MCID: TRN009
MIFTS: 31

Transient Hypogammaglobulinemia of Infancy

Categories: Blood diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Transient Hypogammaglobulinemia of Infancy

MalaCards integrated aliases for Transient Hypogammaglobulinemia of Infancy:

Name: Transient Hypogammaglobulinemia of Infancy 11 58 14 75
Thi - [transient Hypogammaglobulinaemia of Infancy] 33
Immunoglobulin Maturational Delay 33

Classifications:

Orphanet: 58  
Rare immunological diseases


External Ids:

Disease Ontology 11 DOID:624
NCIt 49 C27071
SNOMED-CT 68 88714009
ICD10 31 D80.7
ICD10 via Orphanet 32 D80.7
UMLS via Orphanet 72 C0272238
Orphanet 58 ORPHA169139
ICD11 33 1686370790
UMLS 71 C0272238

Summaries for Transient Hypogammaglobulinemia of Infancy

Orphanet: 58 A rare primary immunodeficiency characterized by a delay in the maturation of immunoglobulin production, leading to prolongation of the physiologic hypogammaglobulinemia of the newborn period beyond six months of age. Patients present recurrent respiratory infections, otitis media, bronchitis, gastroenteritis, or allergic symptoms in the first two to four years of life, before the condition resolves spontaneously. Some children may remain asymptomatic, and severe or life-threatening infections are rare. The capacity to synthesize specific antibodies in response to vaccines is usually normal.

MalaCards based summary: Transient Hypogammaglobulinemia of Infancy, also known as thi - [transient hypogammaglobulinaemia of infancy], is related to agammaglobulinemia and primary agammaglobulinemia. An important gene associated with Transient Hypogammaglobulinemia of Infancy is RNU4ATAC (RNA, U4atac Small Nuclear), and among its related pathways/superpathways are Innate Immune System and Akt Signaling. Affiliated tissues include b cells and t cells, and related phenotypes are immune system and hematopoietic system

Wikipedia: 75 Transient hypogammaglobulinemia of infancy is a form of hypogammaglobulinemia appearing after birth,... more...

Related Diseases for Transient Hypogammaglobulinemia of Infancy

Diseases in the Transient Hypogammaglobulinemia family:

Congenital Hypogammaglobulinemia Transient Hypogammaglobulinemia of Infancy

Diseases related to Transient Hypogammaglobulinemia of Infancy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 91)
# Related Disease Score Top Affiliating Genes
1 agammaglobulinemia 30.5 CR2 CD40LG CD19 BTK
2 primary agammaglobulinemia 30.4 TNFRSF13C TNFRSF13B
3 agammaglobulinemia, x-linked 30.2 TNFRSF13B LRBA CD40LG CD19 BTK
4 dysgammaglobulinemia 30.1 TNFRSF13B LRBA
5 autoimmune disease 29.7 TNFRSF13C TNFRSF13B CR2 CD40LG
6 immune deficiency disease 29.6 TNFRSF13B CR2 CD40LG CD27 BTK
7 immunoglobulin alpha deficiency 29.2 TNFRSF13C TNFRSF13B MSH5 LRBA CD40LG CD27
8 immunoglobulin a deficiency 1 29.1 TNFRSF13B RNU4ATAC MSH5 CD40LG BTK
9 transient hypogammaglobulinemia 28.9 TNFRSF13C TNFRSF13B RNU4ATAC MSH5 LRBA CR2
10 common variable immunodeficiency 27.8 TNFRSF13C TNFRSF13B NFKB2 MSH5 LRBA CR2
11 tetanus 10.3
12 haemophilus influenzae 10.3
13 selective igg deficiency disease 10.2 TNFRSF13C TNFRSF13B
14 ige responsiveness, atopic 10.2
15 otitis media 10.2
16 dermatitis, atopic 10.2
17 diphtheria 10.2
18 dermatitis 10.2
19 bronchitis 10.2
20 lymphoproliferative syndrome 2 10.1 TNFRSF13B CD27
21 lobomycosis 10.1 CR2 CD19
22 igg4-related disease 10.1 CD27 CD19
23 west nile encephalitis 10.1 RNU4ATAC CD19
24 asymmetric motor neuropathy 10.1 CD40LG CD27
25 immunodeficiency with hyper-igm, type 5 10.1 TNFRSF13B CD40LG
26 herpangina 10.1 RNU4ATAC CD27 CD19
27 chickenpox 10.1 RNU4ATAC CD27 CD19
28 good syndrome 10.1 TNFRSF13C TNFRSF13B CD19
29 thrombocytopenia due to platelet alloimmunization 10.1 TNFRSF13B CD19
30 immunodeficiency 14 10.0 LRBA CD27
31 smith-magenis syndrome 10.0
32 asthma 10.0
33 autoimmune disease 4 10.0
34 selective igm deficiency disease 10.0
35 pericarditis 10.0
36 gastroenteritis 10.0
37 pleural empyema 10.0
38 measles 10.0
39 rubella 10.0
40 viral infectious disease 10.0
41 bacterial meningitis 10.0
42 immunodeficiency, common variable, 1 10.0 TNFRSF13B NFKB2
43 esophageal tuberculosis 10.0 LRBA CR2
44 immunodeficiency with hyper-igm, type 2 10.0 TNFRSF13B CD40LG
45 selective immunoglobulin deficiency disease 10.0 TNFRSF13B LRBA CD27
46 immunodeficiency with hyper-igm, type 4 10.0 TNFRSF13C CD40LG CD27
47 immunodeficiency with hyper-igm, type 3 10.0 TNFRSF13C TNFRSF13B CD40LG
48 evans' syndrome 10.0 TNFRSF13B LRBA CD19
49 monoclonal gammopathy of uncertain significance 9.9 CD27 CD19
50 t cell deficiency 9.9 CD40LG CD27 CD19

Graphical network of the top 20 diseases related to Transient Hypogammaglobulinemia of Infancy:



Diseases related to Transient Hypogammaglobulinemia of Infancy

Symptoms & Phenotypes for Transient Hypogammaglobulinemia of Infancy

MGI Mouse Phenotypes related to Transient Hypogammaglobulinemia of Infancy:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.65 BTK CD19 CD27 CD40LG CR2 LRBA
2 hematopoietic system MP:0005397 9.32 BTK CD19 CD27 CD40LG CR2 LRBA

Drugs & Therapeutics for Transient Hypogammaglobulinemia of Infancy

Search Clinical Trials, NIH Clinical Center for Transient Hypogammaglobulinemia of Infancy

Genetic Tests for Transient Hypogammaglobulinemia of Infancy

Anatomical Context for Transient Hypogammaglobulinemia of Infancy

Organs/tissues related to Transient Hypogammaglobulinemia of Infancy:

MalaCards : B Cells, T Cells

Publications for Transient Hypogammaglobulinemia of Infancy

Articles related to Transient Hypogammaglobulinemia of Infancy:

(show top 50) (show all 70)
# Title Authors PMID Year
1
One-year intravenous immunoglobulin replacement therapy: efficacy in reducing hospital admissions in pediatric patients with Inborn Errors of Immunity. 62
34273274 2022
2
Regulatory T and B cells in transient hypogammaglobulinemia of infancy. 62
35611411 2022
3
Invasive Haemophilus influenzae type b infection in a patient with transient hypogammaglobulinemia of infancy. 62
34376350 2021
4
Transient hypogammaglobulinemia of infancy may be associated with reduced switched memory B cells and del (16) (p11.2p12). 62
34188919 2021
5
Vaccination in PADs. 62
34207916 2021
6
Diagnostic approach of hypogammaglobulinemia in infancy. 62
32017208 2020
7
B lymphocyte subsets and outcomes in patients with an initial diagnosis of transient hypogammaglobulinemia of infancy. 62
30152873 2018
8
[Transient hypogammaglobulinemia of infancy]. 62
28722362 2017
9
Decreased IgM, IgA, and IgG response to pneumococcal vaccine in children with transient hypogammaglobulinemia of infancy. 62
26233927 2016
10
Transient hypogammaglobulinemia and severe atopic dermatitis: Open-label treatment with immunoglobulin in a case series. 62
27470901 2016
11
Fcγ receptor polymorphisms in patients with transient hypogammaglobulinemia of infancy presenting with mild and severe infections. 62
26708396 2015
12
Transient hypogammaglobulinemia of infancy: predictive factors for late recovery. 62
27735798 2015
13
Severe atopic dermatitis associated with transient hypogammaglobulinemia of infancy. 62
25600656 2015
14
BCGitis in a patient with transient hypogammaglobulinemia of infancy. 62
23253103 2014
15
Quality of life in children with primary antibody deficiency. 62
25005831 2014
16
Neonatal Lupus or Not? An Intriguing Immunological Dilemma. 62
35923013 2014
17
Primary antibody deficiencies at Queen Rania Children Hospital in Jordan: single center experience. 62
24632588 2014
18
Phenotypic parameters predict time to normalization in infants with hypogammaglobulinemia. 62
24014079 2013
19
Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy. 62
22820757 2013
20
The elevated number of circulating regulatory T cells in patients with transient hypogammaglobulinemia of infancy is not associated with any abnormalities in the genes encoding the TGF-β receptors. 62
23899993 2013
21
Severe parainfluenza pneumonia in a case of transient hypogammalobulinemia of infancy. 62
23814123 2013
22
Primary Immunodeficiency May Be Misdiagnosed as Cow's Milk Allergy: Seven Cases Referred to a Tertiary Pediatric Hospital. 62
24198970 2013
23
Does intravenous immunoglobulin therapy prolong immunodeficiency in transient hypogammaglobulinemia of infancy? 62
24198926 2013
24
B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency. 62
23654075 2013
25
The efficacy of Pelargonium sidoides in the treatment of upper respiratory tract infections in children with transient hypogammaglobulinemia of infancy. 62
22809962 2012
26
The number of circulating CD4+ CD25high Foxp3+ T lymphocytes is transiently elevated in the early childhood of transient hypogammaglobulinemia of infancy patients. 62
21531630 2011
27
TNFRSF13B/TACI alterations in Greek patients with antibody deficiencies. 62
