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Prolazna hipogamaglobulinemija
dojenčeta (PHGD)
Domina Petrić
Domina Petrić
Uvod
• Prolazna hipogamaglobulinemija je
stanje dojenčeta koje nastaje zbog
zakašnjelog početka sinteze vlastitih
imunoglobulina u dojenačkoj dobi.
• Umjesto da sinteza imunoglobulina
počne odmah poslije rođenja, u te
djece sinteza počinje tek između 9. i 15.
mjeseca života.
PHGD
• Normalna razina gama-globulina se ostvari
između 2. i 4. g. života.
• Javlja se podjednako često u muške i
ženske djece, a osobito u nedonoščadi.
• Etiologija bolesti nije poznata.
• Ne smije se zamijeniti s fiziološkim
prolaznim sniženjem konc. imunoglobulina
između 3. i 5. mjeseca života koje nije
praćeno kliničkim simptomima povećane
sklonosti infekcijama.
Klinička slika
• Može i ne mora postojati povećana
sklonost bakterijskim infekcijama kože,
pluća, meninga i respiratornog sustava
(pretežno gram pozitivne bakterije).
• Djeca uz to slabije napreduju.
• Učestale blage virusne respiratorne
infekcije nisu uzrokovane prolaznom
hipogamaglobulinemijom i nisu
simptom ove bolesti.
Dijagnostika
• Bolest se potvrđuje nalazom
snižene konc. imunoglobulina u
plazmi (<4,0 g/L).
• Biopsija limfnih čvorova ili
sluznice rektuma daje normalan
histološki nalaz za razliku od
Brutonove bolesti.
Liječenje
• Postoje mišljenja da je liječenje
nadomještanjem imunoglobulina
indicirano samo u akutnim vitalnim
indikacijama, dok je u većine djece
dovoljno adekvatno antimikrobno
liječenje, jer davanje pripravaka
imunoglobulina potiskuje sintezu
vlastitih imunoglobulina.
• Literatura:
Duško Mardešić i suradnici.
Pedijatrija
Školska knjiga,
Zagreb, 2003.

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Prolazna hipogamaglobulinemija dojenčeta

  • 2. Uvod • Prolazna hipogamaglobulinemija je stanje dojenčeta koje nastaje zbog zakašnjelog početka sinteze vlastitih imunoglobulina u dojenačkoj dobi. • Umjesto da sinteza imunoglobulina počne odmah poslije rođenja, u te djece sinteza počinje tek između 9. i 15. mjeseca života.
  • 3. PHGD • Normalna razina gama-globulina se ostvari između 2. i 4. g. života. • Javlja se podjednako često u muške i ženske djece, a osobito u nedonoščadi. • Etiologija bolesti nije poznata. • Ne smije se zamijeniti s fiziološkim prolaznim sniženjem konc. imunoglobulina između 3. i 5. mjeseca života koje nije praćeno kliničkim simptomima povećane sklonosti infekcijama.
  • 4. Klinička slika • Može i ne mora postojati povećana sklonost bakterijskim infekcijama kože, pluća, meninga i respiratornog sustava (pretežno gram pozitivne bakterije). • Djeca uz to slabije napreduju. • Učestale blage virusne respiratorne infekcije nisu uzrokovane prolaznom hipogamaglobulinemijom i nisu simptom ove bolesti.
  • 5. Dijagnostika • Bolest se potvrđuje nalazom snižene konc. imunoglobulina u plazmi (<4,0 g/L). • Biopsija limfnih čvorova ili sluznice rektuma daje normalan histološki nalaz za razliku od Brutonove bolesti.
  • 6. Liječenje • Postoje mišljenja da je liječenje nadomještanjem imunoglobulina indicirano samo u akutnim vitalnim indikacijama, dok je u većine djece dovoljno adekvatno antimikrobno liječenje, jer davanje pripravaka imunoglobulina potiskuje sintezu vlastitih imunoglobulina.
  • 7. • Literatura: Duško Mardešić i suradnici. Pedijatrija Školska knjiga, Zagreb, 2003.