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UF study sheds light on cystic fibrosis-related diabetes
July 07, 2006Gainesville, Fla. — A growing number of cystic fibrosis patients are battling a second, often deadly complication: a unique form of diabetes that shares characteristics of the type 1 and type 2 versions that strike many Americans.
Many of these patients are teens who take enzymes to help digest their food and undergo daily physical therapy to loosen the thick, sticky mucus that clogs their lungs. But despite treatments that are helping thousands to live decades longer than ever before, when diabetes strikes, their life expectancy plummets — on average by two years for men and an astounding 16 for women.
Now a University of Florida study in animals suggests diabetes in cystic fibrosis patients is not caused by the destruction of insulin-producing cells in the pancreas — as is often the case in patients with the traditional form of type 1 diabetes — but by differences in how these cells function. The findings were published this month in the American Diabetes Association's journal Diabetes.
Cystic fibrosis patients with diabetes produce some insulin on their own, but they require daily injections to boost their levels when eating so they can properly use sugar and other food nutrients for energy. At times they also become very resistant to the insulin they do make, similar to people with type 2 diabetes.
"For the longest time, the development of diabetes in cystic fibrosis has been thought to be chronic destruction of pancreas, so eventually you get loss of the insulin-producing beta cells," said Michael Stalvey, M.D., an assistant professor of pediatrics at UF. "Our study provides some early evidence to suggest there is an inherent difference in beta cell function."
Cystic fibrosis patients suffer recurrent episodes of infection and inflammation that slowly destroy the lungs. The pancreas is also affected, interfering with proper digestion. The disease stems from a faulty gene that blocks the normal passage of salt and water through the body's cells. It is this gene deficiency that is proposed to cause insulin-producing cells to malfunction, Stalvey said.
About 30,000 Americans have cystic fibrosis, making it the nation's most common lethal hereditary disorder. On average, they will not live past 35, though some are living through their 40s and even into their 60s. As each year passes, the likelihood they will develop diabetes increases. As many as 16 percent of all patients with cystic fibrosis also have diabetes, a number that is expected to rise as overall life expectancy for cystic fibrosis patients increases. Half will show signs of diabetes by age 30 and will suffer a rapid decline in overall health and lung function, muscle mass and body mass index.
"It's becoming more and more frequent because of the increasing age of patients," Stalvey said. "That's part of the reason why new recommendations call for screening patients 14 years and older yearly with an oral glucose tolerance test. Each year we know their likelihood of developing diabetes gets higher and higher.
"These young people, teenagers or young adults in their early 20s, have been fighting all their lives to stay healthy and keep their nutrition up," he added. "Now they've just been given something that potentially will overwhelm them. It's a huge thing for them, given the consequences that diabetes means to their underlying condition."
In the UF study, researchers developed the first animal model for the study of cystic fibrosis-related diabetes. They used mice that scientists from the University of North Carolina engineered to be missing the gene that makes the protein responsible for transporting salt and water across the cell membrane. People with cystic fibrosis have a mutated form of this protein.
UF scientists administered a low dose of a chemotherapy drug that weakened insulin-producing cells but did not destroy them. They then tested the animals' ability to regulate their blood sugar while fasting and after receiving glucose, simulating the rise in blood sugar that occurs after eating food.
Animals with the protein deficiency were more sensitive to the effects of the chemotherapy drug and had more difficulty regulating blood sugar levels, both while fasting and after receiving glucose. Mice that were still able to produce the crucial protein that prevents cystic fibrosis were able to maintain normal blood sugar levels, even after the drug had damaged some of their insulin-producing cells.
"This goes beyond improving our understanding of patients with cystic fibrosis-related diabetes; it also will help us improve our understanding of other forms of diabetes and help us work on strategies for a future cure," Stalvey said.
"Twenty-five percent of adolescents and 40 percent of adults with cystic fibrosis have diabetes, and diabetes is associated with poorer survival in this population," said Antoinette Moran, M.D., division head of pediatric endocrinology and director of the Pediatric Diabetes Program at the University of Minnesota Medical School. "The cause of cystic fibrosis-related diabetes is not completely understood, but it is clearly different from other forms of diabetes. The study by Stalvey and colleagues is important because it is the first to show that there are intrinsic abnormalities in the insulin-producing cells of the pancreas related to the genetic defect that causes cystic fibrosis.\\\
University of Florida
Cystic fibrosis patients with diabetes produce some insulin on their own, but they require daily injections to boost their levels when eating so they can properly use sugar and other food nutrients for energy. At times they also become very resistant to the insulin they do make, similar to people with type 2 diabetes.
"For the longest time, the development of diabetes in cystic fibrosis has been thought to be chronic destruction of pancreas, so eventually you get loss of the insulin-producing beta cells," said Michael Stalvey, M.D., an assistant professor of pediatrics at UF. "Our study provides some early evidence to suggest there is an inherent difference in beta cell function."
Cystic fibrosis patients suffer recurrent episodes of infection and inflammation that slowly destroy the lungs. The pancreas is also affected, interfering with proper digestion. The disease stems from a faulty gene that blocks the normal passage of salt and water through the body's cells. It is this gene deficiency that is proposed to cause insulin-producing cells to malfunction, Stalvey said.
About 30,000 Americans have cystic fibrosis, making it the nation's most common lethal hereditary disorder. On average, they will not live past 35, though some are living through their 40s and even into their 60s. As each year passes, the likelihood they will develop diabetes increases. As many as 16 percent of all patients with cystic fibrosis also have diabetes, a number that is expected to rise as overall life expectancy for cystic fibrosis patients increases. Half will show signs of diabetes by age 30 and will suffer a rapid decline in overall health and lung function, muscle mass and body mass index.
"It's becoming more and more frequent because of the increasing age of patients," Stalvey said. "That's part of the reason why new recommendations call for screening patients 14 years and older yearly with an oral glucose tolerance test. Each year we know their likelihood of developing diabetes gets higher and higher.
"These young people, teenagers or young adults in their early 20s, have been fighting all their lives to stay healthy and keep their nutrition up," he added. "Now they've just been given something that potentially will overwhelm them. It's a huge thing for them, given the consequences that diabetes means to their underlying condition."
In the UF study, researchers developed the first animal model for the study of cystic fibrosis-related diabetes. They used mice that scientists from the University of North Carolina engineered to be missing the gene that makes the protein responsible for transporting salt and water across the cell membrane. People with cystic fibrosis have a mutated form of this protein.
UF scientists administered a low dose of a chemotherapy drug that weakened insulin-producing cells but did not destroy them. They then tested the animals' ability to regulate their blood sugar while fasting and after receiving glucose, simulating the rise in blood sugar that occurs after eating food.
Animals with the protein deficiency were more sensitive to the effects of the chemotherapy drug and had more difficulty regulating blood sugar levels, both while fasting and after receiving glucose. Mice that were still able to produce the crucial protein that prevents cystic fibrosis were able to maintain normal blood sugar levels, even after the drug had damaged some of their insulin-producing cells.
"This goes beyond improving our understanding of patients with cystic fibrosis-related diabetes; it also will help us improve our understanding of other forms of diabetes and help us work on strategies for a future cure," Stalvey said.
"Twenty-five percent of adolescents and 40 percent of adults with cystic fibrosis have diabetes, and diabetes is associated with poorer survival in this population," said Antoinette Moran, M.D., division head of pediatric endocrinology and director of the Pediatric Diabetes Program at the University of Minnesota Medical School. "The cause of cystic fibrosis-related diabetes is not completely understood, but it is clearly different from other forms of diabetes. The study by Stalvey and colleagues is important because it is the first to show that there are intrinsic abnormalities in the insulin-producing cells of the pancreas related to the genetic defect that causes cystic fibrosis.\\\
University of Florida
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Cystic Fibrosis: A Guide for Patient and Family by David M Orenstein (Editor) Univ. of Pittsburgh, PA. Text is designed specifically for patients with cystic fibrosis and their families. Explains the disease process, outlines the fundamentals of diagnosing and screening, and addresses the challenges of treatment for those living with CF. Includes new material on carrier testing, infection control, and more. Previous edition: c1997. Softcover. |
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Cystic Fibrosis: Everything You Need To Know (Your Personal Health) by Wayne Kepron MD FRCPC (Author) Cystic fibrosis afflicts approximately 30,000 Americans. The average age of survival has been steadily increasing, but not quickly enough: a child born with cystic fibrosis today can only expect to live 35 to 40 years. In this valuable new addition to the Your Personal Health series, Dr. Wayne Kepron offers a comprehensive look at the disease that afflicts so many young people. Topics include: - Symptoms of cystic fibrosis - Making a diagnosis - Complications of the disease - Treatments (including lung transplants) - Techniques and precautions in lung care - Transition into adulthood - Prospects for gene therapy - End-of-life issues Using diagrams, charts and case studies, Cystic Fibrosis is designed for patients, their families, and caregivers. It is both a quick... |
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With Every Breath: stories by and about people living with cystic fibrosis by Katherine Russell and Margot Russell (Author), Katherine Russell and Margot Russell (Editor) Over 30,000 people in the US have cystic fibrosis, a life-threatening genetic disease that affects the lungs and digestive system. Physically, fighting it is a very perilous struggle. Emotionally, coping can oftentimes be even harder. With Every Breath consists of stories by and about people living with cystic fibrosis. Created to motivate, inspire, and generate positivity for those living with cystic fibrosis, this book is something you can open time and time again. Designed for all ages, this collection of diverse stories offers unique perspectives from patients, a CF doctor and nurse, and family members of those living with the illness. Patients aren't the only ones who will want to read With Every Breath as family members, friends, and doctors can all find inspiration when they... |
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Cystic Fibrosis (Facts) by Ann Harris (Author), Anne Thomson (Author) Cystic Fibrosis: The Facts provides a much needed simple and understandable source book about this disease. It is aimed at those living with Cystic Fibrosis (CF), either themselves or members of their families or their friends. The book explains clearly what is happening to the body in CF, what causes it and what treatment options are available for the different aspects of the disease. There are more detailed chapters for those wanting to find out about the genetics of the disease and specific aspects such as how it affects life choices and employment. It looks to the future in terms of potential new therapies for CF and provides useful information on organizations that can provide help and further information across those areas of the world where the disease is prevalent. |
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The Power of Two: A Twin Triumph over Cystic Fibrosis by Isabel Stenzel Byrnes; Anabel Stenzel (Author) The tragedy of cystic fibrosis has been touchingly recounted before, but this is the first book to portray the symbiotic relationship between twins who share this life-threatening disease through adulthood. Isabel Stenzel Byrnes and Anabel Stenzel tell of their struggle to pursue normal lives while grappling with the realization that they might die young. Their story reflects the physical and emotional challenges of a particularly aggressive form of CF and tells how the twins bicultural heritage Japanese and German influenced the way they coped. The Power of Two is an honest and gripping portrayal of day-to-day health care, the impact of chronic illness on marriage and family, and the importance of a support network to continuing survival. These two remarkable sisters have much to teach... |
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The Stones Applaud: How Cystic Fibrosis Shaped My Childhood by Teresa Anne Mullin (Author) Teresa Mullin was diagnosed with cystic fibrosis at four years of age, but it was not until she was nine that she learned most children with the disease were not expected to live to adulthood. What had been a nuisance soon became a force that molded her childhood, youth, and future. In The Stones Applaud, Mullin writes of absences from school, serving as a poster child, frequent hospitalizations, medical treatments, and most painful the isolation that came with cystic fibrosis, an inherited condition that damages the lungs and affects the digestive system. With dry humor and sharp insights, Mullin describes her battles with the disease, teachers, fellow students, and even medical professionals who tried to hold her back from experiencing life. Alternately funny, frank, poignant, and... |
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Cadberry's Letters by Jennifer Racek (Author) Cadberry's mom is very forgetful. Every time they visit his doctor together she talks about C & F. She never remembers the other letters. When Cadberry draws a set of letters to help his mother remember them all, he discovers what C & F really mean and how those two tiny letters affect so much of his life. Developed for pre-school-age children, Cadberry's Letters uses simple, easy-to-follow language to explain Cystic Fibrosis and the daily care that goes along with it in terms young children can understand. Children will learn along with Cadberry about things like Pancreatic Enzymes, Chest Physical Therapy and more. Bright, full-page illustrations bring the story to life and feature a lot of the equipment and medications CF patients may use in their daily lives. |
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Cystic Fibrosis by Margaret Hodson (Author), Duncan Geddes (Author), Andrew Bush (Author) This international and authoritative work, which brings together current knowledge in the field of cystic fibrosis, has become established in previous editions as a leading reference in the field. The third edition continues to provide everything that the clinician or allied health professional treating patients with cystic fibrosis will need in a single manageable volume. Thoroughly revised and updated throughout, it reflects the significant advances that have been made in the field since the second edition published in 2000. Cystic Fibrosis outlines in detail the basic science that underlies the disease and its progression, putting it into a clinical context. Diagnostic and clinical aspects are covered in depth, as are monitoring the condition and the importance of... |
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Taking Cystic Fibrosis to School by Cynthia S. Henry (Author), Cynthia S. Henry (Author), Tom Dineen (Author), Tom Dineen (Illustrator) These beautifully illustrated and fun-to-read storybooks simplify and normalize complicated childhood conditions, like cystic fibrosis. When read aloud, other children can identify why a peer may be treated differently and begin to empathize with them. In addition, children whose conditions set them apart as being different begin to feel accepted and safe. Each book includes a Kids' Quiz to reinforce new information and Ten Tips for Teachers to provide additional facts and ideas for teacher use. In Taking Cystic Fibrosis to School, Jessie explains to her classmates that even though she has cystic fibrosis, she can still attend school. |
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Understanding Cystic Fibrosis (Understanding Health and Sickness Series) by Ph.D., Karen Hopkin (Author) Since the discovery of the CF gene in 1989, scientists have learned a great deal about the biology of this disease which strikes one child in every 3,300 births. With the gene pinpointed, scientists are now working on ways to replace it and are developing better tests for earlier diagnosis. Understanding CF charts the progress that has been made in a disease whose symptoms can range from mild respiratory distress to life-threatening lung infections |
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