Lectures in Pediatrics.doc

8/9/2019 Lectures in Pediatrics.doc

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Protein Energy Malnutrition (PEM)Defnition:

The


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%. Early detection o PEM: The =r$t indicator o PEM i$ underweight or age. The o$t practical ethod todetect thi$ i$ to aintain growth chart$. The eHtent o weight lo$$ and growth ratevarie$ with $everity o PEM.

1. Early $tage$ o PEM : ,ailure to aintain weight or growth rate2. Progre$$ive PEM : o$$ o weight? lo$$ o $u&cutaneou$ at and u$cle

a$$ and dy$unction o any vital organ$%. Severe PEM: increa$ing ailure o hoeo$tatic echani$$ o &ody and

daage to iune deen$e$ ay lead to inection? $hocC and death. Types o PEM:

1. Mara$u$2. Fwa$hiorCor

%. Mara$ic Fwa$hiorCor

linical eatures o PEM

!eatures

linical

Maras"us #$as%ior&or'l$ays Present

Mu$cle wa$ting &viou$ Soetie$ hidden &y edea and at

,at wa$ting Severe lo$$ o $u&cutaneou$at

,at oten retained &ut not =r

Edea None Pre$ent in lower leg and u$ually in aceand lower ar$


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Seru al&uin Noral or $lightly

decrea$ed

ow (le$$ than %g61""

l &lood)0rinary urea per gcreatinine

Noral or decrea$ed ow

;ydroHyproline 6 creatinineratio

ow ow

Pla$a 6 aino acid ratio Noral Elevated3neia 0ncoon 7ooniver &iop$y Noral or atrophic ,atty change$

lassifcation o PEM:

I'P classifcation:

Nutritional

*rade

Percentage o standard $eig%t

or ageNoral K "Grade 1 /1 L "Grade 2 +1 L /"Grade % 1 L +"Grade ' e$$ than "

+aterlo$,s classifcation:

Para"eter Nor"al +asted tunted


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Thi$ i$ a valua&le indeH o a$$e$$ent o nutritional $tatu$. 26% o children with PEMdoe$ not pre$ent with clinical $ign$ &ut are diagno$ed &y anthropoetry.

/. +eig%t or age: o$t $en$itive ethod when recorded $erially. 3 decrea$e in weight gain 6lo$$ in weight can &e $een within 1 onth.

0. 1eig%t or age:

copare$ the child$ height with the eHpected height or the age.2. +eig%t or %eig%t:

thi$ copare$ a child$ weight with the eHpected weight o the $aeheight. 8t i$ u$eul or diIerentiating &etween acute and chronicalnutrition.

3. 'cute "alnutrition:

child i$ wa$ted i.e. weight or age and height i$ low &ut height or age i$noral.

4. %ronic "alnutrition:

child i$ $tunted i.e. weight or age i$ low and height or age i$ low.

5. Mid upper ar" circu"erence (M6'): Noral M037 or a child &etween 1# year$ o age i$ greater than 1%. c.8 the M037 i$ 12.#1%.? the child ha$ ild to oderate alnutrition and iit i$ le$$ than 12. c it i$ $ugge$tive o $evere alnutrition. Thi$ i$ u$eulor $creening a large nu&er o children &ut le$$ u$eul in long ter growthonitoring.

7. %est 1ead circu"erence

7he$t circuerence ;ead circuerence at one year o age. 7he$t circuerence K ;ead circuerence ater 2 year$ o age.

8n PEM? che$t circuerence i$ le$$ than ;ead circuerence even ater 2year$ o age.

8. &in old t%ic&ness: 8t i$ an indication o the availa&ility o caloric $tore$ in the or o$u&cutaneou$ at. Site$ or ea$ureent are tricep$ and $u&$capularregion.

9. T%e "ost i"portant "easure"ent or "alnutrition is t%e gro$t%

cure: weight or age i$ plotted a$ percentile$ curve$ and growth i$ onitoredover a period o tie: it i$ al$o called road to health chart


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Maras"us:

1. 8t re$ult$ ro inadeBuate intaCe o energy or &oth energy and protein$2. 0$ual age i$ le$$ than 1 year3. Marasmus is typically observed in infants who are breastfeeding when the amount of milk is markedly

reduced or, more frequently, in those who are artificially fed4. It is a form adaptation syndrome to chronic underlnutrition

. ;ence it i$ a non oedeatou$ or+. There i$ $evere wa$ting $Cin &ecoing wrinCled and loo$e/. 5uccal pad o at i$ lo$t giving a wi$e an>$ appearance. 8nant have con$tipation or $tarvation diarrhoea*. ,lat or di$tended a&doen with vi$i&le peri$tal$i$1".Su&noral teperature and low pul$e

Fwa$hiroCor!1. ethargy? apathy? irrita&ility are the initial eature$2. Mu$cle wa$ting and edea%. ;epatoegaly'. SCin!

a. Depigentataion&. Dry and cracCling! JaCy paint and paddy =eld deratiti$. ;air $ign$!

a. Ea$y plucCa&ility&. 5rowni$hc. ,lag $ign

+. Theorie$ o 7au$e$ o edea!a. educed al&uin $ynthe$i$&. 3JatoHin poi$oningc. 8paired renal unctiond. Decreaed Na# F 3TPa$e acivitye. 8ncrea$ed ree radical$: ethionine level$ are low which i$ a precur$or

o glutathione an antioHidanto"plications o PEM:

1. Dehydration and diarrhea2. ;ypotheria%. ;ypoglyceia'. 8nection$. 7onge$tive cardiac ailure+. -itain de=ciency/. Multiple nutritional de=ciencie$. Electrolyte i&alance

Manage"ent:

Goals:1. To inii9e weight lo$$2. To aintain &ody a$$%. To encourage &ody a$$ growth

Principles:

1. Patient evaluation: Severity o PEM ± $y$teic inection$? a$$ociatednutritional de=cit$

2. 8ntaCe o ood: 7ulturally accepta&le and aIorda&le ood u$t &e prooted.%. Prevention o coplication$ and death: 5y careul $urveillance 4 propt

action.


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'. 3ttept to eliinate epideiological actor$Manage"ent o "ild to "oderate PEM:

Thi$ i$ anaged at hoe &y parent$ and onitored through weeCly hoevi$it$ &y paraedical per$onnel? vi$it$ to the ho$pital or nutritionalreha&ilitation centre every weeC. The goal i$ to provide adeBuate calorie$ toreplace lo$$e$ 4 to proote growth.

Caution: The calories and proteins should be gradually built up.;eco""endations:

O Energy 12"#1" Ccal6Cg6dayO Protein 2#% g6Cg6day

!oods adocated:

O Dou&le or triple iHe$ o cereal$ and pul$e$ liCe dal? rice? Chichdi?$ea$onal green leay vegeta&le$? root vegeta&le$? $ugar? @aggery? ilC? ilCproduct$ and nut$ liCe groundnut$.

O il 6 ghee or &utter are added to increa$e calorie$.O ,reBuent $all eed$ with calorie$ and protein$ di$tri&uted

proportionately are encouraged.Miscellaneous:

O Parent$ $hould &e educated regarding the hygienic way o preparing andhandling ood: per$onal hygiene and iportance o $ae and cleandrinCing water.

O 5a$ic advice regarding S (oral rehydration $olution) u$e or diarrhoea?vitain $uppleent$? treatent o inection$ and ine$tation$ $hould &egiven

O inc $uppleent$ are given to proote growth once po$itive nutrition&alance $tart$ (increa$e in weight gain)

O 7hild u$t &e iuni9ed a$ per $chedule.eere PEM:

1ospital Manage"ent:

e$u$citation ! + # 2' hour$ 3cute Pha$e ! 1 day # 1 weeCeha&ilitation ! 2nd # %rd weeC$

;esuscitation - Treat"ent o o"plications:

De%ydration! 3$$e$$ent! ,luid and $odiu given $hould not eHceed / o allowance$calculated on the &a$i$ o weight.

1ypot%er"ia:

8t i$ coon in Mara$u$. 8t i$ u$ually a $ign o inection? hypoglyceia or$evere energy de=cit. The child i$ Cept war? given reBuent eed$ andanti&iotic$ or inection.

1ypoglyce"ia:

O 8t i$ de=ned a$ a &lood $ugar o le$$ than " g6dl.O More coon in Mara$u$O 1#2 l6Cg o 1" deHtro$e a$ &olu$: then aintenance with 1"

deHtro$e and $aline over 2' hour$.Inection:


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2. eturn o appetite.%. Gain in weight o "#/" g$6day.'. Di$appearance o edea (/#1" day$) and hepatoegaly.. i$e in $eru al&uin over the =r$t 2 weeC$ o therapy

ecovery i$ coplete when the child reache$ hi$6her $tandard weight? whichu$ually taCe$ +# weeC$.

Prognosis:Mortality 1"#%"ong ter $eBuelae! 8rrever$i&le $tunting and ental ipairent.

Preentie Measures:

The ollowing i$ adapted ro the th ,36


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*ro$t% and Deelop"ent1. Percentile # 3 percentage $cale ro " to 1"" that i$ a$$ociated with an ite to

$how which percentage o the di$tri&ution i$ a&ove and &elow the ite.2. Standard deviation!

The darC &lue 9one repre$ent$ o&$ervation$ within one $tandard deviationto either $ide o the ean? which account$ or a&out +.2 o thepopulation. Two $tandard deviation$ ro the ean (ediu and darC

&lue) account or a&out *.'? and three $tandard deviation$ (light?ediu? and darC &lue) a&out **./

%. +%at is c%ild deelop"ent> Child development reer$ to how a child &ecoe$ a&le to do ore copleHthing$ a$ they get older. Developent i$ diIerent than growth. Growth onlyreer$ to the child getting &igger in $i9e.

1. *ross "otor: u$ing large group$ o u$cle$ to $it? $tand? walC? run?etc.? Ceeping &alance? and changing po$ition$.

2. !ine "otor: u$ing hand$ to &e a&le to eat? draw? dre$$? play? write?and do any other thing$.

http://en.wikipedia.org/wiki/File:Standard_deviation_diagram.svg


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%. Language: $peaCing? u$ing &ody language and ge$ture$?counicating? and under$tanding what other$ $ay.

'. ognitie: ThinCing $Cill$! including learning? under$tanding? pro&le#$olving? rea$oning? and ree&ering.

. ocial: 8nteracting with other$? having relation$hip$ with aily?riend$? and teacher$? cooperating? and re$ponding to the eeling$ o

other$.'. Growth i$ increa$e in $i9e o the &ody (phy$ical aturation). Developent i$ phy$iological and p$ychological aturation+. +%at are deelop"ental "ilestones>

Developmental milestones are a $et o unctional $Cill$ or age#$peci=c ta$C$that o$t children can do at a certain age range.

*ro$t% and deelop"ent - ? to 0 yearsIntroduction:

1. There are de=nite and predicta&le pattern o growth and developent thatare continuou$? orderly and progre$$ive.

Q 7rawl 7reep $ ace:

preerence or ace$. ;earing! developed well: preerential turn toward eale voice


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+. Ga9e &acC at other/. 3utonoic in$ta&ility! Ju$hing? ottling? perioral pallor? hiccupping?

voiting? uncontrolled li& oveent$ and incon$ola&le crying. 5ehavioural $tate!

i. uiet $leep! not arou$a&le &y heel $ticCii. 3ctive $leep! arou$a&le &ut get$ ha&ituated &y repeated heel $ticCiii. Drow$y! repeated heel $ticC pu$he$ the inant to u$$y or crying

$tateiv. 3lert! =Hate on o&@ect or ace and ollow hori9ontally and vertically:

turn toward a novel $oundv. ,u$$y

vi. 7rying'. "#2 onth$!

1. Phy$ical!i.


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ii. Say$ inga # % oiii. ough$ aloud # ' oiv. EHcited at $ight o ood# ' ov. Show$ acial iitation and hand gae$

vi. Show $adne$$ when parent$ are unavaila&le+. +#12 onth$!

1. Phy$ical!i. 3t 1 year the &irth weight triple$: length increa$e &y ": ;7

increa$e$ &y 1" cii. Sit$ $upported +#/ o: without $upport 1" o: pivot$ while $itting *#

1" o: eHaine$ $everal o&@ect$ at a tieiii. 7rawl and pulling to $tand oiv. Thu& =nger gra$p #* o: pincer gra$p &y 12 o:v.


