Transient Neonatal Diabetes Mellitus malady

NIH Rare Diseases: 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.³⁰

MalaCards: Transient Neonatal Diabetes Mellitus, also known as diabetes mellitus, transient neonatal, is related to type 2 diabetes mellitus and gestational diabetes. An important gene associated with Transient Neonatal Diabetes Mellitus is TNDM (diabetes mellitus, transient neonatal), and among its related pathways are Inwardly rectifying K+ channels and Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics. The drug chromium picolinate and the compounds cibenzoline and mgadp have been mentioned in the context of this disorder.

Wikipedia: Transient neonatal diabetes mellitus (TNDM) is a form of diabetes mellitus presenting at birth that is...⁴⁴ more...

OMIM: 601410

transient neonatal diabetes mellitus 30 diabetes mellitus, transient neonatal 30 33 chromosome 6-associated transient diabetes mellitus 30 diabetes mellitus, 6q24-related transient neonatal 30 neonatal diabetes mellitus 43

diabetes mellitus 43 tndm1 30 dmtn 30 tndm 30

Disease types for transient neonatal diabetes mellitus family:

diabetes mellitus, transient neonatal, 3

Diseases related to transient neonatal diabetes mellitus by text searches and GeneDecks gene sharing:

(show top 50)    (show all 392)

id	Related Disease	Score	Top Affiliating Genes
1	type 2 diabetes mellitus	39.3	ABCC8, TPO, IGF1, INS, KCNJ11
2	gestational diabetes	37.5	ABCC8, INS, KCNJ11
3	diabetes mellitus, 6q24-related transient neonatal	36.1	HYMAI, PLAGL1
4	permanent neonatal diabetes mellitus	34.3	ABCC8, INS
5	hypoglycemia	32.4	KCNJ11, INS, IGF1, ABCC8
6	hyperglycemia	31.6	ABCC8, TPO, IGF1, INS, KCNJ11, ZFP57
7	celiac disease	31.3	TPO, IGF1, INS, KCNJ11
8	myocardial infarction	30.9	KCNJ11, ZACN, INS, IGF1, TPO
9	acromegaly	30.7	PLAGL1, IGF1, INS
10	diabetes, type 2	30.6	ABCC8, INS, KCNJ11
11	glucose intolerance	30.6	KCNJ11, INS, IGF1, ABCC8
12	polycystic ovary syndrome	30.0	IGF1, INS
13	acanthosis nigricans	29.9	INS, IGF1
14	pancreatic agenesis	29.6	KCNJ11, INS, ABCC8
15	neonatal diabetes mellitus	29.4	KCNJ11, ZACN, INS, IGF1, HYMAI, TPO
16	twinning	29.2	INS, IGF1, TPO, ABCC8
17	type 1 diabetes mellitus	29.2	INS, IGF1, TPO
18	diabetes mellitus	29.1	ABCC8, PLAGL1, TNDM, TPO, HYMAI, IGF1
19	rabson-mendenhall syndrome	28.9	IGF1, INS
20	diabetes insipidus	28.7	INS, IGF1, TPO
21	acute myocardial infarction	28.7	TPO, IGF1, INS, KCNJ11
22	morbid obesity	28.7	ABCC8, IGF1, INS
23	alopecia	28.5	INS, IGF1, TPO
24	hyperinsulinism	28.4	KCNJ11, INS, IGF1, ABCC8
25	insulinoma	28.4	KCNJ11, INS, IGF1, ABCC8
26	hyperinsulinemic hypoglycemia	27.9	ABCC8, INS
27	hypoparathyroidism	27.9	TPO, IGF1, INS
28	down syndrome	27.9	PLAGL1, TPO, IGF1, INS
29	autoimmune polyendocrine syndrome	27.7	TPO, INS
30	fetal macrosomia	27.5	INS, IGF1
31	anovulation	27.3	IGF1, INS
32	turner syndrome	26.9	TPO, IGF1
33	combined immunodeficiency	26.4	TPO, IGF1, INS
34	severe combined immunodeficiency	26.4	INS, IGF1, TPO
35	hyperthyroidism	26.2	TPO, IGF1, INS
36	adenoma	25.0	ABCC8, PLAGL1, TPO, IGF1, INS
37	hyperinsulinism, focal	13.1	KCNJ11, ABCC8
38	diabetes, permanent neonatal	13.1	ABCC8, KCNJ11
39	leucine-sensitive hypoglycemia of infancy	13.1	KCNJ11, ABCC8
40	persistent hyperinsulinemic hypoglycemia of infancy	13.1	ABCC8, KCNJ11
41	ketoacidosis due to scot deficiency	12.8	ABCC8, INS
42	endogenous depression	12.7	INS, TPO
43	hyperammonemia with hypoornithinemia	12.7	ABCC8, KCNJ11
44	acute insulin response	12.6	KCNJ11, INS, ABCC8
45	hypoglycemia of infancy	12.6	ABCC8, INS, KCNJ11
46	donohue syndrome	12.6	INS, IGF1
47	sheehan syndrome	12.6	INS, IGF1
48	insulin resistance	12.6
49	pituitary gland disease	12.5	IGF1, INS
50	diabetic autonomic neuropathy	12.5	IGF1, INS

