chronic granulomatous disease report on a.3

8/17/2019 Chronic Granulomatous Disease Report on a.3

1/15

0025-7974/00/7903--0!55/0

MEoicora-79:155-69, 2000Co pyright Q 2000 by Lippincott iiarn" & i#n", !n$%

Vol. 79, No. 3 Prisued in U.S.A.

Chronic Granulomatous Disease

Report on a National Registry of 368

Patients

&E''( A )*+ELE)*, M.'( C% M.')*, ' )C.' % &* &'%, &* (LE, &rn C'*E,&* l. .LL)*, .' '( L% M.LEC, EE* M% LL.*, .* 8C, .L )E,

' EECC. % C+LE(, C.'LE % E', E;E &% C.*C+, .* .' )C+LE'

Introductio

n

Chronic gr an

hy#rog$n p$roBi#$% . "% hr $$ oth$r ?or" o? th$#i"$a"$ ar $ #,


2/15

156 )*+ELE)* E .L 2C' *)C '.*LM. )E.E 'E)'(

pag$ G a patD$nt" ha# or$ than 1 a bnora t$"t%

oth$r "ho@$# 2 #D"tDnct pop


3/15

.LE 2% $ographic char act$ri"tic" 5o? pati$nt" !ith C

$ogra phic "#$ .' nno@n ota

Total pati$nt" 259 A1 2A 36A %

$a$ 2P 50 o 52&l

'

."ian 3 3 o 6 (oMiB$# 2 3 o 5 >

- c::& Totalc::& " llnked re)essl*e

- A+tosoal re)essi*e

..

lr,

*ati$ .$rican o 1 o 1 "$:

l-.bbr $iation": JL' Q J-in $# r $c$""i$ .' Q .


4/15

15A )*+ELE)* E .L 4C' *)C '.*LM. )E.E 'E)'(

.LE 5% r$a$nc$ o? in?$ction by "it$ in C

JL' .' nno@n otayp$ o? )n? $ction =nQ259> =nQA1> =nQ2A> =nQ36A>

*o%=O>P *o%=O>P *o%=O>P *o%=O>P

n$ 62 =77O> 21 =75O> 290 =79O>

. b"c$"" =any in#> 175 =6AO> 57 =70O> 1A =64O> 250 =6AO>U 26 =32O> 15 =56O> 194 =53O>"t$oy$iti" 69 =27O> 17 =21O> 4 =14O> 90 =25O>act$r $ia/?t A =10O> 3 =11O> 65 =1AO>C$ 4=5O> 1 =4O> 15 =4O>th$rH 70 =27O> 33 =41O> 9 =32O> 112 =30O>

. bbr $iation": JL' Q J-in $# r $c$""i$L .' Q a 6 =29O> 120 =41O>#taphylococcus" pp%23 =11O> A =13O> 3 =14O> 34 =12O> 'ur ( hol deria cepacia15 =7O> 7 =11O> 2 =10O> 24 =AO>

)ocardia "pp% 13 =6O> A =13O> o 21 =7O> *yco+act erial " p p% 7=3O> 2 =3O> 3 =14O> 12 =4O>

.typica ycobact$ria 4=2O> o 1 =5O> 5 =2O># erratia "pp% 9=4O> 3 =5O> 2 =10O> 14 =5O> le+ siella " pp% 7=3O> o o 7=3O>

P seudomonas"pp% 5=3O> 1 =2O> 1 =5O> 7 =3O>and ida " p p% 5=2O> o o 5=2O> P aecilom yces" p p% 2 =1O> 2 =3O> o 4 =1O>th$r V 35 =17O> 10 =16O> 2 =10O> 47 =17O>

. bbr $iation": $$ pr $io


5/15

15A )*+ELE)* E .L 5C' *)C '.*LM. )E.E 'E)'( per g i Uus =ab$ 6>% In ?act, abo =nQ57> =nQA> =nQ250>

o%=O>P *o%=O>P *o%=O>P *o%=O>P

7 =21O> 4 =40O> 42 =27O>#erratia "pp% 20 =19O> 3 =9O> o 23 =15O>

Asper gill us " pp% A =7O> o o A=5O> ,le+ siella "pp% 5 =4O> 2 =6O> o 7 =5O>andida "pp% 5 =4O> o 1 =10O> 6=4O>th$r V 23 =24O> 4 =4O> 1 =10O> 2A =1AO>

Li$r ota 67 27 4 9A

#t.aphywcoccus " pp% 35 =52O> 14 =52O> =25O> 50 =50O>#erratia " p p% 4=6O> =4O> o 5=5O># t reptococcus " pp% 5 =7O> o o 5 =5O> )ocard ia " pp% 3=4O> o o 3 =3O> Asper gillus " p p% 2 =3O> 1 =4O> o 3 =3O>andida " pp% 2 =3O> o o 2 =2O>th$r t A =12O> o o A=AO>

L 1 =9O> 1 =13O> 4=7O># t aphywcoccus " pp% 4 =AO> o 1 =13O> 5=AO>

'ur ( hold eria cepocia 1 =2O> 2 =1AO> 1 =13O> 4=7O>th$r U 6 =13O> 2 =1AO> 1 =13O> 9 =15O>

$rir$ctaota 45 6 6 57

#t.aphyl ococcus " p p% 4=9O> 1 =15O> o 5=9O> le+ siella " pp% 2 =4O> o o 2 =2O> E . col i 2 =4O> o o 2 =2O>th$rH 4=9O> o 1 =15O> 5=9O>

rainota A 4 o 12

Asper g ill us "pp% 6 =75O> 1 =25O> o 7 =5AO>#t.aph ywcoccus "pp% o 1 =25O> o 1 =AO>