21547394 2011
28
Study of patients with Hyper-IgM type IV phenotype who recovered spontaneously during late childhood and review of the literature. 62
21274562 2011
29
New laboratory findings in Turkish patients with transient hypogammaglobulinemia of infancy. 62
21131704 2010
30
Infants with low immunoglobulin levels: Isolated low IgA level vs other immunoglobulin abnormalities. 62
20934629 2010
31
Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'similarities and differences'. 62
20609138 2010
32
Transient hypogammaglobulinemia of infancy: intravenous immunoglobulin as first line therapy. 62
20378022 2010
33
Clinical features and follow-up of Chinese patients with symptomatic hypogammaglobulinemia in infancy. 62
19781364 2009
34
The B-cell compartment in the peripheral blood of children with different types of primary humoral immunodeficiency. 62
19342988 2009
35
Massive empyema associated with transient hypogammaglobulinemia of infancy and IgA deficiency. 62
19399287 2009
36
A prospective study on children with initial diagnosis of transient hypogammaglobulinemia of infancy: results from the Italian Primary Immunodeficiency Network. 62
18547478 2008
37
Atopic dermatitis and transient hypogammaglobulinemia of infancy improved simultaneously. 62
17532847 2007
38
Two children with invasive gastrointestinal aspergillosis. 62
17536141 2007
39
The four most common pediatric immunodeficiencies. 62
17712988 2007
40
Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy. 62
17165264 2006
41
Infants presenting with recurrent infections and low immunoglobulins: characteristics and analysis of normalization. 62
16418798 2006
42
Transient hypogammaglobulinemia of infancy presenting as Staphylococcus aureus sepsis with deep neck infection. 62
15843860 2005
43
Symptomatic hypogammaglobulinemia in infancy and childhood - clinical outcome and in vitro immune responses. 62
15498106 2004
44
Transient hypogammaglobulinemia of infancy and early childhood: outcome of 30 cases. 62
15214739 2004
45
Immunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan. 62
15366655 2004
46
A case of IgG subclass deficiency with the initial presentation of transient hypogammaimmuno-globulinemia of infancy and a review of IgG subclass deficiencies. 62
12948264 2003
47
Spontaneous resolution of profound hypogammaglobulinemia. 62
11811863 2001
48
Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases. 62
11192522 2000
49
[Transient hypogammaglobulinemia of infancy]. 62
11212827 2000
50
[Cytokines in children with immunodeficiencies]. 62
10909468 1999

Variations for Transient Hypogammaglobulinemia of Infancy

Expression for Transient Hypogammaglobulinemia of Infancy

Search GEO for disease gene expression data for Transient Hypogammaglobulinemia of Infancy.

Pathways for Transient Hypogammaglobulinemia of Infancy

Pathways related to Transient Hypogammaglobulinemia of Infancy according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.52 BTK CD19 CD27 CD40LG CR2 NFKB2
2
Show member pathways
13.06 TNFRSF13C TNFRSF13B CD40LG CD27 BTK
3
Show member pathways
12.93 TNFRSF13C TNFRSF13B NFKB2 CD40LG CD27
4
Show member pathways
12.9 TNFRSF13C TNFRSF13B CD40LG CD27 BTK
5
Show member pathways
12.25 TNFRSF13C TNFRSF13B CD40LG CD27
6 12.14 NFKB2 CD40LG CD19 BTK
7
Show member pathways
11.59 NFKB2 CR2 CD19 BTK
8
Show member pathways
11.3 TNFRSF13B NFKB2 CD40LG
9 11.23 NFKB2 CD40LG
10
Show member pathways
11.21 TNFRSF13C TNFRSF13B NFKB2 CR2
11 11.18 CR2 CD40LG CD19
12
Show member pathways
11.09 CD19 BTK
13 10.97 CR2 CD19

GO Terms for Transient Hypogammaglobulinemia of Infancy

Biological processes related to Transient Hypogammaglobulinemia of Infancy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of B cell proliferation GO:0030889 9.56 TNFRSF13B BTK
2 immunoglobulin mediated immune response GO:0016064 9.46 CD27 CD19
3 positive regulation of B cell differentiation GO:0045579 9.26 CD27 BTK
4 immune system process GO:0002376 9.02 TNFRSF13C TNFRSF13B CR2 CD19 BTK

Sources for Transient Hypogammaglobulinemia of Infancy

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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