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8/18/2019 Pediatrics Modern Day Analysis
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The Newborn
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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&
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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,
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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%
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Caputsuccedaneum
;Edema%4rossesstre
lines%<
Cephalo-hematoma
;=lctance%>oesn-tcross
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Sim)lex+Salmon Patch,
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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
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>escribed as ;thic8$yellow@whiteoily scale on an inFammatory
base
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Neonatal Screen
•
Two disorders screened for in everystate becase they are disastros if notcaght early +and ha))en to be acontraindication to breast feeding1,
Phenylketonuria%• >e0cient Phe
hydrolxalase%• Sxs 2R$ vomiting$
athetosis$ seires$develo)mental delayover!st few mos
• Signs fair hair$eyes$
s8in$ msty smell%• 3ow Phe diet%
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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%
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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%
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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
h tt)?@@www % e m e d ici n e %co m @r a dio @to ) ic 9!#% h tm
Next bestste)&4om)licatio
ns&
ntbate I sction beforestimlation
Plmonary artery HTN$)nemonitis
h tt)?@@www % a d h b %g ovt %n @n e wb or n@ T e a c h ing Res o r c e s@Ra diolo g y @3 n gP a re n c h y m a%h tm R> S%
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G
disorders
Gastroschisis
• >efect lateral +sallyR, of the midline$ 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@&
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>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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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&
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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
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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%
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• Newborn child withambigos genitalia% /nemonth later has vomiting I
JNa W7 and acidosis%
Congenital !drenal+yperplasia
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– 244ase&
(! Hydroxylase de0ciency% +atosomalrecessive,
– >efinitive
test&
!95/H )rogesterone before and after
A4TH bols –
Tx&Hydrocortisone and Fdrocortisone +W doses intimes of stress,
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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,
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•
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(
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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&
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Gro) K Stre)$ E% 4oli$ 3ysteria monocytogenes%
• Em)iric
treatment&
Am) : gent ntil 'hr cx arenegative% 4efotaxime : Am) if
meningitis ss)ected
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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%
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• 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%
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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
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Genetic >iseases ISyndromes
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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&
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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%
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• 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
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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$
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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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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$diarrhea%
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Selective gA de0ciency
–
4om)lication&Ana)hylaxis reaction if given blood containinggA
•
"w8 old 2 with seire$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
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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
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recrrent earinfxns%
*isc8ott5Aldrich Syndrome%/ften )resent w@ )rolongedbleedingafter circmcision%
– g ma8e)&
3ow g2$ high gA and gE$ slightly low gG%
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Growth and>evelo)ment
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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&
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• 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
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Abnormal Growth• ! y@o boy$ always been
below.[ in height% Parents aretall Iwere ;late bloomers
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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,%
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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
ww w %n r s e %c m %a c %th@%%%@edcate@lesson!@.+!,%C)g
id it
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side$ o))osite armFexes andi)silateral arm extends
=rom birth X @6mo
• *hen a fall issimlated$ arms areextended%
Parachte%From 0-1mo 2por vida
ww w %n r s e %c m %a c %th@%%%@edcate@lesson!@.+!,%C)g
• 4NS origin of these
reFexes&
Krainstem and vestiblar nclei
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>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 ;
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• *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
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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
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• =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
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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 ,
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• >e beforeage (&
>ta) and (nd He)A +6mo after the !st one,
3 t P >t 22R d i ll
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• >e before8indergarden&
3ast P$ >ta)$ 22R and varicella
•
>e at age!(&
Tda) booster$ meningococcal vaccine$ andHP +girls,
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Heart >isease
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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%
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• 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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• (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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*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(%
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: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
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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
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• 2ost common defectin Trner-s baby%>ecreased femoral
)lses$ ;reverse "sign
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/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
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• 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,
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Res)iratory>isease
• Signs atbirth&
4ystic =ibrosis2econim iles dilated loo)s$;grond glass
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• n early childhood$ 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$4=TR )rotein%
•
>iagnosis&
•
Treatment&
Sweat test M6#mE@3 chloride isdiagnostic
– =or thic8 res)%
secretions&
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>NAse +mcolytic,$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
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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,
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• 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
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Endocrine
>iabet
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>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,
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• >iagnostic criteria fordiabetes&
=asting glc M!(. +twice,(hr /GTT +9.g, M (##Any glc M (## :
sym)toms
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Renal >isease
A 8iddo is )eeing blood
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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%
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Sbe)ithelial gG hm)s
• Hematria :
Hemo)tysis&
Good)astre-s Syndrome% Abs tocollagen
• Hematria :>eafness&
Al)ort Syndrome% O3R mtation incollagen
7idney Stones
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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,
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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
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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 (@(
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• 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%
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Heme5/nc
An African American +or
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+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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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
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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%
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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
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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
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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%
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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$blish s8in nodles and antender abdominal mass%
Neroblastoma
– >iagnostict t &
Wrine homovanillic or vanillylmandelic acid%
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tests&
• " year old girl w@ a lim) I left leg
)ain$ 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
)alms@soles,
Roseola5 .th
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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
lett =
e )
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ver +gro
) A stre),
tonge% Sorethroat !5(w8s)rior%
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ealth>e)artment@4>BE)id@images@ScarletB=everBTonge%C)g
–
Treatm
ent
P4N )revents rhematicfever% +won-t hel)
redce changes ofAPSGN,
ww w %o hio h e a lt h% co m@%%%@r9Bscarletfever%C)g
• 4ogh$ rnnynose$ fever maclar rash
beginsbehin
d earsIs)read
s down% Gray
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s down% Grays)ots on thebccal mcosa%
2easles+)aramyx
ovirs,htt)?@@www% ohiohealth%com @bodym ayo%cfm
•
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
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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
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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
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• ( 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
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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
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• 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 :
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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
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/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%
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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
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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
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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
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('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
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• 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$immne d
• Dmo infant with severe coghing s)ells with lodins)iratory whoo)s and vomiting afterwards% (
wee8s ago
she had rnny nose and dry cogh%=hooping Cough
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– Res)onsiblebg&
Kordetella )ertssis
– 3abfindings&
4K4 shows lym)hocytosis
–
Treatment&Erythromycin for ! days
– =amily members and 8ids in her
daycare&Erythromycin for !days
QT
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• n neonates5 sxs are vage5 fever$ 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
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e c oafter (5"w8s
of tx
– 2ost seriosseellae&
4oronary artery anerysm or 2
Kone Pain de to 4ancer
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• 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
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• 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
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Seir
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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
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– 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
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>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
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• "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%