Transient neonatal diabetes

Medical condition

Transient neonatal diabetes

Transient neonatal diabetes mellitus is inherited in an autosomal dominant manner
Specialty	Pediatrics

Transient neonatal diabetes mellitus (TNDM) is a form of neonatal diabetes presenting at birth that is not permanent. This disease is considered to be a type of maturity onset diabetes of the young (MODY).

Types

Type	OMIM	Gene	Locus	Description
TNDM1	601410	ZFP57, PLAGL1	6p22.1, 6q24.2
TNDM2	610374	ABCC8	11p15.1	Due to the mutations of the other subunit of the K_ATP channel, SUR1, which is encoded by the ABCC8 gene.
TNDM3	610582	KCNJ11	11p15.1

Cause

This condition has to do with genetics and is often associated with having an added Chromosome 7 gene (mostly from the paternal side).

The form on chromosome 6 can involve imprinting.

Diagnosis

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Management

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References

"OMIM Entry - # 610582 - DIABETES MELLITUS, TRANSIENT NEONATAL, 3". omim.org. Retrieved 12 September 2017.
de Wet H, Proks P, Lafond M, et al. (May 2008). "A mutation (R826W) in nucleotide-binding domain 1 of ABCC8 reduces ATPase activity and causes transient neonatal diabetes". EMBO Rep. 9 (7): 648–54. doi:10.1038/embor.2008.71. PMC 2475326. PMID 18497752.
Raphaël Scharfmann (2007). Development of the Pancreas and Neonatal Diabetes. Karger Publishers. pp. 113–. ISBN 978-3-8055-8385-5. Retrieved 10 November 2010.
"Other Forms (MODY, LADA, Neonatal)". www.DiabetesCare.net. DiabetesCare.net. Retrieved 20 November 2014.

External links

Classification	D ICD-10: P70.2 OMIM: 601410 610374 610582 MeSH: C563322
External resources	GeneReviews: Diabetes Mellitus, 6q24-Related Transient Neonatal

v t e Diabetes
Types	Type 1 (SAID cluster) Type 1 diabetes (LADA cluster) Type 2 (SIDD, SIRD, MOD and MARD clusters) Type 4 (Gestational diabetes) Diabetes and pregnancy Prediabetes Impaired fasting glucose Impaired glucose tolerance Insulin resistance Ketosis-prone diabetes (KPD) Type 3a (MODY) Type 1 2 3 4 5 6 Neonatal Transient Permanent Type 3c (pancreatogenic) Type 3 MIDD
Blood tests	Blood sugar level Biomarkers Glycated hemoglobin Glucose tolerance test Postprandial glucose test Fructosamine Glucose test C-peptide Noninvasive glucose monitor Insulin tolerance test Metabolic Score for Insulin Resistance (METS-IR) Homeostatic model assessment SPINA-GBeta SPINA-GR Disposition index
Management	Prevention Metabolic assessment and monitoring Blood glucose monitoring Ambulatory glucose profile Diet in diabetes Diabetes medication Insulin therapy intensive conventional pulsatile Diabetic shoes Cure Embryonic stem cells Artificial pancreas Other Gastric bypass surgery
Complications	Diabetic comas Hypoglycemia Ketoacidosis Hyperosmolar hyperglycemic state Diabetic foot ulcer Neuropathic arthropathy Organs in diabetes Blood vessels Muscle Kidney Nerves Retina Heart Diabetes-related skin disease Diabetic dermopathy Diabetic bulla Diabetic cheiroarthropathy Diabetic foot ulcer Hyperglycemia Hypoglycemia
Advocacy & Organizations	T1International Open Insulin Project Breakthrough T1D International Diabetes Federation World Diabetes Day Diabetes UK
Other	Outline of diabetes Glossary of diabetes Epidemiology of diabetes History of diabetes Notable people with type 1 diabetes

Disorders due to genomic imprinting

Chromosome 6

Transient neonatal diabetes mellitus

Chromosome 11

Beckwith–Wiedemann syndrome ♀ / Silver–Russell syndrome ♂
Myoclonic dystonia

Chromosome 15

Angelman syndrome ♀ / Prader-Willi syndrome ♂

Chromosome 20

Pseudohypoparathyroidism ♀ / Pseudopseudohypoparathyroidism ♂

v t e Genetic disorder, membrane: ABC transporter disorders
ABCA	ABCA1 (Tangier disease) ABCA3 (Surfactant metabolism dysfunction 3) ABCA4 (Stargardt disease 1, Retinitis pigmentosa 19) ABCA12 (Harlequin-type ichthyosis, Lamellar ichthyosis 2)
ABCB	ABCB4 (Progressive familial intrahepatic cholestasis 3) ABCB7 (ASAT) ABCB11 (Progressive familial intrahepatic cholestasis 2)
ABCC	ABCC2 (Dubin–Johnson syndrome) ABCC6 (Pseudoxanthoma elasticum) ABCC7 (Cystic fibrosis) ABCC8 (HHF1, TNDM2) ABCC9 (Dilated cardiomyopathy 1O)
ABCD	ABCD1 (Adrenoleukodystrophy, Adrenomyeloneuropathy)
ABCG	ABCG5 (Sitosterolemia) ABCG8 (Gallbladder disease 4, Sitosterolemia)
see also ABC transporters

Genetic disorders relating to deficiencies of transcription factor or coregulators

(1) Basic domains

1.2	Feingold syndrome Saethre–Chotzen syndrome
1.3	Tietz syndrome

(2) Zinc finger
DNA-binding domains

2.1	(Intracellular receptor): Thyroid hormone resistance Androgen insensitivity syndrome PAIS MAIS CAIS Kennedy's disease PHA1AD pseudohypoaldosteronism Estrogen insensitivity syndrome X-linked adrenal hypoplasia congenita MODY 1 Familial partial lipodystrophy 3 SF1 XY gonadal dysgenesis
2.2	Barakat syndrome Tricho–rhino–phalangeal syndrome
2.3	Greig cephalopolysyndactyly syndrome/Pallister–Hall syndrome Denys–Drash syndrome Duane-radial ray syndrome MODY 7 MRX 89 Townes–Brocks syndrome Acrocallosal syndrome Myotonic dystrophy 2
2.5	Autoimmune polyendocrine syndrome type 1

(3) Helix-turn-helix domains

3.1	ARX Ohtahara syndrome Lissencephaly X2 MNX1 Currarino syndrome HOXD13 SPD1 synpolydactyly PDX1 MODY 4 LMX1B Nail–patella syndrome MSX1 Tooth and nail syndrome OFC5 PITX2 Axenfeld syndrome 1 POU4F3 DFNA15 POU3F4 DFNX2 ZEB1 Posterior polymorphous corneal dystrophy Fuchs' dystrophy 3 ZEB2 Mowat–Wilson syndrome
3.2	PAX2 Papillorenal syndrome PAX3 Waardenburg syndrome 1&3 PAX4 MODY 9 PAX6 Gillespie syndrome Coloboma of optic nerve PAX8 Congenital hypothyroidism 2 PAX9 STHAG3
3.3	FOXC1 Axenfeld syndrome 3 Iridogoniodysgenesis, dominant type FOXC2 Lymphedema–distichiasis syndrome FOXE1 Bamforth–Lazarus syndrome FOXE3 Anterior segment mesenchymal dysgenesis FOXF1 ACD/MPV FOXI1 Enlarged vestibular aqueduct FOXL2 Premature ovarian failure 3 FOXP3 IPEX
3.5	IRF6 Van der Woude syndrome Popliteal pterygium syndrome

(4) β-Scaffold factors
with minor groove contacts

4.2	Hyperimmunoglobulin E syndrome
4.3	Holt–Oram syndrome Li–Fraumeni syndrome Ulnar–mammary syndrome
4.7	Campomelic dysplasia MODY 3 MODY 5 SF1 SRY XY gonadal dysgenesis Premature ovarian failure 7 SOX10 Waardenburg syndrome 4c Yemenite deaf-blind hypopigmentation syndrome
4.11	Cleidocranial dysostosis

(0) Other transcription factors

0.6	Kabuki syndrome

Ungrouped

Transcription coregulators

Coactivator:	CREBBP Rubinstein–Taybi syndrome
Corepressor:	HR (Atrichia with papular lesions)

Diseases of ion channels

Calcium channel

Voltage-gated	CACNA1A Familial hemiplegic migraine 1 Episodic ataxia 2 Spinocerebellar ataxia type-6 CACNA1C Timothy syndrome Brugada syndrome 3 Long QT syndrome 8 CACNA1F Ocular albinism 2 CSNB2A CACNA1S Hypokalemic periodic paralysis 1 Thyrotoxic periodic paralysis 1 CACNB2 Brugada syndrome 4
Ligand gated	RYR1 Malignant hyperthermia Central core disease RYR2 CPVT1 ARVD2