Deficit Immunitaire Hereditaire Flashcards

0
Q

Bilan 1ère intention suspicion DIH

A

NFP +++ (+ frottis sg si infection invasive)
Dosage pondéral des Ig
Étude sero post vaccinales

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1
Q

Signes appel DIH chez enfant

A
Atcd fam
chez 8 OMA/an / chez >4ans : >4OMA/an
>2 sinusites ou pnp/an
Inf sévère ou invasive (sepsis, méningite), inf a Pyo récurrentes, inf a germe opportuniste, diarrhée infectieuse persistante, candidose récidivante
Cassure SP, eczéma, adp, hmg, smg
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2
Q

Bilan DIH quand anomalies bilan 1ère intention

A
Phénotypage lymphocytaire
Profil lymphocytaire (si suspicion DIC)
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3
Q

Bilan DIH : LT= N, prolif T= N et LB N ou ↘️

A

Déficit immunité humorale

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4
Q

Bilan DIH : LB = 0

A

Agammaglobulinemie

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5
Q

Bilan DIH : LT= N ou ↘️, prolif T ↘️

A

Déficit immunitaire combine

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6
Q

Bilan DIH : LT = 0

A

DIC sévère

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7
Q

Bilan DIH quand bilan première intention N + infection bact invasive

A

Frottis sg, voies du complément, dosage sous classe Ig, sero

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8
Q

Bilan DIH quand bilan première intention N + int tissulaires bact ou fongiques récurrentes

A

Étude fonction phagocytaire : NBT, DHR, chimiotactisme,

Dosage des IgE (sd hyperIgE ?)

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9
Q

↘️ NBT ou DHR

A

Granulomatose septique chronique

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10
Q

↘️ chimiotactisme

A

Défaut d’adhésion leucocytaire

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