dextrose if (#

–

+ypocalcemia%4om)lications& Neonatal seire +always chec84aV,

– Polycythemia%*hy&

Kig baby needs more /($ hy)oxia WEP/

•

4om)lications&

Renal or s)lenic vein thromboses

– Jaundice%*hy&

2ore RK4s to bread down% Ris8 for8ernicters

– 'D(%*hy&

Winslin interferes w@ cortisol srge )rior to birth

thatnormally stimlates lng matration% 4hec8 3@Sratio M(


31/144

Neonatal =ever *or8 )•

f a baby (' days has a feverM!##% se)sis ntil )rovenotherwise%

• Sxs might inclde irritability, poor

feeding%• *hat tests do yo order&4K4 w@ diff$ 4OR$ blood cltres$ rine cltres +secatheter,$ 3P

• Ris8 factors for neonatal se)sis&Prematrity$ chorioamnionitis$ intra)artm fever$maternal le8ocytosis$ )rolonged r)tre of membranes+M!'hrs,$ GKS: mom%• 2ost common bgs&


32/144

Gro) K Stre)$ E% 4oli$ 3ysteria monocytogenes%

• Em)iric

treatment&

Am) : gent ntil 'hr cx arenegative% 4efotaxime : Am) if

meningitis ss)ected


33/144

T/R4H infections• 2aclo)a)lar rash on

)alms andsoles$ snfFes$ )eriostitis%

Sy)hilis% Tx w@ P4N

• Hydroce)hals$intracranial

calci0cations andchorioretinitis%

Toxo)lasmosis% Tx w@slfadiaine : lecovorin%

• 4ataracts$ deafness and heartdefects +es) P>A$ S>,$

extramedllaryhemato)oeisis%

Rbella% No tx%

• 2icroce)haly$)eriventriclarcalci0cations$ deafness$

thrombo5cyto)enia and

)etechiae%


34/144

• 3imb hy)o)lasia$ctaneos scars$ cataracts$chorioretinits$ cortical

atro)hy%

42% Tx w@ ganciclovir$btwon-t )revent 2R

4ongenital aricella ifmom infected !st or (nd

trimester% f mom isex)osed . days before X

( days after delivery$baby getsG%


35/144

Neonatal conCnctivitis

• >/3 !5"$ redconCnctiva

and tearing%

• >/3 "5.$ bilateral)rlentconCnctivitis can

case corneallceration%

Chemical con&unctivitiscased by silver nitratedro)s% Not commonanymore b@c we seerythromycin%

Gonococcal con&unctivitis txw@ to)ical erythromycin and "rd gen ce)h%

• >/3 95!$

red

h tt)?@@e m e d ici n e %m e d sc a )e %co m @a rticle @!!D(!D #5m e d ia

Chlamydia con&unctivitis tx w@

oral

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conCnctiva w@mcoid

discharge I lidswelling

erythromycin% 4om)lication ischlamydial )nemonia cogh$nasal drainage$ scattered crac8les

: bilat in0ltrates on 4OR


37/144

Genetic >iseases ISyndromes


38/144

A newborn baby has decreased tone$oblie

)al)ebral 0ssres$ a simian crease$ bigtonge$white s)ots on his

iris• *hat can yo tell his

mother abot hisex)ected U&

• 4ommon medicalcom)licatio

ns&

>own-s Syndrome

He will li8ely havemoderate 2R% S)eech$gross and fine motor s8illdelay

–

Heart&


39/144

S>$endocardialcshion

defects –

G&Hirschs)rng-s$ intestinal atresia$ im)erforate ans$annlar )ancreas

–

Endocrine&Hy)othyroidism

–

2s8&Atlanto5axial instability

–

Nero&

–

4ancer&

ncr ris8 of Alheimer-s by "#5".% +APP is on 4hr(!,

!#x increased ris8 of A33

• /m)halocele$ roc8er5bottom feet@ hammer toe$

microce)haly and

clenchedhand$ mlti)le

others%


40/144

• Holo)rosence)haly$ severemental retardation andmicroce)haly$ cleft

li)@)alate$ mlti)le others%• ! year old girl with no

breast develo)ment$ shortstatre and high =SH%

Edward-s syndrome+Trisomy !',

Pata-s syndrome+Trisomy !",

Trner-s

syndrome% O/%24 genoty)eof abortedfetses

– Assoc anomalies& Horseshoe 8idney$ coarctation of aorta$

bics)id aortic valve –

Tx&Estrogen re)lacement for secondary sex char$ and avoidosteo)orosis

• !' year old tall$ lan8y boy

with mild 2R has

gynecomastia

and


41/144

hy)ogonadism% increasedris8 for gonadalmalignancy

7linefelter-ssyndrome

• 4afZ5a5lait s)ots$ seireslarge head%

Atosomal dominant

• 2andiblar hy)o)lasia$glosso)tosis$ cleft soft )alate%*@ =AS or Edwards%

• Kroad$ sare face$ shortstatre$ self5

Nerofibromatosis

Pierre RobinSeence

inCrios behavior% >eletionon 4hr!9

• Hy)otonia$ hy)ogonadism$hy)er)hagia$ s8in )ic8ing$

agression% >eletion on

)aternal4hr!.%

•

Seires$


42/144

strabisms$ sociable w@e)isodic laghter%>eletion on maternal

4hr!.%• El0n5a))earance$ friendly$

increased em)athy andverbal reasoning ability%

>eletion on 4hr9%

Smith2agenis

Prader5*illi

Angelman

*illiams

ww w %) re ) sm le %co m @for m@ th rea d @D"'

beehive%thisishll%co%8@defalt%as)&*4>is)

• QGR$ hy)ertonia$ distinctivefacies$ limb malformation$ self5inCrios behavior$hy)eractive%

4ornelia de 3ange

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• 2icroce)haly$ smooth )hiltrm$thin ))er li)$ A>H>5li8ebehavior% 2ost common caseof mental retardation%

