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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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Keeping kids fit for life: with the dramatic rise in adult onset diabetes in American youth, a new initiative is teaching kids the tools for lifetime fitness
From Saturday Evening Post, 7/1/03 by Patrick Perry

Public-health officials are weighing in on the tidal wave of obesity in American kids, and the message is alarming. According to the CDC, one out of three kids aged 6 to 19 is overweight or obese, and pediatricians are witnessing an associated dramatic increase in health problems--high cholesterol, high blood pressure, sleep apnea, and type 2 diabetes.

Across the country, physicians are struggling with the unprecedented number of preteens and adolescents presenting with type 2 diabetes, recognizing the tremendous toll the disease places on both the lives of their young patients and the United States healthcare system. Typically a disease of middle-aged and older people, adult-onset diabetes was once a medical rarity among the young.

"There was a time when you would virtually never see type 2 diabetes in clinics that take care of children," Dr. C. Ronald Kahn, president of the Joslin Diabetes Center, told the Post. "Now, for example, at Children's Hospital in Boston, I would estimate that between 20 and 25 percent of the children seen actually have type 2 diabetes."

The twin evils of inactivity and overeating are cited as the root of the epidemic. Cutbacks in education resulted in school districts giving physical education classes the boot. With the trend showing no signs of stopping, many groups have stepped forward to take action before American youth reach adulthood.

One organization tackling the problem head-on is P.E.4Life, a nonprofit group that is spearheading a national initiative to restore physical education classes--with a modern twist--to American school systems. The organization was formed to promote and expand quality physical education programs in schools and to advocate mandatory physical education classes that focus on keeping all kids active, rather than sidelining most of the class while a handful of athletes sweats it out on the basketball court or gridiron.

To learn more about the promising new program that addresses the epidemic of adult-onset diabetes by teaching the importance of health and fitness for lifetime well-being, we spoke with Phil Lawler, director of the P.E.4Life Institute and physical education coordinator for Community Unit School District 203 and Madison Junior High School, Naperville, Illinois. The Naperville public-school P.E. program was named a model program by the CDC and is serving as a national prototype of the "New P.E."

Post: What is the P.E.4Life initiative, and how does it differ from previous physical education efforts?

Lawler: The bottom line is that 50 to 60 percent of the United States population who were not athletes had some bad experience in physical education and carry lifetime memories that they will never forget. Now they are parents, school-board members, and administrators who say, "I don't think it is necessary for my child to go through physical education classes. It's a glorified recess. I would rather have them in another English, math, or science class."

In District 203, we have completely changed our perspective. When we shifted the paradigm to health, wellness, and lifestyle management, we found that we met the needs of every kid who walked in the door, which was critical. I would debate with anyone the value of a quality physical education program on the life of a child as it prepares him or her for the 21st century over the span of their life. If done correctly, it is of equal importance than math, science or English.

Post: When did physical fitness become secondary to athletic achievement as a goal of America's school physical education programs?

Lawler: It's still happening. The athletic delivery of physical education still has the stronghold on the country. I am not devaluing the importance of sports. I am a coach. There are some young people whose lives have been touched by sports who would never be touched by any other avenue in a school system--it is their talent.

We are criticized at times for taking the competitive edge away from the physical education program. My response to that is--"Take on the challenge of managing your own health, weight, blood pressure, and cholesterol. Where are you in that competition?" Most Americans are not winning. I tell critics, "This is not about winning a game. This program is about competing to win back health for the rest of your life." Our program still uses team sports as a form of physical activity because more and more kids are enjoying sports now that the focus is not on athletic ability, but playing the sport for movement and enjoyment.

Post: You live in a community that is healthier than many in other parts of the country. Do you think that parents are upset by the lack of P.E. in the schools?

Lawler: If their children are not getting a quality physical education program, they should be frustrated and do something about it. In P.E.4Life, we try to empower parents across the country by asking, "Does your child receive a quality physical education program in the school system?"

Post: How did P.E.4Life come into existence?

Lawler: Naperville School District 203 pioneered the movement, and most of the national attention has been focused on our program. We began the program about ten years ago. The nonprofit organization P.E.4Life came on board in the last two years. The founder of P.E.4Life is Jim Baugh, the CEO of Wilson Sporting Goods, based nearby in Chicago. After looking at the data, they noted the obesity crisis in America's children, as well as the trends in physical education--programs are being cut. Kids are inactive.

