Clinical phenotype of common variable immunodeficiency: a single centre analysis of 140 patients
Gespeichert in:
| 1. Verfasser: | |
|---|---|
| Format: | Abschlussarbeit Buch |
| Sprache: | English |
| Veröffentlicht: |
Freiburg im Breisgau
2020
|
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis Inhaltsverzeichnis |
| Beschreibung: | IX, 92 Seiten Diagramme 30 cm |
Internformat
MARC
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| 100 | 1 | |a Pietsch, Larissa |e Verfasser |0 (DE-588)1211991806 |4 aut | |
| 245 | 1 | 0 | |a Clinical phenotype of common variable immunodeficiency |b a single centre analysis of 140 patients |c vorgelegt von Larissa Monika Pietsch |
| 264 | 1 | |a Freiburg im Breisgau |c 2020 | |
| 300 | |a IX, 92 Seiten |b Diagramme |c 30 cm | ||
| 336 | |b txt |2 rdacontent | ||
| 337 | |b n |2 rdamedia | ||
| 338 | |b nc |2 rdacarrier | ||
| 502 | |b Dissertation |c Albert-Ludwigs-Universität Freiburg im Breisgau |d 2020 | ||
| 655 | 7 | |0 (DE-588)4113937-9 |a Hochschulschrift |2 gnd-content | |
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Datensatz im Suchindex
| _version_ | 1804181617297588224 |
|---|---|
| adam_text | CONTENTS
ABBREVIATIONS
..............................................................................................................................
VIII
1
INTRODUCTION
...............................................................................................................................
1
1.1
DEFINITION
AND
DIAGNOSIS
OF
CVID
....................................................................................
1
1.2
PATHOGENESIS
OF
CVID
........................................................................................................
6
1.3
GENETIC
BACKGROUND
OF
CVID
...........................................................................................6
1.3.1
ICOS
................................................................................................................................
8
1.3.2
TACI
AND
BAFF-R
DEFICIENCY
......................................................................................
8
1.3.3
PIK3CD
..........................................................................................................................
9
1.3.4
CTLA4
...........................................................................................................................
9
1.3.5
NFKB2
DEFICIENCY
.........................................................................................................
9
1.4
CLINICAL
SPECTRUM
OF
CVID
.............................................................................................
10
1.4.1
INFECTIONS
......................................................................................................................
10
1.4.2
RESPIRATORY
COMPLICATIONS
..........................................................................................
10
1.4.3
AUTOIMMUNITY
..............................................................................................................
10
1.4.4
CVID-ENTEROPATHY
........................................................................................................
11
1.4.5
LYMPHOPROLIFERATIVE
DISORDERS
....................................................................................
11
1.5
LABORATORY
CHANGES
IN
CVID
...........................................................................................
12
1.6
TREATMENT
OF
CVID
.........................................................................................................
12
1.6.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
......................................................................
12
1.6.2
ANTIBIOTICS
....................................................................................................................
12
1.6.3
IMMUNOMODULATING
MEDICATIONS
................................................................................
13
1.7
SCORING
SYSTEMS
................................................................................................................
14
1.7.1
SEVERITY
SCORE
...............................................................................................................
14
1.7.2
TREATMENT
SCORE
............................................................................................................
14
2
PATIENTS,
MATERIAL
AND
METHODS
...........................................................................................
16
2.1
STUDY
DESIGN
.......................................................................................................................
16
2.2
ETHICAL
APPROVAL
AND
CONSENT
FORMS
..............................................................................
16
2.3
SELECTION
OF
PATIENTS
........................................................................................................
16
2.4
DATA
COLLECTION
..................................................................................................................
16
2.4.1
COLLECTION
OF
DATA
IN
THE
DEPARTMENTAL
DATABASE
.......................................................
17
2.4.2
SCORE
ASSESSMENT
.........................................................................................................
18
2.5
STATISTICS
...........................................................................................................................
18
V
3
RESULTS
......................................................................................................................................19
3.1
DEMOGRAPHICS
AND
DIAGNOSIS
............................................................................................
19
3.2
AGE
AT
ONSET
OF
CVID
.......................................................................................................
19
3.3
AGE
AT
DIAGNOSIS
AND
DIAGNOSTIC
DELAY
..........................................................................
21
3.4
PRESENTING
SYMPTOMS
.......................................................................................................
26
3.5
SERUM
IMMUNOGLOBULIN
LEVELS
AT
DIAGNOSIS
..................................................................
29
3.6
INFECTIONS
..........................................................................................................................
30
3.6.1
NUMBER
OF
INFECTIONS
PER
SIX
MONTHS
PERIOD
...........................................................
