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J Radana Zachov Institute of Immunology Faculty hospital Prague, Motol  KJ" 2  Complement system $  part of hummoral innate immune system group of serum and cell surface proteins important in antiinfectious and inflammatory immune response activated by a cascade of reactions during activation generates active components . !6   K  x Complement activation pathways  $  Alternative pathway (pathogen surface) Mannan binding lectin pathway (pathogen surface) Classical pathway (antigen-antibody complexes) 6&    K Complement activation pathways$  `  Primary complement deficiencies $  x1) Components of activation pathways C1q, C1r,C1s,C4,C2,C3, MBL, D,B,C5,C6,C7,C8,C9 2) Control proteins soluble control proteins C1 inhibitor, factor I, factor H,C4b binding protein,S protein, SP-40,40 membrane regulatory proteins CD 55(DAF), MCP CD 46, CD 59, HRF/C8bp 3) Receptors for complement C1q receptor, CR1(CD 35), CR2 (CD21), CR3 (CD11b/CD18), CR4 (CD11c-CD18) `iZZIZZZ!.H&H y  6Primary complement deficiencies clinical manifestation47 $$ 7 3Increased susceptibility to infections with systemic course - bacteremia+meningitis S. pneumoniae, S.pyogenes, H.influenzae (early components , defect in opsonization) Neisseria meningitidis (defect in terminal components ) Autoimmune disorders defective immune complex clearance Angioedema (Z2Z)Z/!ZZ#!Z P$!P P Z(2p   # B           !     %  .  Complement genes$8Complement system proteins  members of various gene families Some groups of complement proteins are in close chromosomal linkage chromosome 1 chromosome 6 ,X, DPrimary complement deficiencies in ESID registry from Czech republic&E $%6  #?Table 1.Group of patients with primary complement deficiencies d@ccc8!%$Table 4. C4 deficiencies  "+Primary complement deficiencies Conclusions,,$+nRelatively uncommon diagnosed group of diseases Can be the underlying disease in autoimmunity and recurrent infections Laboratoty investigation of complement components is not easy, but C3 and C4 is normal available Most frequent is C2 deficiency Angioedema in C1 inhibitor deficiency appears in more generations in one family, differential diagnosis to allergyoZo /'()*+,-./0 1 2 3 4   ` ̙33` ` ff3333f` 333MMM` f` f` 3>?" dd@,|?" dd@   " @ ` n?" dd@   @@``PR    @ ` ` p>> S(    6C P  RKlepnutm upravte styl pYedlohy nadpisu.* * ~  0DC   Klepnutm upravte styly pYedlohy textu. Druh roveH TYet roveH tvrt roveH Pt roveH)    ]   0B ``  Y*    0B `   [*    0$B `   [*  B  s *޽h ? Default Designd0 $@(    0< PP   _*   0$<  P  a* d  c $ ?P    0;  @  Klepnutm lze upravit styly pYedlohy textu. Druh roveH TYet roveH tvrt roveH Pt roveH,    `  69 pP   _*   6 p   a* H  0޽h ? ̙33L 0@( &5% @ @ 0> P;   [*    @ 0=  ;  ]*    @ 6D= jP   [*    @ 6< j   ]*   H @ 0޽h ? ̙33  D(  l  C >`    C d> `  <$0  H  0޽h ? ̙33  z`(  l  C 4%v`  v l  C %v v B  s *޽h ?   ( `r@@@ l  C &vP  v l  C  v v H  0޽h ? x  ( (  l  C $  v l  C  v   C pAXC:\WINDOWS\Plocha\complement activation.gifH  0޽h ?    ( `z@@H@  l  C D`p  v l  C 0P  v H  0޽h ? D  $(  $l $ C $  v  $ c 0e0e  v H $ 0޽h ?   x0(  xx x c $P  v x x c $d v H x 0޽h ?  {s 8 ( w 8x 8 <A ??[K x 8 <A &??@*V & 8 6D Pp mPrimary complement deficiencies $  H 8 0޽h ?   @X@(  Xx X c $P  v  X0 <A "?{d " vH X 0޽h ? f   ee`iXe(    S   v   S $ v   dF  i  7 Xc@  g1@  N  S @    6 kType of deficiency <'' Z  s *l@ b  b N  S b @ b  b   6F   Number of families P ' '  Z  s *b b@ b  b N  S b @ b  b    6d~   Number of patients P ' ' Z  s *b &@  f  fN  S  f@  f  f  6 J `Males >' ' Z  s * f(@ f ƍfN ō S f@ f čf  6I bFemales > ' ' Z Í s *f6@  ʍN ɍ S @  ȍ  6I pC1 inhibitor deficiency <' ' Z Ǎ s *"@ b  ΍b N ͍ S b |@ b  ̍b   64JF  \3 >'' Z ˍ s *b "@ b   ҍb  N э S b  |@ b   Ѝb    6J~   \5 >'' Z ύ s *b  "@  f ֍ fN Ս S  f|@  f ԍ f  6J J \4 >'' Z Ӎ s * f"@ f ڍfN ٍ S f|@ f ؍f  6TK \1 >'' Z ׍ s *f-@ y ލyN ݍ S y@ y ܍y  6Ly gC4 deficiency <' ' Z ۍ s *y@ b y b yN  S b yx@ b y b y  6tLF y X (&  Z ߍ s *b y@ b  y b  yN  S b  yx@ b  y b  y  6L~  y X (&  Z  s *b  y@  fy  fyN  S  fyx@  fy  fy  64M Jy X (&  Z  s * fy@ fy fyN  S fyx@ fy fy  6My X (&  Z  s *fy2@ yY yYN  S yY@ yY yY  6TNyY lC4A*Q0 heterozygous <' ' Z  s *yY"@ yb Y yb YN  S yb Y|@ yb Y yb Y  6NyF Y \2 >'' Z  s *yb Y"@ b y Y b y YN  S b y Y|@ b y Y b y Y  6O~ y Y \1 >'' Z  s *b y Y"@  yfY  yfYN  S  yfY|@  yfY  yfY  6tO yJY \- >'' Z  s * yfY"@ fyY fyYN  S fyY|@ fyY fyY  6OyY \1 >'' Z  s *fyY2@ Y9  Y9 N  S Y9 @ Y9  Y9   6PY9  lC4B*Q0 heterozygous <' ' Z  s *Y9 "@ Yb 9   Yb 9 N   S Yb 9 |@ Yb 9  Yb 9   6PYF 9  \1 >'' Z  s *Yb 9 "@ b Y 9  b Y 9 N   S b Y 9 |@ b Y 9   b Y 9   6TQ~ Y 9  \2 >'' Z   s *b Y 9 "@  Yf9   Yf9 N  S  Yf9 |@  Yf9   Yf9   6Q YJ9  \- >'' Z  s * Yf9 "@ fY9  fY9 N  S fY9 |@ fY9  fY9   6RY9  \2 >'' Z  s *fY9 _@ 9 ?  9 ? N  S 9 ? @ 9 ?  9 ?   6R9 ?  C4A*Q0 C4B*Q0 heterozygous b ' '  ' ' Z  s *9 ? "@ 9 b ?  9 b ? N  S 9 b ? |@ 9 b ?  9 b ?   64S9 F ?  \1 >'' Z  s *9 b ? "@ b 9 ?  "b 9 ? N ! S b 9 ? |@ b 9 ?   b 9 ?   6S~ 9 ?  \1 >'' Z  s *b 9 ? "@  9 f?  & 9 f? N % S  9 f? |@  9 f?  $ 9 f?   6S 9 J?  \- >'' Z # s * 9 f? "@ f9 ?  *f9 ? N ) S f9 ? |@ f9 ?  (f9 ?   6TT9 ?  \1 >'' Z ' s *f9 ? ,@ ?   .?  N - S ?  @ ?   ,?    6)?   fC2 deficiency < ' ' Z + s *?  @ ? b  2? b N 1 S ? b x@ ? b  0? b   6t)? F  X (&  Z / s *? b @ b ?  6b ? N 5 S b ? x@ b ?  4b ?   6)~ ?  X (&  Z 3 s *b ? @  ? f  : ? f N 9 S  ? f x@  ? f  8 ? f   64* ? J  X (&  Z 7 s * ? f @ f?   >f?  N = S f?  x@ f?   <f?    6*?   X (&  Z ; s *f?  @   B N A S  @   @ 6  6*  (C2 deficiency I. type (28 bp deletion) b)' ' ' '  Z ? s * "@  b  F b N E S  b |@  b  D b   6T+ F  \1 >'' Z C s * b "@ b  Jb N I S b |@ b  Hb   6+~  \- >'' Z G s *b "@  f N fN M S  f|@  f L f  6, J \- >'' Z K s * f"@ f  Rf N Q S f |@ f  Pf   6t,  \2 >'' Z O s *f [@  VN U S @  T  64- C2 deficiency II.type b' ' ' ' Z S s *"@ b  Zb N Y S b |@ b  Xb   6-F  \1 >'' Z W s *b "@ b   ^b  N ] S b  |@ b   \b    6-~   \1 >'' Z [ s *b  "@  f b fN a S  f|@  f ` f  6T. J \- >'' Z _ s * f"@ f ffN e S f|@ f df  6. \1 >'' Z c s *fT h c $H  0޽h ? e~  ~ ~с}(    640 w C1 inhibitor deficiency < " # #  ڀ 60[ N "   ܀ B0pD  B#  2zF  Ё fPy@  ΁@  N  S x@   ހ 6T1 X (&  Z  s *F@  N  S @   ߀ <27 zPatient Nr. 1 .'Z  s *P@    N  S  @      622  Patient Nr. 2 > ' ' Z  s * P@    N  S  @      63-  Patient Nr. 3 > ' ' Z  s * P@   ! N   S  @      6T4( Patient Nr. 4 > ' ' Z  s * P@  %N $ S @  #  6ܲ# Patient Nr. 5 > ' ' Z " s *$@  )N ( S ~@  '  6$ݲ ^Sex >' ' Z & s *%@  -N , S @  +  6ݲ7 _Male >' ' Z * s *%@   1 N 0 S  @   /   6ݲ2  _Male >' ' Z . s * '@    5  N 4 S   @    3    6D޲-   aFemale >' ' Z 2 s *  %@   9 N 8 S  @   7   6޲( _Male >' ' Z 6 s * %@  =N < S @  ;  6߲# _Male >' ' Z : s *,@  AN @ S @  ?  6߲ f Age (years) >  ' ' Z > s *#@  EN D S }@  C  6$7 ]43 >' ' Z B s *#@   I N H S  }@   G   62  ]18 >' ' Z F s * #@    M  N L S   }@    K    6-   ]11 >' ' Z J s *  #@   Q N P S  }@   O   6D( ]26 >' ' Z N s * #@  UN T S }@  S  6# ]10 >' ' Z R s *M@  YN X S @  W  6d  Family Nr. >  ' ' Z V s *"@  ]N \ S |@  [  67 \1 >' ' Z Z s *"@   a N ` S  |@   _   6$2  \1 >' ' Z ^ s * "@    e  N d S   |@    c    6-   \1 >' ' Z b s *  "@   i N h S  |@   g   6( \2 >' ' Z f s * "@  mN l S |@  k  6D# \3 >' ' Z j s *-@   q N p S  @   o   6  gFamily history <' ' Z n s * q@   u N t S  @   s   6d7  1Edema of upper airways (mother died), oncle, son <20' ' %Z r s * }@    y  N x S   @    w  %  62   ;Recurrent edema of the upper airways (grand mother, father `<$' ' ' ' Z v s *  =@     }   N | S    @     {     6-    wFrequent respiratory infection < ' ' Z z s *   D@      N  S   @        6D(  ~Glomerullonephritis <' '  Z ~ s *  H@    N  S  @      6#  *Frequent respiratory infection, sinusitis <+)' ' Z  s * 2@      N  S   @        6O   lClinical expression <' ' Z  s *  U@      N  S   @        6P7   7Frequent respiratory infections, abdominal pain, edema <86' ' Z  s *  N@      N  S   @        6tP2  0Frequent respiratory infections, abdominal pain <1/' ' Z  s *  `@        N  S    @          64Q-    Frequent respiraotry infections <!' '   Z  s *   l@        N  S    @          6Q(   ,Edema of cheeksafter stomatologic treatment <-+' '  Z  s *   O@      N  S   @        6Q#   1Recurrent respiratory infections, abdominal pain <20' ' Z  s *  )@    N  S  @      6R  c Laboratory <  ' ' Z  s * @    N  S  b@      0S7  HC3 C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA IgG,IgM normal U'  '                  '     b   Z  s * @      N  S   @      f  0tS2  C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA, IgG IgM normal [ ' ' '  ' ' ' ' ' ' / ' ' ' ' '  '  '     b   Z  s *  @    N  S  @    f  0S-  C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA IgG,IgM normal [ ' ' '  ' ' ' ' ' ' / ' ' ' ' ' '  '     b   Z  s * @      N  S   @      f  04T(  C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA IgG,IgM normal [ ' ' '  ' ' ' ' ' ' / ' ' ' ' ' '  '     b   Z  s *  @    N  S  @    f  0T#  C3 normal C4 not detecable C1 INH not detecable CH100 0 CIK normal IgA IgG,IgM normal [ ' ' '  ' ' ' ' ' ' / ' ' ' ' ' '  '     b   Z  s * &@  N  S @    6TU `Therapy < ' ' Z  s *q@  N  S @     6V7 Danazol C1 inhibitor `' '  ' ' Z  s *+@    N  S  @       6V2  e C1 inhibitor < ' ' Z  s * +@    Ł  N ā S   @    Á     6W-   e C1 inhibitor < ' ' Z  s *  N@   Ɂ N ȁ S  @   ǁ    6TX( Danazol, C1 inhibitor <' ' Z Ɓ s * +@  ́N ́ S @  ˁ   6Y# e C1 inhibitor < ' ' Z ʁ s *T ρ c $ с S Y@`   p Table 2. C1 inhibitor deficiency!! H  0޽h ? uN  %NNM(    <Z  pJ Table 3. C2 deficiencies@ '  zLL 5 # 8HKN 2  2N     6T[ R ""  Z  s *N      6d0   Patient Nr.1 4 ## Z   s *N         6$1   Patient Nr.2 4 ## Z   s * N       61'   Patient Nr.3 4 ## Z  s * N %  %  624   Patient Nr.4 4 ## Z  s *%N %2  %2  6d3A  Patient Nr.5 4 ## Z  s *%2N t  t  63t TSex 4## Z  s *tN t  t  6$4 t WFemale 4## Z  s *tN  t   t  64 t WFemale 4## Z  s * tN  t    t ! 64' t WFemale 4## Z " s * tN %t # %t $ 6D54 t WFemale 4## Z % s *%tN %2t & %2t ' 65At WFemale 4## Z ( s *%2tN t ) t * 6d6t `Clinical course 4## Z + s *tN t , t - 6$7 t jNo clinical manifestation 4# # Z . s *tN t  / t  0 67t  jMeningitis in newborn age 4# # Z 1 s *t N  t 2  t 3 <8' t cNeonatal infection 4# #Z 4 s * tN t% 5 t% 6 694t  /Girl died at the age 1 month due to meningitis 40.# # Z 7 s *t%N %t2 8 %t2 9 6d9At (Otitis, sinusitis, recurrent meningitis 4)'# # #Z : s *%t2N   ;   < 6$:  iLaboratory investigation 4## Z = s * N   >   ? 6:   pIgG, IgA, IgE , C3, C4 normal, C2, CH 100 normal 9"### 6*Z @ s * N   A  , B 6:  vIgG, IgA, IgM normal, IgE , C3,C4 normal, CH 100 normal <###  H"Z C s * N    D    E 6D;'   IgG , IgA not detecable, IgM normal, C3,C4 normal, CH 100 normal F#/#  H )Z F s *  N %  G %  H 6;4   jIgG, IgG1-3 , IgM, IgA , C3,C4 normal CH 100 6 # ## # 6 Z I s *% N %2  J %2  K 6A  IgG, IgA, IgM IgG1-4 normal, C3, C4 normal, CH 100 , C2 A."