低ガンマグロブリン血症、低γグロブリン血症
- 関
- hypogammaglobulinemia、hypogammaglobulinemic
WordNet
- an abnormally low concentration of gamma globulin in the blood and increased risk of infection
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/26 04:33:30」(JST)
[Wiki en表示]
| Hypogammaglobulinemia |
| Classification and external resources |
| ICD-10 |
D80.0-D80.1 |
| ICD-9 |
279.00 |
| DiseasesDB |
6426 |
| MedlinePlus |
001307 |
| eMedicine |
med/1120 ped/54 |
| Patient UK |
Hypogammaglobulinemia |
| MeSH |
D000361 |
Hypogammaglobulinemia is a type of primary immune deficiency disease.[1]
Hypogammaglobulinemia is a characteristic of common variable immunodeficiency.[2]
Contents
- 1 Terminology
- 2 Types
- 3 References
- 4 External links
Terminology
"Hypogammaglobulinemia" is largely synonymous with "agammaglobulinemia". When the latter term is used (as in "X-linked agammaglobulinemia") it implies that gamma globulins are not merely reduced, but completely absent. Modern assays have allowed most agammaglobulinemias to be more precisely defined as hypogammaglobulinemias,[3] but the distinction is not usually clinically relevant.
"Hypogammaglobulinemia" is distinguished from dysgammaglobulinemia, which is a reduction in some types of gamma globulins, but not others.[4][5]
Types
| Type |
OMIM |
Gene |
| AGM1 |
601495 |
IGHM |
| AGM2 |
613500 |
IGLL1 |
| AGM3 |
613501 |
CD79A |
| AGM4 |
613502 |
BLNK |
| AGM5 |
613506 |
LRRC8A |
| AGM6 |
612692 |
CD79B |
References
- ^ "hypogammaglobulinemia" at Dorland's Medical Dictionary
- ^ "common variable immunodeficiency" at Dorland's Medical Dictionary
- ^ "agammaglobulinemia" at Dorland's Medical Dictionary
- ^ "Dysgammaglobulinemia" at Dorland's Medical Dictionary
- ^ ‹The template EMedicineDictionary is being considered for deletion.› Dysgammaglobulinemia at eMedicine Dictionary
External links
- "Hypogammaglobulinemia" at Dorland's Medical Dictionary
- Mark E. Rose & David M. Lang: "Evaluating and managing hypogammaglobulinemia", Cleveland Clinic Journal of Medicine, Vol. 73, No. 2 (February 2006), pp. 133–144. Accessed 2009-07-17.
- Robert Y Li, et al.: "Hypogammaglobulinemia", Medscape. Accessed 2009-07-17.
- Saul Greenberg: "Hypogammaglobulinemia ". Accessed 2009-07-17.
|
Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4)
|
|
| Primary |
| Antibody/humoral (B) |
| Hypogammaglobulinemia |
- X-linked agammaglobulinemia
- Transient hypogammaglobulinemia of infancy
|
|
| Dysgammaglobulinemia |
- IgA deficiency
- IgG deficiency
- IgM deficiency
- Hyper IgM syndrome (2
- 3
- 4
- 5)
- Wiskott-Aldrich syndrome
- Hyper-IgE syndrome
|
|
| Other |
- Common variable immunodeficiency
- ICF syndrome
|
|
|
| T cell deficiency (T) |
- thymic hypoplasia: hypoparathyroid (Di George's syndrome)
- euparathyroid (Nezelof syndrome
- Ataxia telangiectasia)
peripheral: Purine nucleoside phosphorylase deficiency
|
|
| Severe combined (B+T) |
- x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
- Omenn syndrome
- ZAP70 deficiency
- Bare lymphocyte syndrome
|
|
|
| Acquired |
|
|
Leukopenia:
Lymphocytopenia |
- Idiopathic CD4+ lymphocytopenia
|
|
| Complement deficiency |
- C1-inhibitor (Angioedema/Hereditary angioedema)
- Complement 2 deficiency/Complement 4 deficiency
- MBL deficiency
- Properdin deficiency
- Complement 3 deficiency
- Terminal complement pathway deficiency
- Paroxysmal nocturnal hemoglobinuria
- Complement receptor deficiency
|
|
|
Index of the immune system
|
|
| Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
| Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
| Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|
|
Disorders of globin and globulin proteins
|
|
| Globin |
- Hemoglobinopathy
- Thalassemia
- alpha
- beta
- delta
- Sickle-cell disease
- HPFH
|
|
