NDM
MCID: NNT009
MIFTS: 54

Neonatal Diabetes Mellitus (NDM) malady

Endocrine diseases category

Summaries for Neonatal Diabetes Mellitus

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63Wikipedia, 32MalaCards
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Wikipedia:63 Neonatal diabetes mellitus (NDM) is a monogenic form of diabetes that occurs in the first 6 months of... more...

MalaCards: Neonatal Diabetes Mellitus, also known as diabetes mellitus syndrome in newborn infant, is related to diabetes mellitus and permanent neonatal diabetes mellitus. An important gene associated with Neonatal Diabetes Mellitus is KCNJ11 (potassium inwardly-rectifying channel, subfamily J, member 11), and among its related pathways are ATP sensitive Potassium channels and Antiarrhythmic Pathway, Pharmacodynamics. The compounds cibenzoline and minoxidil have been mentioned in the context of this disorder. Affiliated tissues include testes, kidney and pancreas, and related mouse phenotypes are renal/urinary system and no phenotypic analysis.

Aliases & Classifications for Neonatal Diabetes Mellitus

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Sources:
8Disease Ontology, 22GTR, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 27ICD9CM, 56SNOMED-CT, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Endocrine diseases


Characteristics (Orphanet epidemiological data):

48
neonatal diabetes mellitus:
Inheritance: Autosomal dominant,Autosomal recessive,Sporadic; Age of onset: Neonatal/infancy; Age of death: Normal


Aliases & Descriptions:

neonatal diabetes mellitus 8 22 10 44 48 60
diabetes mellitus syndrome in newborn infant 8
ndm 48


External Ids:

Disease Ontology8 DOID:11717
ICD9CM27 775.1
ICD10 via Orphanet26 P70.2
SNOMED-CT56 49817004
SNOMED-CT via Orphanet57 49817004
UMLS via Orphanet61 C0158981

Related Diseases for Neonatal Diabetes Mellitus

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17GeneCards, 18GeneDecks
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Diseases in the Diabetes Mellitus, 6q24-Related Transient Neonatal family:

neonatal diabetes mellitus Transient Neonatal Diabetes Mellitus
Diabetes Mellitus, Kcnj11-Related Transient Neonatal Abcc8-Related Transient Neonatal Diabetes Mellitus 2
Diabetes Mellitus, Transient Neonatal 2 Diabetes Mellitus, Transient Neonatal, 3

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

(show top 50)    (show all 88)
idRelated DiseaseScoreTop Affiliating Genes
1diabetes mellitus31.8KCNJ11, FOXP3, KCNJ5, HNF1B, PDX1, IGF1
2permanent neonatal diabetes mellitus31.4KCNJ11, INS, ABCC8
3diabetes mellitus, 6q24-related transient neonatal31.0PLAGL1, HYMAI
4pancreatitis30.8INS
5hypothyroidism30.3TPO, INS, IGF1
6pancreatic agenesis30.1PDX1
7beckwith-wiedemann syndrome30.1PLAGL1, ZFP57, ABCC8
8epilepsy syndrome30.1ABCC8, ZACN, KCNJ5, KCNJ11
9maturity-onset diabetes of the young30.1INS, HNF1B, PDX1, GCK, KCNJ11
10hyperinsulinism30.1KCNJ5, ABCC8, INS, IGF1, GCK, KCNJ11
11type 2 diabetes mellitus30.1KCNJ11, IGF1, INS, ABCC8, SLC2A2, PDX1
12transient neonatal diabetes mellitus10.6
13pancreatic beta cell agenesis with neonatal diabetes mellitus10.5
14diabetic ketoacidosis10.3
15type 1 diabetes mellitus10.3
16pneumonia10.3
17congenital hypothyroidism10.2
18hepatitis10.2
19abcc8-related transient neonatal diabetes mellitus 210.2
20abcc8-related permanent neonatal diabetes mellitus10.2
21gck-related permanent neonatal diabetes mellitus10.2
22kcnj11-related permanent neonatal diabetes mellitus10.2
23pdx1-related permanent neonatal diabetes mellitus10.2
24ins-related permanent neonatal diabetes mellitus10.2
25syndromic diarrhea10.1
26fanconi syndrome10.1
27intestinal atresia10.1
28exocrine pancreatic insufficiency10.1
29congenital diaphragmatic hernia10.1
30hepatitis a10.1
31congenital heart defect10.1
32diarrhea10.1
33glaucoma10.1
34hyperimmunoglobulin syndrome10.1
35organic acidemia10.1
36pervasive developmental disorder10.1
37thrombocytopenia10.1
38triple x syndrome10.1
39congenital hepatic fibrosis10.1
40diabetes mellitus, permanent neonatal, with cerebellar agenesis10.1
41diabetes mellitus, kcnj11-related transient neonatal10.1
42diabetes mellitus, neonatal, with congenital hypothyroidism10.1
43diabetes mellitus, insulin-dependent, neonatal10.1
44calciphylaxis10.0
45nondystrophic myotonia10.0
46morbid obesity10.0INS
47essential hypertension10.0INS
48acute myocardial infarction10.0KCNJ5
49donohue syndrome10.0INS
50turner syndrome10.0IGF1

