Transient neonatal diabetes mellitus

Title

Other Names:

TNDM1; TNDM; DMTN; TNDM1; TNDM; DMTN; Diabetes mellitus, transient neonatal; Chromosome 6-associated transient diabetes mellitus; Diabetes mellitus, 6q24-related transient neonatal See More

Categories:

Congenital and Genetic Diseases; Endocrine Diseases; Metabolic disorders

Summary Summary

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.^[1] Affected individuals have slow growth before birth followed by hyperglycemia, dehydration and failure to thrive in infancy.^[2] 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.^[3]^[4] These cases are referred to as 6q24-related TNDB; most (but not all) of these cases are not inherited.^[4] Other genetic causes include mutations in the KCNJ11 and ABCC8 genes, which usually cause permanent neonatal diabetes.^[3] Treatment may include rehydration and intravenous insulin at the time of diagnosis, followed by subcutaneous insulin.^[3]

Last updated: 8/28/2012

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormality of the pancreatic islet cells		0006476
Dehydration		0001944
Failure to thrive	Faltering weight Weight faltering [ more ]	0001508
Glycosuria		0003076
Hypovolemia	Depleted blood volume	0011106
Intrauterine growth retardation	Prenatal growth deficiency Prenatal growth retardation [ more ]	0001511
Weight loss		0001824
30%-79% of people have these symptoms
Arthrogryposis multiplex congenita		0002804
Bilateral ptosis	Drooping of both upper eyelids	0001488
Contractures of the joints of the lower limbs		0005750
Downturned corners of mouth	Downturned corners of the mouth Downturned mouth [ more ]	0002714
Global developmental delay		0001263
Intellectual disability	Mental deficiency Mental retardation Mental retardation, nonspecific Mental-retardation [ more ]	0001249
Ketonuria		0002919
Motor delay		0001270
Prominent metopic ridge		0005487
5%-29% of people have these symptoms
Abnormal heart morphology	Abnormality of the heart Abnormally shaped heart [ more ]	0001627
Apraxia		0002186
Coma		0001259
Generalized myoclonic seizures		0002123
Generalized tonic-clonic seizures	Grand mal seizures	0002069
Hearing impairment	Deafness Hearing defect [ more ]	0000365
Muscular hypotonia	Low or weak muscle tone	0001252
Neonatal onset		0003623
Renal tubular dysfunction		0000124
Steatorrhea		0002570
1%-4% of people have these symptoms
Type II diabetes mellitus	Noninsulin-dependent diabetes Type 2 diabetes Type II diabetes [ more ]	0005978
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance		0000006
Elevated hemoglobin A1c		0040217
Hyperglycemia	High blood sugar	0003074
Maternal diabetes	gestational diabetes	0009800
Severe failure to thrive	Severe faltering weight Severe weight faltering [ more ]	0001525
Transient neonatal diabetes mellitus		0008255

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 6/1/2018

Do you have updated information on this disease? We want to hear from you.

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Transient neonatal diabetes mellitus. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

International Diabetes Federation (IDF)
166 Chaussee de la Hulpe
B-1170 Brussels, Belgium
Belgium
Telephone: +32-2-5385511
Fax: +32-2-5385114
E-mail: info@idf.org
Website: http://www.idf.org/
International Society for Pediatric and Adolescent Diabetes (ISPAD)
c/o KIT Kurfurstendamm 71
10709 Berlin
Germany
Telephone: 49 30 24603213
Fax: 49 30 24603200
E-mail: secretariat@ispad.org

Organizations Providing General Support

American Diabetes Association
2451 Crystal Drive
Suite 900
Arlington, VA 22202
Toll-free: 1–800–DIABETES (342–2383)
Fax: 703–549–6995
E-mail: AskADA@diabetes.org
Website: http://www.diabetes.org

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Genetics Home Reference (GHR) contains information on Transient neonatal diabetes mellitus. This website is maintained by the National Library of Medicine.
The National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC), part of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), conducts and supports research on a broad spectrum of diseases affecting public health. Click on the link to view information on this topic.

In-Depth Information

GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Transient neonatal diabetes mellitus 1
Transient neonatal diabetes mellitus 2
Transient neonatal diabetes mellitus 3
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Transient neonatal diabetes mellitus. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My son was diagnosed with neonatal diabetes mellitus. He is 15 days old and we are not sure if this is permanent or transient. I want to know what the lifespan is of babies with this illness. Also how can we find out if this diabetes is transient or permanent? See answer

Have a question? Contact a GARD Information Specialist.

References References

Michel Polak. Neonatal diabetes mellitus. Orphanet. March 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=224. Accessed 8/24/2012.
Permanent neonatal diabetes mellitus. Genetics Home Reference. July 2011; http://ghr.nlm.nih.gov/condition/permanent-neonatal-diabetes-mellitus. Accessed 8/24/2012.
Isabel Karen Temple and Deborah JG Mackay. Diabetes Mellitus, 6q24-Related Transient Neonatal. GeneReviews. December 23, 2010; http://www.ncbi.nlm.nih.gov/books/NBK1534/. Accessed 8/24/2012.
6q24-related transient neonatal diabetes mellitus. Genetics Home Reference. February 2011; http://ghr.nlm.nih.gov/condition/6q24-related-transient-neonatal-diabetes-mellitus. Accessed 8/28/2012.