If I am head of Wilson Sporting Goods--which sells basketballs, footballs, and sports equipment--and discover that long-term physical education programs are being cut and kids are less active, that trend doesn't bode well for my long-term business plan. Jim Baugh said that he was going to go get more physical education in our schools to improve athletics and sell equipment. But once Jim Baugh started investigating physical education, then discovered our program in Naperville, he contacted us. He found out that our main mission is about the health of kids. Baugh created P.E.4Life with an executive director, Anne Flannery, in Washington, D.C. He decided that to make changes, we had to be present in the political world. P.E.4Life also made a decision that if they were going to help people understand the vision, they needed a place to demonstrate it. They picked Naperville to be that demonstration site. Schools come here from all over the country to look at our program. I had 15 schools visit us in November alone and have had probably over 200 schools in the last five years from all over the United States, as well as schools from China and Mexico.

Post: Do you test cholesterol levels before students enter the program?

Lawler: Yes. The very first thing that I did when I envisioned expanding this program was to create a business partnership with a local cardiologist, Vincent Bufalino, former board member of the American Heart Association, to help us out. He became somewhat of a spokesperson for P.E.4Life. He was one of the first doctors in the country to start studying cholesterol levels of children. The partnership began in 1994, when we started annual cholesterol screening of our junior-high and high-school kids. We offered the screening to them; it is not mandated, but is a service.

When parents started getting their kids' cholesterol scores, they said, "Wait a second! My kid's scores are high. How do we change this?" We told parents the bottom line is that to reduce bad cholesterol, you have to eat correctly, and if you exercise, you can increase the good cholesterol.

In 1994, when our program was not yet in full swing, out of our 950 kids, 450 underwent cholesterol screening. Forty percent of our 11-, 12-, and 13-year-old kids had elevated cholesterol levels. The Cooper Institute in Dallas recommends 150 mg/dl for children. Dr. Bufalino arrived at a happy medium by using 175 mg/dl. Even at 175, 40 percent of the children tested had elevated cholesterol. The results certainly heightened awareness. The good news is, that number has been declining every year.

The point is: You can put all the data out there you want about kids' health, but when it's personalized and becomes "my" child, it takes on a whole different viewpoint.

Post: We are seeing such an unprecedented rise in adult-onset diabetes in our youth.

Lawler: Yes. A doctor from Texas told me that because this adult onset diabetes is happening at a younger and younger age, it has reached a point where life expectancy is shortened by almost 17 years now in these children.

Post: What are the students' reactions to this whole concept?

Lawler: They embrace it as much as their maturity allows. One of the ultimate goals of our program is for graduating seniors entering the real world to take ownership of their health responsibility. And it's working for us. The kids are starting to understand it.

Post: What activities do you employ to stir enthusiasm about sport activities?

Lawler: First of all, we have state-of-the-art equipment and facilities that many programs won't have. But you don't need all the bells and whistles to be successful. At the same time, a model program is needed. When I taught physical education and team sports, it was by the book. In football, the team consists of 11 on 11. In softball, it's nine on nine. Soccer is 11 on 11. Volleyball is six on six.

P.E. class is only 42 minutes long. When kids went to P.E. class, we would take attendance; then teams were picked. The team put on tennis shoes and got on the field, gathered into a huddle, called a play, and went to the line of scrimmage. The best athlete was quarterback and his best friend receiver. When play began, 15 to 20 kids were standing around, doing nothing. We spent a lot time arguing about who was offside and about conflict management.

We analyzed the 42-minute period to discover just how many kids were truly moving in class and physically active--how many kids actually touched the ball.

Something was wrong with this picture.

We threw out the whole concept of teaching sports. We still talk to them about sports, and we still have that athletic arena for people who really want to get into athletics. But when we play football, we play four on four. Sometimes we don't even have a line of scrimmage. If you throw a ball and it's incomplete, the other team gets and runs with it. If they are tagged, they throw it. We modify all the rules. We play soccer five on five. Basketball is no more than four on four. The games are modified so that there is more movement among all students involved. That is our ultimate goal.

Post: What about other activities, such as rock climbing, rope ladders, and others?

Lawler: When we built the program in the early 1990s, it was all based on cardiovascular fitness, with the ultimate goal to get every kid moving all the time. We have since learned some new dimensions. Dr. Kenneth Cooper, who invented the word aerobics, invited me to visit the Cooper Institute in Dallas last spring. He is a very strong supporter of what we are doing here and is very familiar with the program. He came to Naperville and gave a presentation to our community last year. He said that when he invented aerobics, he was convinced that aerobics was all that was necessary to remain healthy. But now we know that some type of strength and resistance training is necessary to burn calories and make us better movers, which is why we put in a fitness center. We want aerobic fitness and strength training.

Post: Are kids in the program using pedometers?

Lawler: We use pedometers at all levels--from elementary through high school. But pedometers are a tool that we use at the elementary level more than anywhere else, and kids like using them. We use the heart rate monitor for a little more accurate data. I think that pedometers are also a great tool for adults. They provide you with feedback.

Post: When did you begin to embrace different activities in your P.E. program? "

Lawler: We entered another dimension, called Adventure Education. Rock climbing is part of it. Naperville has a very heavy corporate ladder that stretches throughout the community. One time our district conducted a survey of businesses asking the question: In preparing future workers, what do we need to do differently to make these young people successful workers in your company? Ironically, they said that once they hire them, they can train them, but they wanted healthy workers because health insurance is becoming such an important issue. They also wanted people with good listening and team-building skills so they could teach leadership skills, as well as people who are willing to be risk-takers.

We embraced the concept of adventure education, which teaches all those concepts. We offer team-building to the sophomore class right now. We pick kids for the class. They have to demonstrate leadership skills, either in their church, community, or school. We train them in team-building skills and on how to become better leaders. CEOs talk to the kids about leadership skills, and people share the benefits and importance of good listening and motivational skills in the real world.

Post: Does exercise also have an impact on other arenas?

Lawler: We are developing lifetime skills that will make these kids more effective workers, communicators, husbands and wives, and better human beings.

Recently, researchers are finding out the importance of physical activity in the development of the brain. In Plato's time, they knew about the mind-body connection. But through the centuries, we lost sight of that connection. Dr. John J. Ratey is a Harvard brain-research specialist, and his research is fascinating. He said that in layman's terms, exercise is the fertilizer for the brain. He said that the fertilizer is so good, he calls it "miracle grow."

We now know from brain research that the body has almost enough electricity to light a 20-watt bulb, with most of that electricity used in the brain. We know that moisture is a great conductor of electricity. We know that if the body is hydrated while in school, students learn better. Exercise increases the blood flow and supply of oxygen to the brain. With exercise, brain functions better--memory and attention span, for example.

Post: Treadmills and other aerobic machinery are great to work out, but they can be boring. Do you use monitors and television to help keep the kids on the treadmill and exercising?

Lawler: Yes, we do. Our T-shirts read, "Getting Fit for Life at the Madison Health Club." We don't have Phys-Ed abbreviated with a school name. On the sleeve of every shirt in all of the schools in the district, it reads: "The Surgeon General has determined that an inactive lifestyle can be detrimental to your health." We are constantly sending messages to the parents about the importance of P.E.

Post: How do you track a student's progress?

Lawler: We are very fortunate tn that we have the technology to do this. We use heart-rate monitors for day-to-day tracking of the personalized fitness plan that is best for the kids. For cardiovascular fitness, we tell them to get their heart rate tn the healthy target zone for so many minutes. We also do long-term tracking of fitness scores. We pre-test and post-test sixth through 12th grade students. As kids proceed through school, they can see their personal profile.

Think about the adult population at 35 or 40 years old. All of sudden problems begin to arise--they are overweight, have onset diabetes, and suffer from high blood pressure. When they go to the doctor, he gives them information on what steps they need to address the problem--nutrition, diet, and exercise information. Physical education classes for them have failed.

In our system, we believe in cognitive tests. We test them on the information that they should know about exercise and nutrition. If every school in our country had quality physical education, we could show the nation that every child who graduates from our public and private schools would have the knowledge to take care of themselves as they leave to begin the rest of their lives. Using that system, it would more than pay for itself. We could have all the bells and whistles in every school in the country and still save billions of dollars in healthcare costs.

Is every kid going to remain fit? No. But when the readiness is there, they are already equipped.

To learn more about the P.E.4Life program, visit their Web site at pe4life.org or www.ncusd203.org/madison/newpe.

COPYRIGHT 2003 Saturday Evening Post Society
COPYRIGHT 2003 Gale Group

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