• 2ost common ty)e of 2R inboys$

4GG re)eats on the O5chr w@

=etalAlcoholSyndrome

) syc h net 5 8 %co m

antici)ation% 2acroce)haly$macro5orchidism$ large ears%

• Atosomal dominant$ or assoc

w@ advanced )aternal age%Short )al)ebral 0ssres$ whiteforeloc8 and deafness%

=ragile O Syndrome

*aardenbrgSyndrome

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44/144

mmne >e0ciency• ( y@o 2 w@ mlti)le ear

infxns$ diarrheal e)isodes I)nemonias% No tonsilsseen on exam%

Krton

agammagloblinemia5x5lin8ed5infx start L 65Dmo+why&,

–

3abs&

Absence of K cells on flow cytometry$ low levels of all

gs• !9 y@o = with decreased

levels of gG$ g2$ gE$ andgA bt normal nmbers of K cells%

4ombined variableimmne de0ciency%+acired,

–

4om)lication&ncreased lym)hoid tisse

increased ris8 forlym)homa

• 2ost common K5celldef ect%

Recrrent QRs$diarrhea%


45/144

Selective gA de0ciency

–

4om)lication&Ana)hylaxis reaction if given blood containinggA

•

"w8 old 2 with seire$trncs arterioss$micrognathia%

>iGeorge Syndrome

– Geneticdefect&

2icrodeletion on 4hr((

– *hat ty)es of infxns inchildhood&

4andida$ virses$ P4P)nemonia

• nfant w@ severeinfxns$ no thyms or

tonsils% Severelym)ho)enia%

S4>%See infxns w@ bacterial$ viral and

o))ortnistic bgs%

–

nheritance&

24 is O3R% AR is an A>A de0ciency

–

Tx&

P di t i


46/144

PediatricemergencyV Needbonemarrow

trans)lant by age! or

death%

• " y@o 2 child w@

recrrent swollen$infected lym)h nodesin groin and sta)hares s8inabscesses%

4hronic granlomatos diseaseO3R% P2Ns can ingest bt not 8ill

catalase : bgs%

• How todiagnose&

Nitrotetraolim ble +yellow means theyhave thed,% New test is =low cytometry w@ >HR5!("

• !'mo 2 baby w@severe

ecema$ )etechiae$ and


47/144

recrrent earinfxns%

*isc8ott5Aldrich Syndrome%/ften )resent w@ )rolongedbleedingafter circmcision%

– g ma8e)&

3ow g2$ high gA and gE$ slightly low gG%


48/144

Growth and>evelo)ment


49/144

Growth I Ntrition• Newborns lose !#[ of

birthweight in !st wee8%

*hy&

• Shold regain birth

weight by&

>iresis of extravasclar

Fid

( wee8s

• Shold dobleweight by&

• Shold tri)le weightby&

6 months

! year

• ncreased .#[ oflength by&

•

>oble length by&

• Kreast mil8 is best forbabies%

Tre or false&


50/144

• 4ontraindications tobreast5 feeding%

! year

. years

Tre% >h%

Galactosemia$ P7Q$ H$ HSon the breast$ chemo$ 3i$odide$ alcohol%

• Kreast mil8 vs%=ormla5

Kreast mil8 is whey dominant$ morelactose$ more 34=A$ less =e bt itsbetter absorbed%

b l G h


51/144

Abnormal Growth• ! y@o boy$ always been

below.[ in height% Parents aretall Iwere ;late bloomers


52/144

Constitutional Gro"th DelayKone age Real age%4hild is li8ely to have normal0nal adlt height%

Familial (hort (tatureKone age Real age%

/besityKone age M Real age%

Precocios )berty$ 4AH$Hy)erthyroidism

Pathologic (hort(tature 4onsidercranio)haryngioma+vision )roblems$ chect4T,$ Hy)othyroidism+chec8 T=Ts,$Hy)o)ititarism +chec8g=!,$ Trners +chec88aryoty)e,%


53/144

Primitive ReFexes• *hen head is

extended$ armsand legs both Fex%

• *hen yo )lace yor0nger

in )alm$ Fexes hand%• Rb chee8$ head

trns to that side%

• *hen stimlatedorsm of foot$ ste)s)%

• *hen nec8 is trnedto one

2oro%

=rom birth X@6mo

Gras)%=rom birth X@6mo

Rooting%=rom birth X@6mo

Placing%

=rom birth X@6mo

Tonicnec8%

ww w %n r s e %c m %a c %th@%%%@edcate@lesson!@.+!,%C)g


id it

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54/144

side$ o))osite armFexes andi)silateral arm extends

=rom birth X @6mo

• *hen a fall issimlated$ arms areextended%

Parachte%From 0-1mo 2por vida


• 4NS origin of these

reFexes&

Krainstem and vestiblar nclei

http://www.nurse.cmu.ac.th/http://www.nurse.cmu.ac.th/


55/144

>evelo)mental2ilestones

• Rollover&

0mo% Also$ sits w@ s))ort$ cree)@crawl$ strangeranxiety%

• S8i)s I co)ies atriangle&

03mo% Also draws a )erson w@ '5!#)arts%

• *al8alone&

45mo% Also$ bilds " cbe tower and scribbles w@crayon%

• *al8 )stairs w@alternating feet&

63mo% Also$ stands on ! foot$8nows name$

refers to self as ;


56/144

• *al8s downstairs$ co)iesa circle

and can Cm) with both

feet%

60mo% Also$ 8nows age andsex% Qnderstands ta8ing trns%4onts to "%

• \ of s)eech iscom)rehensible

I (5"word sentences&

97mo% Also$ rns well$ bilds9 cbe tower$ holds s)oon$hel)s ndress%

•

Social smile$ start tocoo&9mo

% Also$ sstains head in )lane ofbody$follows an obCect !'#deg$ some vowelsonds


57/144

Potty Training

• Qrinary continence shold beattained by?

. years

• Primary if continence never achieved$Secondary if

after a 6mo )eriod of dryness%

• 2edical cases tor@o&

QT +do a QA,$ consti)ation+disim)act, or>iabetes +chec8 sgar,

•

Tx ofEnresis&!

st

5 behavioral5 reward system$ )eebefore bed$ bell5 alarm )ad%(nd5 )harmacological5 >>AP orimi)ramine


58/144

• =ecal continence shold beattained by?

years

• 2ost commoncase&

4onsti)ation$ fecal retention%

•

Treatment&

>isim)act$ stool softeners$ high 0ber diet

• Kehavioralmodi0cation&

Post5)randial toilet sitting%

i i


59/144

mmniations• >e atbirth&

He)K +remember to give He)K if mom is

HbsAg :,

• >e at (mo$ mo and6mo&

He)K$ Rota$ >ta)$ HiK$ P4 andP

• Starting a 6mo andthen yearly&

nFena

– 4ontraindications to flvac&

Egg allergy$ also 4 for yellowfever vac

• >e at!(mo&

22R$ varicella$ He)A +live vaccines not for8iddos!(mo,

– 4ontraindications to22R&

Neomycin or stre)tomycin allergy

>t d (nd H A +6 ft th !st ,


60/144

• >e beforeage (&

>ta) and (nd He)A +6mo after the !st one,

3 t P >t 22R d i ll


61/144

• >e before8indergarden&

3ast P$ >ta)$ 22R and varicella

•

>e at age!(&

Tda) booster$ meningococcal vaccine$ andHP +girls,


62/144

Heart >isease


63/144

Kenign 2rmers•

Not all mrmrs are a case foralarm$ M"#[of 8iddos have

them%

• Good characteristics – Stills mrmr5 SYST/34$ @$ soft$vibratory and

msical$ heard best L lower mid5sternm

– enos hm5 best heard in anteriornec8$ disa))ears when Cglar veinis com)ressed%


64/144

• Never normal – Anything >AST/34%

– Anything M@ – Get an echo

• Newborn is cyanoticL

birth$ /( does notim)rove%

:ranspositiono the

Great!rteries

– 2ostcommon in&

nfants of diabetic mothers

– Associatedmrmr&

– mmediate tx&

N/NEV +nless P>Aor S>, www %radswi8i%net@m ain@im ag es@thm b@d@df@

PGE! to 8ee)P>A )atent

T r a n s

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65/144

• (y@o child whogets

:etralogy o Fallot

cyanotic and hy)ernea S> : RA hy)ertro)hy : over

riding aorta$ )lmonary stenosiswhile )laying$ sats down% – Associatedmrmr&

Harsh SE2 :single S(

!%b)%blogs)ot%com @%%%@s"(#@T /=thoraxra

–

Treatment&

/( and 8nee5chest )osition$ srgical

correct

y

i

%

o

C)g

n%

• Ki)olar womangives birth to achild w@

holosystolicmrmr worse onins)iration%

Ebstein !nomaly- Trics)id ins0cciency (@( Tdis)lacement into R%

– Associated

arrhythmia&

%

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66/144

*olff5Par8inson5*hite

• 4yanosis L birthwith holosystolic

mrmr$ de)endson S> or AS> forlife% E7G shows 3H%

• Heart defectassociated with

>iGeorgesyndrome% 4ORshows W)lmblood Fow and bi5ventriclarhy)ertro)hy%

• ! congenital heartlesion%

Harsh holosystolicmrmr

over 33 sternalborder$ lod

P(%


67/144

:ricuspid atresia%Give PGE! ntil srgery

:runcus arteriosis%Eisenmenger

develo)s early%>o srg in !st

few wee8s of life

;entriculoseptaldeect%

– f @ in a (moold&

f no sxs$ contine to monitor% 2ostclose by !5(yr

– Gold standarddx test&

Echo

– *hen is srgeryindicated&

=TT$ 65!(mo w@ )HTN$ M(yrs w@U)@Us M(?!

– s loder better orworse&

Ketter% t means the defect is small%2ost often membranos% 2oreli8ely to s)ontaneosly close%

• 3od S! w@ 0xed ands)lit S(%

/lder child w@ exercise

intolerance%

• 2ost common

defect in >own


68/144

Syndrome baby% =ixedI s)lit S( : SE2 w@diastolic rmble%

!(D

Endocardial CushionDeect

–

Tx&L ris8 for early Eisenmengers% Srgery before )HTNL 65!(mo%

• 4ontinos machine5li8e mrmr w@bonding )lses andwide )lse )ressre%

PD!

–

Associations&

Prematrity$ congenital rbella syndrome

–

Treatment&f not closed by !w8$ give indomethacin orsrgically close


69/144

• 2ost common defectin Trner-s baby%>ecreased femoral

)lses$ ;reverse "sign


70/144

/ther cardiac diseases?• !. year old athlete com)lains of occasional

)al)ations angina and diiness% 3ast wee8he fainted dring the !st

inning of his baseballgame%

+/C*

–

2rmr&SE2$ better w@ W )reload +sat$ handgri), loderw@ valsalva$

standing$ exercise +J)reload,

– Treatment of this

child&

Keta bloc8ers or 44K +no diretics or

dig5 why&, Alcohol ablation or srgicalmyotomy

–

Restrictions&No s)orts or heavy exerciseV

• 9 year old girl )resents with vage chest )ain


71/144

• 9 year old girl )resents with vage chest )ain$)ain in several different Coints over the )ast fewdays$ and a rash% Her ESR is elevated$ and her

E7G shows )rolongation of thePRinterval%

!cute 'heumatic Fever

–

Treatment&

/ral P4N +erythromycin, for !# days$ then)ro)hylactic till (#

–

4om)lications&

2itral stenosis$ +then aortic or trics)idinvolvment,


72/144

Res)iratory>isease

• Signs atbirth&

4ystic =ibrosis2econim iles dilated loo)s$;grond glass


73/144

• n early childhood$ ss)ect it when?failre to thrive +.th [ weight Iheight,$ fol5smelling$ bl8y$Foating stools$ recrrent res)iratoryinfections and nasal )oly)s%

