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Flashcards > Pediatrie patologii Imune > Flashcards

Pediatrie patologii Imune Flashcards

1
Q

Sdr DiGeorge

A

Deletie cromozom 22q11.2
Hipoplazie timica si paratiroidiana
Afectiuni cardiace congenitale
Tetaie si structura faciala anormala
Infectii fungice recurente

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2
Q

Afectiuni ale limfocitelor T

A

Sdr DiGeorge si candidoza cutaneomucoasa cronica

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3
Q

Semne/simptome DiGeorge

A

Tetanie si anomalii faciale
Calciu scazut
Anomalii cardiace
Rx absenta profilului timic
Creening genetic - anomaliicromozomiale

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4
Q

Tratament DiGeorge

A

Calciu
Vit D
Transplant timic/medular
Corectie chirg malf cardiace
Imunoglobuline IV
Antibioterapia profilactica poate fi de ajutor

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5
Q

Candidoza cutaneomucoasa cronica

A

Infectie persistenta tegumente mucoase si unghii ( Candida albicans
Deficienta limf T
Patologie suprarenala frecvent asociata

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6
Q

DG Candidoza cutaneomucoasa cronica

A

Reactie slaba test anergic ( posibil deficit IgG

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7
Q

Tratament Candidoza cutaneomucoasa cronica

A

Agenti antifungici (ex fluconazol)

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8
Q

Afectiuni limfocite B

A

Agamaglobulinemia X linkata
Deficit IgA
Sdr Hiper IgM
Imunodeficienta comuna variabila

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9
Q

Agamaglobulinemia X linkata

A

Diferentierea anormala a limfocitelor B
Nivel scazut de limfocite B si anticorpi
Baieti infectii bacteriene recurente dupa varsta de 6 luni

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10
Q

Dg Agamaglobulinemia X linkata

A

Lipsa limfocitelor B pe frotiu
Nivel total scazut de imunoglobuline

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11
Q

Tratament Agamaglobulinemia X linkata

A

Imunoglobulina iv
Trat antibiotic
Tratament respirator suportiv

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12
Q

Deficit IgA

A

Scazuta productia de imunoglobuline
infectii respiratorii si gastrointestinale

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13
Q

Dg Deficit IgA

A

Nivel scazut de IgA, restul normale

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14
Q

Tratament Deficit IgA

A

Antibioterapie profilactica
Adm Ig iv ( risc scazut de anafilaxie)

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15
Q

Sdr Hiper IgM

A

Anomalie Ligand CD40 al limfocitelor T –> interactiune slaba cu limf B
Nivel scazut de IgG si productie excesiva IgM
infectii cauzate de bacterii incapsulate ( pulmonare si gastrointestinale

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16
Q

Diagnostic Sdr Hiper IgM

A

Nivel scazut IgG si IgA
Valori crescute IgM
Valori posibil scazute ale Hb si Ht, trombocitelor si neutrofilelor

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17
Q

Tratament Sdr Hiper IgM

A

Ig iv
Antibiotice profilactic
Transplant medular

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18
Q

Imunodeficienta comuna variabila

A

afectiune autozomala a diferentierii limf b – > nivele scazute de imunoglobuline
Infectii respiratorii si gastrointestinale frecvente in a 2a decada a vietii
Risc crescut de afectiuni neoplazice si autoimune

19
Q

Dg Imunodeficienta comuna variabila

A

Nivel scazut imunoglobuline
Raspuns precar la vaccinare
Raport CD4/CD8 scazut
Afectarea ambelor sexe

20
Q

Tratament Imunodeficienta comuna variabila

A

Ig iv
Antibiotic corespunzator

21
Q

In care afectiuni ale limfocitelor B se administreaza antiobioterapie profilactica?