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



Diseases related to neonatal diabetes mellitus

Clinical Features for Neonatal Diabetes Mellitus

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Drugs & Therapeutics for Neonatal Diabetes Mellitus

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Neonatal Diabetes Mellitus

Drug clinical trials:

Search ClinicalTrials for Neonatal Diabetes Mellitus

Search NIH Clinical Center for Neonatal Diabetes Mellitus

Search CenterWatch for Neonatal Diabetes Mellitus

Genetic Tests for Neonatal Diabetes Mellitus

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22GTR
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Genetic tests related to Neonatal Diabetes Mellitus:

id Genetic test Affiliating Genes
1 Neonatal Diabetes Mellitus22

Anatomical Context for Neonatal Diabetes Mellitus

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32MalaCards
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MalaCards organs/tissues related to Neonatal Diabetes Mellitus:

32
Testes, Kidney, Pancreas, Brain, Heart

Animal Models for Neonatal Diabetes Mellitus or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Neonatal Diabetes Mellitus:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053679.1HNF1B, PDX1, FOXP3, IGF1, INS, GCK
2MP:00030128.9KCNJ11, PDX1, FOXP3, INS, ABCC8, PLAGL1
3MP:00053708.7GCK, HNF1B, PDX1, FOXP3, INS, PTF1A
4MP:00053858.2KCNJ5, KCNJ11, FOXP3, IGF1, INS, PLAGL1
5MP:00053797.8TPO, KCNJ11, GCK, HNF1B, PDX1, FOXP3
6MP:00053787.8FOXP3, PDX1, HNF1B, GCK, KCNJ11, IGF1
7MP:00053767.6FOXP3, PDX1, HNF1B, GCK, KCNJ11, IGF1
8MP:00107687.6FOXP3, PDX1, HNF1B, GCK, KCNJ11, IGF1

Publications for Neonatal Diabetes Mellitus

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50PubMed
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Articles related to Neonatal Diabetes Mellitus:

(show top 50)    (show all 192)
idTitleAuthorsYear
1
Decreased concentrations of the lipoprotein lipase inhibitor angiopoietin-like protein 4 and increased serum triacylglycerol are associated with increased neonatal fat mass in pregnant women with gestational diabetes mellitus. (23744407)
2013
2
Large- and small-for-gestational-age neonates born by women with gestational diabetes mellitus diagnosed by the new IADPSG criteria: a case-control study of 289 patients and 1 108 controls. (23450330)
2013
3
KCNJ11 in-frame 15-bp deletion leading to glibenclamide- responsive neonatal diabetes mellitus in a Chinese child. (23640936)
2013
4
Neonatal Diabetes Mellitus Due to a Novel ABCC8 Gene Mutation Mimicking an Organic Acidemia. (23783767)
2013
5
Transient neonatal diabetes mellitus in a Turkish patient with three novel homozygous variants in the ZFP57 gene. (23748067)
2013
6
Disproportionate body composition and neonatal outcome in offspring of mothers with and without gestational diabetes mellitus. (24159180)
2013
7
Neonatal diabetes mellitus in China: a case report and review of the Chinese literature. (22144719)
2012
8
Perinatal maternal and neonatal outcomes in women with gestational diabetes mellitus according to fetal sex. (23089258)
2012
9
Improved long-term glucose control in neonatal diabetes mellitus after early sulfonylurea allergy. (22768668)
2012
10
Neonatal diabetes mellitus and congenital diaphragmatic hernia: coincidence or concurrent etiology? (22781086)
2012
11
Permanent neonatal diabetes mellitus--the importance of diabetes differential diagnosis in neonates and infants. (21054355)
2011
12
Intranasal administration of insulin eliminates the deficit of long-term spatial memory in rats with neonatal diabetes mellitus. (22095122)
2011
13
Neonatal diabetes mellitus due to L233F mutation in the KCNJ11 gene. (21210267)
2011
14
KCNJ11 activating mutations cause both transient and permanent neonatal diabetes mellitus in Cypriot patients. (21352428)
2011
15
Mutations in pancreatic A9-cell Glucokinase as a cause of hyperinsulinaemic hypoglycaemia and neonatal diabetes mellitus. (20878480)
2010
16
Partial paternal uniparental disomy of chromosome 6 in monozygotic twins with transient neonatal diabetes mellitus and macroglossia. (20412110)
2010
17
A Turkish newborn infant with cerebellar agenesis/neonatal diabetes mellitus and PTF1A mutation. (19650412)
2009
18
Transfer from insulin to sulfonylurea treatment in a chinese patient with permanent neonatal diabetes mellitus due to a KCNJ11 R201H mutation. (19247925)
2009
19
Compound heterozygosity for mutations in PAX6 in a patient with complex brain anomaly, neonatal diabetes mellitus, and microophthalmia. (19876904)
2009
20
Placental mesenchymal dysplasia associated with transient neonatal diabetes mellitus and paternal UPD6. (18486206)
2008
21
Neonatal diabetes mellitus due to pancreatic agenesis. (18426328)
2008
22
Neonatal diabetes mellitus: insights for more common forms of diabetes. (17933979)
2007
23