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'. ingui$tic!i. eceptive language precede$ eHpre$$ive languageii. Ploy$yla&ic @argoniii. e$pond$ appropriately &y word$ liCe no? &ye#&ye? give#e etc #12

oiv. Nae$ &ody part$ and u$e$ '#+ word$ correctly# 1 o

. Second year! 1#2' o!1. Phy$ical!

i. Gain$ R and l&ii. 3t 2' o they their adult heightiii. *" head circuerence achieved in 2 yr

2. 7ognitive!i. 0$e$ a $ticC to get a toy out o reach L 1 oii. 7an wind a echanical toyiii. Sy&olic play! ,eed$ a toy ro epty plate

%. Eotional!i. Show$ $eparation anHiety when parent$ are i$$ingii. 0$e$ a toy or &lanCet (tran$itional o&@ect) while $leeping to repre$ent

a&$ent parentiii. ooC$ at the irror and reove an unu$ual thing ro hi$ no$eiv. ;and over a &roCen toy to parent$ to =Hv. Say$ hi$el Uno? noR when touche$ a or&idden o&@ect

'. ingui$tic!i. 1#2" word$ at 1 o and "#1"" word$ at 2 year$ii. 7o&ine two word$iii. 0nder$tand$ 2 L$tep coand$ liCe give the &all and taCe the $hoe$

o"e Paediatric


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/. Stre$$! Maternal deprivation? parental $eparation? parental neglect? childa&u$e? and in$uWcient aount$ o parent6child interaction$ have &eena$$ociated with pica.

. Pregnancyi$C!

1. ead poi$oning

2. 8ron de=ciency aneia%. Para$itic ine$tation'. Such a$ hyperCaleia ollowing cautopyreiophagia (inge$tion o &urnt

atch head$).. geophagia ($oil or clay inge$tion) ha$ &een a$$ociated with $oil#&orne

para$itic inection$? $uch a$ toHopla$o$i$ and toHocaria$i$+. ToHocaria$i$ (vi$ceral larva igran$? ocular larva igran$) and

a$caria$i$ are the o$t coon $oil#&orne para$itic inection$a$$ociated with pica.

/. Sypto$ o toHocaria$i$ are diver$e and appear to &e related to thenu&er o larvae inge$ted and the organ$ to which the larvae igrate.

. Phy$ical =nding$ a$$ociated with vi$ceral larva igran$ ay includeever? hepatoegaly? alai$e? coughing? yocarditi$? and encephaliti$.

*. cular larva igran$ can re$ult in retinal le$ion$ and lo$$ o vi$ion.1".di$place noral dietary intaCe or interere with the a&$orption o

nece$$ary nutritional $u&$tance$.11.Dental eIect$! Pica ha$ &een reported to re$ult in $evere tooth a&ra$ion?

a&raction? and $urace tooth lo$$ Te$t$!

1. $creening o &lood!• lead concentration$• inte$tinal para$ite$• 3neia

2. 3&doinal radiography%. 0pper and lower G8 &ariu eHaination$'. epeated iaging at regular interval$ to tracC change$ in location o

inge$ted aterial$. 0pper G8 endo$copy to diagno$i$ &e9oar oration? identiy a$$ociated

le$ion$? or &oth Treatent!

1. 3ddre$$ any identi=ed nutritional de=ciencie$: however? nutritional anddietary approache$ have deon$trated $ucce$$ related to the prevention

o pica in only a very liited nu&er o patient$.2. Treatent involve$ &ehaviour and developent? environental? and ailyeducation approache$. ther $ucce$$ul treatent$ include a$$ociating thepica &ehaviour with &ad con$eBuence$ or puni$hent (ild aver$iontherapy) ollowed &y po$itive reinorceent or eating the right ood$.

%. Medication$ ay help reduce the a&noral eating &ehaviour? i pica occur$a$ part o a developental di$order $uch a$ ental retardation.

0.Enuresis:

http://emedicine.medscape.com/article/907543-overviewhttp://emedicine.medscape.com/article/1000028-overviewhttp://emedicine.medscape.com/article/999850-overviewhttp://emedicine.medscape.com/article/897591-overviewhttp://emedicine.medscape.com/article/1000028-overviewhttp://emedicine.medscape.com/article/999850-overviewhttp://emedicine.medscape.com/article/897591-overviewhttp://emedicine.medscape.com/article/907543-overview


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1+.Dia&ete$ ellitu$ i$ al$o a$$ociated with a&noralitie$ in the aIerent$en$ory pathway$ to the &ladder? which ay contri&ute to enure$i$.

1/.Dia&ete$ in$ipidu$ ay &e central or nephrogenic. 7entral dia&ete$in$ipidu$ ay re$ult ro an intracranial tuor? head traua? encephaliti$?or eningiti$. Nephrogenic dia&ete$ in$ipidu$ ay re$ult ro any cau$e o renal ailure? diIu$e renal cortical or edullary daage? hypoCaleia?

hypercalceia? or nephrotoHic drug$.tudies:

La


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o The intrana$al orulation ha$ a &lacC &oH warning &y the ,D3 and i$ nolonger recoended

o The recoended $tarting do$e or the ta&let i$ ".2 g? adini$tered 1hour &eore &edtie

o hyponatreic#related $ei9ure$ a$$ociated with the u$e o de$opre$$ino 3 aHiu o 1 cup o Juid $hould &e oIered at the evening eal? no

ore than 1 cup &etween ealtie and &edtie? and nothing to drinCwithin 2 hour$ prior to &edtie.

/. 3nticholinergic therapy!o helpul in $oe patient$? e$pecially tho$e with overactive &ladder?

dy$unctional voiding? or neurogenic &laddero The$e edication$ reduce uninhi&ited detru$or contraction$? increa$e

the thre$hold volue at which an uninhi&ited detru$or contractionoccur$? and enlarge the unctional &ladder capacity.

o The$e edication$ $hould not &e adini$tered during a ever &ecau$e adecrea$e in $weating i$ an anticholinergic eIect

o The do$age o oHy&utynin i$ 2.# g adini$tered at &edtie. 8ipraine therapy!

o iipraine i$ eIective to reduce &edwetting.o The u$ual do$e? taCen 1#2 hour$ &eore &edtie? i$ 2 g or patient$

aged +# year$ and "#/ g or older children and adole$cent$.o 3dver$e eIect$ include con$tipation? diWculty initiating voiding?

irrita&ility? drow$ine$$? reduced appetite? and per$onality change$.8ipraine overdo$e can &e atal? and a cautionary warning i$nece$$ary with every pre$cription

2.Inantile colic:,eature$!

1. Severe paroHy$al crying in late aternoon2. Fnee$ drawn up: =$t$ clenched: pa$$ Jatu$ or $tool$ or urinate: No re$pon$e to$oothing

%. 2 weeC$ # 2 hr6day : + weeC$ # % hr6day : % onth$# 1 hr6day : 7ontinue till '#onth$ in %"#'"

'.


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7au$e$!7au$e$ include the ollowing!

1. Ga$troe$ophageal reJuH? overeeding? undereeding?2. MilC protein allergy? and early introduction o $olid$.%. Parental anHiety and parental $tre$$ ha$ &een a $u&@ect o any $tudie$.

Po$tpartu depre$$ion ay lead to $tre$$ in parent$? which ay &e tran$erred tothe inant? re$ulting in eHce$$ crying.

'. ther cau$e$ include ineHperienced parent$ or incoplete or no &urping atereeding.

. ecent epideiologic evidence $ugge$t$ that eHpo$ure to cigarette $oCe and it$eta&olite$ ay &e related to colic. Maternal $oCing during pregnancy ay &ea$$ociated with colic.'

+. Soe evidence ha$ linCed per$i$tent crying in young inant$ to ood allegy. ana$$ociation &etween colic and cow$ ilC allergy (ca) ha$ &een po$tulated.+

/. data ro one $tudy $ugge$ted an a$$ociation &etween low &irth weight and

increa$ed incidence o colic.. ecently? $oe report$ have ocu$ed on inte$tinal icroJora and it$ a$$ociationwith colic. lower count$ o inte$tinal lacto&acilli were o&$erved in inant$ with coliccopared with inant$ without colic

Treatent!1. Dicycloine hydrochloride i$ an anticholinergic drug that ha$ &een proven in

clinical trial$ to &e eIective in the treatent o colic. ;owever? &ecau$e o$eriou$? although rare? adver$e eIect$ (eg? apnea? &reathing diWculty?$ei9ure$? $yncope)? it$ u$e cannot &e recoended.

2.


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. The only tie it ight cau$e concern i$ i it goe$ on &eyond + to year$ o age. 3tthi$ tie? it ay aIect the $hape o the oral cavity or dentition.

Tip$ ro the 3erican Dental 3$$ociation!1. Prai$e children or not $ucCing? in$tead o $colding the when they do.2. 8 a child i$ $ucCing it$ thu& when eeling in$ecure or needing coort? ocu$

in$tead on correcting the cau$e o the anHiety and provide coort to your child.

%. 8 a child i$ $ucCing on it$ thu& &ecau$e o &oredo? try getting the child$attention with a un activity.

'. 8nvolve older children in the $election o a ean$ to cea$e thu& $ucCing.. The pediatric denti$t can oIer encourageent to a child and eHplain what could

happen to it$ teeth i it doe$ not $top $ucCing.+. nly i the$e tip$ are ineIective? reind the child o it$ ha&it &y &andaging the

thu& or putting a $ocC6glove on the hand at night.Suary o 5e$t Practice$ ecoendation$!

1. 3erican 3cadey o Paediatric$ ! Mo$t children $ucC their thu&$ or =nger$ at$oe tie in their early lie. The only tie it ight cau$e concern i$ i it goe$ on

&eyond + to year$ o age or aIect$ the $hape o the child$ outh and thepo$ition o teeth.2. 3erican Dental 3$$ociation ! 7hildren $ucC on o&@ect$ a$ a natural reJeH:

however? during and ater the eruption o the peranent teeth? $uch $ucCing aycau$e pro&le$ with the $Celetal developent o the outh and alignent o theteeth.

4.!ood reusal:1. Aoung children ay go through period$ o u$$y eating. They have a $hort period

where they don>t want to eat certain ood$2. ,ood reu$al ight &e a noral part o growing up or $oe children: &ut it al$o

could &e $oetie$ indicating early $tage anoreHia.

%. Growing child want$ to &e ore independent. They ay declare it &y reu$ing toeat.

'. 8t can &e diWcult or ailie$ to get the &alance &etween reedo and parentalcontrol.

. Parent$ need to avoid &ecoing anHiou$ &ecau$e the toddler can re$pond to thi$&y &ecoing ore adaant in not wanting to eat.

+. Aoung children liCe routine $o parent$ need to have a regular eal $chedule./. Parent$ $hould try and eat with their toddler. 8t i$ al$o $ugge$ted that parent$ oIer

encourageent and prai$e i their child eat$ well.. Meal tie $hould &e un and relaHed with no other di$traction$.

*. Parent$ $hould not try and ru$h their child a$ $oe can &e Buite $low eater$: it i$recoended that twenty#=ve inute$ i$ an accepta&le aount o tie.

5. reat% %olding spell

1. 8ncidence ".1 to 2. 3ge + o to + year$%. eJeHive holding o &reath in eHpiration ollowing a period o provoCed cry'. 5ecoe$ $ilent? apneic? turn$ pale (pallid) or &lue (cyanotic). o$$ o con$ciou$ne$$ and opi$thotonu$? &ody @erC$? urination and ranC

convul$ion$ ay occur

http://en.wikipedia.org/wiki/Habit_(psychology)http://en.wikipedia.org/wiki/Habit_(psychology)


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+. e$olve$ $pontaneou$ly/. DD! Spell$ occur in!

