Humoral immunodeficiencies:
Gespeichert in:
| Format: | Buch |
|---|---|
| Sprache: | English |
| Veröffentlicht: |
Philadelphia [u.a.]
Saunders
2001
|
| Schriftenreihe: | Immunology and allergy clinics of North America
21,1 |
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | X, 190 S. Ill., graph. Darst. |
Internformat
MARC
| LEADER | 00000nam a2200000 cb4500 | ||
|---|---|---|---|
| 001 | BV013564984 | ||
| 003 | DE-604 | ||
| 005 | 00000000000000.0 | ||
| 007 | t | ||
| 008 | 010201s2001 ad|| |||| 00||| eng d | ||
| 035 | |a (OCoLC)45885119 | ||
| 035 | |a (DE-599)BVBBV013564984 | ||
| 040 | |a DE-604 |b ger |e rakwb | ||
| 041 | 0 | |a eng | |
| 049 | |a DE-355 |a DE-29 | ||
| 050 | 0 | |a QC983 | |
| 245 | 1 | 0 | |a Humoral immunodeficiencies |c Chaim M. Roifman, guest ed. |
| 264 | 1 | |a Philadelphia [u.a.] |b Saunders |c 2001 | |
| 300 | |a X, 190 S. |b Ill., graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
| 337 | |b n |2 rdamedia | ||
| 338 | |b nc |2 rdacarrier | ||
| 490 | 1 | |a Immunology and allergy clinics of North America |v 21,1 | |
| 650 | 2 | |a Déficits immunitaires | |
| 650 | 4 | |a Antibody Formation |x immunology | |
| 650 | 4 | |a Immunodeficiency | |
| 650 | 4 | |a Immunologic Deficiency Syndromes |x immunology | |
| 650 | 0 | 7 | |a Humorale Immunität |0 (DE-588)4160794-6 |2 gnd |9 rswk-swf |
| 650 | 0 | 7 | |a Immundefekt |0 (DE-588)4026622-9 |2 gnd |9 rswk-swf |
| 655 | 7 | |0 (DE-588)4143413-4 |a Aufsatzsammlung |2 gnd-content | |
| 689 | 0 | 0 | |a Humorale Immunität |0 (DE-588)4160794-6 |D s |
| 689 | 0 | 1 | |a Immundefekt |0 (DE-588)4026622-9 |D s |
| 689 | 0 | |5 DE-604 | |
| 700 | 1 | |a Roifman, Chaim M. |e Sonstige |4 oth | |
| 830 | 0 | |a Immunology and allergy clinics of North America |v 21,1 |w (DE-604)BV000645505 |9 21,1 | |
| 856 | 4 | 2 | |m HBZ Datenaustausch |q application/pdf |u http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009264367&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |3 Inhaltsverzeichnis |
| 999 | |a oai:aleph.bib-bvb.de:BVB01-009264367 | ||
Datensatz im Suchindex
| _version_ | 1804128365295173632 |
|---|---|
| adam_text | J1UMOKA1. lMMUNllDlvnCIKNCIlsS
CONTENTS
Preface xi
Common Variable Immunodeficiency 1
A. David B. Webster
The diagnosis of common variable immunodeficiency (CVID) re¬
mains unsatisfactory and depends on the exclusion of other
known single gene disorders causing hypoimmunoglobulinemia.
The pattern, however, in a substantial proportion of patients is
one of chronic immune activation, failure of T cell help for anti¬
body production, and a marked skewing toward a TH1 cytokine
profile manifested clinically as a widespread mucosal inflamma¬
tion and multiorgan granulomas. Genetic studies confirm that
many patients have a complex polygenic disease, with a major
susceptibility locus in the major histocompatibility complex on
chromosome 6. In general, the prognosis is good provided that
meticulous attention is paid to preventing and treating complica¬
tions such as Haemophilus influenzae colonization of the lungs,
mycoplasma infection in the joints, lungs, and urinary tract, and
enteroviral infection in the central nervous system. Although
steroids are useful for the unexplained chronic mucosal inflam¬
mation in the bowel and multisystem granulomas that are now a
major cause of morbidity and mortality, new therapeutic ap¬
proaches for these complications are needed.
