DMTN
MCID: TRN032
MIFTS: 36

Transient Neonatal Diabetes Mellitus (DMTN) malady

Summaries for Transient Neonatal Diabetes Mellitus

Sources:
43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Transient neonatal diabetes mellitus (tndb) is a type of diabetes that appears within the first few weeks of life but is transient; affected infants go into remission within a few months, with possible relapse to permanent diabetes in adolescence or adulthood. affected individuals have slow growth before birth followed by hyperglycemia, dehydration and failure to thrive in infancy. approximately 70% of cases are caused by the overactivity of certain genes in a region of the long (q) arm of chromosome 6 called 6q24. these cases are referred to as 6q24-related tndb; most (but not all) of these cases are not inherited. other genetic causes include mutations in the kcnj11 and abcc8 genes, which usually cause permanent neonatal diabetes. treatment may include rehydration and intravenous insulin at the time of diagnosis, followed by subcutaneous insulin. last updated: 8/28/2012

MalaCards: Transient Neonatal Diabetes Mellitus, also known as diabetes mellitus, transient neonatal, is related to neonatal diabetes mellitus and diabetes mellitus, 6q24-related transient neonatal. An important gene associated with Transient Neonatal Diabetes Mellitus is PLAGL1 (pleiomorphic adenoma gene-like 1), and among its related pathways are Inwardly rectifying K+ channels and ATP sensitive Potassium channels. The compounds minoxidil and cibenzoline have been mentioned in the context of this disorder. Related mouse phenotype endocrine/exocrine gland.

Wikipedia:64 Transient neonatal diabetes mellitus (TNDM) is a form of diabetes mellitus presenting at birth that is... more...

Description from OMIM:47 601410

Aliases & Classifications for Transient Neonatal Diabetes Mellitus

Sources:
43NIH Rare Diseases, 47OMIM, 61UMLS
See all sources

Aliases & Descriptions:

transient neonatal diabetes mellitus 43
diabetes mellitus, transient neonatal 43 47
chromosome 6-associated transient diabetes mellitus 43
diabetes mellitus, 6q24-related transient neonatal 43
diabetes mellitus, transient neonatal, 1 61
dmtn 43
tndm 43


Related Diseases for Transient Neonatal Diabetes Mellitus

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the diabetes mellitus, 6q24-related transient neonatal family:

neonatal diabetes mellitus transient neonatal diabetes mellitus
diabetes mellitus, neonatal, with congenital hypothyroidism diabetes mellitus, transient neonatal 2
diabetes mellitus, transient neonatal, 3

Diseases related to Transient Neonatal Diabetes Mellitus via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 52)
idRelated DiseaseScoreTop Affiliating Genes
1neonatal diabetes mellitus31.6PLAGL1, ABCC8, INS, KCNJ11
2diabetes mellitus, 6q24-related transient neonatal31.1HYMAI, PLAGL1
3permanent neonatal diabetes mellitus30.3ABCC8, INS
4beckwith-wiedemann syndrome30.3ZFP57, ABCC8, PLAGL1
5macroglossia10.5
6n syndrome10.5
7abcc8-related transient neonatal diabetes mellitus 210.4
8diabetes mellitus, transient neonatal 210.4
9diabetes mellitus, transient neonatal, 310.4
10klinefelter's syndrome10.2
11diabetes mellitus, kcnj11-related transient neonatal10.2
12donohue syndrome10.0INS
13fetal macrosomia10.0INS
14morbid obesity10.0INS
15celiac disease10.0TPO
16myocardial infarction10.0TPO
17familial hyperinsulinism10.0ABCC8, KCNJ11
18goiter10.0TPO
19down syndrome10.0TPO
20dwarfism10.0IGF1
21sheehan syndrome10.0IGF1
22diabetic ketoacidosis10.0INS
23laron syndrome10.0IGF1
24hyperammonemia multi-gene panels10.0ABCC8, KCNJ11
25autonomic neuropathy10.0INS
26hyperinsulinemic hypoglycemia10.0ABCC8, INS
27silver-russell syndrome10.0ZFP57
28gestational diabetes10.0KCNJ11, INS, ABCC8
29insulinoma10.0KCNJ11, INS, ABCC8
30pituitary adenoma10.0ABCC8, IGF1
31rabson-mendenhall syndrome10.0INS, IGF1
32pituitary tumors10.0IGF1, PLAGL1
33epilepsy syndrome10.0ABCC8, ZACN, KCNJ11
34pituitary gland disease10.0IGF1, INS
35acromegaly10.0INS, IGF1
36type 1 diabetes10.0INS, TPO
37anovulation10.0IGF1, INS
38thyrotropin deficiency, isolated10.0TPO, IGF1
39acanthosis nigricans10.0IGF1, INS
40cystic fibrosis10.0ABCC8, INS, KCNJ11
41polycystic ovary syndrome10.0IGF1, INS
42turner syndrome10.0TPO, IGF1
43thyrotoxicosis10.0TPO, INS
44diabetes mellitus10.0ABCC8, INS, IGF1, KCNJ11
45glucose intolerance10.0KCNJ11, IGF1, INS, ABCC8
46hyperinsulinism10.0KCNJ11, IGF1, INS, ABCC8
47hypoglycemia10.0ABCC8, INS, IGF1, KCNJ11
48hyperthyroidism10.0TPO, INS, IGF1
49insulin resistance10.0IGF1, INS, ABCC8
50adenoma10.0IGF1, INS, PLAGL1, TPO

Graphical network of the top 20 diseases related to Transient Neonatal Diabetes Mellitus:



Diseases related to transient neonatal diabetes mellitus

Clinical Features for Transient Neonatal Diabetes Mellitus

Sources:
47OMIM
See all sources

Clinical features from OMIM:

601410

Drugs & Therapeutics for Transient Neonatal Diabetes Mellitus

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Transient Neonatal Diabetes Mellitus

Drug clinical trials:

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Search NIH Clinical Center for Transient Neonatal Diabetes Mellitus

Search CenterWatch for Transient Neonatal Diabetes Mellitus

Genetic Tests for Transient Neonatal Diabetes Mellitus

Anatomical Context for Transient Neonatal Diabetes Mellitus

Animal Models for Transient Neonatal Diabetes Mellitus or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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MGI Mouse Phenotypes related to Transient Neonatal Diabetes Mellitus:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053798.4TPO, ABCC8, INS, IGF1, KCNJ11

Publications for Transient Neonatal Diabetes Mellitus

Sources:
51PubMed
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Articles related to Transient Neonatal Diabetes Mellitus:

(show top 50)    (show all 53)
idTitleAuthorsYear
1
Clinical presentation of 6q24 transient neonatal diabetes mellitus (6q24 TNDM) and genotype-phenotype correlation in an international cohort of patients. (23385738)
2013
2
Transient neonatal diabetes mellitus in a Turkish patient with three novel homozygous variants in the ZFP57 gene. (23748067)
2013
3
Transient neonatal diabetes mellitus gene Zac1 impairs insulin secretion in mice through Rasgrf1. (22547676)
2012
4
Clinical and molecular basis of transient neonatal diabetes mellitus in Brazilian children. (22749773)
2012
5
Transient neonatal diabetes mellitus caused by a de novoABCC8 gene mutation. (21738553)
2011
6
Treatment of transient neonatal diabetes mellitus with subcutaneous insulin glargine in an extremely low birth weight neonate. (22768014)
2011
7
KCNJ11 activating mutations cause both transient and permanent neonatal diabetes mellitus in Cypriot patients. (21352428)
2011
8
A case of transient neonatal diabetes mellitus. (21131807)
2010
9
Partial paternal uniparental disomy of chromosome 6 in monozygotic twins with transient neonatal diabetes mellitus and macroglossia. (20412110)
2010
10
Transient neonatal diabetes mellitus type 1. (20803656)
2010
11
Transient neonatal diabetes mellitus in an extremely preterm infant. (21686427)
2009
12
An oral sulfonylurea in the treatment of transient neonatal diabetes mellitus. (19446154)
2009
13
Transient neonatal diabetes mellitus in extremely preterm infant. (18192332)
2008
14
Placental mesenchymal dysplasia associated with transient neonatal diabetes mellitus and paternal UPD6. (18486206)
2008
15
Microarray-based DNA methylation analysis of imprinted loci in a patient with transient neonatal diabetes mellitus. (19012334)
2008
16
Double paternal nondisjunction in an infant with transient neonatal diabetes mellitus and Klinefelter syndrome. (17366578)
2007
17
Transient neonatal diabetes mellitus in an infant with paternal uniparental disomy of chromosome 6 including heterodisomy for 6q24. (17220064)
2006
18
Epimutation of the TNDM locus and the Beckwith-Wiedemann syndrome centromeric locus in individuals with transient neonatal diabetes mellitus. (16402210)
2006
19
Assessment of the role of common genetic variation in the transient neonatal diabetes mellitus (TNDM) region in type 2 diabetes: a comparative genomic and tagging single nucleotide polymorphism approach. (16873690)