Sei9ure$? 7NS tuor$? ailial dy$autonoia etc

. Manageent!i. ea$$ure parent$ii. 8gnore $pell$ and onitor or any coplication$

iii. Support the child &ut do not &ecoe $u&i$$iveiv. 0ncon$ciou$ne$$!v. Place the child on hi$ $ide

vi. Maintain airwayvii. 3tropine ."1 g 6 Cg S7 or &radycardia or a$y$tole

7. Te"per Tantru"s

1. 1#' year$: once a weeC in "#" o thi$ age group2. 7linical!

i. 7hild throw$ hi$el down? CicC and $crea: hold$ hi$ &reathii. 3 anie$tation achieving autonoy and a$tery over the environent

iii. 3nnoying at hoe? e&arra$$ing in pu&lic%. 7au$e! not ature enough to cope with diWcultie$'. Manageent

i. Minii9e the need to $ay no &y coorta&le environentii. Di$tractioniii. ewardiv. ,ight the &attle that need$ to won and avoid conJict$v. 3llow hi to achieve a$tery and autonoy

vi. Set rea$ona&le liit$. 3ter an attacC

i. Stay near&y: do not a&andonii. No threat$iii. Do not u$e negative ter$iv. Do not let hi hurt hi$el v. Do not hold a grudge ater the event

vi. P$ychiatry in re$i$tant ca$e$

8. 'ttention defcit %yper actie disorder 1. Neuro deveolpental di$order: 2#1" o $chool children2. Triad!

i. 8pul$ivity! act without thinCing =r$t? not learning ro pa$t i$taCe$

ii. 8nattention!iii. ;yperactivity

%. 8pul$ivityi. 3ct without thinCing =r$t?ii. Not learning ro pa$t i$taCe$iii. SpeaC


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vi. 7ognitively ipul$ive 3D;D Cid$ will aCe a ultiple nu&er o gue$$e$ ina $hort period o tie.

'. 8nattentioni. No attention to detail$ii. Short attention $paniii. Ea$y di$tracti&ility

iv. ,orgetulne$$v. ,ailure to li$ten when $poCen to

vi. ,ailure to engage in ta$C$. ;yperactivity

i. ,idgetine$$ii. DiWculty in reaining $eated in cla$$iii. EHce$$ive running and cli&ingiv. Not awaiting turn$

v. EHce$$ive talCing ! 3n$wer &eore Bue$tion i$ coplete+. Etiology

a. Genetic ! ultiactorial&. 3$$ociated with !i. ,ragile Z $yndroeii.


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2%

%. Epideiolgy! #1" $chool children'. Pathogene$i$!

a. Deect in let teporo # parieto#occipital &rain region$ which doe$ notunction properly while reading

&. 7linicali. DiWculty in teHt reading L $low reader$

ii. 0nder achiever in $chooliii. DiWculty in coprehendingiv. DiWculty in writingv. 8na&ility to eori9e

vi. DiWculty in $pellingvii. DiWculty in doing $iple atheatical pro&le$viii. DiWculty earning any new language

. Diagno$i$i. 8t i$ a clinical diagno$i$ii. ,aily hi$tory

iii. Teacher o&$ervationiv. ral reading to ea$ure reading accuracy and Juency u$ing $et ograded reader$ under tied condition$

v. 7opare with peer$+. ele


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2'

ii. Strong genetic &a$i$# ultiactorialiii. Diagno$ed &y % oiv. -aria&le $everity# $pectruv. ./ per 1" """ children

vi. MM vaccine# not proved%. &$ervation$!

i. Structural change$ in cingulate gyru$ii. Early accelerated &rain growth and ollowed &y $low growth

'. clinicali. 3&noral eye contactii. ,ailure to orient to naeiii. ,ailure to u$e ge$ture to point or $howiv. ,ailure to $ilev. acC o $haring

vi. acC o peer playvii. 3&$ence o iaginary play

viii. 0nu$ual u$e o language. DDi. ,ragile Z $yndroeii. Tu&erou$ $clero$i$iii. 3ngel an $yndroe

+. Diagno$i$!i. Screening u$ing checCli$tii. 2" have icrocephalyiii. Treatentiv. 5ehavior therapyv. Special $cooling

vi. Drug$!i. 7loipraine (tricyclic antidepre$$ant)ii. 7lonidineiii. Stiulant$! aphetaine: ethylphenidate

/. Progno$i$!i. May &e a&le to live $el $uWcient? eployed and live in counityii. Schi9ophrenia i$ a coplication


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2

Deliery roo" "anage"ent o Ne$


26/217

2+

4. 7au$e$1. Maternal!

1. Microva$cular i$chaeia (P8;)2. ow oHygen carried &y r&c ($evere aneia)%. 3cute &leeding (placenta previa? placental a&ruption)'. ShocC and acute inection

2. Placental#u&ilical cord actor$!1. &$tructed o u&ilical &lood Jow2. Dy$unction o placenta%. Po$t aturity

%. ,etal actor$!1. Maloration$ o cardiova$cular $y$te2. 8ntrauterine inection

5. 3nte partu a$$e$$ent1. ,etal oveent ()

• ,M or etal CicC$

• 1" CicC$ in '#+ hour window period• CicC$ recorded a$ H in the corre$ponding tie2. ,etal heart onitoring

• Non $tre$$ te$t• 7ontraction $tre$$ te$t

%. 5iophy$ical pro=le'. iBuor anii

7. ,;!1. Tool$!

• Monaural (pinard$) $tetho$cope?• ;and#held ultra$ound doppler apparatu$.• 7ardioto chograph#7TG

8. ,;


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Early ge$tation!• ,; high under $ypathetic control

ater ge$tation!• decrea$e in hr ater para $ypathetic aturation

,etal heart pattern$1. 5a$al etal heart rate

Noral 12"#1+"6t 5radycardia [ 12"

Tachycardia K 1+"

2. 5eat to &eat varia&ility ! &etween contraction$%. 3cceleration ! 16t la$ting or 1 $ec.'. 5a$eline &radycardia

Mean ,; [ 11" &p

Etiology!

;eart &locC

cciput po$terior or tran$ver$e po$ition?

,etal hypoHia. Zylocain during pudental &locC

. 3cceleration$ 3cceleration$# tran$ient increa$e in hr o 1 &p or ore la$ting or 1

$ec. Good outcoe. 7au$e$!

• EarlyhypoHia

• Maternal ever

• ,etal aneia• ,etal inection

+. Deceleration

,; &elow the &a$eline level o ore than 1 &p and la$ting or 1 $ec.Early!

Synchronou$ with uterine contraction

7au$ed &y head copre$$ion and vagal $tiulation

-aria&le ! Slowing oh h even ater uterine relaHation

7au$ed &y cord copre$$ion o the u&ilical cord

ate! Prolonged and per$i$t$ ater uterine contraction

7au$ed &y etal hypoHia

/. Stre$$ te$t1. 0terine contraction $pontaneou$ or induced &y nipple $tiulation or

oHytocin challenge.2. Po$itive!

% contraction$ in 1" inute$ ollowed &y late deceleration


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2

Prolonged deceleration!

5iophy$ical pro=le H2 1" point$

1. ,etal &reathing # %" $econd$ o $u$tained etal &reathing in %" inute$


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2*

2. ,etal oveent # % or ore gro$$ &ody oveent$ in %" inute$%. ,etal tone # one epi$ode o li& otion ro JeHion to eHten$ion to JeHion'. 3niotic Juid # pocCet o Juid ea$uring at lea$t one centieter. ,; 12"#1+" 6 t

3niotic e&rane and liBuor anii!1. 3&$ent e&rane endanger$ etal lie

2. Meconiu $tained aniotic Juid indicate$ e$ta&li$hed etal di$tre$$,etal P;

1. 3 icrotechniBue o $apling &lood ro the etal $calp2. Ph [ /.2 indicate$ etal di$tre$$

Neonatal resuscitation Perinatal phy$iology! $eBuence o event$ during &irth!

1. ,ir$t cry2. ung eHpan$ion%. 7learing o lung Juid'. 3ir eHchange. Terination o t to t $hunt$

+. 8ncrea$e in Po2 ro 2 to +" ;g

/. Decrea$e in pulonaryva$cular re$i$tance

. 8ncrea$e in t atrial Jow*. 8ncrea$e in $y$teic &p

7irculatory tran$ition

• ,atal tran$itional neonatal• 7oproi$ed &y actor$!

1. ,etal2. Placental

%. Maternal

,etal actor$1. Preature delivery2. Pulonary hypopla$ia%. 8ntra uterine inection$'. 7;D

. ;ydrop$ etali$+. 7hroo$oal anoalie$/. Maternal $edation


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%1

'ctivity (Mu$cle Tone) 3&$ent 3r$ 4 leg$eHtended

3ctive oveentwith JeHed ar$ 4leg$

Pul$e (;eart ate) 3&$ent 5elow 1"" 5PM 3&ove 1"" 5PM

*riace (e$pon$e

Stiulation or eJeH8rrita&ility)

No

e$pon$e

,acial griace Snee9e? cough? pull$

away

'ppearance (SCin 7olor) 5lue#gray?pale all over

PinC &ody and&lue eHtreitie$

Noral over entire&ody L 7opletelypinC

;e$piration (5reathing) 3&$ent Slow? irregular Good? crying

Pri"ary secondary apnoea:

Priary apnea!


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%2

1. 8ntu&ate2. 1"" o2

;r [ +"!%. 7ardiac a$$age with &ag! %!1 ratio'. 7ardiac a$$age

'. Medication!

1. 7athedri9e u&. -ein with $aline =lled cath.2. Epinephrin 1!1"""" .1 to .% l6Cg%. Noral $aline 1" l6Cg'. 5icar&onate ' l6Cg o . g6l. Dopaine drip! #2" ic.g6Cg6t

. Meconiu a$piration!1. No &ag and a$C2. -igorou$ $uction%. 8ntu&ation 4 $uction o econiu through ET

,E-E

1. Noral teperature!1. 3dult core (rectal) tep! %+.' c to %+.* c with .' c Juctuation2. 8nant$ rectal tep! %/ c and . c Juctuation during $leep

%. 7ircadian rhyth! lowe$t at ' a: highe$t tep at + p'. EHternal tep . le$$ than core tep0. !eer defnition: core te"p B28 c%. Theroeter$!

1. Mercury!i. 3Hillary: oralii. ectaliii. ow reading

2. Eletronic! Typanic%. 7ry$tal ! ,orehead

'. ,ever echani$!


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%%

1. Theroregulatory centre in ant hypothalau$ i$ $et to higher level &yi. Endogenou$ Pyrogen$! cytoCine$: intereron: tuour necro$i$ actorii. EHogenou$ Pyrogen$!

1. Micro&e$ icro&ial toHin$ $tiulation o

acrophage$endogenou$ pyrogen$2. 5acterial endotoHin $tiulate$ Thero$tat directly

%. 3ntigen anti&ody copleH al$o $tiulate$ endogenopu$pyrogen$2. Pyrogen$ $tiulate preoptic area o hypothalau$ to relea$e pro$taglandin

E2 which act$ on the hypothalau$ to rai$e the $et point:%. The hypothalau$ aintain$ the $et point at the elevated level until it i$

re$et downward &y the di$appearance o endogenou$ pyrogen$ or theinhi&ition o pro$taglandin $ynthe$i$ (e.g.? &y antipyretic agent$).

'. Theroregulatory re$pon$e o the &ody!i. -a$odilatation or con$trictionii. 8ncrea$e or decrea$e in $weatingiii. -a$opre$$in altering the volue o E7,iv. 8ncrea$e in u$cle contraction$#$hiveringv. 5ehaviour! to ove to war or cool place

vi. 7hill$ ay &e provoCed &y antipyretic$ that cau$e $udden decrea$eo &ody teperature.

. 7ircadian rhyth!i. 1\c le$$ in early orningii. 1\c ore in evening

. 7au$e$ o ever!1. 8nection$2. -accine$%. Ti$$ue in@ury! inarction? pulonary e&oli$? traua etc'. Malignancy ! lyphoa? leuCeia? hepatoa

. 3utoiune di$order$! heuatoid arthriti$? heuatic ever? SE+. Drug$! cocaine? atropine? 3photericin 5 and drug ever/. 8nJaation! ulcerative coliti$? regional ileiti$. Granuloatou$ di$ea$e! $arcoido$i$! $arcoido$i$ i$ a nonca$eating

granuloa that i$ ored in re$pon$e to an eHaggerated iune reactionediated &y T#helper cell$. The etiology i$ unCnown? although increa$ediunoglo&ulin M (8gM) and hyperglo&ulineia again$t variou$ inectiou$agent$ are pre$ent.

*. Endocrine! thyrotoHico$i$? pheochroocytoa (ro adrenal edulla)1".Meta&olic ! ureia? gout11.Genetic di$order$!

1. Nephrogenic Dia&ete$ 8ncipitu$2. eiley Dey $yndroe! iley#Day $yndroe? i$ an auto$oal rece$$ivedi$order $een predoinantly in ]ew$ o ea$tern European de$cent.Patient$ pre$ent with $en$ory and autonoic di$tur&ance$: a decrea$eda&ility to eel pain or teperature $en$ation$: inappropriate &loodpre$$ure and &ody teperature Juctuation$: trou&le with eeding?$wallowing and ga$trointe$tinal otility: hypotonia: developentaldelay$: recurrent pneuonia$ (ro a$piration): $colio$i$ and Cypho$i$:increa$ed $weating: tran$ient $Cin &lotching: and decrea$ed $tature.


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%'

%. congenital icthyo$i$'. ;ereditary periodic ever$!

1. ,ailial Mediteranean ever! The $alient eature$ o ,M, include &rierecurrent epi$ode$ o peritoniti$? pleuriti$? and arthriti$? u$ually withaccopanying ever. ,M, occur$ within ailie$ and i$ uch orecoon in individual$ o Mediterranean de$cent than in per$on$ o

any other ethnicity.utation in chroo$oe 1+ cau$e decrea$e inpyrin that i$ nece$$ary or inhi&ition o inJaatory ediator$:auto$oal rece$$ive

2. Tuor necro$i$ actor (TN,) receptorLa$$ociated periodic $yndroe(T3PS)

%. MucCle#


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%

iv. ;eat $troCe+. 5ene=cial eIect$ o ever!

1. ,ever i$ e$ta&li$hed a$ a phylogenetically ancient ho$t re$pon$e that i$con$erved highly in all aal$

2. 8t i$ an adaptive re$pon$e and not a di$ea$e%. the anial$ =nd the ware$t $pot in the environent and reain there

while their &ody teperature increa$e$ in re$pon$e to the eHternal $tiulu$'. it decerea$e$ icro&ial reproduction. increa$e$ the rate o iune reaction$+. $tiulate$ lyphocyte production/. increa$e in intereron production. increa$e phagocytic activity*. increa$e$ $eru erritin level that inhi&it$ icro&ial growth

/. 3dver$e eIect$!1. 8ncrea$e!

i. 2 con$uptionii. 72 productioniii. 7ardiac output

iv. 8ncrea$ed in heart &eat$ &y 1" to 1 &eat$ or 1 .:v. 77, in aneia and 7;Dvi. Pulonary in$uWciency in chronic lung di$ea$evii. Meta&olic coplication$ in dia&ete$

2. ,e&rile $ei9ure$ in + onth$ to year$%. 8n epilep$y? prone or $evere $ei9ure$'. Dehydration. 3 ever greater than 1"+ degree$ ,ahrenheit can re$ult in &rain daage and

death+. 8ncrea$e the cata&oli$ o protein$ leading to lo$$ o protein o %"" to

'""gra$ per day/. TN,# alpha and 8#1 cau$e the relea$e o endor=n$ in the &rain that

produce deliriu. 1erpes la


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%+

iv. Aellow wverv. 7olorodo ticC ever

5. Periodic eer:i. ,ailial Mediteranean everii. Tuor necro$i$ actor (TN,) receptorLa$$ociated periodic $yndroe

(T3PS)

iii. MucCle#


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!eer o 6ncertain Arigin (!6A)/. De=nition! Peter$dor and 5ee$on (1*+1)!i. !eer %ig%er t%an 28.2o on seeral occasions recorded


38/217

%

iv. 3&$ence o $weating!1. Nephrogenic dia&ete$ in$ipitu$2. 0nhydrotic ectoderal dy$pla$ia%. ,ailial dy$autonoia(eily Dey)

v. ed weeping eye$! polyarteriti$ nodo$avi. Palpe&ral con@unctiviti$!

1. Mea$le$2. 7ocC$acCie%. T5'. 8nectiou$ ononucleo$i$

vii. 5ul&ar con@unctiviti$!1. Fawa$Ci2. epto$piro$i$

viii. 7on@unctival heorrhage! inective endocarditi$iH. 0veiti$!

1. Sarcoido$i$2. ]3%. SE

H. 7horioretiniti$!1. 7M-2. ToHopla$a

Hi. Propto$i$! or&ital celluliti$Hii. acC o tear$!

1. ,ailial dy$autonoia(eily Dey)2. S@ogren $yndroe! (Sicca $yndroe) i$ an autoiune di$order in

which iune cell$ attacC and de$troy the eHocrine gland$X2Y that producetear$ and $aliva. The hallarC $ypto$ o the di$order are dry outh and dry eye$

Hiii. Sinu$ tenderne$$! $inu$iti$Hiv. ,ever &li$ter$! rule$ out enteric ever: $taph inection

Hv. 7on@e$ted pharynH!1. 8nectiou$ ononucleo$i$2. Fawa$Ci

Hvi. 5one pain and tenderne$$!1. $teoyeliti$2. euCeia

Hvii. eerred pain over trape9iu! $u&diaphraatic a&$ce$$Hviii. Tenderne$$ per rectu! pelvic a&$ce$$

8. a&!i. 757!

%. Poly [ """!a. no &acterial etiology&. enteric ever

'. PolyK1"""" and K &and$ ! &acterial $ep$i$ii. ES!

. K %" ! inallaatory condition$+. K1"" !

c. 3utoiuned. T5e. Fawa$aCi

iii. 5lood culture! aero&ic? anero&ic? enteric and 3,5iv. 0rine culture! id $trea o $uprapu&ic

http://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Immune_cellhttp://en.wikipedia.org/wiki/Exocrine_glandhttp://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome#cite_note-pmid18289371-1http://en.wikipedia.org/wiki/Tearshttp://en.wikipedia.org/wiki/Salivahttp://en.wikipedia.org/wiki/Dry_mouthhttp://en.wikipedia.org/wiki/Dry_eyeshttp://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Immune_cellhttp://en.wikipedia.org/wiki/Exocrine_glandhttp://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome#cite_note-pmid18289371-1http://en.wikipedia.org/wiki/Tearshttp://en.wikipedia.org/wiki/Salivahttp://en.wikipedia.org/wiki/Dry_mouthhttp://en.wikipedia.org/wiki/Dry_eyes


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%*

. MantouH! po$itive priary T5: Negative in $arcoido$i$+. Zray ! 7he$t? $inu$ and a$toid/. 5one arrow! euCeia and MP. yuphnode! &iop$y or ,N37! T5 and lyphoa

9. 0ltiate cau$e$!

8nection$ %" # '"

Malignancie$ 2" L 2

7ollagen -a$cularDi$ea$e

1" L 2"

Mi$cellaneou$ 1 L 2"

0ndiagno$ed 1" L 1

/?. Treatent!1. 3void epirical trial$: it o&$cure$ diagno$i$ o inective endocarditi$ ?

eningiti$ etc2. 3TT i the child i$ critically ill and T5 i$ $u$pected

//. Progno$i$!1. 7hildren with ,0 ha$ &etter progno$i$ than adult$2. More oten ,0 i$ due to atypical pre$entation o a coon illne$$%. 8n 2 ca$e$ the diagno$i$ reain$ unclear


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'"

!loppy inant syndro"e1. De=nition! eer$ to an inant with generali$ed hypotonia pre$enting at &irth or in

early lie.0. 1ITA;H:

a. ,etal oveent$ are dini$hed in utero&. The incidence o &reech pre$entation i$ higher in oetu$e$ with

neurou$cular di$order$ a$ turning reBuire$ adeBuate etal o&ility.c. 5irth anoHia? a$ hypoHic#i$chaeic encephalopathy reain$ an iportant

cau$e o neonatal hypotonia.d. Neonatal $ei9ure$ and an encephalopathic $tate oIer urther proo that the

hypotonia i$ o central origin.e. The on$et o the hypotonia i$ al$o iportant a$ it ay di$tingui$h &etweencongenital and aBuired aetiologie$.

. EnBuire a&out con$anguinity and identiy other aIected aily e&er$ inorder to reach a de=nitive diagno$i$? u$ing a detailed aily pedigree toa$$i$t uture genetic coun$elling.

2. linical clues on neurological e@a"ination:a. There are two approache$ to the diagno$tic pro&le.

i. The =r$t i$ &a$ed on identiying the neuro#anatoical $ite o thele$ion or in$ult. 7areul neurological eHaination $hould? in o$tca$e$? locali$e the $ite o the le$ion to the upper otor neuron (0MN)

or lower otor neuron (MN) unitii. The $econd i$ to deterine whether or not the hypotonia i$accopanied &y weaCne$$. ). 3pply

pre$$ure to the trachea and wait or a $ingle cough that clear$$ecretion$. 8 ore than one cough i$ needed to clear $ecretion$? thi$i$ indicative o weaCne$$.

ii. Poor $wallowing a&ility a$ indicated &y drooling and oropharyngealpooling

o $ecretion$.iii. The character o the cry ̀ inant$ with con$i$tent re$piratory

weaCne$$ have a weaC cry.iv. ParadoHical &reathing pattern ` interco$tal u$cle$ paraly$ed with

intactdiaphrag.


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'1

v. ,rog#liCe po$ture and Buality o $pontaneou$ oveent$ ` poor$pontaneou$ oveent$ and the rog#liCe po$ture are characteri$tico MN condition$.

vi. EHce$$ive head lag will &e evident on _pull to $it>.vii. Minial $upport with a $en$ation o _$lipping through the hand$>

during vertical $u$pen$ion te$ting and inverted 0 po$ition on ventral

$u$pen$ion are urther indicator$c. 3 di$tinct pattern o weaCne$$ ay avour certain aetiologie$!i. 3Hial weaCne$$ i$ a $igni=cant eature in central hypotonia.ii. Generali$ed weaCne$$ with $paring o the diaphrag? acial u$cle$?

pelvi$ and $phincter$ $ugge$t$ anterior horn cell involveent.iii. $ di$ea$e.vii. 3n E7G ay &e helpul in deon$trating &a$eline a$ciculation$ o

the interco$tal$ u$cle$.viii. The pre$ence o a acial diplegia (yopathic acie$: a$C liCe)

$ugge$t$ either a congenital yopathy or ya$thenia gravi$.iH. e$piratory and &ul&ar weaCne$$ can accopany &oth condition$.H. ,luctuation in $trength would avour ya$thenic $yndroe$Hi.


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'2

iii. ellweger $yndroe! high orehead? wide patent ontanelle$ and$uture$? $hallow or&ital ridge$? epicanthu$ and icrognathia.Neurological anie$tation$ are doinated &y $evere hypotonia withdepre$$ed or a&$ent tendon reJeHe$? and poor $ucCing and$wallowing. ;epatoegaly and liver dy$unction are con$i$tent=nding$.

&. 3 Caryotype i$ a u$t in the dy$orphic hypotonic child. Thi$ can &eco&ined with ,8S; te$ting i Prader#$ $yndroe. 3n eHce$$ive range o @oint o&ility? unu$ual @ointpo$ture$? and the a&ility to do variou$ contortion$ o the li&$ and6orhyperela$ticity o the $Cin are iportant diagno$tic clue$.

%. 3lthough eta&olic di$order$ are well recogni$ed a$ the cau$e or centralhypotonia? &ecau$e o the rarity o the condition$? the diagno$tic yield i$ low. -erylong#chain atty acid$ (-7,3) te$ting $hould &e perored i a peroHi$oaldi$order $uch a$ ellweger i$ $u$pected.

6PETED PE;IP1E;'L '6E

1. genetic analy$i$! o the u$cular dy$trophie$? $pinal u$cular atrophie$ andhereditary neuropathie$.

2. EHaple$ include!a. $pinal u$cular atrophy! polyera$e chain reaction (P7) te$ting or a

deletion o the $urvivor otor neuron (SMN) gene and&. congenital yotonic dy$trophy! eHpan$ion o cyto$ine thyine guanine

(7TG) triplet repeat$.c. Duchenne u$cular dy$trophy! DN3 Zp21 deletion te$ting (P7 a$$ay$) cancon=r the diagno$i$ in + o ale$ with and " o ale$ with 5ecCer>$u$cular dy$trophy.

d. nly in the reaining ca$e$ i$ u$cle &iop$y $till advocated.e. Mu$cle en9ye$ level$ (creatine Cina$e a$$ay) are rarely helpul in the

Joppy inant? with the eHception o u$cle di$order$ where creatine Cina$evalue$ are elevated? $uch a$ congenital u$cular dy$trophie$ and in $oeo the congenital $tructural yopathie$.