A

Deficit IgA
Sdr Hiper-IgM

22
Q

Afectiuni combinate ale limfocitelor T si B

A

Sdr de imunodeficienta combinata severa
Sdr Wiskott Aldrich
Ataxia teleangiectazia

23
Q

Sdr de imunodeficienta combinata severa
IDCS

A

Absenta limf T si disfunctionalitate anticorpi
Infectii recurente severe inca de la o varsta frageda
Frecvent fata la varste mici

24
Q

Dg Sdr de imunodeficienta combinata severa
IDCS

A

leucocite semnifictiv scazute
Imunoglobine scazute

25
Q

Tratament Sdr de imunodeficienta combinata severa
IDCS

A

Ig iv
Antibiotice
Transplant medular
Vaccinuri vii sau vii atenuate nu trebuie administrate

26
Q

Sdr Wiskott Aldrich

A

Afectiune X linkata
Suscebilitate severa la infectii cu bacterii incapsulte si patogeni oportunisti
Asociata cu eczema si trombocitopenie

27
Q

Dg Sdr Wiskott Aldrich

A

Infectii recurente
Eczema
sangerari facile
Trombocite scazute
Niv IgM scazute
normale sau crescute celelalte Ig
testare genetica

28
Q

Tratament Sdr Wiskott Aldrich

A

Splenectomia
Antibioterapia profilactica
Ig iv
Transplant medular

29
Q

Care patologii imune produc Trombocitopenie?

A

Sdr Hiper IgM
Sdr Wiskott Aldrich

30
Q

Ataxia- teleangiectazia

A

afectiune autozomal recesiva
difunctie cerebelara
teleangectazie cutanata
risc crescut de cancer
Dezvoltare defectuasa a leucocitelor si a IgA

31
Q

Afectiuni cel fagocitare

A

Boala granulomatoasa cronica
Sdr Hiper IgE ( job)
Sdr Chediak Higashi
Deficit al adeziunii leucocitare tip 1 si 2

32
Q

Afectiuni ale complementului

A

Deficienta complement
cauzeaza infectii bacteriene recurente
Predispozitie de a dezvolta afectiuni autoimune ( LES)
TRat antibioterapia corespunzatoare
Tratarea afectiunilor autoimune

33
Q

Boala granulomatoasa cronica

A

Neutrofilele nu pot digera bacteriile ingerate
Infectii bacteriene si fungice recurente

34
Q

Dg Boala granulomatoasa cronica

A

Abcese cutanate pulmonare si perirectale
Limfadenopatie cronica
Investigatii genetice

35
Q

Tratament Boala granulomatoasa cronica

A

Antibioterapia profilactica
gama interferon
cortico
transplant medular

36
Q

Sdr Hiper IgE ( job)

A

Anomalie chemiotaxie neutrofile
Anomalii cai de semnalizare limf T
Supraproductie de IgE
Dermatita cronica
Abcese cutanate recurente
Infectii pulmonare
Dentitie primara persistenta

37
Q

Dg Sdr Hiper IgE ( job)

A

IgE crescut
Eosinofile crescute
Raspuns chemotactic deficitar al neutrofilelor la stimulare

38
Q

Tratament Sdr Hiper IgE ( job)

A

Antibioterapie profilactica
Hidratarea pielii
Utilizarea de emolienti

39
Q

Sdr Chediak Higashi

A

autosomal recesiva
Disfunctie a neutrofilelor –> infectii recurente cu stafilococi streptococi, bacterii G- si infectii fungice
Trombocite anormale
albinism
disfunctii neurologice

40
Q

DG Sdr Chediak Higashi

A

Granulatii gigante la niv granulocitelor pe frotiu periferic

41
Q

Tratament Sdr Chediak Higashi

A

Antibioterapie profilactica
Transplant medular

42
Q

Deficit al adeziunii leucocitare tip 1 si 2

A

Inabilitatea neutrofilelor de a parasi circulatia - anomalii al integrinelor (tip1) sau E selectina ( tip2) leucocitare
Infectii bacteriene recurente ale tract resp superior si ale tegumentului
Detasare intarziata a bontului ombilical
Statura mica
Facies anormal
Deficite cognitive tip 2

43
Q

Dg Deficit al adeziunii leucocitare tip 1 si 2

A

Neutrfile serice crescute
raspuns chemotactic deficitar a neutrofilelor la stimulare

44
Q

Tratament Deficit al adeziunii leucocitare tip 1 si 2

A

Antibioterapie profilactica
Tip 1 transplant medular
Tip 2 fucoza

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