Clinical features from OMIM: 601410

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 chromium picolinate

Articles related to transient neonatal diabetes mellitus:

id	Title	Authors	Year	Affiliating Genes
1	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)	Gloyn A.L.... Frayling T.M.	2006	PLAGL1, ZNF673, HYMAI
2	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)	Mackay D.J.... Robinson D.O.	2005	TNDM
3	Impaired glucose homeostasis in transgenic mice expressing the human transient neonatal diabetes mellitus locus, TNDM. (15286800)	Ma D.... Kelsey G.	2004	PLAGL1, ZACN, HYMAI
4	Transient neonatal diabetes mellitus, type 4, type 1 diabetes mellitus, or MODY: which disease is it, anyway? (12014522)	AvRuskin T.W.... Juan C.S.	2002	TPO
5	Relaxation of imprinted expression of ZAC and HYMAI in a patient with transient neonatal diabetes mellitus. (11935319)	Mackay D.J.... Robinson D.O.	2002	PLAGL1, HYMAI
6	A conserved imprinting control region at the HYMAI/ZAC domain is implicated in transient neonatal diabetes mellitus. (11448939)	Arima T.... Surani M.A.	2001	INS, PLAGL1
7	Characterization of the methylation-sensitive promoter of the imprinted ZAC gene supports its role in transient neonatal diabetes mellitus. (11297535)	Varrault A.... Journot L.	2001	PLAGL1
8	Ultralente insulin treatment of transient neonatal diabetes mellitus. (8636827)	Mitamura R.... Okuno A.	1996	INS
9	Transient neonatal diabetes mellitus: a relapse after 10 years of complete remission. (7949222)	Vanelli M.... Chiari G.	1994	IGF1

Genes related to transient neonatal diabetes mellitus according to GeneCards:

(show all 11)

id	Symbol	Description	Score	GeneCards Section Context
1	TNDM	diabetes mellitus, transient neonatal	11.1	Publications, Summaries
2	HYMAI	hydatidiform mole associated and imprinted (non-protein coding)	11.1	Publications, Summaries
3	ZFP57	zinc finger protein 57 homolog (mouse)	11.1	Summaries
4	PLAGL1	pleiomorphic adenoma gene-like 1	11.1	Publications, Summaries
5	KCNJ11	potassium inwardly-rectifying channel, subfamily J, member 11	11.1	Summaries
6	ZACN	zinc activated ligand-gated ion channel	11.0	Publications
7	ZNF673	zinc finger family member 673	11.0	Publications
8	ABCC8	ATP-binding cassette, sub-family C (CFTR/MRP), member 8	11.0
9	TPO	thyroid peroxidase	11.0	Publications
10	INS	insulin	11.0	Publications
11	IGF1	insulin-like growth factor 1 (somatomedin C)	11.0	Publications