X linked Agammaglobulinemia 23
Hubert B. Gaspar and Christine Kinnon
X linked agammaglobulinemia (XLA) is the prototypk humoral
immunodeficiency arising from a defect in B cell maturation and
the consequent failure of immunoglobulin production. The defec¬
tive gene in XLA has now been identified and termed BTK. In
IMMUNOUXiY AND ALLERGY CUNICS OF NORTH AMERICA
VOLUME 21 • NUMBER 1 • FEBRUARY 2001 vii
this article, the clinical manifestations of XLA and the role of Btk
protein in intracellular signaling and its interactions are dis¬
cussed. This discussion leads to an increased understanding of
the molecular mechanisms in the development of B cells and
has allowed the authors to develop more accurate methods for
diagnosis of humoral abnormalities.
Agammaglobulinemia Caused by Defects Other than
BTK 45
Eyal Grunebaum
In recent years, patients with an immunodeficiency comprised of
agammaglobulinemia and reduced circulating B cell numbers
were identified in which the pattern of inheritance, a lack of BTK
abnormality or a female sex, indicated that mutations in genes
other than BTK may result in a similar phenotype. Better under¬
standing of B cell development and function has allowed delinea¬
tion of some of these mutations, including the |x heavy chain, Iga
and surrogate chains, and BLNK adaptor protein. Further re¬
search in humans, and knowledge gained from animal models,
will help define additional causes of agammaglobulinemia.
CD154 Deficiency and Related Syndromes 65
Francisco A. Bonilla and Raif S. Geha
The X linked hyper IgM syndrome results from mutations in the
CD154 gene. The contact of CD154 with its ligand, CD40, under¬
lies a wide variety of immunoregulatory cellular dialogues. This
article provides an overview of the physiology of CD40 154 inter¬
actions and presents a detailed description of the clinical and
immunologic features of more than 100 reported cases of XHIM.
Aspects of the genetics, diagnosis, and treatment of XHIM and
the clinical and immunologic features of the autosomal recessive
forms of hyper IgM syndrome in comparison with XHIM are also
discussed.
Syndromic Immunodeficiencies with Humoral Defects 91
Jeffrey E. Ming and E. Richard Stiehm
Although infection is generally the predominant manifestation
in primary immunodeficiencies, immune defects may also be
associated with abnormalities in other organ systems, often as
part of recognizable syndromes. The authors term these condi¬
tions syndromic immunodeficiencies. Humoral (antibody) defects are
reported in association with growth deficiency, dermatologic ab¬
normalities, gastrointestinal anomalies, and chromosomal aberra¬
tions. The authors review the clinical manifestations and genetic
aspects of each syndrome and delineate the associated immune
defects. The authors focus on conditions with recent genetic ad¬
vances, including Nijmegen Breakage syndrome, ICF syndrome,
viii CONTENTS
and ataxia telangiectasia. Recognition of these conditions involv¬
ing the immune and other organ systems may facilitate accurate
diagnosis and management and yield information regarding
genes critical for the development of the involved systems.
Humoral Immunodeficiencies Associated with Bone
Dysplasias 113
Adelle R. Atkinson
During the last four decades, a growing number of descriptions
in the literature of immunodeficiencies have been associated with
a variety of bone dysplasias. This article describes the phenotypic
characteristics of B cell, T cell, and combined immunodeficiencies
found to be associated with bone dysplasias. This association has
led to a variety of hypotheses about a developmental relationship
between these two systems. The athymic murine model (nu/nu)
and the motheaten murine model (me/me) demonstrate abnormali¬
ties with T cell maturation and B cell differentiation, respectively.
Both models demonstrate bone abnormalities. Other models, in¬
cluding the microphthalmic mouse (mi) and the osteoprotegerin
ligand (OPGL) knockout mouse, also have bone abnormalities.
The PAX family of genes exhibits some developmental control
over skeletal and immunologic maturation and offers an area
worthy of investigation.
Hypogammaglobulinemia of Infancy 129
Ilan Dalai and Chaim M. Roifman
Although transient hypogammaglobulinemia of infancy (THI) has
been recognized for many years, little is known about many
aspects of this disorder, including the long term outcome. THI
likely represents a heterogeneous group of errors in the immune
system presenting with similar clinical and laboratory findings.
A major initial step in genotype identification is the careful analy¬
sis of the various phenotypes. The authors have identified three
different patterns of evolution in Ig concentrations and antibody
production in patients presenting with recurrent infections and
hypogammaglobulinemia in infancy and early childhood. In
group I, Ig and antibody production eventually normalized, but
the process may span over a decade and may include a transient
phase of IgG subclasses deficiency (only then can they be given
the diagnosis of THI). In group II, patients continued to have low
IgG levels and poor antibody production (consistent with CVID).
In group III, patients IgG levels normalized, but antibody levels
were transient (consistent with dysgammaglobulinemia).
Secondary Hypogammaglobulinemia 141
Elizabeth F. Jaffe, M. Christine Lejtenyi,
Francisco J. D. Noya, and Bruce D. Mazer
This article reviews the available data on a common but poorly
understood problem, secondary hypogammaglobulinemia. Pa
CONTENTS x
dents are found to have low immune globulins following an
infection, as a consequence of a disease, or as a side effect of a
medication. Yet the significance of these depressed immune glob¬
ulin levels is unclear. The authors review diseases of the kidneys
and gastrointestinal tract, malignancies, and rheumatologic disor¬
ders that have hypogammaglobulinemia as a frequent finding.
The authors also delineate viral infections and common medica¬
tions that can contribute to this problem. Finally, the approach to
the individual who has depressed serum IgG, with or without
infections, and how the patient should be evaluated and treated
is discussed.
Replacement Intravenous Immune Serum Globulin
Therapy in Patients with Antibody Immune Deficiency 165
Sutthi Thampakkul and Mark Ballow
Passive immunotherapy with plasma derived immunoglobulins
has been used successfully for the treatment of immunodeficiency
diseases since the 1950s. Intravenous immune serum globulin
(IVIG), available since the early 1980s, is a safe and effective
therapy in patients with humoral immune or antibody deficienc¬
ies. The rationale for the use of IVIG is the prevention of acute
and chronic infections. This article discusses the use of IVIG
for patients with immunodeficiency disorders. Detail of dosages,
various routes of administration, metabolism, products available
in the United States, and possible adverse effects of IVIG are
discussed.
Index 185
Subscription Information Inside back cover
X CONTENTS
|
| any_adam_object | 1 |
| building | Verbundindex |
| bvnumber | BV013564984 |
| callnumber-first | Q - Science |
| callnumber-label | QC983 |
| callnumber-raw | QC983 |
| callnumber-search | QC983 |
| callnumber-sort | QC 3983 |
| callnumber-subject | QC - Physics |
| ctrlnum | (OCoLC)45885119 (DE-599)BVBBV013564984 |
| format | Book |
| fullrecord | <?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01626nam a2200409 cb4500</leader><controlfield tag="001">BV013564984</controlfield><controlfield tag="003">DE-604</controlfield><controlfield tag="005">00000000000000.0</controlfield><controlfield tag="007">t</controlfield><controlfield tag="008">010201s2001 ad|| |||| 00||| eng d</controlfield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(OCoLC)45885119</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-599)BVBBV013564984</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-604</subfield><subfield code="b">ger</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1="0" ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="049" ind1=" " ind2=" "><subfield code="a">DE-355</subfield><subfield code="a">DE-29</subfield></datafield><datafield tag="050" ind1=" " ind2="0"><subfield code="a">QC983</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Humoral immunodeficiencies</subfield><subfield code="c">Chaim M. Roifman, guest ed.</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="a">Philadelphia [u.a.]</subfield><subfield code="b">Saunders</subfield><subfield code="c">2001</subfield></datafield><datafield tag="300" ind1=" " ind2=" "><subfield code="a">X, 190 S.</subfield><subfield code="b">Ill., graph. Darst.</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="b">n</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="b">nc</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="490" ind1="1" ind2=" "><subfield code="a">Immunology and allergy clinics of North America</subfield><subfield code="v">21,1</subfield></datafield><datafield tag="650" ind1=" " ind2="2"><subfield code="a">Déficits immunitaires</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Antibody Formation</subfield><subfield code="x">immunology</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Immunodeficiency</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Immunologic Deficiency Syndromes</subfield><subfield code="x">immunology</subfield></datafield><datafield tag="650" ind1="0" ind2="7"><subfield code="a">Humorale Immunität</subfield><subfield code="0">(DE-588)4160794-6</subfield><subfield code="2">gnd</subfield><subfield code="9">rswk-swf</subfield></datafield><datafield tag="650" ind1="0" ind2="7"><subfield