30
3.6.2
SERIOUS
INFECTIONS
PER
LIFETIME
...................................................................................
31
3.7
RESPIRATORY
COMPLICATIONS
...............................................................................................
31
3.8
AUTOIMMUNITY
..................................................................................................................32
3.9
CVID-ENTEROPATHY
.............................................
33
3.10
LYMPHOPROLIFERATIVE
DISORDERS
.......................................................................................
34
3.11
BASIC
LABORATORY
VALUES
IN
CVID
...................................................................................
34
3.12
ASSOCIATIONS
BETWEEN
CVID
COMPLICATIONS
.................................................................
35
3.12.1
ASSOCIATION
BETWEEN
COMMON
INFECTIONS
AND
CVID
COMPLICATIONS
.................
37
3.13
TREATMENT
.........................................................................................................................
37
3.13.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
.................................................................
37
3.13.2
ANTIBIOTICS
................................................................................................................
38
3.13.3
IMMUNOMODULATING
DRUGS
......................................................................................
40
3.14
QUALITY
OF
LIFE
...................................................................................................................
44
3.14.1
ASSOCIATION
BETWEEN
CVID
COMPLICATIONS
AND
QUALITY
OF
LIFE
...........................
46
3.14.2
ASSOCIATIONS
BETWEEN
CVID
TREATMENTS
AND
QUALITY
OF
LIFE
...............................
48
3.15
CVID
SCORES
....................................................................................................................
50
3.15.1
SEVERITY
SCORE
...........................................................................................................
50
3.15.2
TREATMENT
SCORE
.......................................................................................................
53
3.15.3
SCORE
CORRELATIONS
....................................................................................................
56
3.15.3.1
CORRELATIONS
BETWEEN
SCORES
AND
QUALITY
OF
LIFE
MEASURES
.
56
3.15.3.2
CORRELATIONS
BETWEEN
SCORES
AND
IMMUNOGLOBULIN
LEVELS
AT
DIAGNOSIS
....
56
3.15.3.3
CORRELATIONS
BETWEEN
SCORES
AND
LABORATORY
VALUES
.57
4
DISCUSSION
.................................................................................................................................
58
4.1
DISCUSSION
OF
METHODS
....................................................................................................58
4.1.1
CVID
DIAGNOSTIC
CRITERIA
............................................................................................
58
4.1.2
LIMITATIONS
...................................................................................................................
58
VI
4.2
DISCUSSION
OF
RESULTS
.....................................................................................................59
4.2.1
DEMOGRAPHICS
AND
DIAGNOSTIC
DELAY
............................................................................
59
4.2.2
CVID
DIAGNOSIS
...........................................................................................................
60
4.2.3
CVID
COMPLICATIONS
....................................................................................................
61
4.2.3.1
INFECTIONS
..............................................................................................................
61
4.2.3.2
RESPIRATORY
COMPLICATIONS
..................................................................................
61
4.2.3.3
AUTOIMMUNITY
......................................................................................................62
4.2.3.4
CVID-ENTEROPATHY
...............................................................................................
62
4.2.3.5
LYMPHOPROLIFERATIVE
DISORDERS
...........................................................................
63
4.2.3.6
ASSOCIATIONS
BETWEEN
CVID
COMPLICATIONS
......................................................
63
4.2.4
LABORATORY
CHANGES
IN
CVID
.....................................................................................
64
4.2.5
TREATMENTS
....................................................................................................................
64
4.2.5.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
..............................................................
64
4.2.5.2
PROPHYLACTIC
ANTIBIOTICS
.......................................................................................
65
4.2.53
IMMUNOMODULATING
MEDICATION
.........................................................................
65
4.2.6
QUALITY
OF
LIFE
...............................................................................................................
66
4.2.7
SEVERITY
SCORING
SYSTEMS
IN
CVID
............................................................................67
4.2.7.1
ASSOCIATIONS
BETWEEN
SCORES
AND
QUALITY
OF
LIFE
AND
LABORATORY
VALUES
.......68
4.2.7.2
LIMITATIONS
OF
SEVERITY
SCORES
...........................................................................68
5
SUMMARY
..................................................................................................................................
69
6
ZUSAMMENFASSUNG
..................................................................................................................
70
REFERENCES
........................................................................................................................................
71
FIGURES
...............................................................................................................................................
78
TABLES
.................................................................................................................................................
80
SUPPLEMENTS
..........................................................................................................
81
SUPPLEMENTARY
TABLES
...................................................................................................................
81
PATIENT
INTERVIEW
QUESTIONNAIRE
...................................................................
85
ERKLARUNG
ZUM
EIGENANTEIL
..........................................................................................................
89
EIDESSTATTLICHE
VERSICHERUNG
........................................................................................................