2006
20
A maternal hypomethylation syndrome presenting as transient neonatal diabetes mellitus. (16816970)
2006
21
Monozygous triplets discordant for transient neonatal diabetes mellitus and for imprinting of the TNDM differentially methylated region. (15924231)
2005
22
Transient neonatal diabetes mellitus is associated with a recurrent (R201H) KCNJ11 (KIR6.2) mutation. (16205880)
2005
23
Bisulphite sequencing of the transient neonatal diabetes mellitus DMR facilitates a novel diagnostic test but reveals no methylation anomalies in patients of unknown aetiology. (15635480)
2005
24
Impaired glucose homeostasis in transgenic mice expressing the human transient neonatal diabetes mellitus locus, TNDM. (15286800)
2004
25
Transient neonatal diabetes mellitus: a patient report. (14513885)
2003
26
Transient neonatal diabetes mellitus, type 4, type 1 diabetes mellitus, or MODY: which disease is it, anyway? (12014522)
2002
27
Relaxation of imprinted expression of ZAC and HYMAI in a patient with transient neonatal diabetes mellitus. (11935319)
2002
28
Neonatal diabetes mellitus: chromosomal analysis in transient and permanent cases. (12378186)
2002
29
A conserved imprinting control region at the HYMAI/ZAC domain is implicated in transient neonatal diabetes mellitus. (11448939)
2001
30
Characterization of the methylation-sensitive promoter of the imprinted ZAC gene supports its role in transient neonatal diabetes mellitus. (11297535)
2001
31
Transient neonatal diabetes mellitus in a child with paternal uniparental disomy of chromosome 6. (11515730)
2001
32
Transient but not permanent neonatal diabetes mellitus is associated with paternal uniparental isodisomy of chromosome 6. (10617703)
2000
33
Variable features of transient neonatal diabetes mellitus with paternal isodisomy of chromosome 6. (10757646)
2000
34
An imprinted locus associated with transient neonatal diabetes mellitus. (10699182)
2000
35
Paternal uniparental disomy of chromosome 6 and transient neonatal diabetes mellitus. (9894800)
1998
36
Transient neonatal diabetes mellitus or an exaggerated steroid response? A case study. (9592462)
1998
37
Transient neonatal diabetes mellitus in a child with invdup(6)(q22q23) of paternal origin. (9450188)
1997
38
Paternal uniparental isodisomy of chromosome 6 in transient neonatal diabetes mellitus. (9296544)
1997
39
Transient neonatal diabetes mellitus and macroglossia. (8866300)
1996
40
Ultralente insulin treatment of transient neonatal diabetes mellitus. (8636827)
1996
41
Transient neonatal diabetes mellitus: a relapse after 10 years of complete remission. (7949222)
1994
42
Two interesting cases of transient neonatal diabetes mellitus. (8186824)
1994
43
Diabetes Mellitus, 6q24-Related Transient Neonatal (20301706)
1993
44
Transient neonatal diabetes mellitus in a pair of twins. (2741692)
1989
45
Transient neonatal diabetes mellitus. (3235149)
1988
46
Transient neonatal diabetes mellitus in half sisters. A sequel. (7091092)
1982
47
Transient neonatal diabetes mellitus. Treatment with chlorpropamide. (760514)
1979
48
Macroglossia, transient neonatal diabetes mellitus and intrauterine growth failure: a new distinct entity? (1110857)
1975
49
Transient neonatal diabetes mellitus in sibs. (5440211)
1970
50
Transient neonatal diabetes mellitus in half sisters. (6067278)
1967

Genetic Variations for Transient Neonatal Diabetes Mellitus

Expression for genes affiliated with Transient Neonatal Diabetes Mellitus

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Transient Neonatal Diabetes Mellitus

Search GEO for disease gene expression data for Transient Neonatal Diabetes Mellitus.