| Globulin |
|
|
| Serpin |
- Serpinopathy: Alpha 1-antitrypsin deficiency
- Antithrombin III deficiency
- Hereditary angioedema
- FENIB
|
|
- See also
- globular proteins
- globins
- antibodies
- serpins
|
Index of cells
|
|
| Description |
- Structure
- Organelles
- peroxisome
- cytoskeleton
- centrosome
- epithelia
- cilia
- mitochondria
- Membranes
- Membrane transport
- ion channels
- vesicular transport
- solute carrier
- ABC transporters
- ATPase
- oxidoreduction-driven
|
|
| Disease |
- Structural
- peroxisome
- cytoskeleton
- cilia
- mitochondria
- nucleus
- scleroprotein
- Membrane
- channelopathy
- solute carrier
- ATPase
- ABC transporters
- other
- extracellular ligands
- cell surface receptors
- intracellular signalling
- Vesicular transport
- Pore-forming toxins
|
|
|
|
Cell surface receptor deficiencies
|
|
G protein-coupled receptor
(including hormone) |
| Class A |
- TSHR (Congenital hypothyroidism 1)
- LHCGR (Male-limited precocious puberty)
- FSHR (XX gonadal dysgenesis)
- EDNRB (ABCD syndrome, Waardenburg syndrome 4a, Hirschsprung's disease 2)
- AVPR2 (Nephrogenic diabetes insipidus 1)
- PTGER2 (Aspirin-induced asthma)
|
|
| Class B |
- PTH1R (Jansen's metaphyseal chondrodysplasia)
|
|
| Class C |
- CASR (Familial hypocalciuric hypercalcemia)
|
|
| Class F |
- FZD4 (Familial exudative vitreoretinopathy 1)
|
|
|
Enzyme-linked receptor
(including
growth factor) |
| RTK |
- ROR2 (Robinow syndrome)
- FGFR1 (Pfeiffer syndrome, KAL2 Kallmann syndrome)
- FGFR2 (Apert syndrome, Antley–Bixler syndrome, Pfeiffer syndrome, Crouzon syndrome, Jackson–Weiss syndrome)
- FGFR3 (Achondroplasia, Hypochondroplasia, Thanatophoric dysplasia, Muenke syndrome)
- INSR (Donohue syndrome
- Rabson–Mendenhall syndrome)
- NTRK1 (Congenital insensitivity to pain with anhidrosis)
- KIT (KIT Piebaldism, Gastrointestinal stromal tumor)
|
|
| STPK |
- AMHR2 (Persistent Müllerian duct syndrome II)
- TGF beta receptors: Endoglin/Alk-1/SMAD4 (Hereditary hemorrhagic telangiectasia)
- TGFBR1/TGFBR2 (Loeys-Dietz syndrome)
|
|
| GC |
- GUCY2D (Leber's congenital amaurosis 1)
|
|
|
| JAK-STAT |
- Type I cytokine receptor: GH (Laron syndrome)
- CSF2RA (Surfactant metabolism dysfunction 4)
- MPL (Congenital amegakaryocytic thrombocytopenia)
|
|
| TNF receptor |
- TNFRSF1A (TNF receptor associated periodic syndrome)
- TNFRSF13B (Selective immunoglobulin A deficiency 2)
- TNFRSF5 (Hyper-IgM syndrome type 3)
- TNFRSF13C (CVID4)
- TNFRSF13B (CVID2)
- TNFRSF6 (Autoimmune lymphoproliferative syndrome 1A)
|
|
| Lipid receptor |
- LRP: LRP2 (Donnai–Barrow syndrome)
- LRP4 (Cenani–Lenz syndactylism)
- LRP5 (Worth syndrome, Familial exudative vitreoretinopathy 4, Osteopetrosis 1)
- LDLR (LDLR Familial hypercholesterolemia)
|
|
| Other/ungrouped |
- Immunoglobulin superfamily: AGM3, 6
- Integrin: LAD1
- Glanzmann's thrombasthenia
- Junctional epidermolysis bullosa with pyloric atresia
EDAR (EDAR Hypohidrotic ectodermal dysplasia)
- PTCH1 (Nevoid basal cell carcinoma syndrome)
- BMPR1A (BMPR1A Juvenile polyposis syndrome)
- IL2RG (X-linked severe combined immunodeficiency)
|
|
- See also
- cell surface receptors
|
Index of cells
|
|
| Description |
- Structure
- Organelles
- peroxisome
- cytoskeleton
- centrosome
- epithelia
- cilia
- mitochondria
- Membranes
- Membrane transport
- ion channels
- vesicular transport
- solute carrier
- ABC transporters
- ATPase
- oxidoreduction-driven
|
|
| Disease |
- Structural
- peroxisome
- cytoskeleton
- cilia
- mitochondria
- nucleus
- scleroprotein
- Membrane
- channelopathy
- solute carrier
- ATPase
- ABC transporters
- other
- extracellular ligands
- cell surface receptors
- intracellular signalling
- Vesicular transport
- Pore-forming toxins
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin.
- Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R.SourceDepartment of Virology and Immunology, Auckland City Hospital, Auckland, New Zealand; Department of Clinical Immunology, Auckland City Hospital, Auckland, New Zealand.
- Clinical and experimental immunology.Clin Exp Immunol.2013 Nov;174(2):203-11. doi: 10.1111/cei.12178.
- Common variable immune deficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. The standard of care is intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (scIG) therapy. The cause of CVID is currently unknown, and there is no universally accepted definit
- PMID 23859429
- Heterogeneous clinical presentation in ICF syndrome: correlation with underlying gene defects.
- Weemaes CM, van Tol MJ, Wang J, van Ostaijen-Ten Dam MM, van Eggermond MC, Thijssen PE, Aytekin C, Brunetti-Pierri N, van der Burg M, Graham Davies E, Ferster A, Furthner D, Gimelli G, Gennery A, Kloeckener-Gruissem B, Meyn S, Powell C, Reisli I, Schuetz C, Schulz A, Shugar A, van den Elsen PJ, van der Maarel SM.SourceDepartment of Pediatric Infectious Diseases and Immunology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
- European journal of human genetics : EJHG.Eur J Hum Genet.2013 Nov;21(11):1219-25. doi: 10.1038/ejhg.2013.40. Epub 2013 Mar 13.
- Immunodeficiency with centromeric instability and facial anomalies (ICF) syndrome is a primary immunodeficiency, predominantly characterized by agammaglobulinemia or hypoimmunoglobulinemia, centromere instability and facial anomalies. Mutations in two genes have been discovered to cause ICF syndrome
- PMID 23486536
- Hypogammaglobulinemia in pediatric systemic lupus erythematosus.
- Lim E, Tao Y, White A, French A, Cooper M.Source1Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, MO, USA; and.
- Lupus.Lupus.2013 Oct 8. [Epub ahead of print]
- ObjectiveSystemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospe
- PMID 24106215
Japanese Journal
- Hypogammaglobulinaemia with absent B lymphocytes and agranulocytosis after carbamazepine treatment
- Nodular lymphoid hyperplasia of the bowel in primary hypogammaglobulinaemia : Study of in vivo and in vitro lymphocyte function
- Nodular lymphoid hyperplasia with hypogammaglobulinaemia
Related Links
- Hypogammaglobulinaemia hypogammaglobulinemia hypogammaglobulinemia hypogammaglobulinemia hypogammaglobulinemia hypogammagloulinaemia due to TACI deficiency Hypogammglobulinaemia hypoganglionosis ...
- What drug can cause Hypogammaglobulinaemia as their side effect? Check drug and medication side effect reports associated with Hypogammaglobulinaemia ... All medicines have benefits and risks. The risks of medicines are the ...
Related Pictures
★リンクテーブル★
[★]
- 英
- hypogammaglobulinemia、hypogammaglobulinaemia
- 関
- 低ガンマグロブリン症、低ガンマグロブリン血症
- 無γグロブリン血症 agammaglobulinemia
[★]
- 関
- hypogammaglobulinaemia、hypogammaglobulinemia