hemophilia pediatric-cases phtdi makassar 2014

8/10/2019 Hemophilia Pediatric-Cases PHTDI Makassar 2014

1/51

PEDIATRIC CASE REPORTS

Stefanus Gunawan

Hemophilia Treatment Center

RD Kandou Hospital

Faculty of Medicine Sam Ratulangi Uniersity


2/51

CASE 1

!"# Minahasan# male# $ months

Referred from surgeon

%rolonged &leeding ' %ost(op cleft(lip

)


3/51

PRIOR DATA (PRE-OP)

H& *$+* g,dl

Hct $-+. /

01C 2+344,mm$

%lt 52.+444,mm

1T .6 789$(*46 :

CT ;6 7893(*46 :

$


4/51

FURTHER EXAMINATION 8o history of &leeding

8o family history

3


5/51

PHYSICAL FINDINGS

Compos mentis

-+) ,m Temperature

$;+;?C

@nemic 7(:

Upper "ip9

&leeding from

surgical site Thora> A

@&domen9 no

a&normality

S


6/51

LAB FINDINGS

@fter consultation9

H& **+* g,dl

Hct $3+. /

01C -.44,mm$

%lt 33;#444,mm$

%T *)B 7*4+2(*3+3:= 8R *+4)

aPTT 65 7);+3($-+;: FVIII 2!"

;


7/51

A##$##%$&'

Mild(&leeding post(

op la&ioschiEis e+c

Haemophilia @7moderate:

T$a'%$&'

Factor 9 )4

U,amic acid54mg,


8/51

FOLLO* UP 8o more &leeding after ) days

Discharged

2


9/51

COST ANALYSIS INJKN*

Bleeding type

F VIII PASIEN

FVIII cost

Tariff (INA-CBG+Top p!

B class

"ospital #eg-$

Balance

Targetdesiredle%el

(&!

'eigt()g!

* Fa)torVIII

needed

(I!

Surgery (Minor)

Bleeding

Dy 1-2 , ./0 12 0313 43,,,3$ 7,944,300#

(e5cl/ oter 6edicines7 ser%ice!

.


10/51

LEARNING POINT

E+$,'.$ #/0$

Comprehensie medical history including

family history should &e ta


11/51

CASE 2 "S# male# Chinese# 5 years

Diagnosed as hemophilia from * yr 7F U:%rolonged wound healing

Hemophilia @ 7moderate: ' phimosis 8eed to circumsiEed

**


12/51

PEDIGREE

*)


13/51

PHYSICAL FINDINGS

Compos mentis

). ,m Temperature $;?C

@nemic 7(:

Thora> A @&domen

no a&normality

detected I>tremity no

hemarthrosis

Genital9 phimosis

*$


14/51

LABORATORY FINDINGS

H& *$+* g,dl

Hct $-+. /

01C 2344,mm$

%lt 52.#444,mm$

%T*)+.B

7*4+2(*3+3:

8R *+4)

a%TT ;;+- 7);+3($-+;:

Factor )/

*3


15/51

A##$##%$&' Hemophilia @ 7moderate: 'phimosis# pro circumcision

P+a&&&0 @docation to &oard of director

F replacement pre(post circumcision

*5


16/51

FOLLO* UP 3FVIII REPLACEMENT SCHEME

(* hr9 F -54 U 7)5 U,Kg&w:

4 hr9 circumcision done

'* hr9 no &leeding

F -54 U,*) hour 7)5 U,Kg&w:Trane>amic acid $ > 544 mg

@mo>icillin $ > 544 mg %+J

0ound care

'5 hr94+$$&07ooEing:R,F -54 U 7)5U,Kg&w:

*;


17/51

FOLLO* UP 3FVIII REPLACEMENT SCHEME

Day '*

B+$$&0 ()

F 54 U,


18/51

LEARNING POINT 0FH %rotocol for circumcision

@lternatie protocols

*2


19/51

CIRCUMCISION IN HEMOPHILIA*FH277!