code="a">Immundefekt</subfield><subfield code="0">(DE-588)4026622-9</subfield><subfield code="2">gnd</subfield><subfield code="9">rswk-swf</subfield></datafield><datafield tag="655" ind1=" " ind2="7"><subfield code="0">(DE-588)4143413-4</subfield><subfield code="a">Aufsatzsammlung</subfield><subfield code="2">gnd-content</subfield></datafield><datafield tag="689" ind1="0" ind2="0"><subfield code="a">Humorale Immunität</subfield><subfield code="0">(DE-588)4160794-6</subfield><subfield code="D">s</subfield></datafield><datafield tag="689" ind1="0" ind2="1"><subfield code="a">Immundefekt</subfield><subfield code="0">(DE-588)4026622-9</subfield><subfield code="D">s</subfield></datafield><datafield tag="689" ind1="0" ind2=" "><subfield code="5">DE-604</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Roifman, Chaim M.</subfield><subfield code="e">Sonstige</subfield><subfield code="4">oth</subfield></datafield><datafield tag="830" ind1=" " ind2="0"><subfield code="a">Immunology and allergy clinics of North America</subfield><subfield code="v">21,1</subfield><subfield code="w">(DE-604)BV000645505</subfield><subfield code="9">21,1</subfield></datafield><datafield tag="856" ind1="4" ind2="2"><subfield code="m">HBZ Datenaustausch</subfield><subfield code="q">application/pdf</subfield><subfield code="u">http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009264367&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA</subfield><subfield code="3">Inhaltsverzeichnis</subfield></datafield><datafield tag="999" ind1=" " ind2=" "><subfield code="a">oai:aleph.bib-bvb.de:BVB01-009264367</subfield></datafield></record></collection> |
| genre | (DE-588)4143413-4 Aufsatzsammlung gnd-content |
| genre_facet | Aufsatzsammlung |
| id | DE-604.BV013564984 |
| illustrated | Illustrated |
| indexdate | 2024-07-09T18:48:05Z |
| institution | BVB |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-009264367 |
| oclc_num | 45885119 |
| open_access_boolean | |
| owner | DE-355 DE-BY-UBR DE-29 |
| owner_facet | DE-355 DE-BY-UBR DE-29 |
| physical | X, 190 S. Ill., graph. Darst. |
| publishDate | 2001 |
| publishDateSearch | 2001 |
| publishDateSort | 2001 |
| publisher | Saunders |
| record_format | marc |
| series | Immunology and allergy clinics of North America |
| series2 | Immunology and allergy clinics of North America |
| spelling | Humoral immunodeficiencies Chaim M. Roifman, guest ed. Philadelphia [u.a.] Saunders 2001 X, 190 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Immunology and allergy clinics of North America 21,1 Déficits immunitaires Antibody Formation immunology Immunodeficiency Immunologic Deficiency Syndromes immunology Humorale Immunität (DE-588)4160794-6 gnd rswk-swf Immundefekt (DE-588)4026622-9 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Humorale Immunität (DE-588)4160794-6 s Immundefekt (DE-588)4026622-9 s DE-604 Roifman, Chaim M. Sonstige oth Immunology and allergy clinics of North America 21,1 (DE-604)BV000645505 21,1 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009264367&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Humoral immunodeficiencies Immunology and allergy clinics of North America Déficits immunitaires Antibody Formation immunology Immunodeficiency Immunologic Deficiency Syndromes immunology Humorale Immunität (DE-588)4160794-6 gnd Immundefekt (DE-588)4026622-9 gnd |
| subject_GND | (DE-588)4160794-6 (DE-588)4026622-9 (DE-588)4143413-4 |
| title | Humoral immunodeficiencies |
| title_auth | Humoral immunodeficiencies |
| title_exact_search | Humoral immunodeficiencies |
| title_full | Humoral immunodeficiencies Chaim M. Roifman, guest ed. |
| title_fullStr | Humoral immunodeficiencies Chaim M. Roifman, guest ed. |
| title_full_unstemmed | Humoral immunodeficiencies Chaim M. Roifman, guest ed. |
| title_short | Humoral immunodeficiencies |
| title_sort | humoral immunodeficiencies |
| topic | Déficits immunitaires Antibody Formation immunology Immunodeficiency Immunologic Deficiency Syndromes immunology Humorale Immunität (DE-588)4160794-6 gnd Immundefekt (DE-588)4026622-9 gnd |
| topic_facet | Déficits immunitaires Antibody Formation immunology Immunodeficiency Immunologic Deficiency Syndromes immunology Humorale Immunität Immundefekt Aufsatzsammlung |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009264367&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| volume_link | (DE-604)BV000645505 |
| work_keys_str_mv | AT roifmanchaimm humoralimmunodeficiencies |