Pathways for genes affiliated with Transient Neonatal Diabetes Mellitus

Sources:
54Reactome, 30KEGG, 50PharmGKB, 38NCBI BioSystems Database
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Compounds for genes affiliated with Transient Neonatal Diabetes Mellitus

Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 50PharmGKB, 60Tocris Bioscience, 24HMDB, 2BitterDB
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Compounds related to Transient Neonatal Diabetes Mellitus according to GeneCards/GeneDecks:

(show all 35)
idCompoundScoreTop Affiliating Genes
1minoxidil45 29 1112.4ABCC8
2cibenzoline4510.2KCNJ11, ABCC8
3nicorandil45 2911.1ABCC8, KCNJ11
4mgadp4510.1ABCC8, KCNJ11
5cromakalim45 2911.1ABCC8, KCNJ11
6meglitinide4510.0ABCC8, INS
7mitiglinide45 1111.0INS, ABCC8
8Glyburide119.9ABCC8, KCNJ11
9glipizide45 1110.9INS, ABCC8
10chlorpropamide45 1110.9INS, ABCC8
11mecasermin45 1110.8IGF1, INS
12oxandrolone45 1110.8IGF1, INS
13nateglinide45 50 1111.8ABCC8, INS
14acipimox45 2910.7IGF1, INS
15repaglinide45 50 1111.7ABCC8, INS, KCNJ11
16glimepiride45 50 1111.7KCNJ11, ABCC8, INS
17gliclazide45 50 1111.7ABCC8, INS, KCNJ11
18sulfonylurea459.7KCNJ11, ABCC8, INS
19katp459.7ABCC8, KCNJ11, INS
20diazoxide45 60 29 1112.7ABCC8, KCNJ11, INS
21tolbutamide45 50 29 1112.7ABCC8, KCNJ11, INS
22glibenclamide45 29 50 6012.7KCNJ11, ABCC8, INS
23clomiphene citrate459.6INS, IGF1
24octreotide45 60 29 1112.6IGF1, INS, PLAGL1
25cortisone45 2410.5IGF1, INS
26starch459.5IGF1, INS
27hydrocortisone45 2 60 1112.3TPO, IGF1, INS
28potassium45 11 2411.3ZACN, ABCC8, TPO, KCNJ11
29thyroxine45 2410.3IGF1, INS, TPO
30metformin45 50 1111.2KCNJ11, INS, IGF1, ABCC8
31c-peptide459.2IGF1, TPO, INS, KCNJ11
32acth459.0TPO, INS, IGF1
33testosterone45 60 11 2411.8IGF1, TPO, ABCC8, INS, PLAGL1
34arginine458.7TPO, IGF1, KCNJ11, INS, ABCC8
35cholesterol45 29 11 2411.4IGF1, INS, ABCC8, TPO

GO Terms for genes affiliated with Transient Neonatal Diabetes Mellitus

Sources:
16Gene Ontology
See all sources

Biological processes related to Transient Neonatal Diabetes Mellitus according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of glycolysisGO:0458219.6INS, IGF1
2positive regulation of glycogen biosynthetic processGO:0457259.6IGF1, INS
3potassium ion transmembrane transportGO:0718059.5ABCC8, KCNJ11
4positive regulation of glucose importGO:0463269.5IGF1, INS
5positive regulation of DNA replicationGO:0457409.5IGF1, INS
6regulation of insulin secretionGO:0507969.5KCNJ11, ABCC8, INS
7energy reserve metabolic processGO:0061129.4ABCC8, KCNJ11, INS
8positive regulation of mitosisGO:0458409.4IGF1, INS

Molecular functions related to Transient Neonatal Diabetes Mellitus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1insulin receptor bindingGO:0051589.5INS, IGF1
2insulin-like growth factor receptor bindingGO:0051599.2INS, IGF1

Products for genes affiliated with Transient Neonatal Diabetes Mellitus

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  • Lysates
  • Antibodies

Sources for Transient Neonatal Diabetes Mellitus

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet