HNO.90471063.ger.abstr-passage-0
abstract
Das Hyperimmunglobulin-E-Syndrom ( Job's Syndrom , Hiob-Syndrom , Buckley-Syndrom ) ist eine seltene Erkrankung des Immunsystems , die im HNO-Bereich charakteristische Manifestationen zeigen kann . Das Krankheitsbild ist gekennzeichnet durch rezidivierende urtikarielle Exantheme , Fieber , Lymphadenitis , Hyperplasie lymphatischer Organe und bakteriellen Haut- und Organinfektionen . Diagnostisch richtungsweisend sind stark erhoehte IgE-Konzentrationen im Serum beim gleichzeitigem Fehlen von Hinweisen auf allergische und parasitaere Erkrankungen . In der vorgestellten Kasuistik konnte die Diagnose eines Hyper-IgE-Syndroms trotz mehrjaehrigen Krankheitsverlaufs erst bei einer HNO-aerztlichen Behandlung wegen einer Zungengrundhyperplasie gestellt werden . Therapeutisch erwies sich eine Behandlung mit hochdosiertem intravenoesem Immunglobulin ( IVIG ) als ausgezeichnet vertraeglich und gut wirksam . Mehr als ein Jahr nach der einmaligen Therapie ist die Symptomatik anhaltend gebessert . Der derzeitige Kenntnisstand und die zur Verfuegung stehenden Therapieoptionen beim Hyper-IgE-Syndrom werden dargestellt und diskutiert . Beim Hyper-IgE-Syndrom sollte ein Therapieversuch mit IVIG eine Option der ersten Wahl darstellen .
HNO.90471063.ger.abstr-s2-r1-t2.1-t6.1
associated_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r2-t4.1-t6.1
degree_of
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r3-t2.1-t4.1
associated_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r4-t6.1-t3.1
degree_of
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r5-t2.1-t3.1
associated_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r6-t2.1-t1.1
associated_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r7-t1.1-t5.1
evaluation_of
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r8-t4.1-t1.1
degree_of
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r9-t1.1-t3.1
degree_of
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r10-t3.1-t6.1
associated_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r11-t3.1-t5.1
associated_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r12-t3.1-t4.1
associated_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r13-t3.1-t2.1
associated_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-r14-t3.1-t2.1
co-occurs_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-r15-t3.1-t1.1
associated_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r16-t4.1-t5.1
associated_with
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r17-t5.1-t6.1
location_of
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r18-t3.1-t5.1
evaluation_of
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r19-t4.1-t3.1
associated_with
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r20-t6.1-t4.1
degree_of
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r21-t1.1-t3.1
co-occurs_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r22-t3.1-t6.1
degree_of
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r23-t4.1-t2.1
associated_with
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-r24-t4.1-t1.1
associated_with
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r25-t5.1-t6.1
associated_with
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r26-t1.1-t4.1
degree_of
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r27-t5.1-t4.1
associated_with
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r28-t3.1-t1.1
degree_of
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r29-t2.1-t3.1
co-occurs_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r30-t5.1-t4.1
location_of
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r31-t5.1-t3.1
associated_with
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r32-t5.1-t1.1
associated_with
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r33-t5.1-t3.1
location_of
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r34-t6.1-t5.1
associated_with
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r35-t6.1-t3.1
associated_with
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r36-t2.1-t5.1
evaluation_of
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r37-t6.1-t2.1
associated_with
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s2-r38-t1.1-t6.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s2-r39-t6.1-t1.1
associated_with
HNO.90471063.ger.abstr-s2-t6
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r40-t3.1-t1.1
co-occurs_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r41-t1.1-t5.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s2-r42-t1.1-t4.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s2-r43-t5.1-t1.1
location_of
HNO.90471063.ger.abstr-s2-t5
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s2-r44-t1.1-t3.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s2-r45-t1.1-t2.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s4-r1-t3.1-t4.1
location_of
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s4-r2-t4.1-t1.1
associated_with
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s4-r3-t3.1-t1.1
location_of
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s4-r4-t1.1-t4.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s4-t4
HNO.90471063.ger.abstr-s5-r1-t1.1-t3.1
uses
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s5-r2-t3.1-t2.1
interacts_with
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s5-r3-t1.1-t2.1
uses
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s5-r4-t2.1-t3.1
interacts_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t3
HNO.90471063.ger.abstr-s7-r1-t1.1-t1.2
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s7-t1
HNO.90471063.ger.abstr-s7-r2-t1.2-t1.1
associated_with
HNO.90471063.ger.abstr-s7-t1
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-r2-t2.2-t1.1
diagnoses
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-r3-t2.2-t1.1
indicates
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-r4-t2.1-t1.1
associated_with
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-r5-t1.1-t2.2
produces
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-r6-t1.1-t2.1
associated_with
HNO.90471063.ger.abstr-s8-t1
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-r7-t2.2-t1.1
affects
HNO.90471063.ger.abstr-s8-t2
HNO.90471063.ger.abstr-s8-t1