Pathways related to transient neonatal diabetes mellitus according to GeneDecks:

id	Pathway	Score	Top Affiliating Genes
1	Inwardly rectifying K+ channels38	9.6	KCNJ11, ABCC8
2	Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics34	8.7	KCNJ11, INS, ABCC8
3	Type II diabetes mellitus20	8.5	KCNJ11, INS, ABCC8

Compounds related to transient neonatal diabetes mellitus according to GeneDecks:

(show all 31)

id	Compound	Score	Top Affiliating Genes
1	cibenzoline32	9.9	KCNJ11, ABCC8
2	mgadp32	9.9	ABCC8, KCNJ11
3	Glyburide9 9	10.6	KCNJ11, ABCC8
4	meglitinide32	9.5	INS, ABCC8
5	mitiglinide32 9 9	11.5	INS, ABCC8
6	mecasermin32 9 9	11.4	INS, IGF1
7	oxandrolone32 9 9	11.4	INS, IGF1
8	glipizide32 9 9	11.4	ABCC8, INS
9	chlorpropamide32 9 9	11.4	ABCC8, INS
10	acipimox32	9.3	IGF1, INS
11	clomiphene citrate32	9.2	IGF1, INS
12	repaglinide32 42 9 9	12.1	KCNJ11, INS, ABCC8
13	glimepiride32 34 9 9	12.1	INS, ABCC8, KCNJ11
14	gliclazide32 34 9 9	12.1	KCNJ11, INS, ABCC8
15	starch32	9.1	INS, IGF1
16	nateglinide32 42 9 9	12.1	ABCC8, KCNJ11, INS
17	diazoxide32 42 9 9	12.1	KCNJ11, INS, ABCC8
18	sulfonylurea32	9.1	ABCC8, KCNJ11, INS
19	katp32	9.1	INS, ABCC8, KCNJ11
20	tolbutamide32 34 9 9	12.1	INS, KCNJ11, ABCC8
21	octreotide32 42 9 9	12.1	IGF1, INS, PLAGL1
22	glibenclamide32 34	10.1	KCNJ11, ABCC8, INS
23	cortisone32 18	10.0	IGF1, INS
24	potassium32 9 18 9	11.8	ABCC8, ZACN, TPO, KCNJ11
25	hydrocortisone32 9 9	10.7	TPO, INS, IGF1
26	metformin32 34 9 9	11.6	ABCC8, KCNJ11, IGF1, INS
27	thyroxine32 18	9.6	IGF1, TPO, INS
28	c-peptide32	8.5	KCNJ11, INS, IGF1, TPO
29	acth32	8.2	INS, IGF1, TPO
30	testosterone32 9 18 9	10.9	INS, ABCC8, PLAGL1, IGF1, TPO
31	arginine32	7.8	TPO, ABCC8, INS, KCNJ11, IGF1

Biological processes related to transient neonatal diabetes mellitus according to GeneDecks:

(show all 7)

id	Name	GO ID	Score	Top Affiliating Genes
1	potassium ion transmembrane transport	GO:071805	9.7	ABCC8, KCNJ11
2	positive regulation of glycolysis	GO:045821	9.2	INS, IGF1
3	positive regulation of mitosis	GO:045840	9.1	INS, IGF1
4	positive regulation of glycogen biosynthetic process	GO:045725	9.0	IGF1, INS
5	regulation of insulin secretion	GO:050796	8.9	ABCC8, INS, KCNJ11
6	positive regulation of glucose import	GO:046326	8.8	INS, IGF1
7	energy reserve metabolic process	GO:006112	8.8	KCNJ11, ABCC8, INS

Molecular functions related to transient neonatal diabetes mellitus according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	ion channel binding	GO:044325	9.6	KCNJ11, ABCC8
2	insulin-like growth factor receptor binding	GO:005159	9.0	INS, IGF1
3	insulin receptor binding	GO:005158	8.8	INS, IGF1