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HEMOPHILIA

Created by : Muhanmmad FahryzalFK UNISSULA 2012

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DEFINITION & TERMINOLOGY

Hemophla !heem"o"FILL"ee"ah# $ a rarebleedn% d$order n &h'h the blood doe$n(t'lot normally)http:**&&&)nhlb)nh)%o+*health*health"top'$*top'$*hemophla*

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DEFINITION CONT.

Hemophla $ a %enet' d$order,u$uallynherted,o- the me'han$m o- blood'lottn%) .ependn% on the de%ree o- the

d$order pre$ent n an nd+dual/ e'e$$bleedn% may o''ur only a-ter $pe'-'/pred'table e+ent$ !$u'h a$ $ur%ery/ dental

pro'edure$/ or nury#/ or o''ur$pontaneou$ly/ &th no no&n ntatn% e+ent

http:**med'al"d'tonary)the-reed'tonary)'om*hemophla

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DEFINITION CONT.

Haemophilia !*h m - l *4 al$oː əˈ ɪ ə$pelled hemophilia n North Amer'a/ -romthe 5reehaima 6 76 (blood( andἷ  philia 89;6

(lo+e(<1=

# $ a %roup o- heredtary %enet'd$order$ that mpar the body($ ablty to'ontrol blood 'lottn% or 'oa%ulaton/ &h'h$ u$ed to $top bleedn% &hen a blood

+e$$el $ broen)http$:**en)&peda)or%*&*Haemophla

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CAUSE & EFFECT  - you ha+e nherted hemophla/ you(re born

&th the d$order) It($ 'au$ed by a de-e't none o- the %ene$ that determne ho& the

body mae$ blood 'lottn% -a'tor >III or I?)@he$e %ene$ are lo'ated on the ?'hromo$ome$ !KB"muh"$omz#)

  Chromo$ome$ 'ome n par$) Female$ ha+e

t&o ? 'hromo$ome$/ &hle male$ ha+e one ?and one 'hromo$ome) Bnly the ?'hromo$ome 'arre$ the %ene$ related to'lottn% -a'tor$)

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CLASSIFICATION

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HEMOPHILIA A

Haemophilia A $ a re'e$$+e ?"lned %enet'd$order n+ol+n% a la' o- -un'tonal'lottn% Fa'tor >III and repre$ent$ D0E o-

haemophla 'a$e$)

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HEMOPHILIA B

Haemophilia B $ a re'e$$+e ?"lned %enet'd$order n+ol+n% a la' o- -un'tonal'lottn% Fa'tor I?) It 'ompr$e$

appromately 20E o- haemophla 'a$e$

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HEMOPHILIA C

Haemophilia C $ an auto$omal %enet'd$order !)e) not ?"lned# n+ol+n% a la' o--un'tonal 'lottn% Fa'tor ?I) Haemophla C

$ not 'ompletely re'e$$+e/a$ heterozy%ou$ nd+dual$ al$o $ho&n'rea$ed bleedn%)

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SIGN & SYMPTHOM

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EXCESSIE BLEEDING

S%n$ o- eternal bleedn% may n'lude:

leedn% n the mouth -rom a 'ut or bte or -rom'uttn% or lo$n% a tooth

No$ebleed$ -or no ob+ou$ rea$on

Hea+y bleedn% -rom a mnor 'ut

leedn% -rom a 'ut that re$ume$ a-ter $toppn%-or a $hort tme

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CONT.

S%n$ o- nternal bleedn% may n'lude:

lood n the urne !-rom bleedn% n the dney$or bladder#

lood n the $tool !-rom bleedn% n thente$tne$ or $toma'h#

Lar%e bru$e$ !-rom bleedn% nto the lar%emu$'le$ o- the body#

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BLEEDING IN THE !OINTS

leedn% n the nee$/ elbo&$/ or other ont$$ another 'ommon -orm o- nternal bleedn%n people &ho ha+e hemophla) @h$bleedn% 'an o''ur &thout ob+ou$ nury)

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BLEEDING IN THE BRAIN  Lon%"la$tn%/ pan-ul heada'he$ or ne' pan

or $t--ne$$

  epeated +omtn%

  Sleepne$$ or 'han%e$ n beha+or  Sudden &eane$$ or 'lum$ne$$ o- the arm$

or le%$ or problem$ &aln%

  .ouble +$on

  Con+ul$on$ or $ezure$

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PATHOPHYSIOLOGY  Fr$t/ the blood +e$$el 'on$tr't$ to lmt the

+olume o- blood that $ lo$t)

  Se'ond/ 'r'ulatn% platelet$ -orm a plu% at

the $te o- nury)  Fnally/ the blood under%oe$ 'oa%ulaton)

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TREATMENT  ou or your 'hld 'an %et Gu'er treatment

&hen bleedn% happen$) arly treatmentlo&er$ the r$ o- 'ompl'aton$)

 

Fe&er +$t$ to the do'tor or emer%en'yroom are needed)

  Home treatment 'o$t$ le$$ than treatment na med'al 'are $ettn%)

  Home treatment help$ 'hldren a''epttreatment and tae re$pon$blty -or thero&n health)

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PREENTION & EDUCATION  eople &ho ha+e hemophla $hould a+od

$tuaton$ that m%ht 'au$e bleedn%) @hey$hould be 'are-ul about dental 'are $o they&on(t need to ha+e teeth etra'ted) eople &ho

ha+e hemophla $hould al$o a+od 'ertan dru%$that 'an a%%ra+ate bleedn% problem$: A$prn

Heparn

Jar-arn  Certan anal%e$'$/ $u'h a$ non$terodal ant"

n-lammatory dru%$ !e%/ @ylenol and Ibupro-en #

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CONT.  5enet' 'oun$elor$ 'an mae a detaled

-amly h$tory) @hey &ll d$'u$$ r$$ anda+alable te$tn% opton$)

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PUSTA"A LIST  H$tory o- leedn% .$order$) Jhat Are leedn%

.$order$) 200) Natonal Hemophla Foundaton) 1 .e'200D

   %%$ / .ou%la$ AS/ Ma'Farlane 5/ .a'e O>/ tney J/Mer$ey C/ B(ren O !1PQ2#) Chr$tma$ d$ea$e: a'ondton pre+ou$ly m$taen -or haemophla) Br Med

 J # !RPP#: 1DTD2) do:10)11*bm)2)RPP)1D) MC 20220) MI. 12PPP0)

  $ M'hael r'e !D B'tober 200P#) Ca$e Clo$ed: Famou$oyal$ Su--ered From Hemophla) ScienceNOW Daily News)AAAS) etre+ed P B'tober 200P)

  $ +%eny I) o%ae+ et al) !D B'tober 200P#) 5enotypeAnaly$$ Ident-e$ the Cau$e o- the oyal .$ea$e)S'en'e) etre+ed P B'tober 200P)