Sunday, June 12, 2016

PKD in Kids, Diagnosing PKD, PKD: Kidney Search Starts Over; Gift of Life: Santa Clarita, Milwaukee

Living with PKD

From BMJ (British Medical Journal), United Kingdom

Autosomal dominant polycystic kidney disease in children

Article authors:
Satyamaanasa Polubothu, academic clinical fellow1,
Amanda Richardson, paediatric nephrology specialist trainee2,
Larissa Kerecuk, consultant paediatric nephrologist3,
Manish Sinha, consultant paediatric nephrologist2

Screen now to save later?

Autosomal dominant polycystic kidney disease (ADPKD) was previously known as “adult PKD” because most cases come to medical attention during adulthood. However, clinically important morbidity such as hypertension and cardiovascular disease occurs much earlier. Screening children could detect those affected, and randomised controlled trials show that early intervention reduces disease progression. Despite this, very few children from families with the condition are currently assessed in the United Kingdom.

ADPKD is the most common inherited kidney disease and accounts for 10% of people under 65 years on renal replacement therapy in the UK.1 Often asymptomatic during the first three decades of life, the condition typically presents with abdominal pain or an incidental finding of hypertension. Renal disease is characterised by gradual bilateral cystic dilation of the renal tubules, with eventual compression of normal renal parenchyma and kidney enlargement. After patients develop hypertension, the disease has an accelerated trajectory, with a … [Read more]




From News-Medical, By Sally Robertson, BSc

Diagnosing Kidney Cysts

Kidney (renal) cysts are fluid-filled sacs that form on one or both of the kidneys. Usually, these cysts do not cause symptoms or kidney damage, in which case they are referred to as simple kidney cysts.

Simple kidney cysts are very different from the type of cysts that develop when a person has the genetic condition polycystic kidney disease (PKD), which causes scarring of the kidney tissue that can eventually damage the kidney and lead to kidney failure.

Simple cysts sometimes cause symptoms if they have become large enough or have started to press on other organs. They can also become infected, leading to pain and fever.

Diagnosing Simple Kidney Cysts

Since these cysts do not generally cause symptoms, they are usually only discovered when a test or scan is performed for some other condition. When a cyst is found, imaging tests are performed to determine whether it is a simple cyst or a sign of a more serious condition. The imaging tests that may be employed are described below.

Ultrasound

Ultrasound employs a device called a transducer to bounce sound waves off organs and generate an image of their structure. Images of the whole urinary tract can be created and used to distinguish between cysts that are harmless and those that signal more serious conditions.

Computerized tomography

In a computerized tomography (CT) scan, a combination of X-rays and computer technology is used to generate 3D images. This may involve a dye being injected, which is referred to as a contrast medium. For these scans, patients are required to lie down on a table, which is moved into a device shaped like a tunnel where the X-rays are obtained. These scans can reveal tumors and cysts present in the kidneys.

Magnetic resonance imaging

Magnetic resonance imaging (MRI) scans use magnets and radiation to create very detailed images of soft tissue and organs. This process may also involve the use of a contrast medium. Here again, a person lies on a table that is moved into a tunnel-like machine. These scans also reveal the presence of any cysts or tumors.

Diagnosing PKD

PKD is usually diagnosed using ultrasound, which is the most non-invasive and cost-effective way of diagnosing the condition. If a person is at risk of PKD, but is aged older than 40 years and has an ultrasound scan that shows the kidneys are normal, then they are unlikely to have PKD. Occasionally, a CT scan or MRI scan may be used to find cysts that are too small to be detected by ultrasound. When cysts are found, MRI is the technique used to monitor the growth and volume of both the kidneys and the cysts.

Sometimes, genetic testing is performed, which involves a sample of blood being taken and checked for gene mutations known to cause PKD. This is not always recommended because it is expensive and not always reliable. Genetic testing fails to detect PKD in around 15% of patients who undergo the test. Situations where genetic testing may be recommended include the following:
Where there is uncertainty in diagnosis after imaging techniques have been used
Where a person is younger than 30 years of age and has a family history of PKD, a negative ultrasound, and intends to have a children
Where a person with a family history of the condition wishes to donate a kidney

Reviewed by Susha Cheriyedath, MSc

Sources
https://www.kidney.org/atoz/content/polycystic
http://www.niddk.nih.gov/health-information/health-topics/kidney-disease/polycystic-kidney-disease-pkd/Pages/facts.aspx
http://pkdcharity.org.uk/
https://www.kidney.org/atoz/content/simple-kidney-cysts
Further Reading
Kidney Cyst Removal




From Boston Globe, By Eric Moskowitz, Globe Staff

Cambridge educator must start search for kidney anew

Cambridge, Massachusetts -- 1/19/2016- Parent Julia Kanno (R) hopes to be able to donate a kidney to after-school program director Christine Tierney the two pose for a portrait at Cambridgeport School in Cambridge, Massachusetts January 19, 2016. Jessica Rinaldi/Globe Staff Topic: 21kidney Reporter:

Christine Tierney has to restart her search for a kidney donor after a parent who appeared to be a potential match was ruled out because of hypertension.


