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Maturity onset diabetes of the young

Maturity onset diabetes of the young (MODY) refers to any of several rare hereditary forms of diabetes mellitus due to dominantly inherited defects of insulin secretion. As of 2004, six types have been enumerated, but more are likely to be added. MODY 2 and MODY 3 are the most common forms. The severity of the different types varies considerably, but most commonly MODY acts like a very mild version of type 1 diabetes, with continued partial insulin production and normal insulin sensitivity. It is not type 2 diabetes in a young person, as might erroneously be inferred from the name. more...

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History of the concept and treatment of MODY

The term MODY dates back to 1964, when diabetes mellitus was considered to have two main forms: juvenile-onset and maturity-onset, which roughly corresponded to what we now call type 1 and type 2. MODY was originally applied to any child or young adult who had persistent, asymptomatic hyperglycemia without progression to diabetic ketosis or ketoacidosis. In retrospect we can now recognize that this category covered a heterogeneous collection of disorders which included cases of dominantly inherited diabetes (the topic of this article, still called MODY today), as well as cases of what we would now call type 2 diabetes occurring in childhood or adolescence, and a few even rarer types of hyperglycemia (e.g., mitochondrial diabetes or mutant insulin). Many of these patients were treated with sulfonylureas with varying degrees of success.

By the 1990s, as our understanding of the pathophysiology of the various forms of diabetes has increased, the concept and usage of "MODY" have become refined and narrower. It is now used as a synonym for dominantly inherited, monogenic defects of insulin secretion occurring at any age, and no longer includes any forms of type 2 diabetes.

Signs, symptoms and differential diagnosis

There are two general types of clinical presentation. Some forms of MODY produce significant hyperglycemia and the typical signs and symptoms of diabetes: increased thirst and urination (polydipsia and polyuria). In contrast, however, many people with MODY have no signs or symptoms and are diagnosed by either (1) accident, when a high glucose is discovered during testing for other reasons, or (2) screening of relatives of a person discovered to have diabetes. Discovery of mild hyperglycemia during a routine glucose tolerance test for pregnancy is particularly characteristic.

MODY cases may make up as many as 5% of presumed type 1 and type 2 diabetes cases in a large clinic population. While the goals of diabetes management are the same no matter what type, the two primary advantages of confirming a diagnosis of MODY are that (1) insulin may not be necessary and it may be possible to switch a person from insulin injections to oral agents without loss of glycemic control, and (2) it may prompt screening of relatives and discovery of other cases in family members.

As it occurs infrequently, many cases of MODY are initially assumed to be more common forms of diabetes: type 1 if the patient is young and not overweight, type 2 if the patient is overweight, or gestational diabetes if the patient is pregnant. Standard diabetes treatments (insulin for type 1 and gestational diabetes, and oral hypoglycemic agents for type 2 are often initiated before the doctor suspects a more unusual form of diabetes. In some forms of MODY, standard treatment is appropriate, though exceptions occur. For example, in MODY2, oral agents are relatively ineffective and insulin is unnecessary, while in MODY1 and MODY3, insulin may be more effective than drugs to increase insulin sensitivity. Sulfonylureas are effective in the KATP channel forms of MODYX.

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RCN paediatric and adolescent diabetes group.
From Journal of Diabetes Nursing, 9/1/03

Due to lack of numbers our study day on 11 June, 2003, had to be cancelled. Apologies to those of you who wanted to attend and to the speakers who had kindly agreed to speak. We did receive several contacts and forms after the closing date but unfortunately we do have to stick to a deadline otherwise it becomes very expensive and wasteful when we have to cancel.

[ILLUSTRATION OMITTED]

We need to review whether we should continue with the study day each year and so it would be helpful if you could tell us if there was a particular reason for not applying. Was the reason you did not attend due to:

* Cost?

* Time of year?

* Content?

* Location?

* Other conferences?

* Finance delaying return of form?

* Other--please specify?

I look forward to seeing some of you at the De Vere Belfry, North Warwickshire for the November conference. Please remember that we need payment by the closing date. The final closing date is 3 October, 2003.

Do you have patients with a family history of diabetes?

Diabetes is on the increase in the UK and worldwide. While some research focuses on current treatment options for diabetes, other studies have attempted to gain a better insight into the causes of diabetes. We know that some types of diabetes run in families, suggesting a genetic cause.

One particular type of diabetes that is inherited is maturity onset diabetes of the young (MODY). This was first noticed in the 1970s when Tattersall and Fajan found that some people they had diagnosed with type I diabetes did not seem to require insulin (Tattersall, 1998). People with MODY have diabetes that presents at a young age (less than 25 years). MODY is inherited in an autosomal dominant fashion. Some type 2 diabetes is also inherited in this way, with a relatively early age of onset. Research has focused on finding genes for diabetes in these families as this may not only tell us about how diabetes is caused, but could also help in the design of new treatments for diabetes.

Since the 1970s, six genes have been found for MODY, but 20% of people with MODY do not have any of these genes (Frayling et al, 2001). Unfortunately, few useful genes have been found for type 2 diabetes. In adults it can be hard to disentangle inherited causes of diabetes from those caused by other disease, diet and lifestyle. As diabetes has become more common in children, interest has turned to looking for genes in younger people, as they will have fewer environmental factors clouding the causes for their diabetes.

At Birmingham Children's Hospital we are currently running a nationwide study in collaboration with Prof. Andrew Hattersley's team at the Peninsular Medical School, Exeter, sponsored generously by Diabetes UK, to look at young people (diagnosed before 16 years old) who have inherited diabetes from one parent that is not type 1. We are taking DNA from all members of these families with or without diabetes to see if we can find genes that pass down families: children, parents and grandparents.

If you think that one of your families could be eligible for this study please contact Dr John Porter at Birmingham Children's Hospital: 0121 3339267 or email j.porter@bham.ac.uk.

Frayling et al (2001) B cell genes and diabetes: molecular and clinical characterisation of mutations in transcription factors. Diabetes 50: S94-100

Tattersall R (1998) Maturity-onset diabetes of the young: a clinical history. Diabetic Medicine 15: 11-14

COPYRIGHT 2003 S.B. Communications
COPYRIGHT 2004 Gale Group

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