Neonatal diabetes mellitus: a disease linked to multiple mechanisms. (17349054)
2007
24
A mutation in the TMD0-L0 region of sulfonylurea receptor-1 (L225P) causes permanent neonatal diabetes mellitus (PNDM). (17317760)
2007
25
Glucose-monitoring with continuous subcutaneous microdialysis in neonatal diabetes mellitus. (16819705)
2006
26
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
27
Assessment of insulin sensitivity in adults with permanent neonatal diabetes mellitus due to mutations in the KCNJ11 gene encoding Kir6.2. (17491708)
2006
28
A maternal hypomethylation syndrome presenting as transient neonatal diabetes mellitus. (16816970)
2006
29
Sulfonylurea treatment outweighs insulin therapy in short-term metabolic control of patients with permanent neonatal diabetes mellitus due to activating mutations of the KCNJ11 (KIR6.2) gene. (16816952)
2006
30
Neonatal diabetes mellitus: Insulin pump as an alternative management strategy. (16150072)
2005
31
Impaired glucose homeostasis in transgenic mice expressing the human transient neonatal diabetes mellitus locus, TNDM. (15286800)
2004
32
Neonatal diabetes mellitus and neonatal polycystic, dysplastic kidneys: phenotypically discordant recurrence of a mutation in the hepatocyte nuclear factor-1beta gene due to germline mosaicism. (15181075)
2004
33
Continuous subcutaneous insulin infusion in neonatal diabetes mellitus. (15601363)
2004
34
Permanent neonatal diabetes mellitus manifesting as diabetic ketoacidosis. (14976569)
2003
35
Neonatal diabetes mellitus, congenital hypothyroidism, hepatic fibrosis, polycystic kidneys, and congenital glaucoma: a new autosomal recessive syndrome? (12966531)
2003
36
Relaxation of imprinted expression of ZAC and HYMAI in a patient with transient neonatal diabetes mellitus. (11935319)
2002
37
Transient but not permanent neonatal diabetes mellitus is associated with paternal uniparental isodisomy of chromosome 6. (10617703)
2000
38
Variable features of transient neonatal diabetes mellitus with paternal isodisomy of chromosome 6. (10757646)
2000
39
HLA-DRB1 and DQB1 genotypes in patients with insulin-dependent neonatal diabetes mellitus. A study of 13 cases. (11034557)
2000
40
Neonatal diabetes mellitus with hypergalactosemia. (10526252)
1999
41
Permanent neonatal diabetes mellitus: epidemiology, mode of presentation, pathogenesis and growth. (10798084)
1999
42
Neonatal diabetes mellitus with severe hypertriglyceridemia. (8887156)
1996
43
Transient neonatal diabetes mellitus and macroglossia. (8866300)
1996
44
Neonatal diabetes mellitus associated with severe diarrhea, hyperimmunoglobulin E syndrome, and absence of islets of Langerhans. (8597835)
1995
45
Neonatal diabetes mellitus. (7769543)
1995
46
Permanent neonatal diabetes mellitus and pancreatic exocrine insufficiency resulting from congenital pancreatic agenesis. (8506821)
1993
47
Permanent neonatal diabetes mellitus: lack of diabetic complications after a 20-year follow up. (1537364)
1992
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 Neonatal Diabetes Mellitus