%. Electroyography (EMG)!Neuropathic di$ea$e!

• 3n action potential aplitude that i$ twice noral due to the increa$ednu&er o =&re$ per otor unit &ecau$e o reinnervation o denervated=&re$

• 3n increa$e in duration o the action potential• 3 decrea$e in the nu&er o otor unit$ in the u$cle (a$ ound u$ing

otor unit nu&er e$tiation techniBue$)Myopathic di$ea$e!

• 3 decrea$e in duration o the action potential• 3 reduction in the area to aplitude ratio o the action potential

http://en.wikipedia.org/wiki/Action_potentialhttp://en.wikipedia.org/wiki/Amplitudehttp://en.wikipedia.org/wiki/Fibreshttp://en.wikipedia.org/wiki/Reinnervationhttp://en.wikipedia.org/wiki/Timehttp://en.wikipedia.org/wiki/Motor_unithttp://en.wikipedia.org/wiki/Motor_unit_number_estimationhttp://en.wikipedia.org/wiki/Areahttp://en.wikipedia.org/wiki/Amplitudehttp://en.wikipedia.org/wiki/Action_potentialhttp://en.wikipedia.org/wiki/Amplitudehttp://en.wikipedia.org/wiki/Fibreshttp://en.wikipedia.org/wiki/Reinnervationhttp://en.wikipedia.org/wiki/Timehttp://en.wikipedia.org/wiki/Motor_unithttp://en.wikipedia.org/wiki/Motor_unit_number_estimationhttp://en.wikipedia.org/wiki/Areahttp://en.wikipedia.org/wiki/Amplitude


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• 3 decrea$e in the nu&er o otor unit$ in the u$cle (in eHtreely $evereca$e$ only)

'. Nerve conduction $tudie$ are u$eul in the inve$tigation o hereditary otor$en$ory neuropathie$ and di$tingui$hing aHonal ro deyelinating di$order$.

. Molecular DN3 te$ting can then &e u$ed or $peci=c deyelinating di$order$.+. Mu$cle &iop$y!

a. -ariation in $i9e and $hape o u$cle =&er$&. 8ncrea$e in connective ti$$uec. ,atty in=ltrationd. 3rea$ o degeneration and regeneratione. 8nJaatory change$. 7entrali9ed nuclei

P;INIPLE A! M'N'*EMENT1. egular phy$iotherapy will prevent contracture$.ccupational therapy i$ iportant

in acilitating activitie$ o daily living.2. -igorou$ treatent o re$piratory inection$ i$ indicated.%. 3nnual Ju vaccination i$ nece$$ary.'. 3nnual orthopaedic review i$ reBuired to onitor or $colio$i$ and to eHclude hip

di$location6 $u&luHation.. ,eeding intervention &y na$oga$tric tu&e or ga$tro$toy will &ene=t the

undernouri$hed child. Maintenance o ideal weight i$ iportant? a$ eHce$$iveweight gain will eHacer&ate eHi$ting weaCne$$.

+. 7hildren with neurou$cular di$order$ de$erve $pecial attention when it coe$ toanae$the$ia. The anae$theti$t $hould &e orewarned a&out the po$$i&ility o anunderlying u$cle di$ea$e even i the child ha$ very ild or non#eHi$ting$ypto$. 3 aily hi$tory o u$cle di$ea$e or ild hyper#7F#aeia ay &e oiportance.

/. Mu$cle relaHant$ $hould only &e u$ed i e$$ential &ecau$e o their ore prooundand prolonged eIect in yopathic children.. 3ll children with neurou$cular di$ea$e $hould al$o &e con$idered potentially

$u$cepti&le to alignant hypotheria (the $tronge$t correlation i$ with centralcore di$ea$e) and iplicating agent$ $hould thereore &e avoided.

*. Ethical con$ideration$ $uch a$ the appropriatene$$ o cardiopulonaryre$u$citation in the event o cardiac arre$t or acute re$piratory ailure need to &eaddre$$ed $en$itively.

lincical clues and inestigations o t%e "ore co""on p%enotypes o t%e

oppy $ea& inant

pinal cord transection or 1aeangioa or tut o hair in idlineM8 $pinal cord

disease Scolio$i$

yringo"yelia or ot%er or"s Evidence o &ladder or &owel dy$unctionM8 $pinal cord

o spinal dysrap%is" MiHed deep tendon reJeHe$ with a&$enta&doinal and anal reJeHe$


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''

pinal "uscular atrop%y Tongue atrophy and a$ciculation$ E7G!5a$eline a$ciculation$

ParadoHical &reathing pattern Deletion o the$urvival

Severe proHial u$cle weaCne$$ with otor neuron

(SMN) gene &y P7a&$ent tendon reJeHe$ te$tingPre$erved $ocial interaction

Perip%eral neuropat%y


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'

3$$ociated &rain and eye pro&le$ 5rain M8 or$tructural and

white attera&noralitie$

Mu$cle &iop$y! ero$in$tain

ongenital "yotonic Polyhydranio$ with reduced etal MolecularDN3 te$ting &ydystrop%y oveent$ deterining nu&er o 7TG

8nverted -#appearance o the outh repeat$ (noralrange # %*

EHaination o other>$ ace $how$ ina&ility repeat$)to &ury her eyela$he$ and grip yotonia EMG o the

other

Preature cataract $urgery in the other

ongenital structural Slender $tature 7F andEMG u$ually not"yopat%y ;ypotonia with eeding pro&le$ at &irth helpul


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'+

Mu$cle &iop$y u$uallynot

nece$$ary


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'cute


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2. Sinu$iti$%. Pneuona'. $teoyeliti$. Endocarditi$+. 3&$ce$$

0. Pat%ology

a. Meningeal eHudation&. -entriculiti$c. -a$culiti$d. Thro&o$i$e. 8ncrea$e in 87P. 7ranial neuropathyg. ;ydrocephalu$h. Su&dural eIu$ion

2. linical "aniestations

a. arely draatic on$et and $udden death in 2' hr$

&. 8n$idiou$ on$et!i. ,everii. ;ead acheiii. Photopho&iaiv. 8rrita&ilityv. ethargy

vi. 3ltered $en$oriuvii. 7onvul$ion$viii. 7oa

3. linical signs

i. Nugal rigidityii. Fernig $ign ! ,leHion o hip to *"\ and eHten$ion o Cnee produce

painiii. 5rud9inCi $ign! ,leHion o necC produce$ JeHion o hip and Cnee$iv. 5ulging ontannelv. Sutural widening

vi. 8v ? vi? vii? viii cranial nerve pal$yvii. ,ocal de=cit$viii. Tache cere&rale! StroCe $Cin with a &lunt in$truent %"#+" $ec

rai$ed red ra$h

iH. 7onvul$ion$

4. auses or onulsions i. 7ere&riti$ii. -entriculiti$iii. 7ere&ral a&$ce$$iv. 8narctionv. Syndroe o 8nappropriate 3D; $ecretion 4 electrolyte

di$tur&ance$vi. ;ypoglyceiavii. -a$cular thro&o$i$


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'*

viii. Su&dural eIu$ion5. u&ar puncture

a. Noral 7S,i. 7olour! clearii. Gluco$e! +" o &lood level: " to " g6dliii. Protein! 1#% g6dl

iv. 7hloride! 11+#122v. Pre$$ure! 1""#2"" o water

vi. 7ell$! [ lypvii. 7S, in eningiti$


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i. 7oon in ;.inJuen9aii. 7ollection o Juid in $u&dural $paceiii. 5ulging ontaneliv. Sutural wideningv. 8ncrea$e in head circuerence

vi. 7onvul$ion$ and

vii. ever/?. o"plications

a. Epilep$y&. 7r n pal$ie$c. StroCed. Dural $inu$ thro&o$i$e. S83D;. Deane$$g. Mental retardationh. D8-7

//. yndro"e A Inappropriate 'D1 ecretiona. ,ailure o o$oreceptor$ in the hypothalau$&. EHce$$ive relea$e o 3D; ro the po$t pituitaryc. ,luid retentiond. Delu$ional hyponatreiae. ;eadache. 7onvul$ion$

/0. Meningococce"ia

Nei$$eria eningitide$!a. +ater%ouse-rideric%sen syndro"e!

i. ;eorrhagic adrenaliti$ with adrenocortical in$uWciencyii. verwheling &acterial inectioniii. ShocCiv. Di$$einated i ntrava$cular coagulation(D87)v.


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2

ENEP1'LITI K ENEP1'LAP'T1H/. Introduction:

• Potentially $eriou$ inection or inJaation o 7NS

• 8ncrea$ed ortality

•

;igh rate o coplication$• ong ter or&idity

0. Defnitions:

• Meningiti$!

o 8nJaatory proce$$ o the e&rane$ o &rain and $pinal cord and c$

• Encephaliti$! a$eptic eningiti$ $yndroe

o 8nJaation o the &rain parenchya

• Meningoencephaliti$!

o 8nJaation o the eninge$ and &rain parenchya

• 7ere&riti$!

o 5acterial inection o &rain ti$$ue? and a&ce$$ oration• Encephalopathy!

o Generali9ed di$order o cere&ru without 7S, pleocyto$i$

2. Encep%alitis:a. !eatures:

i. 8nJaation o eninge$ and &rainii. 7S, inJaatory cell$iii. No organi$$ in G $tainiv. 5acterial culture negative


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%

i. Polio? up$? ea$le$

• 3canthaoe&a! $wiing pool 4 contact len$

0. ]3P3NESE EN7EP;38T8S !

• 3 Javi viru$ ($ingle#$tranded rna)

• Tran$i$$ion # c. Tritaeniorhynchu$? culeH vi$hnui (india)?

•

3pliying ho$t$ # pig$ and aBuatic &ird$ ( egret$? heron$)• Su&clinical! re$olve in a ew day$

• 7linial! neurotoHic eIect$ in &rain cell$ and neural $te cell$.

• a@or neurologic $eBuelae including $ei9ure di$order$? otor or cranialnerve pare$i$? or oveent di$order$.

• 7a$e#atality rate$ range ro 1 to +".

2. Encephaliti$!Pathogene$i$!

• ocal replication in the $Cin or uco$a

• ;eatogenou$ di$$eination #]E

• Neuronal retrograde di$$eination# ra&ie$ ! peripheral nerve: ;S- !alactory tract

• priary vireia lead$ to $eeding o the reticuloendothelial $y$te

• Secondary vireia lead$ to $eeding o other $ite$? including the 7NS

• Pathology

• -aria&le degree$ o eningiti$? cere&ral oedea? conge$tion? andhaeorrhage

• Periva$cular cuWng! the accuulation o lyphocyte$ or pla$a cell$ ina den$e a$$ around the ve$$el.

• Deyelination

• ;ydranencephaly! &rain ti$$ue replaced &y $ac$ =lled with c$

• Neuronal daage

• Glio$i$

3. 78N873 ,E3T0ES

• 78N873 SPE7T0M

Su&clinical # ice&erg

-aria&le $everity

Mild illne$$ with coplete recovery or coa and $udden death

• TAP873 8NESS!

;ead ache? ever? irrita&ility nau$ea? voiting

etro &ul&ar pain

Photopho&ia

3ltered $en$oriu

7onvul$ion$

Stupor

7oa


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• P33AS8S

Static ? Progre$$ive or ,luctuating

,laccid or $pa$tic

• 5ladder &owel dy$unction

• Eotional out&ur$t$

• 7ere&ral edea and elevated 87P• EEG!

Slow wave pattern$

• 7T6M8!

5rain edea: necro$i$

• ,ocal le$ion$ in ;S-

ENEP1'LAP'T1H !1. Etiology!

• 8nection$!

T&? enteric ever? $higella

;iv• ToHic!

7o? lead? a$pirin? alcohol

• Meta&olic!

5iliru&in? uraeia? hypoglyceia

• ;ypoHia

;ypoHic i$cheic encephalopathy

• ;yperten$ion

Nephriti$

•

;ereditory! Mitochondrial encephalopathy

• Myoclonic encephalopathy o childhood

• Slow viru$ (prion$)

7reut9eldt#@aCo& di$ea$e

Furu

• Su&acute $clero$ing pan encephaliti$($$pe)

• euCeia

• Punch drunC $yndroe (trauatic)

1. ;EHE HND;AME:

• 3cute non inJaatory encephalopathy and hepatic ailure.• 3ter a viral illne$$ liCe inJuen9a and varicella

• 3$$ociated with the u$e o a$pirin during the illne$$

• 8n&orn error$ o eta&oli$ ay &e a predi$po$ing actor

• Mitochondrial dy$unction

• ,ollow$ an apparent recovery ro viral illne$$

2. Stage$ o eye $yndroeStage 1 # voiting? $leepine$$? and lethargy


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Stage 2 # re$tle$$ne$$? irrita&ility? di$orientation? deliriu?Stage % # o&tunded? coato$e? decorticate rigidity?Stage ' # deep coa? decere&rate rigidity? =Hed and dilated pupil$? lo$$ ooculove$ti&ular reJeHe$?Stage # $ei9ure$? Jaccid paraly$i$? a&$ent deep tendon reJeHe$? no pupillaryre$pon$e? and re$piratory arre$t

%. DI'*NAI A! ;EHE HND;AME• 3ltered level o con$ciou$ne$$: convul$ion$• 8ncrea$e in SGT? SGPT and aonia• ;ypoglyceia• noral 7S, &ut increa$e in 87P• No @aundice• 5rain &iop$y with cere&ral edea without inJaation• Mortality K '"


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'ne"iaIntroduction:

/. Ste cell i$ the precur$or o 57$0. Erythropoie$i$! erythropoietin direct$ $te cell$ into the production o yeloid

precur$or$!a. Proerythro&la$t early erythro&la$t late erythro &la$t noro&la$t

reticulocytepolychroatic cell$ 57

2. Erythropoietin (epo)a. ;orone produced &y the Cidney in n& and &y liver in etu$&. Proote$ the oration o red &lood cell$c. Fidney cell$ aCe and relea$e erythropoietin in re$pon$e to hypoHiad. 3ction through rna

3. ed &lood cell di$tri&ution width!a. 3 ea$ure o the variation o 57 $i9e.

&. ed &lood cell$ o uneBual $i9e$? i$ Cnown a$ ani$ocyto$i$.c. Noral rdw i$ 11 # 1'.d. 8ron de=ciency anaeia $how$ an increa$ed rdw.e. dw ( $tandard deviation o cv ean cv) b 1""

4. Noro&la$t$!a. ,ull eta&olic unction$&. ow o2 carrying capacityc. oo$e$ nucleu$ &y eHtru$ion


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5. eticulocyte$a. 1 o the red cell$&. Develop and ature into 57c. ;ave a reticular networC o ri&o$oal rna

d. -i$i&le with ethylene &lue

7. PoiCilocyto$i$DiIerence in $hape o 57$!eg.

i. Microcyte$ii. Spherocute$iii. valocyte$iv. Tear drop cell$

8. 57!a. oo$e$ nucleu$ to accoodate ;&&. 8ncrea$ed "2 carrying capacityc. ,leHi&le di$cd. 7ontain '" en9ye$e. No itochondria. 3TP &y anaero&ic and pento$e pathwayg. Energy needed to Ceep iron in errou$ $tate and to neutrali9e oHidant$h. +"#*" day$ lie $pan


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i. 0tili9e gluco$e without in$ulin

9. Nor"al alues

1. ;5! ale! 1%. to 1/.2 g6dl: eale! 12.1 to 1.1 g6dl2. 57 count! ale! './ to +.1 illion cell$6cl:

,eale! '.2 to .' illion cell$6cl%. Mcv! " to 1"" etoliter(J)'. Mch! 2/ to %1 picogra$6cell. Mchc! %2 to %+ gra$6dl+. Pac&ed cell olu"e (pc)! ' or en and % or woen.

'ne"ia:

/?. De=nition! hg [ 2 $tandard deviation$ &elow the ean or age with1. educed ;52. educed P7-%. educed 57 count

//. 3naeia i$ a $ign o an underlying pathology (it i$ not a diagno$i$)

/0. lassifcation o ane"ia:

8. Etiologic cla$$i=cationv. 1. 8paired 57 production

vi. 2. EHce$$ive de$tructionvii. %. 5lood lo$$

8i. Morphologic cla$$i=cationviii. 1. Macrocytic aneiaiH. 2. Microcytic hypochroic aneiaH. %. Norochroic norocytic aneia

Type$ o 57$1. Norocytic 2. Microcytic %. Macrocytic

/0. Iron defciency anae"ia

i. alculation o daily reuire"ent:

1. 8ron content o new&orn i$ .g : adult i$ g2. ". g $hould &e a&$or&ed daily or 1 year$ to reach thi$ value.%. o$$ o iron i$ 1 g6day through hair? epitheliu and neil$


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*

'. 1 g 6day i$ the daily reBuireent. nly 1" i$ a&$or&ed ro the gut+. ;ence 1" g i$ the daily reBuireent

ii. auses so iron defcient ane"ia!%" o world population i$ aneicNe$


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+"

i. ;&! 1" gii. Pcv %"

%. 7au$e$ o phy$iological aneia!i. Decrea$ed 57 lie $panii. Decrea$ed eryhtropoietiniii. apid increa$e in &ody weight

/4.La< tests:/. ;50. Pcv2. Seru erritin3. Seru iron4. 8ron &inding capacity5. 5lood $ear $tudy7. Stool occult &lood8. 5one arrow

/5. Dierential diagnosis o Microcytic %ypoc%ro"ic ane"ia:

/. Thala$$eia trait 4 ;& E di$ea$e

a. ;& 32 increa$ed&. ;& , increa$edc. Seru iron and 857 noral

0. ead poi$oning!a. 5a$ophilic $tippling&. ,ree erythro protoporphyrin (,E5) increa$ed

2. Sidero&la$tic aneia!a. 3ino levulinic acid $yntha$e de=ciency&. Deective hee $ynthe$i$c. Sidero&la$t$ in peripheral $ear

3. 3tran$errineiaa. No $taina&le iron in noro&la$t$

5a$ophilic $tippling! Sidero&la$t$!

/7. Treat"ent:

1. ,errou$ $ulphate &e$t a&$or&eda. 5ad ta$te?&. Ga$tric up$etc. 7on$tipationd. 7ontain$ 2" eleental irone. '#+ g 6 Cg in % divided do$e$. Treat or weeC$


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+1

&. e$pon$e!a. ;& rai$e$ &y . g 6 day&. eticount increa$e$

0. lood transusion:a. arely nece$$ary&. Precipitate$ cc

c. Give 2#% l6Cg pacCed cell$/8. Preention:a. 8ron orti=ed orula&. educe ilC intaCe to "" l6dayc. 8ron rich ood!

a. -egetarian! leay vegeta&le$: cereal$d. Non vegetarian$!

a. liver? eat? Cidney etce. 3void iron chelator$

a. Tea? coIee? phytate$ in green leave$

Malariauestions:

/. Descri


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+2

Malaria i$ an acute and chronic illne$$ characteri9ed &y paroHy$$ o ever? chill$?$weat$? atigue? aneia and $plenoegaly.

0. Etiology:

7au$ed &y intracellular para$ite pla$odiu proto9oa: ' $pecie$!1. ,alciparu2. Malariae

%. vale'. -ivaH2. Trans"itted


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+%

. 7yto adherence o inected erythrocyte$ in capillaryendotheliu and va$cular o&$truction due to alciaru $p withleaCage o &lood? Juid and protein$ and re$ult in ti$$uehypoHia.

+. ;ypoglycaeia lactic acido$i$ due to anaero&ic eta&oli$/. No $uWcient iunity ater inection and hence re inection$

can occur. 7uulative eIect$ o a&ove actor$ lead to ulti organ ailure*. ;& , re$i$t p.alciparu: hence new&orn $how$ re$i$tance to

alaria1".Severe di$ea$e in pregnancy and inancy

8. ;esistance to "alaria:

c. ;& $ re$i$t$ alarial inection in generald. acC o &lood group duIy re$i$t$ p.vivaHe. ;&. and ovalocyte$ re$i$t p.alciparu. New&orn i$ re$i$tant due to h&. and aternal anti&odie$.g. % to yr$! lacC o iunity and develop $evere di$ea$eh. Severe di$ea$e in pregnancy

9. linical eatures:

i. Incu


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v. ;epato$lenoegalyvi. 7onvul$ion$vii. ;ypoglycaeiaviii. ;yperCaleiaiH. 3cido$i$H. Dehydration

. ;ecrudescence! relap$e o ever due to dorant para$ite$

a. ,ro liver! p. -ivaH and p.ovale&. ,ro 57! p.alariae

vi. P.alciparu eature$!a. 8nten$e para$iteia K +" (other$ only 2) due to inva$ion o &oth

ature and iature 57$:&. ;igh atality

vii. P.vivale!a. 7an cau$e death due to $plenic rupture&. elap$e ro hepatic pha$e ro + to yr$

viii. P.alariae!

a. ow para$iteia&. 8nect$ only ature 57$c. Milde$t or o alariad. 7hronic inectione. ecrude$cence even ater %" yr$

iH. P.ovale!a. ea$t coon&. 3cute and chronicc. 7an occur in con@unction with P.alciparu

/?. ongenital "alaria:

1. 3&ortion2. Still &irth%. Preaturity and 80G'. Neonatal death. 3Iected N5 ay $how ever? pallor? @aundice? and hepato$plenoegaly.

//. ere


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iii. 7oa per$i$t$ ore than %" inute$ ater a convul$ioni. Deep &reathing due to acido$i$. 7old? clay $Cin due to $hocC

i. pi$thotonu$ po$ture? iicCing either tetanu$ or eningiti$.ii. Neurological $ign$!

a. Syetrical upper otor neuron and &rain $te di$tur&ance$

&. Dicon@ugate ga9e? decere&rate and decorticate po$ture$.c. 0naro$a&le coad. 7orneal reJeH and doll$ eye oveent$ ay &e a&$ent.e. etinal haeorrhage$ and eHudate$ are rarer than in adult$.. 7S, eHaination in cere&ral alaria i$ u$ually noral:g. 8ncrea$e in pre$$ure? protein level and cell#count (o$tly

lyphocyte$? "cell$6l) ay &e $een.2. At%er eatures:

i. EHclu$ion o other cau$e$ o encephalopathy? i.e. -iral or &acterial.ii. Severe anaeia

iii. 3cute renal ailurei. Pulonary oedea or adult re$piratory di$tre$$ $yndroe (ard$).. ;ypoglycaeia

i. 7irculatory collap$e? $hocC? hypoten$ionii. Spontaneou$ &leeding6di$$einated intrava$cular coagulation.iii. ]aundice

/0. Diagnosis:

a. 3ll ever in endeic area$ i$ due to alaria unle$$ proved otherwi$e.&. P.alciparu! ring or$ with dou&le chroatic dot$: 57 with ore than

one para$ite$c. Micro$copy!

T%ic& s"ear 1. MaCe thicC $ear &y @oining the % drop$ o &lood and $preading it with

corner o another $lide. 7orrect thicCne$$ i$ attained when new$print i$&arely legi&le through the $ear.

2. 8t $hould &e 1" diaeter: 1" away ro the edge o the $lide:contain$ 1" layer$ o 57$ and 1" w&c$ $hould &e vi$i&le per oil ier$ion=eld o icro$cope.

T%in s"ear 1.


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3ter deheoglo&ini$ation &y treating with dw or 1" t$? dip the thicC$ear in @$& ii $tain two to three tie$.


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+/

Ta&. PriaBuin in $ingle do$e o "./ g6 Cg &w

%. eere and co"plicated "alaria and cere


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+

? heoglo&inuria? inten$e @aundice? anuria (pa$$age o le$$ than " illilitero urine in a day)? and =nally death in the a@ority o ca$e$.

+. The o$t pro&a&le eHplanation or &lacCwater ever i$ an autoiune reaction.

/. 5lacCwater ever i$ uch le$$ coon today than it wa$ &eore 1*". 8t ay&e that Buinine play$ a role in triggering the condition? and thi$ drug i$ no

longer coonly u$ed or alaria prophylaHi$.. The treatent i$ antialarial cheotherapy? intravenou$ Juid and

$oetie$ $upportive care $uch a$ inten$ive care and dialy$i$./0. To@icity o anti"alarial drugs:

a. uinine:

i. 7inchoni$.!1. Tinnitu$ (a hearing ipairent)? ra$he$? vertigo? nau$ea? voiting

and a&doinal pain are the o$t coon $ypto$.2. Neurological eIect$ are eHperienced in $oe ca$e$ due to the drug$

neurotoHic propertie$.