• Genetic >efect Inheritance&

AR$ mtation on 4hr9$4=TR )rotein%

•

>iagnosis&

•

Treatment&

Sweat test M6#mE@3 chloride isdiagnostic

– =or thic8 res)%

secretions&


74/144

>NAse +mcolytic,$albterol@saline nebs

– =or

)nemonia&

2ost often )sedomonas or

colonied w@ b% ce)acia Tx w@ )i)eracillin : tobramycin orceftaidime

– =or)ancreatic

insff&

Eny re)lacement w@ meals :A>E7 s))lement

– =or electrolyte lossthrogh s8in&

Adeate Fidre)lacementwhen exercisingor when hot%

Asthma

• f )thassxs


75/144

twice a wee8 and P=Ts arenormal&

Albterol only

• f )t has sxs x a wee8$ nightcogh (x a month and

P=Ts arenormal&

Albterol : inhaled 4S

• f )t has sxs daily$ night cogh (x awee8 and =E! is

6#5'#[&

Albterol : inhaled 4S : long5acting beta5ag +salmeterol,


76/144

• f )t has sxs daily$ night cogh x awee8 and =E! is

6#[&

Albterol : inhaled 4S : salmeterol :

montel8ast and oral steroids

• Exacerbation tx w@ inhaled

albterol and P/@steroids% Watch peak ow ratesand blood gas% P4/(shold be low% Normaliing P4/(

means im)endingres)iratory failre NTQKATE%

• 4om)lications AllergicKrocho)lmonary As)ergills


77/144

Endocrine

>iabet


78/144

>iabetes

A !( y@o girl )resents with a ( day history ofvomiting% =or the last wee8s$ she noticedweight loss$ )oly)hagia$ )olydi)sia and )olyria%

Na !"#$ 4l D#$ H4/" !.$ glcose "6%

• Next bestste)&

Start inslin dri) : =% 2onitor KG3 andanion ga)% Start 7% Kridge w@ glargineonce tolerating P/%

•

Patho)hys&

T5cell mediated destrction of islet cells$inslinatoAb$ gltamic acid decarboxylase atoAb

• 3ong termtreatment&

*ill need inslin tx%

>i ti it i f =asting glc M!(. +twice,


79/144

• >iagnostic criteria fordiabetes&

=asting glc M!(. +twice,(hr /GTT +9.g, M (##Any glc M (## :

sym)toms


80/144

Renal >isease

A 8iddo is )eeing blood


81/144

A 8iddo is )eeing blood1• Kest !st

test&

Qrinanalysis

• >ysmor)hic RK4s orRK4

casts&

Glomerlar sorce

•>e0nition ofne)hriticsyndrome&

Proteinria +bt (g@(hrs,$hematria$ edema and aotemia

• !5( days after rnny

nose$sore throat I cogh&

• !5( wee8s after sorethroat or s8in infxn&

Kerger-s > +gA ne)hro)athy,% 24

case%

Post5stre) GN5 smo8y@cola rine$best !st

test is AS/ titer%


82/144

Sbe)ithelial gG hm)s

• Hematria :

Hemo)tysis&

Good)astre-s Syndrome% Abs tocollagen

• Hematria :>eafness&

Al)ort Syndrome% O3R mtation incollagen

7idney Stones


83/144

7idney Stones• =lan8 )ain radiating to groin : hematria%• Kest test& 4T%• Ty)es5

– 2ost commonty)e&

4alcim /xalate% Tx w@ H4T

– 7id w@ family hx ofstones&

4ysteine% 4an-t resorb certain AA%

– 4hronic indwellingfoley and al8aline )ee&

2g@Al@P/ strvite%)rotes$ sta)h$)sedomonas$ 8lebsiella

– f le8emia beingtreated w@ chemo&

Qric Acid Tx by al8aliniing the rine :hydration

– f s@) bowel resection forvolvls&

• Treatment

Pre oxylate stone% 4anot

reabsorbed by gt +)oo)ed ot,


84/144

y g ) )

– Stones.mm –

StonesM(cm

*ill )ass s)ontaneosly% ]st hydrate

/)en or endosco)ic srgical removal

– Stones .mm5(cm

Extracor)oral shoc8 wave lithotro)sy

A 8iddo is )eeing


85/144

A 8iddo is )eeing)rotein1

• Kest !sttest&

Re)eat test in ( wee8s$ then antify w@ (hrrine

• >e0nition ofne)hrotic

syndrome&

• 24 in 8iddos&

M"%.g )rotein@(hrs$hy)oalbminemia$ edema$

hy)erli)idemia +fatty@waxy casts,

2inimal change d5 fsion of foot )rocesses Treat with )rednisone for 56w8s%2ost common com)lication is infection5

2a8e sre immnied against)nemococcs

andvaricella%

• f ne)hrotic )atient Ss)ect renal vein thrombosisV (@(


86/144

• f ne)hrotic )atientsddenly develo)sFan8 )ain&

Ss)ect renal vein thrombosisV (@()eeingot AT$ )rotein 4 and S% >o 4T orQ@SstatV

• /ther randomcases&

/rthostatic 24 in school aged8ids% Normal while s)ine$ increasedwhen standing%


87/144

Heme5/nc

An African American +or


88/144

+2editerranean, 8id

w@ sic8le cell diseasecomes in1• Swollen$ )ainfl handsand feet%

>actylitis% (@( necrosis of smallbones

• Excrciating )ain in theextremities$

lcers$ hi) )ain%

Pain crises% schemicdamage (@( sic8ling%

• Point tenderness on femr$

fever$ and malaise%• Things seen on blood smear&

/steomyelitis%

24 bg is salmonella

Wretics$ nl 24 sic8les$ targets$ H] bodies%htt)?@@em edicine%medsca)e%com @article@D

..D'5media

• Acte dro) in H4T with A)lastic crisis% Parvo K!D

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89/144

JJretics&

• Recrrent RQU )ain after

meals%• Res)iratory distress I

emergent tonsilectomy&

Pigment gallstones% >o 4hole%

*aldyer Ring hy)er)lasia%

• Proteinria and increased

creatinine :recrrent QTs&

7idney infarcts de to

sic8ledRK4s

2ore Sic8le 4ell Pearls


90/144

2ore Sic8le 4ell Pearls• 2ost common case of

se)sis&• Presents w@ fever$ cogh$chest

)ain$ chills$ and S/K&

Stre) Pnemo

Acte 4hest syndrome%Plmonaryinfarction% 24 case of death%

–

Tx& /($ abx and exchange transfsion%

• Acte confsion andfocal nerologicde0cits&

Stro8e

–

Tx&Exchange transfsion +N/T tPAV,

– Assessingris8&

Transcranial do))ler +v (##cm@sec,$ 8ee)HbS "#[

•

accination and )ro)hylaxis&

("5valent )nemococcal vaccine L age ( : H% F and N%meningitides%


91/144

meningitides%P4N )ro)hylaxis from age (mo ntil age 6yrs%• f a )atient )resents w@

fatigeand megaloblastic

anemia&

2ost li8ely folate de0ciency%Has higher need (@( Wretics

•

Treatment&Hydroxyrea incr )rodctionof Hb= Tx infx aggressively

and manage )ain%Kone marrow trans)lant cres$ bt has !#[ )osto) mortality

7ids with Anemia


92/144

7ids with Anemia• *hen is anemia not a big deal&

Physiologic dro) in HIH for !st (5"mo% Transient

Erythroblasto)enia occrs later +"mo56yrs, immnes))ression after viral infxn +not K!D,

• !' mo 8iddo$ )ic8y eater$drin8s

lots of cow-s mil8%

JHIH$ 24 9.$ Jferritin$WTK4

• !' mo 8iddo$ eats a varied

diet%2om is talian%

JHIH$ 24 6#$ JR>*

• ' mo 8iddois irritable$has

glossitis I=TT% Pic8yeater$drin8s lots

of goat-smil8%

Fe-de


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Tx w@ oral ferrossalts%

:halessemia% arying degrees% Txw@ transfsion I deferoxamine%4an see ex)anded medllarys)ace

Folate-deA and low retics%

Tri)halangeal thmbs%

Klac8fan5>iamond

Anemia

–

Tx&

4orticosteroids$ transfsions$ stem cell trans)lant%

• !'mo baby )resents w@ low)lts$ low *K4s and )rofondanemia% He has cafZ5a5laits)ots$ microce)haly$ and

absent thmbs%

=anconi Anemia

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–

>x& –

Tx&

Kone marrow shows hy)o)lasia$ 4ytogenetic stdies forchr brea8s

4orticosteroids$ androgens$ bone marrow trans)lant%

– 4om)lications& ncr ris8 for A23 and other cancers%

• ( y@o baby )resents w@hy)eractivity$ im)aired

growth$ abdominal )ain andconsti)ation%

3ead Poisoning

–

>x& –

Tx&

enos blood sam)le$ chec8 lead level

M.5 tx w@ sccimer% M9#5 admit and tx w@ E>TA :dimerca)rol

–

Screening& Test blood lead levels btwn !(5( mo if low

SES$ live in old hose+!D6#,%

www%w ads w orth %or g @% %%@baso)hilicsti))lingB nw%C)g

A 8iddo wal8s in with

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A 8iddo wal8s in withthrombocyto)enia

):P% Tx w@ G for !5( days$then• !. y@o = recrrente)istaxis$ heavy

menses I )etechiae% J)ltsonly%

• !. y@o = recrrent e)istaxis$heavy menses$ )etechiae$normal )lts$ W bleedingtime and PTT%

)rednisone$ then s)lenectomy%N/)ltsVV

;=D% >>AP for bleeding or)re5o)% Re)lace factor +contains v*=, if bleedingcontines%

• 9 y@o 2 recrrent brising$hematria$

I hemarthroses$ W PTT thatcorrected

w@ mixing stdies%

• !w8 old newborn$born at home$comes in withbleeding from thembilical stm) I

bleeding diathesis

• D y@o = with *ilson-s diseased l d f l i t li

+emophilia% f mild$ tx w@


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develo)ed flminant liverdisease%

>>AP$ otherwise$ re)lacefactors%

;it> de % J $ $ O and O%Samein 4= 8id withmalabsorbtion

Tx w@ ==P actely : vit7

shot – !st factorde)leted&

$ so PT increases !st

– ( factors notde)leted&

and v*= b@c they are made byendothelial cells%

A " y@o child is broght in with)etechiae$ abdominal )ain$ vomitingand lethargy% He had bloody diarrhea. days ago after eating hambrgers at

a family )icinic%

3abs revealthrombocyto)enia andWcreatinine

Hemolytic Qremic Syndrome


97/144

ww w %n e Cm %o r g @%%%@(##.@(##.#'#@images@s%C)g

2ost commoncase&

E% 4oli /!.9H9$ Shigella$ Salmonella$4am)ylobacter

Treatment& N/ )lateletsV Tx w@ aggressive ntrition +TPN, andearly )eritoneal

dialysis% >on-t give abx for bloody diarrhea% 4an W ris8 ofHQS

A . y@o child is broght in with )r)raon his legs and bttoc8s$ abdominal)ain$ Coint )ain$ crrent Celly stool% Hissmear a))ears normal$ as are hiscoaglation stdies and electrolytes% gA

and 4" are de)osited in the8i

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s8in%Henoch Schonlein Pr)ra

2ost commoncase&Qsally follows a QR

Treatment&

Sym)tomatic treatment% 4an se steroids for G or renald%

•

New onset seire$ ataxia andHA worse in the A2 withvomiting for a month%

Krain tmor% 2ost li8elyinfratentorial

– 2ost

common5

Pilocytic astrocytoma of cerebellm% Resect%

^D#[ srvive% – (nd most common$ worse)rognosis5

2edllobastoma% ermis$obstrct th

• Adolescent with height in

.th

[$ w@ bitem)oral

hemiano)sia% See

calci0cations in sellat i

4ranio)haryngioma%


99/144

trcica%

• ( year old hy)ertensive

child with asym)tomaticabdominal mass%

4ranio)haryngioma%S)rasellarA remnant of Rath8e-s )och%

*ilm-s tmor

–

Associations

&

Aniridia$ GQ anoms$ Hemihy)ertro)hy$ Kec8with5*eidemann

– Kesttest&

Abdominal 4T% >o 4OR to chec8 lng involvement

–

Treatment&Srgery$ chemo$ rads

• year old with Cer8ingmovements of eyes and legs$blish s8in nodles and antender abdominal mass%

Neroblastoma

– >iagnostict t &

Wrine homovanillic or vanillylmandelic acid%


100/144

tests&

• " year old girl w@ a lim) I left leg

)ain$ T GDD%D$ HS2$ )etechiae$ I )allor%4ells areshown% 4ells are 4A33A and TdT :%

A33

– Kesttest&

Kone marrow bio)sy

M"#[ lym)hoblasts

– Treatment& >P : 4NS tx w@ intrathecalmethotrexate

– Poor )rognostic factors&! or M!#$ M!##7*K4

• ! y@o boy w@ enlarged$ )ainless$rbbery nodes$ drenching fevers$ and!#[ weight loss%

ww w %n lm %n ih% g ov @%%%@ency@fllsie@!((!%C)g

Hodg8in

3ym)homa

– Kesttest&

Excisional bio)sy%

St i 4T l

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test&

– Andthen&

Staging 4T or la)arosco)y%+determines tx,

h o dg 8i n di s e a se %w or d )re s s %co m

– Treatment&

4hemo : Rads%D#[ cre if

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D#[ cre ifstage or

• 9 year old girl with non5)rodctive

cogh and large anterior mediastinalmass on 4OR%

Non5Hodg8in

3ym)homa

– Kesttest&

Kio)sy of mass$ bone marrow bx for staging

–

Treatment&Srgical excision if abdominal tmor%4an se anti54>(# if K5cell tmor%Rads for some%


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nfectios >isease• (y@o w@ a feverto !#.$ " dayslater gets a

)in8$ mac5 )a)rash on trn8arms and legs%

• (y@o w@ a lowgrade fever$

lacyreticlar rash

onchee8sand))er

body

+s)aresthe

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HH6 >isease@Erythema

nfectiosm5ParvovirsK!D

www%m on tnitta ny %org @%%%@8rames@!((#'9%C)g

– *ho is thisbad for&

Preggos$ sic8lecell$ thalessemia

e a sy ) e d ia tric s% co m @w) 5co n te n t@ ) lo a d s@(#!#@#.

• =ine$ mac5)a) desamatingrash begins onchest ands)reads to

nec8$ trn8$ Iextremities:strawberry

Scar

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ver +gro

) A stre),

tonge% Sorethroat !5(w8s)rior%

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–

Treatm

ent

P4N )revents rhematicfever% +won-t hel)

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• 4ogh$ rnnynose$ fever maclar rash

beginsbehin

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s down% Grays)ots on thebccal mcosa%

2easles+)aramyx

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•

Tx&itamin A : s))ortive care

• Sore throat$ Coint)ain fever

)in)oint rashon the face

Rbella+)aramyxovirs,

and s)reads down% Rose s)otson the )alate%•

4om)lications&

4ongenital rbella syndrome

• Kaby with )oorfeeding%

esicles

in the

moth on Hand5

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moth on)alms and soles: rash onbttoc8s%

=oot5and2oth>isease+4oxsac8ie virsA!6,

h tt)?@@www % to ) ne ws% in@ he a lt h@fi le s@H = 2> %C)g

• !6 year old 2

with swollen)arotid glands$fever I HA%

2m)s+)aramyxovirs,

•

4om)lications&

/rchidis and sterility

• 6y@o 8id fromcentral PA$ went

cam)ing% Hadfever%

3yme a

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y>isease%

Korreli

brgorferi

–

4om)lications&

Arthritis$ heart bloc8$ meningitis$Kells

– Treatment& Amox for

this 8id% >oxy if M'%• 6y@o 8id fromcoastal N4$ went

) hil %c d c %g ov @P H3 B m a g e s@D'9@D '9 Blore s%C ) g

cam)ing% Hadfever$ myalgias$abd )ain%

Roc8y 2ontainS)otted fever%Ric8ettsia ric8ettsii

–

4om)lications&

asclitis and gangrene

– >oxy no matter what age

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Treatment&

y g

•

'y@o 8id$ mlti)le excoriations onarms% tchyat night%

ScabiesV

–

Treatm

ent&

.[ )ermetrin forwhole familyV

ww w %li b% io wa% e d @%%%@dermnet@scabiesBbody#!%C)g

• Honey5colored crsted)lae on face%

m)etigo% 24 bgis sta)h if

–

Treatment&

To)ical

mci)rocin iflocalied

blls%

• nFamedconCnctiva and

mlti)leblisters%Ni8ols8y-s :@

Sta)h Scalded S8in =rom exfoliative

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Syndrome toxin

–

Treatment&

Tx w@ ox or nafcillin

2eningit


111/144

gis

• 2ost 4ommonbgs&• n yong I

immnes))ressed&

• n ))l w@ brainsrg&

Stre) Pnemo$ H% nFena$ N%meningitidis+tx w@ 4eftriaxone and anco,

Add 3ysteria% +tx w@ Am)icillin,

Add Sta)h +tx w@ anco,

•

Randoms& TK +RPE : _roids, and 3yme + ceftriaone,

• Kest !st

ste)&

• >iagnostic test&

Start em)irictreatment +: steroidsif thi 8 it

is bacterial,Then$ chec8 4T if signs of increased 4P


112/144

if yo thin8 it Then$ chec8 4T if signs of increased 4P Then$ do an 3P?

