Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Rabson-Mendenhall syndrome


Other Names for this Disease
  • Mendenhall Syndrome
  • Pineal hyperplasia, insulin-resistant diabetes mellitus, and somatic abnormalities
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview


Rabson-Mendenhall syndrome is a genetic disorder characterized by severe insulin resistance.[1][2] Insulin, a hormone produced by the pancreas, regulates blood sugar levels by promoting the movement of glucose into cells for energy production or into the liver and fat cells for storage.[1] Symptoms of Rabson-Mendenhall syndrome may include intrauterine and postnatal growth retardation, hypertrophy of muscle and fat tissues, abnormalities of the head and face, abnormalities of the teeth and nails, and skin abnormalities such as acanthosis nigricans.[1][2] Additional symptoms may also be present.[1] Rabson-Mendenhall syndrome is inherited in an autosomal recessive manner.[1][2] Treatment is difficult and may include high doses of insulin and/or recombinant insulin-like growth factor.[2]
Last updated: 11/9/2010

References

  1. Cochran E. Rabson-Mendenhall Syndrome. National Organization for Rare Disorders (NORD). 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Rabson-Mendenhall%20Syndrome. Accessed 11/9/2010.
  2. Vigouroux C. Rabson-Mendenhall syndrome. Orphanet. 2009; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=769. Accessed 11/9/2010.
Your Questions Answered
by the Genetic and Rare Diseases Information Center

Please contact us with your questions about Rabson-Mendenhall syndrome. We will answer your question and update these pages with new resources and information.

Basic Information

  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an 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. 
  • 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 Rabson-Mendenhall syndrome. Click on the link to view a sample search on this topic.