%. The$e action$ are ediated through the interaction$ o Buininecau$ing a decrea$e in the eHcita&ility o the otor neuron end plate$.'. Thi$ oten re$ult$ in unctional ipairent o the eight cranial nerve?

re$ulting in conu$ion? deliriu and coa.. uinine can cau$e hypoglycaeia through it$ action o $tiulating

in$ulin $ecretion: thi$ occur$ in therapeutic do$e$ and thereore it i$advi$ed that gluco$e level$ are onitored in all patient$ every 'L+ hour$. Thi$ eIect can &e eHaggerated in pregnancy and thereoreadditional care in adini$tering and onitoring the do$age i$e$$ential.

+. epeated or over#do$age can re$ult in renal ailure and death throughdepre$$iono the re$piratory $y$te.


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+*

c. ;yper$en$itivity reaction$ liCe ra$he$? =Hed drug eruption$? erytheaultiore o the $teven @ohn$on type? eHoliative deratiti$? $eru$icCne$$:

d. iver dy$unction a&noralitie$:e. 3noreHia? voiting. 3cute heolytic aneia can al$o occur.

g. The drug i$ contraindicated in patient$ with Cnown hyper$en$itivity to $ula?inant$ &elow 2 onth$ o age? patient$ with advanced renal di$ea$e and=r$t and la$t trie$ter$ o pregnancy.

e. Meouin: a. Nau$ea? voiting? a&doinal pain and di99ine$$ can occur in do$e$

eHceeding 1 g.&. e$$ reBuently it can cau$e nightare$? $leeping di$tur&ance$?

di99ine$$? ataHia? $inu$ &radycardia? $inu$ arrhythia? po$turalhypoten$ion?

c. 3cute &rain $yndroe con$i$ting o atigue? a$thenia? $ei9ure$ and

p$ycho$i$.d. MeJoBuine $hould &e u$ed with caution in patient$ with heart &locC?patient$ taCing &eta &locCer$? patient$ with hi$tory o epilep$y andp$ychiatric di$ea$e.

e. 8t $hould &e avoided in =r$t trie$ter o pregnancy and pregnancy$hould &e avoided within % onth$ o taCing the drug.

. ontraindications:

1. 8t $hould not &e u$ed or prophylaHi$ in pregnancy? particularlyduring the =r$t trie$ter.

2. 8t i$ contraindicated in patient$ with hi$tory o $ei9ure$? $evereneurop$ychiatric di$tur&ance$? or adver$e reaction$ to Buinolineantialarial$ liCe chloroBuine and Buinine.

%. 8t $hould not &e u$ed concoitantly with the$e drug$ orincrea$ed ri$C o cardiotoHicity and ri$C o convul$ion$.

'. MeJoBuine i$ reported to increa$e the ri$C o $ei9ure$ in patient$taCing valproate.

. 'rte"isinin deriaties !a. The o$t coon toHic eIect$ that have &een identi=ed are nau$ea?

voiting? anoreHia? and di99ine$$:&. Neutropenia? aneia? heoly$i$? and elevated level$ o liver en9ye$?

have &een noted rarely.

c. NeurotoHicity i$ the greate$t concern regarding artei$inin$? $ince theadini$tration o high do$e$ in la&oratory anial$ ha$ led to $evere andirrever$i&le change$ in the &rain.

d. 8n huan$? an epi$ode o ataHia wa$ reported ater treatent with oralarte$unate? and one ca$eLcontrol $tudy $howed hearing lo$$ ater theu$e o arteetherLlueantrine.

e. Treatent$ during the =r$t trie$ter? $howed $iilar level$ o congenitala&noralitie$? $till&irth$? and a&ortion$

. E&ryotoHic eIect$? which have &een deon$trated in anial$.


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g. Pri"auine:

a. 3t larger do$e$? it ay cau$e occa$ional epiga$tric di$tre$$ anda&doinal crap$.

&. Mild aneia? cyano$i$ and etheoglo&ineia ay al$o occur.c. Severe etheoglo&ineia can occur rarely in patient$ with de=ciency

o nadh etheoglo&in reducta$e.

d. Granulocytopenia and agranulocyto$i$ are rare coplication$.e. Patient$ with de=ciency o gluco$e +#pho$phate dehydrogena$e will

develop heolytic aneia on taCing u$ual do$e$ o priaBuine. 8 apatient i$ Cnown to &e $everely G+PD de=cient? then priaBuine $houldnot &e given.

ME'LE (;u


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%. al$o rarely on con@unctiva and vaginal uco$a'. EHanthe!

1. Sypto$ a&ate a$ ra$h appear$2. 5egin$ at #+ day$ ater ever%. ,ir$t $een around hairline and &ehind ear$'. Maculopapular. Spread$ downward$ to trunC and li&$+. ,inally conJuence and ade over / day$/. eave$ &rawny de$Buaation o $Cin

5.u


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(autop$y) $how$ ultinucleated giant cell$ lining the alveoli (air $ac$) o thelung$.

'. Po$t ea$le$ &acterial pneuonia$ e$p $taph.. 5ronchioliti$ o&literan$! rare and lie#threatening or o non#rever$i&le

o&$tructive lung di$ea$e in which the &ronchiole$ are plugged with granulationti$$ue. 7au$e$ death.

+. 7roup? tracheiti$ and &ronchioliti$./. Sinu$iti$ and a$toiditi$.. etropharyngeal a&$ce$$*. 3ctivation o P71".Ga$tro enteriti$ and dehydration11.3ppendiciti$ due to lyphoid hyperpla$ia12.,e&rile $ei9ure$ in %1%.Encephaliti$ 1 in 1"""1'.5lacC ea$le$! &lacC ea$le$? a rare? $evere? oten atal? or o ea$le$ in

which heorrhage into the $Cin le$ion$ and ucou$ e&rane$ i$ a$$ociatedwith a $udden ri$e in teperature? convul$ion$? deliriu? $tupor? coa? andarCed re$piratory di$tre$$. 7alled al$o heorrhagic ea$le$.

1.Myocarditi$1+.Prgnancy! lead$ to etal death? and neonatal ortality1/.Su&ute $clero$ing pan encephaliti$ (SSPE)

1. u


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2. ;ydration? antipyretic$? 2%. -entilator $upport'. -it 3 1#2 unit$. 8- ri&averin tried in iune de.

/0.Prognosis: death 1 in 1""": 1 in iune de./2.Preention:

1. Mea$le$ * onth2. MM in 1 onth$ (Vauti$)/3.Post e@posure:

1. Mea$le$ vaccine within /2 hr$2. 8M68- 8gG within + day$


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M6MPEtiology!

• Single $tranded N3: ,aily Para yHoviridae: Genu$ u&ula viru$• ;uan &eing$ are the only ho$t

Epideiology!

• 3ge! #* yr$: epideic at ' yr$ : $igni=cant reduction ater vaccination• 8nectivity! / day$ &eore and / ater parotid $welling appear$

Pathology!

• 7an involve Salivary gland$: 7NS: Te$te$: Thyroid: varie$: ;eart: Fidney$: iver:

]oint$.• 8nection epitheliu lyphnode vireia target ti$$ue$ necro$i$ with

lyphocytic in=ltration ocal i$cheia healing

7linical eature$!

• 8ncu&ation 12#2 day$• 3$yptoatic• Non $peci=c $ypto$• Typical illne$$!

o Prodroe! 1# 2 day$ o ever: headache and voiting:o 0nilateral or &ilateral (/") $welling o parotid$: tenderne$$: ear paino Parotid $welling!

3ngle o lower @aw o&$cured Ear lo&e lited up and out

Sten$en duct opening red and $wollen Su& andi&ular gland$ can &e involved yphatic o&$truction lead$ to edea over $ternu

o ,ever re$olve$ in % day$ $welling in / day$

Diagno$i$!1. 8ncrea$e in $eru ayla$e2. eucopenia%. elative lyphocyto$i$'. -iral i$olation. P7+. Paired $erology! ri$e in titre o acute and convale$cent $era o 8gG &y

a. 7oplient =Hation&. ;eagglutinationc. En9ye iuno a$$ay and E83d. 8g M &y E83 deon$trate$ recent inection

D.D!1. Parotid $welling occur$ in inJuen9a: 7M-: E.5 viru$: ;8-: $taph inection(poly

increa$ed)2. Sialidiniti$ due to calculu$


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%. S@ogren $yndroe! autoiune di$order in which iune cell$ attacC and de$troythe eHocrine gland$X2Y that produce tear$ and $aliva. The hallarC $ypto$ o thedi$order are dry outh and dry eye$ (part o what are Cnown a$ $icca $ypto$).

'. SE! a chronic autoiune connective ti$$ue di$ea$e that can aIect any part othe &ody.SE i$ one o $everal di$ea$e$ Cnown a$ the great iitator$ &ecau$e itoten iic$ or i$ i$taCen or other illne$$e$.

. Parotid tuour7oplication$!

1. Meningiti$2. Encephaliti$%. Gonadal atrophy'. are! optic neuriti$: nephriti$? pancreatiti$. Nerve deane$$+. Thro&ocytopenia/. Tran$ver$e yeliti$. Pregnancy! etal lo$$*. %" gonadal involveent: $terility i &ilateral: oopheriti$ can iic appendiciti$1".Pancreatiti$ dia&ete$11.Myocarditi$12.Thyroiditi$ yHaedea

Treatent!

• Non $peci=c: pain Ciller$ and re$t

Progno$i$!

• good rarely death due to encephaliti$

Prevention!

• MM at 1 and yr$: contra indicated in egg and neoycin allergy ? pregnancy

and ;8-

'cute !laccid Paralysis

http://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Immune_cellhttp://en.wikipedia.org/wiki/Exocrine_glandhttp://en.wikipedia.org/wiki/Exocrine_glandhttp://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome#cite_note-pmid18289371-1http://en.wikipedia.org/wiki/Tearshttp://en.wikipedia.org/wiki/Salivahttp://en.wikipedia.org/wiki/Dry_mouthhttp://en.wikipedia.org/wiki/Dry_eyeshttp://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Connective_tissue_diseasehttp://en.wikipedia.org/wiki/The_great_imitatorhttp://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Immune_cellhttp://en.wikipedia.org/wiki/Exocrine_glandhttp://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome#cite_note-pmid18289371-1http://en.wikipedia.org/wiki/Tearshttp://en.wikipedia.org/wiki/Salivahttp://en.wikipedia.org/wiki/Dry_mouthhttp://en.wikipedia.org/wiki/Dry_eyeshttp://en.wikipedia.org/wiki/Autoimmunityhttp://en.wikipedia.org/wiki/Connective_tissue_diseasehttp://en.wikipedia.org/wiki/The_great_imitator


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1. De=nition!1. ,laccid paraly$i$ occurring over hour$ or ew day$ with!

1. ;ypotonia2. ;yporeJeHia%. o$$ o power'.


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di$tal one li&Mu$cle tone educed or a&$ent

inaIected li&

Glo&alhypotonia

educed ora&$entin aIected li&

;ypotonia inlower i&$

Deep#tendon

reJeHe$

Decrea$ed toa&$ent

Glo&ally a&$ent Decrea$ed toa&$ent

3&$ent inlower

li&$ early:hyperreJeHialate

Sen$ation Severe yalgia?&acCache? no$en$orychange$

7rap$?tingling?hypoanae$the$ia opal$ and $ole$

Pain in gluteu$?hypotheria

3ne$the$ia olower li&$with$en$ory level

7ranialnerveinvolveent

nly when &ul&arinvolveent i$pre$ent

ten pre$ent?aIecting nerve$-88?8Z? Z? Z8? Z88

3&$ent 3&$ent

e$piratoryin$uWciency

nly when &ul&arinvolveent i$pre$ent

in $evere ca$e$?enhanced &y&acterialpneuonia

3&$ent Soetie$

3utonoic$ign$4 $ypto$

are ,reBuent&lood pre$$urealteration$?$weating?

&lu$hing and&odyteperatureJuctuation$

;ypotheria inaIected li&

Pre$ent

7ere&ro#$pinalJuid

8nJaatory 3l&uin#cytologicdi$$ociation

Noral noral or ildin.7ell$

5ladderdy$unction

3&$ent Tran$ient Never Pre$ent

Nerve

conductionvelocity!thirdweeC

3&noral! anterior

horn cell di$ea$e(noral during the=r$t 2 weeC$)

3&noral!

$lowedconduction?decrea$edotoraplitude$

3&noral! aHonal

daage

noral or

a&noral?nodiagno$ticvalue

EMG atthreeweeC$

3&noral Noral Noral Noral


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SeBuel atthreeonth$ andup toa year

Severe?a$yetricalatrophy? $Celetaldeoritie$developing later

Syetricalatrophy odi$talu$cle$

Moderateatrophy?only in aIectedlower li&

Jaccid diplegiaatrophy ateryear$

'. 8nve$tigation$!1. 7S,2. M8 $pine%. -iral i$olation! throat $wap: $tool$'. EMG. Nerve conduction $tudie$+. Elevation u$cle en9ye$+. 3,P $urveillence!

1. Non#polio 3,P rate in children [1 year$ o age. (Target K 161""?""")

2. eported 3,P ca$e$ inve$tigated ' hour$ o report (Target ")%. eported 3,P ca$e$ with 2 $tool $pecien$ collected 1' day$ $inceon$et. (Target ") 8 no ore than two onth$ have elap$ed $ince theon$et o paraly$i$? collect two $tool $pecien$ ro the patient with aninterval o 2'#' hour$ &etween collection$.

'. eported 3,P ca$e$ with a ollow#up eHa at lea$t +" day$ ater paraly$i$on$et to veriy the pre$ence o re$idual paraly$i$ or weaCne$$. (Target ")

. Specien$ arriving at national la&oratory % day$ o &eing $ent (Target *")

+. Specien$ with a turn#around tie 2 day$ (Target ").The turn#around tie i$ the tie &etween $pecien receipt and reporting ore$ult$

/. Stool $pecien$ ro which non#polio enteroviru$ wa$ i$olated (Target K1").

. 7la$$iy the 3,P ca$e$.*. USu$pected polioR or 3,P i$ a teporary cla$$i=cation which within twelve

weeC$ o paraly$i$ on$et? the eHpert coittee $hould recla$$iy the ca$ea$ con=red polio? polio#copati&le? or di$carded.

PALIAMHELITI

1. -iru$!/. Single $tranded N3: ,aily! Picarnoviridae: genu$!Enteroviru$0. Type 1?2?% are antigenically diIerent2. ;uan$ are the only ho$t3. P-1 i$ highly locali9ed to region$ in 8ndia? PaCi$tan? 3ghani$tan? and

Egypt?


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/*

2. Tran$i$$ion!/. EHcreted ro G8T and tran$itted &y eco oral ethod and di$ea$e occur$

in 7NS%. Epideiology!

/. *"#* $u&clinical or a&ortive0. non paralytic illne$$2. 1in 1""" paralytic polio in inant$: 1 in 1"" in adole$cent$.3. Poor $anitation and overcrowding predi$po$e$ tran$i$$ion4. 8nectivity! 2 weeC$ &eore and $everal weeC$ ater the on$et o illne$$.

'. Pathogene$i$!/. Enter cell$ with $peci=c polio receptor$ in G8T epitheliu regional

lyphadenopathy piary vireia ES 4 7NS through nerve ending$

$pinal otor neuron$ ultiply elea$e &y cell death0. -accine viru$ $iilar cour$e &ut no replication in 7NS2. -accine viru$ ater acBuiring neurovirulance can produce $iilar illne$$

(-3PP). Pathology!

/. Motor neuron$ o $pinal cord invaded: poly and lyphocytic in=ltration and

inJaation: edea: ore neuronal lo$$ due to inJaatory edea.0. 3nterior horn cell? internuncial neuron$ and dor$al ganglion can &e invoveld:2. -eri$ o cere&ellu? $u&$tantia nigra? red nucleu$ o pon$? thalau$?

hypothalau$? pallidu and otor corteH can &e involved to varia&leeHtent.

+. 8unity!/. 8nant$ "#' onth$ have tran$placental 8g.G0. Type $peci=c iunity &y natural and vaccine viru$ through 8gG2. G8T get$ 8g3 or $urace iunity.

/. 8ncu&ation!/. #12 day$ or longer.

. 7linical 7our$e!/. 3&ortive!1. Mild inJuen9a liCe illne$$: ever? alice? head ache and

anoreHia.2. ecovery in 2#% day$.

0. Non Paralytic!1. Minor illne$$! a$ a&ove2. Ma@or illne$$!

/. NecC $tiIne$$0. StiIne$$ o u$cle$ o trunC and li&$# $pinal rigidity2. ,leeting &ladder paraly$i$3. 7on$tipation

4. o$$ o head control5. Depre$$ed tendon reJeHe$7. o$$ o a&doinal and crea$tric reJeHe$8. No $en$ory di$tur&ance$

2. Paralytic Polio! i in 1"" ca$e$1. Spinal2. 5ul&ar%. Spino &ul&ar'. Polio encephaliti$

3. Spinal!


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1. Pha$e 8! Siilar to a&ortive polio2. Pha$e 88!

/. 3pparent recovery ro pha$e 8 ollowed &y0. ,ever? head ache? u$cle pain? pare$the$ia$? u$cle $pa$2. 3$yetrical and $potty paraly$i$3. ProHial ore than di$tal u$cle group$4. Diaphrag and interco$tal$ paraly$i$5. educed DT7. 5ladder and &owel dy$unction8. Provocation paraly$i$ ollowing 8M [email protected]. Sen$ation i$ intact/?. ecovery +#12 onth$

4. 5ul&ar!1. May occur alone2. Na$al voice due to palatal pal$y%. Pooling o $aliva'. 8rregular re$piration. Diini$hed cough reJeH+. Deviation o uvula/. 8ncrea$e in 5P. ;yperpyreHia*. 7ardiac arrhythia$1". SCin ottling due to va$ootor in$ta&ility11. 3phonia due to vocal cord paraly$i$12.


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1

1. -3PP2. Guillain 5arre Syndroe! $yetric: a$cending with $en$ory

involveent%. Tran$ver$e yeliti$'. Trauatic paraly$i$

11. Treatent!/. Syptoatic0. Pain Ciller$2. 5ed re$t3. No 8 in@ and $urgery: ton$illectoy can lead to &ul&ar polio4. ;ot tu& &ath$5. Splinting in neutral po$ition$7. Ph$iotherapy ater pain $u&$ide$8. 3ir way aintenance9. Tracheo$toy/?. -entilatory $upport

12.Prevention!/. The =r$t inactivated viru$ vaccine wa$ developed in 1*2 &y ]ona$ SalC? and

announced to the world on 3pril 12? 1*.X'/Y The SalC vaccine? orinactivated polioviru$ vaccine (8P-)? i$ &a$ed on polioviru$ grown in a typeo onCey Cidney ti$$ue culture (-ero cell line)? which i$ cheicallyinactivated with oralin.X1Y 3ter two do$e$ o 8P- (given &y in@ection)? *"or ore o individual$ develop protective anti&ody to all three $erotype$ opolioviru$? and at lea$t ** are iune to polioviru$ ollowing three do$e$.X'Y

0. Su&$eBuently? 3l&ert Sa&in developed another live? oral polio vaccine (P-).8t wa$ produced &y the repeated pa$$age o the viru$ through non#huancell$ at $uphy$iological teperature$. Three do$e$ o live#attenuated P-

produce protective anti&ody to all three polioviru$ type$ in ore than *o recipient$.

1%.Eradication!1. ;igh inant iuni9ation coverage with our do$e$ o oral polio vaccine (P-)

in the =r$t year o lie in developing and endeic countrie$? and routineiuni9ation with P- and6or 8P- el$ewhere.

2. rgani9ation o UNational iuni9ation day$R to provide $uppleentary do$e$o oral polio vaccine to all children le$$ than =ve year$ o age.

%. 3ctive $urveillance or wild polioviru$ through reporting and la&oratory te$tingo all ca$e$ o acute Jaccid paraly$i$X/Y aong children le$$ than =teen year$ o age.

'. Targeted op#up capaign$ once wild polioviru$ tran$i$$ion i$ liited to a$peci=c ocal area.

. Pulse Polio i$ an iuni9ation capaign e$ta&li$hed &y the governent o8ndia in 1**' to eradicate polioyeliti$ (polio) in 8ndia &y vaccinating annuallyall children under age =ve again$t polioviru$.

Every child receive$ a do$e o ral Polio -accine (P-)? a live? attenuatedviru$ which coloni$e$ the ga$trointe$tinal tract. Thi$ viru$ copetitively inhi&it$the wild? di$ea$e#cau$ing polioviru$. Not only doe$ thi$ prevent perniciou$inection in the ho$t? it preclude$ tran$i$$ion o the wild polioviru$ to other

http://en.wikipedia.org/wiki/Jonas_Salkhttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-Spice-46http://en.wikipedia.org/wiki/Tissue_culturehttp://en.wikipedia.org/wiki/Vero_cellhttp://en.wikipedia.org/wiki/Cell_culturehttp://en.wikipedia.org/wiki/Formalinhttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-Kew_2005-14http://en.wikipedia.org/wiki/Injection_(medicine)http://en.wikipedia.org/wiki/Serotypehttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-PinkBook-3http://en.wikipedia.org/wiki/Albert_Sabinhttp://en.wikipedia.org/wiki/Physiologicalhttp://en.wikipedia.org/wiki/Polio_vaccinehttp://en.wikipedia.org/wiki/Poliomyelitis_eradication#cite_note-6http://en.wikipedia.org/wiki/Immunizationhttp://en.wikipedia.org/wiki/Government_of_Indiahttp://en.wikipedia.org/wiki/Government_of_Indiahttp://en.wikipedia.org/wiki/Poliomyelitishttp://en.wikipedia.org/wiki/Indiahttp://en.wikipedia.org/wiki/Vaccinationhttp://en.wikipedia.org/wiki/Poliomyelitishttp://en.wikipedia.org/wiki/Gastrointestinal_tracthttp://en.wikipedia.org/wiki/Host_(biology)http://en.wikipedia.org/wiki/Jonas_Salkhttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-Spice-46http://en.wikipedia.org/wiki/Tissue_culturehttp://en.wikipedia.org/wiki/Vero_cellhttp://en.wikipedia.org/wiki/Cell_culturehttp://en.wikipedia.org/wiki/Formalinhttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-Kew_2005-14http://en.wikipedia.org/wiki/Injection_(medicine)http://en.wikipedia.org/wiki/Serotypehttp://en.wikipedia.org/wiki/Poliomyelitis#cite_note-PinkBook-3http://en.wikipedia.org/wiki/Albert_Sabinhttp://en.wikipedia.org/wiki/Physiologicalhttp://en.wikipedia.org/wiki/Polio_vaccinehttp://en.wikipedia.org/wiki/Poliomyelitis_eradication#cite_note-6http://en.wikipedia.org/wiki/Immunizationhttp://en.wikipedia.org/wiki/Government_of_Indiahttp://en.wikipedia.org/wiki/Government_of_Indiahttp://en.wikipedia.org/wiki/Poliomyelitishttp://en.wikipedia.org/wiki/Indiahttp://en.wikipedia.org/wiki/Vaccinationhttp://en.wikipedia.org/wiki/Poliomyelitishttp://en.wikipedia.org/wiki/Gastrointestinal_tracthttp://en.wikipedia.org/wiki/Host_(biology)


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ho$t$. Since polioviru$ cannot $urvive out$ide a ho$t or ore than two weeC$?theoretically it would &e eradicated? re$ulting in the eradication opolioyeliti$.

TET'N6uestions:

1. De$cri&e the clinical eature$? coplication$? prevention and anageent otetanu$. Nov 2""1

2. S.N! Neonatal tetanu$. ,e& 2""/Defnition:

Tetanu$ i$ an acute? $pa$tic paralytic illne$$ cau$ed &y the neurotoHin o

7lo$tridiu tetaniacteriology:

• 7lo$tridiu tetani i$ a otile? gra po$itive? $pore#oring? o&ligate anaero&e:dru $ticC appearance in icro$copy.

• 0&iBuitou$ and ound in $oil? du$t? alientary tract o anial$.�

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