• Roommate of the

8id inthe dorms whohasbacterial

meningitisand )etechialrash&

Rifam)inVV

:Gram stain$ M!###*K4 is

diagnostic% High )rotein andlow glcose s))ort bacterial

Ear nfections


113/144

• ( y@o w@ fever to !#($ tgging on his right ear%

Patient-stym)anic membrane is red andblging%

/titis 2edia

– 2ost sensitive dx

test&

3imited mobility on insfFation or air5

Fid level –

R=&JSES$ Native Americans$ formla fed$ tobacco smo8e$arond 8ids

–

Treatment&

Amox or aithromycin for !#days% f no

im)rovement in (5" –

4om)lications&

days$ switch to amox5clav

Effsion5)lace tbes if bilat effsion Mmo or if bilateral

hearing loss%

• !(y@o in smmer swim leage has )ain when


114/144

adCsting his goggle stra)s behind his ear% Thic8 exdates coming from

the ear and tender )osteriorariclar nodes%

/titis Externa

–

Treatment&

To)ical ci)roFoxacin

–

4om)lications&

2alignant external otitis

can invade totem)oral bone facial )aralysis$ vertigo%Need 4T and abx% 2ay need srgery

Sore Throat


115/144

• 9y@o w@ exdative )haryngitis w@ tender cervical

lym)hnodes and fever to!#(%

Sonds li8e GAKHS Pharyngitis

– Kest !st

test& – fnegative&

–

Treatment&

Ra)id stre) antigen

f clinical ss)icion W +ie$ no viral sxs, X docltre

P4N or erythromycin% *hy&

• A child )resents w@ ;mfFed voice for 4IS%GAS :


116/144

anaerobes% "rd

gen ce)h :

am) or clinda – 4om)lications& Retro)haryngeal s)ace commnicates w@

mediastinm

• A child )resents w@ ;hot )otato voice< and)on throat

exam her vla is deviated to the right (@(a blge%

Peri5tonisill

ar

–

Treatment&

As)iration or I> : abx$ tonsillectomy ifrecrrent%

abscess

– ndications fortonsilectomy&

M. e)isodes of stre)@year for (years orM" e)isodes@year for " years%

/lder 8iddo with a sore


117/144

/lder 8iddo with a sorethroat1

• /ther sxs fever$ fatige$ generaliedadeno)athy and

s)lenomegaly +anterior and )osterior

cervical nodes,% Thin8 E)stein5Karr virs

• *hat ha))ens if yo give them am)icillin oramoxicillin&

2aclo)a)lar Rash +immne mediated vasclitic,•

>iagnosis&• Treatment&

Klood smearshows

:Hetero)hile antibody +2onos)ot, test%


118/144

showslym)hocytosisw@ aty)ical

lym)hs

Rest and sym)tomatic thera)y%

•

Precations&

S)lenic hemorrhage or r)tre% +most in(nd wee8, No contact s)orts ntils)lenomegaly resolves%

Res)iratory>istress

•! y@o w@ fever to !##%. I

;bar8ing<cogh and lod noises onins)iration%

4ro)

– 2ost commonbg&

ParainFena virs


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bg&

– O5raybword&

;stee)le sign


120/144

bword&

–

Treatment&

2ist$ e)ine)hrine neb$ steroids

• ( y@o w@ fever to !# Idrooling w@ intercostal

retractions and tri)od)osition%

)load%wi8imedia%org@%%%@4ro)Bstee)leBsign%C)g

E)iglottitis

– 2ost commonbg&

H% =l K only in nimmniedStre) )yo$ stre) )nemo$ sta)h

–

O5raybword&

– Next bestste)&

;thmb)rint sign<Go to /R and intbate

–

TreatmenAnti5sta)h abx : "rd

generationww w %m d co n s lt %co m @da s @b oo8 @b o d y @#@#@!69D@f

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Treatment&

generationce)halos)orin

#@!69D@f5

Pnemonia


122/144

e o a• 7id comes in w@ cogh )rodctive of yellow5

green s)tm$ rnny nose and T !##%'% 3ngexam only reveals somecoarserhonchi%

– Next bestste)&

Acte Kronchitis

S))ortive tx w@ anti5)yretic$ tssives$

histamines%

• 7id comes in w@ similar sxs bt decrease breathsonds

and crac8les in the 333 and*K4 !67%

Pnemonia

– Next bestste)&

4OR to con0rm% Ty)ical vs aty)ical%

– 24 case in neonates('days&

GKS$ E% 4oli$ 3ysteria


123/144

('days&

– 24 case !mo5

"mo&

4% trachomatis$ RS$ )araF$ stre)

)nemo – S)ecific findings for chlamydia)nemonia&

Staccato cogh$eosino)hilia

– 24 case in 8ids mo5.y@o&

RA3V +RS, then s% )nemo

– 24 case in 8idsM.y@o&

2yco)lasma$ s% )nemo

7id with a cogh


124/144

• Dmo infant w@ rnny nose$ wheey cogh$ T

!#!%.$ andRR 6#% Retractions are visible and )lse ox isD![%Bronchiolitis

– 2ost common

bg&

RS% 4on0rm w@ swab

– 4ORfindings&

Hy)erinFation w@ )atchy atelectasis

–

Treatment&Hos)italie if res)iratory distress% Albterol nebs%N/ steroids

– *ho needsvaccine&

Palivimab for )remies$ 4H>$ lng d$immne d

• Dmo infant with severe coghing s)ells with lodins)iratory whoo)s and vomiting afterwards% (

wee8s ago

she had rnny nose and dry cogh%=hooping Cough


125/144

– Res)onsiblebg&

Kordetella )ertssis

– 3abfindings&

4K4 shows lym)hocytosis

–

Treatment&Erythromycin for ! days

– =amily members and 8ids in her

daycare&Erythromycin for !days

QT


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• n neonates5 sxs are vage5 fever$ dehydration$fssy%

– f fever is )resent its )yelo% 4ystitis has N/ fever

•

Kefore age !$ boys are more li8ely than girls to getQT%

• Anatomic ris8 factor forQT&

esicoreteral reFx% Need abx)ro)hylaxis

• >iagnosis ofQT&

4lean catch or cath sam)le$ QA and 4ltre+M!#7 4=Q,

– Needltrasond if?

• Treatment of QT&

Any febrile QT foranatomy$ abscess orh d h i

P/ trim5slfa or nitrofrantoin


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hydrone)hrosis

• Treatment of

)yelo&

! days of ceftriaxone or am) I gent

• =ollow)&

Test of cre to con0rm sterility

• *ho needs4QG&

All males$ females .$ any )yelo$ femalesM. w@ (nd QT

• Role of Tc5labeled >2SAscan&

t is most sensitive andaccrate stdy of scarring and

renal sie$ bt is not 0rst line%


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Kone and ]ointsses

7id with a lim)2 t


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• 2ost common caseoverall&

Trama

• !'mo = w@ asymmetric glteal foldson exam%

Developmentalhip

–

R=&

!st born =$ :=H$ breech)osition

dysplasia

–

>x and Tx&

cln8 on Karlow% Q@S of hi) if nsre% Tx w@Pavil8harness$ srg

• . y@o 2 initially w@ )ainless lim) now has )ain inhisthigh%

egg-Calve,-Perthes Disease% +avasclarnecrosis,%

• . y@o 2 initially w@ a cold !w8 ago now)resents w@ a lim) I effsion in the hi)%O5rays are normal and ESR is ". +W,$ T DD%'$ *K4s !#7%

:ransient(ynovitis

– Next bestste)&

Ked rest for ! w8 : NSA>S

! y@o lan8y 2 w@ nagging 8nee (CFE Remember


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• ! y@o lan8y 2 w@ nagging 8nee)ain and

decreased R/2 of the hi) onexam%

(CFE% Rememberthey-renot always fatV

–

TxSrgically close and )in the e)i)hysis to avoidosteonecrosis%

•

! y@o bas8etball )layer has 8nee )ain andswelling

of the tibialtbercle

/sgood-(chlatter% /verse inCry from Cm)ing

•

!( y@o = w@ ( w8 history of daily feversto !#( and a salmon colored evanescentrash on her trn8$ thighs and sholders%Her left 8nee and right 8nee are swollen%

J'!

– Good Prognosticfactor&

:ANA


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– Kad Prognosticfactor&

:R=$ also )olyarticlar and older ageLons e t

–

Treatment&!st line NSA>s$ (nd line methotrexate$ "rd steroids

• (y@o = w@ a ( w8 history of dailyfevers to !#( and a desamating

rash on the )erinem% She hasswollen hands and feet$ conCnctivitisand nilateral swollen cervical lym)hnode%

>a"asak

i

– /ther lab

0ndings&

W)lts +w8(5",$ W rine *K4$ W3=Ts$ W4S=

)rotein

– Kest !st

test& –

Treatment&

(> echo andE7G% Re)eat

the Echo

Acte G : high dose as)irin% Thenas)irin : warfarin

mailto:@onsetmailto:@onset


132/144

e c oafter (5"w8s

of tx

– 2ost seriosseellae&

4oronary artery anerysm or 2

Kone Pain de to 4ancer


133/144

• f !#$ more li8ely%

2M=%2ore common ifhx of

Ewing Sarcoma

retinoblastoma or)revios

radiation% ;/nions8inning<on xray% +layers of )eriostealdevelo)ment,%

h tt)?@@www %w i8i d oc %o r g @in de x%) h )@Ew ing`s B Sa rcom a

–

Treatment&Rads and@or srgery

• f M!#$ more li8ely%2M=% See ;snbrst<

and ;4odman-striangle< on xray%

/steogenicsarcoma

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sarcoma

–

Treatment&4hemo and@or srgery

• 2ore diffse bone


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• 2ore diffse bone)ain in a )atient w@

)etechiae$ )allor andincreased infections>on-t forget bone )ain can be )resenting sxfor le8emia

h tt)?@@e m e d ici n e %m e d sc a )e %co m @a rticle @"D"D(95m e d ia

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Nerology

Hydroce)hals


137/144

• Anytime yo see a

meningocele ormyelomeningocele1

>o a head 4T loo8ing forhydro5 ce)hals% +Arnold

4hiari ,

• Anytime yo see an infant with

a head circmference MD.th

[%%%

4onsider hydroce)hals%Also blging fontanelle$

W>TRs$ HA$ vomiting%

•

Noncommnicatin

g5

Stenosis of 4A$ tmor@malformation nearth ventr

•

4ommnicating5

SAH$ )nemoncoccal@TK meningitis$ le8emia


138/144

Seir


139/144

es• This morning$ a ! y@o develo)s a fever to

!#(%% =or hors later$ the )arents bring herin after she has a "5 mintetonic5clonicseire%

=ebrile Seire

– Next bestste)&

Give acetameno)hen% N/ Wris8 for e)ile)sy

• An ' year old boy gets in troble in school

becase he is always ;staring into s)ace


140/144

– Kest Tx& Ethosxamide or val)roic acid

•

A 6mo old is broght in for mlti)le symmetriccontraction e)isodes of nec8$ trn8 andextremities that occr in s)ells% –

>x&nfantile S)asms

– 4ommon EEGfinding&

Hy)sarrhythmia asynchronos$chaotic$ bilat

– Kest Tx&

A4TH% Prednisone is (nd line%

Nerodegenerative>isorders


141/144

>isorders•

'y@o w@ dif0clty w@ balance whilewal8ing$ no >TRs$ bilateral Kabins8i and;ex)losive$ dysarthric s)eeche0ciency of arylslfatase A

accm cerebrosideslfate

• !(y@o w@ decreased school)erformance$ behavior changes$ataxia$ s)asticity$

hy)er)igmentation$ W7$JNa$ acidosis%

Adrenole8odystro)hyO3R

–

Prognosis&>eath w@in !# years


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Prognosis&

• Dmo who had )reviosly beenreaching milestones starts to lag%Seires$ hy)otonia$ cherry redmacla%

Tay5Sachs

O3R

–

Patho)hys&>ef of hexosaminidase A accm G2(

Neromsclar >isordersh


143/144

• "mo infant lays in the ;frog5leg< )osition$ .th[

(@( feeding dif0clties$ hy)otonic$ fasiclationsof the tonge and absent >TRs% –

>x&S2A !5 *erdnig Hoffman >isease

–

Prognosis&

2ost die before age (

• 6y@o is broght in (@( ;clmsiness< andfreent falls% The lower leg has decreasedmscle bl8 and a))ears ;stor85 li8ex&2arie54harcot5Tooth >isease

–

Tests&>ecreased motor@sensory nerve vel$ sral nerve bx%4P7 is normal

Stablie an8les w@ srgical fsion Qsally normal


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–

Treatment&Stablie an8les w@ srgical fsion% Qsally normallifes)an and most remain amblatory%

pediatrics modern day analysis

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