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Expression for genes affiliated with Neonatal Diabetes Mellitus

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

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

Pathways for genes affiliated with Neonatal Diabetes Mellitus

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Sources:
53Reactome, 49PharmGKB, 12EMD Millipore, 37NCBI BioSystems Database, 29KEGG
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Compounds for genes affiliated with Neonatal Diabetes Mellitus

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Sources:
44Novoseek, 28IUPHAR, 11DrugBank, 49PharmGKB, 59Tocris Bioscience, 24HMDB
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Compounds related to Neonatal Diabetes Mellitus according to GeneCards/GeneDecks:

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1cibenzoline4410.5KCNJ11, ABCC8
2minoxidil44 28 1112.5KCNJ5, ABCC8
3benzopyran4410.5KCNJ5, ABCC8
4dicyandiamide4410.5ABCC8, KCNJ5
5meglitinide4410.4INS, ABCC8
6chromium picolinate4410.4GCK, INS
7mitiglinide44 1111.4INS, ABCC8
8nicorandil44 2811.4ABCC8, KCNJ11, KCNJ5
9alloxan4410.4GCK, SLC2A2
10mgadp4410.4KCNJ5, KCNJ11, ABCC8
11pinacidil4410.4KCNJ5, ABCC8
12cromakalim44 2811.3ABCC8, KCNJ11, KCNJ5
13gliclazide44 49 1112.3KCNJ11, INS, ABCC8
14levosimendan44 1111.3KCNJ5, KCNJ11
15glipizide44 1111.3ABCC8, INS, KCNJ5
16i-app4410.3GCK, PDX1, INS
17Glyburide1110.3ABCC8, KCNJ11, KCNJ5
18nateglinide44 49 1112.3KCNJ5, INS, ABCC8
19proglucagon4410.3SLC2A2, PDX1, GCK
20mecasermin44 1111.2IGF1, INS
213-o-methylglucose4410.2GCK, SLC2A2
22repaglinide44 49 1112.2ABCC8, INS, KCNJ11, KCNJ5
23glimepiride44 49 1112.2KCNJ5, KCNJ11, INS, ABCC8
24glibenclamide44 28 49 5913.2KCNJ5, KCNJ11, INS, ABCC8
25aicar44 11 2412.1INS, IGF1, GCK
26preproinsulin4410.1SLC2A2, INS, PDX1, GCK
27incretin4410.1GCK, PDX1, INS, SLC2A2
28chlorpropamide44 1111.1INS, ABCC8
29nicotinamide44 1111.1INS, PDX1, HNF1B
30levcromakalim5910.1KCNJ11, KCNJ5
31oxandrolone44 1111.1INS, IGF1
32streptozotocin4410.1GCK, PDX1, INS, SLC2A2
332-deoxyglucose44 1111.0SLC2A2, INS, IGF1, GCK
34diazoxide44 59 28 1113.0KCNJ5, KCNJ11, GCK, INS, ABCC8
35tolbutamide44 49 28 1113.0KCNJ5, KCNJ11, GCK, INS, ABCC8
36octreotide44 59 28 1113.0PLAGL1, INS, IGF1
37metformin44 49 1111.9KCNJ11, GCK, IGF1, INS, ABCC8
38uric acid44 2410.7TPO, INS, HNF1B
39glycogen44 2410.6GCK, INS, ABCC8, SLC2A2, ONECUT1
40sulfonylurea449.6SLC2A2, KCNJ5, KCNJ11, GCK, PDX1, INS
41katp449.6KCNJ5, KCNJ11, GCK, PDX1, INS, ABCC8
42testosterone44 59 11 2412.6KCNJ5, IGF1, INS, ABCC8, PLAGL1, TPO
43cholesterol44 28 11 2412.5GCK, HNF1B, IGF1, INS, ABCC8, TPO
44potassium44 11 2411.5KCNJ5, KCNJ11, GCK, ZACN, ABCC8, SLC2A2
45c-peptide449.5KCNJ11, GCK, PDX1, IGF1, INS, SLC2A2
46glutamate449.5KCNJ5, KCNJ11, GCK, INS, ABCC8, TPO
47oligonucleotide449.3GCK, HNF1B, PDX1, SLC2A2, KRBOX4, TPO
48arginine449.2KCNJ11, GCK, HNF1B, IGF1, INS, ABCC8
49atp44 2810.0KCNJ5, KCNJ11, GCK, ABCC8, SLC2A2, TPO
50glucose448.4KLF11, KCNJ5, KCNJ11, GCK, HNF1B, PDX1

GO Terms for genes affiliated with Neonatal Diabetes Mellitus

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16Gene Ontology
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Cellular components related to Neonatal Diabetes Mellitus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1voltage-gated potassium channel complexGO:0080769.9ABCC8, KCNJ11, KCNJ5

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

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1detection of glucoseGO:05159410.2PDX1, GCK
2positive regulation of glycolysisGO:04582110.1GCK, IGF1, INS
3positive regulation of glycogen biosynthetic processGO:04572510.1INS, IGF1, GCK
4exocrine pancreas developmentGO:03101710.0PTF1A, IGF1, PDX1
5negative regulation of gluconeogenesisGO:04572110.0GCK, INS
6hindbrain developmentGO:03090210.0PTF1A, HNF1B
7energy reserve metabolic processGO:0061129.9KCNJ11, INS, ABCC8, SLC2A2
8glucose transportGO:0157589.9SLC2A2, INS, GCK
9regulation of insulin secretionGO:0507969.8KCNJ11, GCK, INS, ABCC8, SLC2A2
10glucose metabolic processGO:0060069.7KCNJ11, PDX1, INS, ONECUT1
11glucose homeostasisGO:0425939.6INS, PDX1, GCK
12endocrine pancreas developmentGO:0310189.1ONECUT1, PTF1A, SLC2A2, INS, PDX1, HNF1B
13positive regulation of transcription from RNA polymerase II promoterGO:0459449.0HNF1B, PDX1, FOXP3, IGF1, PLAGL1, ONECUT1

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

idNameGO IDScoreTop Affiliating Genes
1insulin-like growth factor receptor bindingGO:00515910.0INS, IGF1

Products for genes affiliated with Neonatal Diabetes Mellitus

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Sources for Neonatal Diabetes Mellitus

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet