Rabson-Mendenhall Syndrome

Important

It is possible that the main title of the report Rabson-Mendenhall Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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• None

Disorder Subdivisions

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• None

General Discussion

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Rabson-Mendenhall syndrome is an extremely rare genetic disorder characterized by severe insulin resistance. Insulin, a hormone produced by the pancreas, regulates blood sugar levels by promoting the movement of glucose (a simple sugar) into cells for energy production or into the liver and fat cells for storage.



Initial symptoms of Rabson-Mendenhall syndrome include abnormalities of the head and face (craniofacial region), abnormalities of the teeth and nails, and skin abnormalities such as acanthosis nigricans, a skin disorder characterized by abnormally increased coloration (hyperpigmentation) and "velvety" thickening (hyperkeratosis) of the skin, particularly of skin fold regions, such as of the neck, groin, and under the arms. In most cases, additional symptoms are present. Rabson-Mendenhall syndrome is inherited as an autosomal recessive trait.

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Resources

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March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)428-7100

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



American Diabetes Association

National Call Center

1701 N. Beauregard Street

Alexandria, VA 22311

Tel: (703)549-1500

Fax: (703)549-6995

Tel: (800)342-2383

Email: askADA@diabetes.org

Internet: http://www.diabetes.org



NIH/Developmental Endocrinology Branch

Bldg 10, Room 10N262

10 Center Drive

Bethesda, MD 20892

Tel: (301)496-4686

Fax: (301)402-0574



NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Endocrine Diseases Metabolic Diseases Branch

2 Information Way

Bethesda, MD 20892-3570

Tel: (301)654-3810

Fax: (301)496-7422

Email: NDDIC@info.niddk.nih.gov

Internet: http://www.niddk.nih.gov



NIH/National Institute of Child Health and Human Development

31 Center Dr

Building 31, Room 2A32

MSC2425

Bethesda, MD 20892

Tel: (301)496-5133

Fax: (301)496-7101

Internet: http://www.nih.gov/hichd/

For a Complete Report

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This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  

Copyright   2001 National Organization for Rare Disorders, Inc.