From News Medical
New technique slows progression of polycystic kidney disease in mice
Polycystic kidney disease (PKD) is a genetic disorder where fluid filled cysts grow in kidneys and destroy normal renal tissue. It is the world’s most common inherited kidney disease affecting between 1 in 400 and 1 in 1000 people worldwide – around 12.5 million individuals. A rarer form of the disease, which occurs in about one in every 20,000 live births in the UK, leads to a third of these babies dying before or just after birth.
Treatment for the condition has traditionally targeted proteins which are thought to play a role in causing the condition and are located in hair-like structures and tissue that line the inside of cysts. These treatments can help alleviate some of the symptoms of PKD but they can’t currently cure the condition.
Researchers have now discovered that the blood and lymphatic system surrounding cysts may also be important in the development of the condition and could be a new target for treating the disease.
By looking at mouse models of both the common and rarer form of the disease, the team noticed that tiny blood vessels surrounding the cysts were altered very early in cyst development. They therefore treated the mice with a potent ‘growth factor’ protein called VEGFC, and found that patterns of blood vessels normalised and the function of the kidneys improved. In the mice with the rare form of the condition, it also led to a modest but significant increase in lifespan.
David Long, lead researcher and Principal Research Associate at the ICH, explains:
"With further testing, treatments that target blood vessels surrounding the kidney cysts, perhaps in combination with currently used drugs, may prove to be beneficial for patients with polycystic kidney disease." [Read more]
"Even though it's adaptive to have this fight or flight response in the situation when you need it, if it's revved up all the time, then it's not good for your body," explained Dr. Jeanie Park, assistant professor at Emory University School of Medicine in Atlanta. Park found that a drug already FDA-approved for a metabolic disorder dials down the adrenaline levels in kidney patients. It's called tetrahydrobiopterin. Park studied 32 men with moderate kidney disease and found a decrease in sympathetic nerve activity in those who took the drug for 12 weeks. "It could be a novel way of reducing cardiovascular risk and potentially reducing blood pressure in patients with hypertension or chronic kidney disease," Park said. For patients like MacKenzie, that would mean managing just one chronic condition, kidney disease, instead of two. Right now, doctors use beta blockers and another drug, clonidine, to treat high blood pressure and the over-activation in the sympathetic nervous system. Park said those drugs are often hard for patients to tolerate. [Read more]
Living With PKD
LONDON, United Kingdom — A personalized exercise program integrated into routine dialysis can improve strength, endurance, and quality of life, new research indicates.
Fitness "improved significantly over a 1-year period, after which patients stabilized," said Kirsten Anding-Rost, MD, from the KfH Dialysis Center in Bischofswerda, Germany.
The program "was a really big success," she explained. "Normally in dialysis patients, health status steadily declines, so if you do something that maintains health status, it's actually very good."
Dr Anding-Rost presented results from the study here at the European Renal Association–European Dialysis and Transplant Association 52nd Congress.
The 46 study participants were typical dialysis patients. Mean age was 63 years, there were a lot of comorbidities, and three patients had undergone leg amputation.
The exercise program consisted of 30 minutes of combined resistance training that worked eight major muscle groups and 30 minutes of endurance training in the supine position on a bicycle ergometer.
Patients trained two times a week for 5 years, and were assessed with maximum strength testing and mean cycling power per training session.
"To adapt the training intensity to the personal fitness of each patient, we had to first measure the personal fitness of each patient," Dr Anding-Rost explained. And throughout the study period, exercise intensity was continuously adjusted to match improvements in fitness.
Of the initial 46 participants, 36 completed 1 year of the study and 20 completed 5 years.
Improvements in strength depended on how adherent patients were to their training sessions. For those with high adherence, improvements were significant in all eight muscle groups targeted by the resistance exercises.
In the moderate-adherence group, "we still saw an improvement in some muscle groups, but it was not as good as in the high-adherence group," Dr Anding-Rost reported.
There was a "nice improvement" in the average cycling power gained during the first 3 months of the exercise program, she said. After that, patient endurance remained more or less stable.
During the first 3 months, there was a 55% improvement in maximum exercise capacity in the high-adherence group and a 45% improvement in the moderate-adherence group.
There were also significant improvements on all three tests of physical function and in quality of life, ranging from 11% to 31%.
Physically weak patients actually had greater improvements in physical function than stronger patients.
From KDKA, CBS Affiliate Pittsburgh, by Brenda Waters
Salvation Army Employee Donates Kidney To Co-Worker
Thousands of people die every year waiting for a kidney transplant, but a minister at the Salvation Army is making sure her co-worker doesn’t end up on that list.
Michael Riemer, Director of Emergency Disaster Services at The Salvation Army’s Western Pennsylvania Division, is to receive a kidney Tuesday from co-worker Kate Esker.
“It’s the biggest day of my life, because I get a second chance at life,” Riemer tells KDKA’s Brenda Waters.
Riemer, 55, found out in his mid-20’s that he was diagnosed with polycystic kidney disease (PKD), a genetic disorder that took the lives of his mother, grandmother, and great-grandmother.
Riemer says PKD’s progression is long and slow, and remained private about his diagnosis for 30 years.
He has devoted his life to helping others. Riemer served Mt. Lebanon as a police officer for 25 years, and currently oversees disaster relief for The Salvation Army’s Western PA Division’s 28 counties.
When Allegheny General Hospital proposed to Riemer several months ago to go public in order to find potential donors, Esker, Core Officer and minister at McKeesport’s Salvation Army Worship and Service Center, immediately wished to help.
“I told my husband on the way home I wanted to see if I was a match,” says Esker. “I know Mike pretty well, and I like the life that he lives… there is so much life ahead of him.”
Riemer says he is forever indebted to Esker and her family.
“How do you begin to say thank you to someone who’s given you life a second time over? There’s not enough words; there’s not enough money in the world for this gift, a second time.”
Esker is “more excited than nervous.” She and Riemer would encourage anyone who knows someone who needs a kidney to check if they are a fit.
From WTSP, CBS Affiliate Tampa Bay, by Bobby Lewis
Friends' kidney swap will save them and strangers
It's one thing to loan a friend some money or a book but you have to have a special bond to offer up a body part.
"She's outstanding," said Subash Vajja. "She started saying, 'I'm going to give you a kidney,' and she never changed her mind. She stick with what she say."
Vajja and Beth Dillon, one of his wife's former co-workers, have become so close that they consider each other family. Vajja has been waiting a year and a half for a new kidney and Dillon didn't hesitate to give up hers for her friend.
The only problem was, the two weren't a match. They did a lot of research and found a Paired Kidney Exchange option – an opportunity to give and receive kidneys for those who are facing illness. Vajja has been fighting Polycystic Kidney Disease.
They went through all of the tests. Even after finding out her kidney wouldn't match Vajja's needs, Dillon still wanted to give up her organ as a way to honor her uncle.
"He was never healthy enough to receive a kidney," she said. "He passed away almost two years ago. June 13, 2013."
Now, two years later, on June 12, 2015, Vajja and Dillon are both having surgery to save and be saved by strangers.
Both will travel to Birmingham to have surgery. They are part of an 11-person kidney swap group that will all have surgery on Friday.
"I'm going to tease the doctor and say, 'Since you're in there, can you do a little lypo or something?'," joked Dillon. "I think it will just enlarge our family because we're all going to be touched by each other and I think it's going to be a life-long connection with people."
From Brookings Register, Brookings, South Dakota, by Eric Sandbulte
A 10-way gift of life
Brookings resident Greg Enz participated in a kidney exchange program at the Mayo Clinic in Rochester, Minn., in March so that his best friend’s mother could receive a lifesaving kidney donation herself. About 10 people were involved in the exchange program at the time, donors and recipients included. Pictured are Greg Enz at right, Kathy Allen at center and her son and Enz’s friend Ethan Allen at left.
“Greg is an absolutely wonderful person. He is just so generous and selfless, and myself and my family are just so grateful to him. He’s just a gem.”
Those are the words of Kathy Allen, who received a kidney transplant thanks to the generosity of Greg Enz and the Mayo Clinic’s kidney exchange program.
Enz, who works as a correctional officer at the Brookings County Detention Center, is a lifelong friend to Allen’s son, Ethan, ever since they met in the seventh grade. But Allen was diagnosed roughly 30 years ago with polycystic kidney disease (PKD), a common but life-threatening genetic kidney disease.
Fitness "improved significantly over a 1-year period, after which patients stabilized," said Kirsten Anding-Rost, MD, from the KfH Dialysis Center in Bischofswerda, Germany.
The program "was a really big success," she explained. "Normally in dialysis patients, health status steadily declines, so if you do something that maintains health status, it's actually very good."
Dr Anding-Rost presented results from the study here at the European Renal Association–European Dialysis and Transplant Association 52nd Congress.
The 46 study participants were typical dialysis patients. Mean age was 63 years, there were a lot of comorbidities, and three patients had undergone leg amputation.
The exercise program consisted of 30 minutes of combined resistance training that worked eight major muscle groups and 30 minutes of endurance training in the supine position on a bicycle ergometer.
Patients trained two times a week for 5 years, and were assessed with maximum strength testing and mean cycling power per training session.
"To adapt the training intensity to the personal fitness of each patient, we had to first measure the personal fitness of each patient," Dr Anding-Rost explained. And throughout the study period, exercise intensity was continuously adjusted to match improvements in fitness.
Of the initial 46 participants, 36 completed 1 year of the study and 20 completed 5 years.
Improvements in strength depended on how adherent patients were to their training sessions. For those with high adherence, improvements were significant in all eight muscle groups targeted by the resistance exercises.
In the moderate-adherence group, "we still saw an improvement in some muscle groups, but it was not as good as in the high-adherence group," Dr Anding-Rost reported.
There was a "nice improvement" in the average cycling power gained during the first 3 months of the exercise program, she said. After that, patient endurance remained more or less stable.
During the first 3 months, there was a 55% improvement in maximum exercise capacity in the high-adherence group and a 45% improvement in the moderate-adherence group.
There were also significant improvements on all three tests of physical function and in quality of life, ranging from 11% to 31%.
Physically weak patients actually had greater improvements in physical function than stronger patients.
Gift of Life
Thousands of people die every year waiting for a kidney transplant, but a minister at the Salvation Army is making sure her co-worker doesn’t end up on that list.
Michael Riemer, Director of Emergency Disaster Services at The Salvation Army’s Western Pennsylvania Division, is to receive a kidney Tuesday from co-worker Kate Esker.
“It’s the biggest day of my life, because I get a second chance at life,” Riemer tells KDKA’s Brenda Waters.
Riemer, 55, found out in his mid-20’s that he was diagnosed with polycystic kidney disease (PKD), a genetic disorder that took the lives of his mother, grandmother, and great-grandmother.
Riemer says PKD’s progression is long and slow, and remained private about his diagnosis for 30 years.
He has devoted his life to helping others. Riemer served Mt. Lebanon as a police officer for 25 years, and currently oversees disaster relief for The Salvation Army’s Western PA Division’s 28 counties.
When Allegheny General Hospital proposed to Riemer several months ago to go public in order to find potential donors, Esker, Core Officer and minister at McKeesport’s Salvation Army Worship and Service Center, immediately wished to help.
“I told my husband on the way home I wanted to see if I was a match,” says Esker. “I know Mike pretty well, and I like the life that he lives… there is so much life ahead of him.”
Riemer says he is forever indebted to Esker and her family.
“How do you begin to say thank you to someone who’s given you life a second time over? There’s not enough words; there’s not enough money in the world for this gift, a second time.”
Esker is “more excited than nervous.” She and Riemer would encourage anyone who knows someone who needs a kidney to check if they are a fit.
It's one thing to loan a friend some money or a book but you have to have a special bond to offer up a body part.
"She's outstanding," said Subash Vajja. "She started saying, 'I'm going to give you a kidney,' and she never changed her mind. She stick with what she say."
Vajja and Beth Dillon, one of his wife's former co-workers, have become so close that they consider each other family. Vajja has been waiting a year and a half for a new kidney and Dillon didn't hesitate to give up hers for her friend.
The only problem was, the two weren't a match. They did a lot of research and found a Paired Kidney Exchange option – an opportunity to give and receive kidneys for those who are facing illness. Vajja has been fighting Polycystic Kidney Disease.
They went through all of the tests. Even after finding out her kidney wouldn't match Vajja's needs, Dillon still wanted to give up her organ as a way to honor her uncle.
"He was never healthy enough to receive a kidney," she said. "He passed away almost two years ago. June 13, 2013."
Now, two years later, on June 12, 2015, Vajja and Dillon are both having surgery to save and be saved by strangers.
Both will travel to Birmingham to have surgery. They are part of an 11-person kidney swap group that will all have surgery on Friday.
"I'm going to tease the doctor and say, 'Since you're in there, can you do a little lypo or something?'," joked Dillon. "I think it will just enlarge our family because we're all going to be touched by each other and I think it's going to be a life-long connection with people."
A 10-way gift of life
Those are the words of Kathy Allen, who received a kidney transplant thanks to the generosity of Greg Enz and the Mayo Clinic’s kidney exchange program.
Enz, who works as a correctional officer at the Brookings County Detention Center, is a lifelong friend to Allen’s son, Ethan, ever since they met in the seventh grade. But Allen was diagnosed roughly 30 years ago with polycystic kidney disease (PKD), a common but life-threatening genetic kidney disease.
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