*) hrs prior tosurgery

Jral trane>amicacid )5($4

mg,


20/51

2 A *; hrs aftersurgery9

F *4 U,


21/51

SEVERE 3 MODERATE CASES

Day 3(-9 seere9 F $4 U,


22/51

MILD CASES

Day 39 F *5 U,


23/51

*FH GUIDELINESON MINOR SURGERY (2712)

SUGGESTED PLASMA FACTOR PEA8 LEVEL AND DURATION OF ADMINISTRATION(*HEN THERE IS SIGNIFICANT RESOURCE CONSTRAINT)

Hemophilia @ Hemophilia 1

Desired leel7U,d":

Duration 7days: Desired leel7U,d":

Duration 7days:

%re(op 34(24 34(24

%ost(op )4(54 *(59

)4(54 *(59

)$

9depends on type of procedure


24/51

ALTERNATIVE PROTOCOLS

I:% ;


25/51

)3 h &efore circumcision9 Jral

trane>amic acid *5 mg,?0 Repeat same dose at *) h after

surgery# a 2-17# a8afil H# et al+ Med Sante Trop+ )4*$=)$9***()+

)5


26/51

) doses of F concentrate 7)5

U,


27/51

Inhancing local hemostasis9 risGA@AHMADA UNIVERSITY

)2

PROTOKOL PEMBERIAN AHF PADA OPERASI SIRKUMSISIPADA PENDERITA HEMOFILIA A

Nama # MR Umur....thn/bln BB.kg

Kadar factor VIII =.% Inhibitor (tgl pmri!"aan

Hari H 0 H +1 H +2 H +3 H +4 H + H +!

Ta"gga#

Jam - 30mnt

O$%ra&i + 12 + 24 + 36 + 48 + 60 + 72 + 84

Tra"%'a(i) a)i*,

iv, bolus

15 mg/kg=...........mg

AHFiv bolus

25 U/kg=.....UPROTOKOL DAPAT DIUBAH SESUAI KONDISI PERDARAHAN

S,$%r-i&r Dk/%r a"g (%raa/

ika (a&i $%r*araa"


29/51


Bleeding type

P'"

FVIII total cost

Tariff (INA-

CBG+Top p!

B class "ospital#eg-$

8eficit

'eigt()g!

* Fa)tor

VIIIneeded9kg

(I!

Circumciion

!" 2012(minor urgery)

S$r%i&o'M

*$&ien&

$40(-0:!

001

0

4.313

$$323

0;3;3

4:3;1:32

-;3:,3,

).


30/51

*FH RECOMMENDATIONSCIRCUMCISION OF P*H

Should not &e considered as a minor procedure

Should not &e performed without taamic acid# DD@%# analgesics#

anti&iotics# etc:

Generally ris< of postoperatie &leeding in %0H *5()4/

$4


31/51

CASE ! D!# male# *4 yrs#Gorontaloneese

Refered from @mpana Hospital Central

Sulawesi9 )3 hrs &y land

Chief complaint9 unconscious

$*


32/51

HISTORY

Head trauma Hit &ed(handle

omit 0ea


33/51

PEDIGREE

$$


34/51

PHYSICAL FINDINGS

Seere ill# GCS . 7I)M;*:

$4 Kg= *$*cm good nutrition

1% **4,-4 mmHg

%ulse *)3 &pm# regular Respiratory )3 >,m# thoracoa&d

Temperature $2+)?C

$3


35/51

PHYSICAL FINDINGS

@nemic 7(:# icteric 7(:

%upil round# isochors# $($ mm# refle> ','

T9 ',' Spastic 7(:# clonus 7(:

$5

1 5

* 5


36/51

LABORATORY FINDINGS

H& *$+* g,dl

Hct $2+) /

01C *)#.44,mm$

%lt 5;4#444,mm$

%T *)+$ B7*4+2(*3+3:= 8R 4+.-

a%TT $*+4 B7);+3($-+;:

Factor */

$;


37/51

$-

I&'a,$$4a+ a$%


38/51

@ssessment9 CH ' seere hemophilia @ '

susp+ &acterial infection Treatment9

J>ygen *",m

8aCl 4+35/ in D5/ 54ml,m

F ) > *544U 754 U,amid acid $ > -54 mg#

Furosemid * > )4 mg#

Mannitol -5 cc in $46$>,dy

Cefota>ime $ > * gr# %aracetamol $ >544 mg prn#

Consult neurosurgery9 Ilectie trepanation

$2


39/51


40/51


41/51


Bleeding type

F VIII PASIEN

FVIII total cost

Tariff (INA-CBG+Top p!

B class

"ospital #eg-$

8eficitTarget

desired

le%el(&!

'eigt

()g!

* Fa)tor

VIII

needed(I!

C+S Bleeding

Dy 1-3

Dy 4-7

Dy -14

1-;$-1

0-,

$ 4:3; :1/,/ 4:/;1:/2 -.1/4;/,

3*


42/51

LEARNING POINTS @pproach %0H with suspect of CH

0FH Guidelines for CH

3)


43/51

8agel K# et al+ 1lood Coagul Fi&rinolysis )4*)=)39)$ N )-+

APPROACH TO SUSPECT ICH

3$


44/51

*FH GUIDELINESON CNS BLEEEDING (2712)

SUGGESTED PLASMA FACTOR PEA8 LEVEL AND DURATION OF ADMINISTRATION(*HEN THERE IS NO SIGNIFICANT RESOURCE CONSTRAINT)


Desired leel7U,d":


Duration 7days:

nitial 24(*44 *(- ;4(24 *(-

Maintenance 54 2()* $4 2()*

33


45/51

*FH GUIDELINESON CNS BLEEDING (2712)

SUGGESTED PLASMA FACTOR PEA8 LEVEL AND DURATION OF ADMINISTRATION(*HEN THERE IS SIGNIFICANT RESOURCE CONSTRAINT)


Desired leel7U,d":


Duration 7days:

nitial 54(24 *($ 54(24 *($

Maintenance $4(54 3(- $4(54 3(-

)4(34 2(*3 )4(34 2(*3

35


46/51

Cermelj M et al.Haemophilia. 2004;10:405-

!u "# et al. $r # %euro&ur'. 200;2:455-

8eurosurgery9Failure of conseratie(replacement

therapy

Seere(life threatening CH

3;


47/51

$ Klinge et al9

$ -33 children with hemophilia$4 episodes of

CH9 5-/ due to head trauma$ Haemophilia 200*;14:52-5

Mortality due to CH in %0H9

$ *.;4(*..*9 ).+)/

$ )44*9 *2+)/$ )44$9 2+;/

+e ,eano&-into. Haemo&ta&i&.12;22:25-/

%u&&. m # Hematol.2001;/*:-42

ntune&. Haemophilia.200;:5-

3-


48/51

*FH RECOMMENDATIONSICH IN P*H meial emer'en3. ,reat ir&t

beore ealuatin' @ll post(traumatic head inOuries#

confirmed or suspected# A

significant headaches must &e

treated as intracranial &leeds Do not wait for further symptoms

to deelop or for la&oratory or

radiologic ealuation 32


49/51

6mmeiatel3 raise the patient6s factor

leel when significant trauma or early

symptoms occur

Further doses will depend on imagingresults

Maintain factor leel until etiology is

defined f a &leed is confirmed# maintain the

appropriate factor leel for *4(*3 days

7"eel 3:

3.


50/51

CT scan or MR of the &rain should

&e performed

8eurological consultation should &e

sought early 7"eel 3:

CH may &e indication for prolonged

)nd(prophyla>is 7$(; mo:# especially

where high ris< of recurrenceo&sered 7e+g+ H ': 7"eel $:

54


51/51

THAN8 YOU

hemophilia pediatric-cases phtdi makassar 2014

Documents