*"##  64Z L s *%2 N    M    N 6Գ   qGenotypisation 4##  Z O s *  N    P    Q 6    C2 180/152 28 bp deletion T ## ##  Z R s *  N   S   T 6T  C2 180/152 28 bp deletion T ## ##  Z U s * N    V    W 6'   ^Not performed 4 ## Z X s *  N  %  Y  %  Z 6Զ4   ^Not performed 4 ## Z [ s * % N % 2  \ % 2  ] 6A   Deletion 28 bp not verified 4##  Z ^ s *% 2 N  I _  I ` 6T I XTherapy 4 # # Z a s * IN  I b  I c <  I T- 6# # Z d s * IN  I e  I f 6t I ~Antimicrobial drugs 4# #  Z g s * IN  I h  I i 64' I Antimicrobial drugs, plasma 4# #  Z j s * IN  %I k  %I  l 64 I Antimicrobial drugs T # # # #   Z m s * %I,N % 2I n % 2Il o 6TA I FAntimicrobial drugs, vaccciination Pneumokok, Haemofilus, Meningokok TG## # !# # @   Z p s *% 2IN I q I r 6I R ""  Z s s *IN I t I u 6 I R ""  Z v s *IN I  w I  x 6tI  R ""  Z y s *I N  I z  I { 6Լ' I R ""  Z | s * IN I% } I% ~ 644I  R ""  Z  s *I%N %I2  %I2  6AI R ""  Z  s *%I2T  c $5H  0޽h ? _   __^'_(  r  S TP     S      6dp CH100 ` " * "    \F  ^ P`:\@  \@  ӐN Ґ S x@  ѐ  6p X (&  Z А s *p@   א N ֐ S  @   Ր   6q   Patient Nr. 1. P ''&Z Ԑ s * q@  s ې sN ڐ S  s@  s ِ s  6Dr W  Patient Nr. 2 R  ''&Z ؐ s * sp@ sD ߐsDN ސ S sD@ sD ݐsD  6s(  Patient Nr.2 R  ''&Z ܐ s *sDh@ D DN  S D@ D D  6s`  Pateint Nr.4 R  '' Z  s *D$@ & &N  S &~@ & &  6$t& ^Sex >' ' Z  s *&'@  &  &N  S  &@  &  &  6t & aFemale >' ' Z  s * &'@  s&  s&N  S  s&@  s&  s&  6t W& aFemale >' ' Z  s * s&'@ sD& sD&N  S sD&@ sD& sD&  6Du(& aFemale >' ' Z  s *sD&'@ D& D&N  S D&@ D& D&  6u`& aFemale >' ' Z  s *D&$@ & &N  S &~@ & &  6v& ^Age >'' Z  s *&"@ &  & N  S & |@ &  &   6dv&  \8 >'' Z  s *& #@  &s  &sN  S  &s}@  &s  &s  6v &W ]54 >'' Z  s * &s"@ s&D s&DN  S s&D|@ s&D s&D  6$w&( \5 >'' Z  s *s&D#@ D&  D&N   S D&}@ D&  D&  6w`& ]31 >'' Z  s *D&(@  N  S @     0Dx hClinical course <' ' Z   s *p@    N  S  @      0x  ZTransposition of great arteries, chronic acute hepatitis with delayed antibody formation :[Y' ' Z  s * @  s  sN  S  sk@  s  s  0y W QwRheumatic fever, meningitis, lupus like skin disease, arthralgias, Anemia of chronic infection, recurrent pyodermia xC' ' '  ' ' 65  Z  s * s6@ sD sDN  S sD@ sD sD  0y( v Septic infection in newborn age :!' ' Z  s *sD@ D DN  S DS@ D D  0$z` 9MMeningitis Recurrent pyodermias Lupus erythematodes, autoimmune cytopenia N ' ' ' ' )' ' & 6 Z  s *D1@ j  #j N " S j @ j  !j   0zj  qLaboratory investigation <' ' Z   s *j @  j  ' j N & S  j @  j  % j 8  0D{ j  CH100 normal C3 normal C4 not detecable IgG, IgA, Ana, ANCA, dsDNA, ASMA, LKNM negative HbsAg positive, HbeAg positive V{' / ' '  ' ' ' ' '  '  ''     b   Z $ s * j @  sj  + sj N * S  sj N@  sj  ) sj  Ð 0{ Wj  4C3 C4 antigen normal C4 hemolytic MBL normla CH100 Factor B normal Nephritic factor negative Facor H normal Factor I normal '  '    '       '          ,)2Z ( s * sj @ sDj  /sDj N . S sDj @ sDj  -sDj 6 Đ 0T¹(j  C3 normal C4, CH100 normal IgG, IgA, IgM normal ANCA type p positive IgG kappa positive ANCA ELISA method normal ru ' ' '  '             P,Z , s *sDj @ Dj  3Dj N 2 S Dj @ Dj  1Dj l Ő 0¹`j  C3 normal, C4, CH100 , IgG, IgA, IgM normal ENA SS-B positive Cardiolipin antibodies positive Coombs test positive z '  '     '           b Z 0 s *Dj R@ j p 7j pN 6 S j p@ j p 5j p Ɛ 6tùj p Genetic phenotypisation >' ' Z 4 s *j p@ j p ;j pN : S j p@ j p 9j p= ǐ 64Ĺj p  C4A3AQ0 B2B2  ' ' ' ' ' Z 8 s *j p@  j sp ? j spN > S  j sp@  j sp = j sp* Ȑ 6Ĺ j Wp  C4A3A3B1BQ0 v ' ' ' '  Z < s * j spw@ sj Dp Csj DpN B S sj Dp@ sj Dp Asj Dp ɐ 6Źj (p  C4B Q0 heterozygous b '  ' ' Z @ s *sj Dpd@ Dj p GDj pN F S Dj p@ Dj p EDj p  ʐ <tƹ`j p  C4AQ0C4BQ0 >  ' & Z D s *Dj p @ p KpN J S pz@ p Ip ː 04ǹp `Therapy < ' ' Z H s *p#@ p  Op N N S p }@ p  Mp  ̐ 0ǹp  c Interferon A : ' ' Z L s *p F@  ps S psN R S  ps@  ps Q ps ͐ 0ȹ pW Antimicrobial therapy :' '   Z P s * ps@ spD WspDN V S spDp@ spD UspD ΐ 0ɹp( V &&  Z T s *spD@ Dp [DpN Z S Dp@ Dp YDpV ϐ 0tɹ`p HCorticosteroids, vaccination (Pneumococcus, Meningiciccus, Haemophilus) :IG' ' H  Z X s *DpT ] c $H  0޽h ?    $(  r  S 4ʹP   r  S ʹ  H  0޽h ? 0 P(  X  C P     S $v @    H  0޽h ? ̙330 p(  X  C P   v  S v @  v  H  0޽h ? ̙330 (  X  C P   v  S t!v @  v  H  0޽h ? ̙330 (  X  C P   v  S  @  v  H  0޽h ? ̙330 (  X  C P   v  S  @  v  H  0޽h ? ̙330 (  X  C P   v  S  @  v  H  0޽h ? ̙330 (  X  C P   v  S  @  v  H  0޽h ? ̙33 0 0(  X  C P   v  S  @  v  H  0޽h ? ̙330 P(  X  C P   v  S d @  v  H  0޽h ? ̙330 p(  X  C P     S t/ @    H  0޽h ? ̙330 (  X  C P     S 4Z @    H  0޽h ? ̙330 (  X  C P     S  @    H  0޽h ? ̙330 (  X  C P     S ɹ @    H  0޽h ? ̙330 (  X  C P     S ˹ @    H  0޽h ? ̙33 hx{pTWn\RB IT $HRcK)䱁$K(XEm-qFЩձu:8j-O)wMB~ mr;=x /=(NC (5Odkp82;l?`K:d!<[KRaitXro-eaB!簐sD qk/8GcB_3Bq!B#BNo2 9(3@<*+Bί 9 CB·_r>",cBBί 9ra?DŽ 9r>),&wByRhJeR%cFUFT^搒B4L  $ڼ#D "$ZBm!0j !'=}p^ ,Pa4x,S^Wܧqz"=Ge_7 Gl}zUlg g{[4r7 : aŠ`֡|>˶l_d:m42](*Zi=%km-qB,%8}- >W^Us'\,}|l…t7267uوWMrCM'B##########BmvC Dz3ɶQemDK Q鼋00ɚ{НfvIfɵgOdiy ASt8+=ԈƜa~C`O.C Hw3+5Ktqcr=>o6`96[81c<=i=u:.>]y !7u_n U==bͤ'cu:xWlE9MWT*)ꉟyRox8㡸sڎUo>qM5Jo>ݔ"&1uV28d[GKϽ[8wiCBXщbزsgddGmΨàt8m!O|NREyNtG>fS;U? 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#$ . .!2 %complement system!$4$!4 $! ! 4.--Q1-- "Arial- .2 *{Radana Zachov  . .Q2 P1Institute of Immunology Faculty hospital Prague,     . .2 P Motol.--"System-&TNPP & ՜.+,D՜.+,D     pedvdn na obrazovceedcB Times New RomanArialSymbolDefault Designdokument Microsoft Wordgraf aplikace Microsoft Excel.Primary deficiencies of the complement systemComplement system Complement activation pathways Complement activation pathways Primary complement deficiencies7Primary complement deficiencies clinical manifestationComplement genes Bez nadpisuEPrimary complement deficiencies in ESID registry from Czech republic@Table 1.Group of patients with primary complement deficiencies !Table 2. 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