90
DANKSAGUNG
......................................................................................................................................
91
LEBENSLAUF
........................................................................................................................................92
VII
|
| adam_txt |
CONTENTS
ABBREVIATIONS
.
VIII
1
INTRODUCTION
.
1
1.1
DEFINITION
AND
DIAGNOSIS
OF
CVID
.
1
1.2
PATHOGENESIS
OF
CVID
.
6
1.3
GENETIC
BACKGROUND
OF
CVID
.6
1.3.1
ICOS
.
8
1.3.2
TACI
AND
BAFF-R
DEFICIENCY
.
8
1.3.3
PIK3CD
.
9
1.3.4
CTLA4
.
9
1.3.5
NFKB2
DEFICIENCY
.
9
1.4
CLINICAL
SPECTRUM
OF
CVID
.
10
1.4.1
INFECTIONS
.
10
1.4.2
RESPIRATORY
COMPLICATIONS
.
10
1.4.3
AUTOIMMUNITY
.
10
1.4.4
CVID-ENTEROPATHY
.
11
1.4.5
LYMPHOPROLIFERATIVE
DISORDERS
.
11
1.5
LABORATORY
CHANGES
IN
CVID
.
12
1.6
TREATMENT
OF
CVID
.
12
1.6.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
.
12
1.6.2
ANTIBIOTICS
.
12
1.6.3
IMMUNOMODULATING
MEDICATIONS
.
13
1.7
SCORING
SYSTEMS
.
14
1.7.1
SEVERITY
SCORE
.
14
1.7.2
TREATMENT
SCORE
.
14
2
PATIENTS,
MATERIAL
AND
METHODS
.
16
2.1
STUDY
DESIGN
.
16
2.2
ETHICAL
APPROVAL
AND
CONSENT
FORMS
.
16
2.3
SELECTION
OF
PATIENTS
.
16
2.4
DATA
COLLECTION
.
16
2.4.1
COLLECTION
OF
DATA
IN
THE
DEPARTMENTAL
DATABASE
.
17
2.4.2
SCORE
ASSESSMENT
.
18
2.5
STATISTICS
.
18
V
3
RESULTS
.19
3.1
DEMOGRAPHICS
AND
DIAGNOSIS
.
19
3.2
AGE
AT
ONSET
OF
CVID
.
19
3.3
AGE
AT
DIAGNOSIS
AND
DIAGNOSTIC
DELAY
.
21
3.4
PRESENTING
SYMPTOMS
.
26
3.5
SERUM
IMMUNOGLOBULIN
LEVELS
AT
DIAGNOSIS
.
29
3.6
INFECTIONS
.
30
3.6.1
NUMBER
OF
INFECTIONS
PER
SIX
MONTHS
PERIOD
.
30
3.6.2
SERIOUS
INFECTIONS
PER
LIFETIME
.
31
3.7
RESPIRATORY
COMPLICATIONS
.
31
3.8
AUTOIMMUNITY
.32
3.9
CVID-ENTEROPATHY
.
33
3.10
LYMPHOPROLIFERATIVE
DISORDERS
.
34
3.11
BASIC
LABORATORY
VALUES
IN
CVID
.
34
3.12
ASSOCIATIONS
BETWEEN
CVID
COMPLICATIONS
.
35
3.12.1
ASSOCIATION
BETWEEN
COMMON
INFECTIONS
AND
CVID
COMPLICATIONS
.
37
3.13
TREATMENT
.
37
3.13.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
.
37
3.13.2
ANTIBIOTICS
.
38
3.13.3
IMMUNOMODULATING
DRUGS
.
40
3.14
QUALITY
OF
LIFE
.
44
3.14.1
ASSOCIATION
BETWEEN
CVID
COMPLICATIONS
AND
QUALITY
OF
LIFE
.
46
3.14.2
ASSOCIATIONS
BETWEEN
CVID
TREATMENTS
AND
QUALITY
OF
LIFE
.
48
3.15
CVID
SCORES
.
50
3.15.1
SEVERITY
SCORE
.
50
3.15.2
TREATMENT
SCORE
.
53
3.15.3
SCORE
CORRELATIONS
.
56
3.15.3.1
CORRELATIONS
BETWEEN
SCORES
AND
QUALITY
OF
LIFE
MEASURES
.
56
3.15.3.2
CORRELATIONS
BETWEEN
SCORES
AND
IMMUNOGLOBULIN
LEVELS
AT
DIAGNOSIS
.
56
3.15.3.3
CORRELATIONS
BETWEEN
SCORES
AND
LABORATORY
VALUES
.57
4
DISCUSSION
.
58
4.1
DISCUSSION
OF
METHODS
.58
4.1.1
CVID
DIAGNOSTIC
CRITERIA
.
58
4.1.2
LIMITATIONS
.
58
VI
4.2
DISCUSSION
OF
RESULTS
.59
4.2.1
DEMOGRAPHICS
AND
DIAGNOSTIC
DELAY
.
59
4.2.2
CVID
DIAGNOSIS
.
60
4.2.3
CVID
COMPLICATIONS
.
61
4.2.3.1
INFECTIONS
.
61
4.2.3.2
RESPIRATORY
COMPLICATIONS
.
61
4.2.3.3
AUTOIMMUNITY
.62
4.2.3.4
CVID-ENTEROPATHY
.
62
4.2.3.5
LYMPHOPROLIFERATIVE
DISORDERS
.
63
4.2.3.6
ASSOCIATIONS
BETWEEN
CVID
COMPLICATIONS
.
63
4.2.4
LABORATORY
CHANGES
IN
CVID
.
64
4.2.5
TREATMENTS
.
64
4.2.5.1
IMMUNOGLOBULIN
REPLACEMENT
THERAPY
.
64
4.2.5.2
PROPHYLACTIC
ANTIBIOTICS
.
65
4.2.53
IMMUNOMODULATING
MEDICATION
.
65
4.2.6
QUALITY
OF
LIFE
.
66
4.2.7
SEVERITY
SCORING
SYSTEMS
IN
CVID
.67
4.2.7.1
ASSOCIATIONS
BETWEEN
SCORES
AND
QUALITY
OF
LIFE
AND
LABORATORY
VALUES
.68
4.2.7.2
LIMITATIONS
OF
SEVERITY
SCORES
.68
5
SUMMARY
.
69
6
ZUSAMMENFASSUNG
.
70
REFERENCES
.
71
FIGURES
.
78
TABLES
.
80
SUPPLEMENTS
.
81
SUPPLEMENTARY
TABLES
.
81
PATIENT
INTERVIEW
QUESTIONNAIRE
.
85
ERKLARUNG
ZUM
EIGENANTEIL
.
89
EIDESSTATTLICHE
VERSICHERUNG
.
90
DANKSAGUNG
.
91
LEBENSLAUF
.92
VII |
| any_adam_object | 1 |
| any_adam_object_boolean | 1 |
| author | Pietsch, Larissa |
| author_GND | (DE-588)1211991806 |
| author_facet | Pietsch, Larissa |
| author_role | aut |
| author_sort | Pietsch, Larissa |
| author_variant | l p lp |
| building | Verbundindex |
| bvnumber | BV046811479 |
| ctrlnum | (OCoLC)1190907933 (DE-599)KXP1700614576 |
| discipline | Medizin |
| discipline_str_mv | Medizin |
| format | Thesis Book |
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| id | DE-604.BV046811479 |
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| index_date | 2024-07-03T14:58:57Z |
| indexdate | 2024-07-10T08:54:30Z |
| institution | BVB |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-032220023 |
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| owner_facet | DE-355 DE-BY-UBR |
| physical | IX, 92 Seiten Diagramme 30 cm |
| publishDate | 2020 |
| publishDateSearch | 2020 |
| publishDateSort | 2020 |
| record_format | marc |
| spelling | Pietsch, Larissa Verfasser (DE-588)1211991806 aut Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients vorgelegt von Larissa Monika Pietsch Freiburg im Breisgau 2020 IX, 92 Seiten Diagramme 30 cm txt rdacontent n rdamedia nc rdacarrier Dissertation Albert-Ludwigs-Universität Freiburg im Breisgau 2020 (DE-588)4113937-9 Hochschulschrift gnd-content B:DE-101 application/pdf https://d-nb.info/1213561051/04 Inhaltsverzeichnis DNB Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=032220023&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Pietsch, Larissa Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| subject_GND | (DE-588)4113937-9 |
| title | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| title_auth | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| title_exact_search | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| title_exact_search_txtP | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| title_full | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients vorgelegt von Larissa Monika Pietsch |
| title_fullStr | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients vorgelegt von Larissa Monika Pietsch |
| title_full_unstemmed | Clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients vorgelegt von Larissa Monika Pietsch |
| title_short | Clinical phenotype of common variable immunodeficiency |
| title_sort | clinical phenotype of common variable immunodeficiency a single centre analysis of 140 patients |
| title_sub | a single centre analysis of 140 patients |
| topic_facet | Hochschulschrift |
| url | https://d-nb.info/1213561051/04 http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=032220023&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| work_keys_str_mv | AT pietschlarissa clinicalphenotypeofcommonvariableimmunodeficiencyasinglecentreanalysisof140patients |
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Inhaltsverzeichnis