E xophiala "pp% o 1 =25O> o 1 =AO>.bbr $iation": $$ pr $io


6/15

160 8IN9:#;T:IN :T A#

a p$, in th$ ca"$ o? "P *o% =O>P *o%=O>P *o%=O>Po? an Asper g3lus a b"c$"" than th$y #i# @ith anyoth$r organi"%

taphyococca "p$ci$" @$r $ a"o th$ o"t proi-n$nt caP *o%=O>P *o%=O>P

Stümoneüa "pp% 11 =20O> 1 =13O> o 12 =1AO> *o%=O>P *o%=O>P *o%=O>P *o%=O>P 'ur(holderia 7 =13O> o 1 =33O> A =12O>#taph ylococcus "pp% 44 =29O> 3 =12O> 3 =20O> 50 =26O> cepacia

# err at ia "pp% 14 =9O> 4 =15O> o 1A =9O> and ida " p p% 5 =9O> 2 =25O> o 7 =11O>and ida "pp% 10 =7O> 1 =4O> 3 =20O> 14 =7O> #taphylococcus "pp% 6 =11O> o o 6=9O> le+siell a "pp% 9 =6O> 1 =4O> o 10 =6O> P seudomonas " p p% 5 =9O> 1 =13O> o 6=9O> )ocard ia "pp% 3 =2O> 1 =4O> o 4 =2O> # err at ia " pp% 4 =7O> o o 4=6O> E . col i 3 =2O> o 1 =6O> 4 =2O> Acineto+act er " p p% 2 =4O> 1 =13O> o 3 =5O>th$r t 11 =AO> 4 =15O> 1 =6O> 16 =AO> l e+ siella "pp% 2 =4O> 1 =13O> o 3 =5O>

.b br $iation": $$ pr $io 3 =3AO> o 12 =1AO>P * o? pati$nt" @ith "


7/15

C'*)C '.*LM. )E.E 'E)'( 161

TAB#: 11% )"oation o? organD"" ?ro C pati$nt" !ith $ningiti "

@ith int$r?$r on-gaa% i?ty-?i$ pati$nt" =15O> ha#r$c$i$# granP *o%=O>P o%=O>P *o%=O>P

Gand i da "pp% 2 =20O> o 1 =100O> 3 =20O>H. imftuen/ae 2 =20O> o o 2 =13O>Bur khold eria 1 =10O> o o 1 =7O>cepacia

E nterovirus 1 =10O> o o 1 =7O>.bbr$iation" : $$ pr$io =nQ2A> =nQ36A>

L%$

0

?

@@..

)=6 Total)=6 "linked re)essi*e

A+tosoal re)essi*e

-

LE in pati$nt o 2 o 2+in#r $#" @ith LE 19/219t ?71 o 20+in#r $#" @ith LE 2/219 ?71 o 3

" % -

::::,# -

b"tr =nQ!> =nQ!5> =A9O> @$r$ b$ing tr$at$#, or ha# b$$n tr$at$# at "orn$

-...

$


8/15

162 8IN9:#;T:IN :T A# AC' *)C '.*LM. )E.E 'E)'(

.LE 13 % M ortai ty in C

$n$tic yp$ Mortaity

%%%%% %N--NN-N-NNNNNNNNNNNNNNN--,

J-in $# r $c$""i$ W%,,

[

-X

$

Wo

- - - JL'C) A$

nno@n g$notyp$ota

Pp 2 0%02 coparing JL' to .' %

A%6O =7/A1>16%7O =4/2A>17%6O =65/36A>

N::, o

=&

/>&

o$

.& CD>

11

1

----------

C

o0%% @oD

@ith th$ a


9/15

"orn$ in"tanc$" th$ in?$ction" @$r$ not con:?in$# to a"ing$ "it$% or $Bap$, atho


10/15

164 8IN9:#;T:IN :T A# 164C'*)C '.*LM. )E.E 'E)'(

thro


11/15

165 8IN9:#;T:IN :T A# 165C'*)C '.*LM. )E.E 'E)'(?orro an# mal es @ith th$ a% )n th$ pr$"$nt "$ri$" it @a"


12/15

th$ "$con# o"t pr$a$nt organi" i"oat$# ?ro pati$nt" @ith pn$, chorior$tiniti"=21, 33, 36, 4A>, ) =3A>, an# $hT$t "yn#ro$ =30>%In ?act, in "orn$ in"tanc$", th$"$ in%?aatory #i"or-#$r" ha$ b$$n th$ ?ir"t cinica ani?$"tation o? Can# ha$ $# to #iagno"i" =13, 49>% i"toogicaiy, th$r$i" % In ?act, in a "% :ort%In th$ pr$"$nt "$ri$", 17%6O o? pati$nt" @$r$ $ith$r #$a# at th$ ti$ th$y @$r$ r$gi"t$r$# or #i$# #


13/15

167C' *)C '.*LM. )E.E 'E)'(16 )*+ELE)* E .L

c$ar$r pict @a" $"tabi"h$# in 1993in ar#$r to $"tiat$ th$ Dni


14/15

167C' *)C '.*LM. )E.E 'E)'(16 )*+ELE)* E .Lhac $?or#, M, Chi#r $n" o"pita o? a"hington ni$r"ity, t% Lo


15/15

169C'*)C '.*LM. )E.E 'E)'(

3A% Mat"

chronic granulomatous disease report on a.3

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