A popular Cambridge educator with a life-threatening kidney disease needs a new donor, after a parent who appeared to be a potential match has been ruled out because of previously undiscovered hypertension.

Christine Tierney, who runs an after-school program celebrated for its arts and enrichment offerings as well as its free-wheeling sense of fun, was diagnosed eight years ago with polycystic kidney disease. The inherited condition causes ever-larger clusters of cysts to form inside the kidneys, eventually overwhelming them and causing them to fail. [Read more]




Gift of Life

From KHTS, AM 1220, Santa Clarita, CA, Posted by: Kimberly Beers

Santa Clarita Man Getting Second Chance Thanks To Organ Donation

James Zimmerman, a long time Santa Clarita resident with polycystic kidney disease, thought he had run out of options and prepared himself and his family for his life on dialysis until a friend decided to give him a second chance at life by donating one of her kidneys.

Debi Rodriguez and James Zimmerman.


James Zimmerman, a long time Santa Clarita resident with polycystic kidney disease, thought he had run out of options and prepared himself and his family for his life on dialysis until a friend decided to give him a second chance at life by donating one of her kidneys.

Hometownstation.com ran a story about James, his disease, and his immediate need for a kidney in February.

“We were hoping to get a donor to keep him off of dialysis,” said Vicci Zimmerman, James’ wife. “Your chances of not rejecting a kidney, of not having complications, and doing better with immunosuppressant drugs post surgery are greatly improved if you don’t go onto dialysis first.”

Polycystic kidney disease, or PKD, affects about 600,000 people in the United States today. PKD produces large cysts on the kidneys that are filled with toxic fluid and make it impossible for the kidneys to drain and remove the excess. An average adult kidney is about the size of a clenched fist, but Zimmerman’s kidneys are much larger, estimated to weigh about 22 pounds each.

“His kidneys are unable to process out the toxins from his blood,” Vicci Zimmerman said. “His quality of life at this point is not very good.”

James was diagnosed when he was 20 years old, and he lived with the disease for over 30 years, not needing a kidney transplant until now due to his healthy lifestyle.

Gerry Rodriguez, a friend of the Zimmermans for years, was on hometownstation.com searching for information about a tragic incident that happened by his home in Canyon Country and stumbled upon a story about James’ need for a kidney. Gerry showed his wife Debi, who immediately decided to give James one of hers.

When asked if he was surprised about his wife’s instantaneous decision to undergo surgery, Gerry said it’s just who his wife is.

“Knowing her, it didn’t surprise me,” said Gerry.

Gerry and Debi said they’ve raised their children to be selfless, and this was evident when their daughter Olivia was “ready to step in” if Debi wasn’t approved to donate her kidney.

“The way we’ve raised our kids, they’ve always had that spirit of giving,” said Gerry. [Read more]




From Milwaukee Journal-Sentinel, by Jim Stingl

Acquaintance donates kidney, solves family dilemma

Paul Bentley meets with Cassie Peterson-Huber in the Preoperative Clinic at Froedtert Hospital. Bentley found a kidney donor in Peterson-Huber, a family acquaintance.

Paul Bentley meets with Cassie Peterson-Huber in the Preoperative Clinic at Froedtert Hospital. Bentley found a kidney donor in Peterson-Huber, a family acquaintance. Credit: Gary Porter



Cassie Peterson-Huber saw my column in March about Paul Bentley needing a kidney, so she gave him one of hers.

You don't expect to get a kidney from one of the Brewers' racing sausages, but more on that in a minute.

Donor and recipient are both recovering nicely from the transplant last week at Froedtert Hospital.

"From what I heard, I had a really great kidney, and it's working just as it's supposed to in the new body," Cassie said.

"It functioned immediately," Paul said.

The surgery was routine, if we can ever call opening people up and swapping parts routine. But the story of Paul and his family is not.

The Oostburg man's polycystic kidney disease, diagnosed when he was in his 40s, was passed genetically to two of his four sons. The hope is that the sons without the disease each will someday donate a healthy kidney to a brother when he needs one. Meanwhile, Paul's wife, Judy, gave him one of her kidneys nine years ago.

Unfortunately, that kidney failed. Then the sons wondered if one of them would need to donate to their 70-year-old father instead.

Judy led an all-out effort to find a live donor, and that resulted in a dozen people coming forward to be screened, including 29-year-old Cassie.

She is not a stranger to Paul and his family. She competed with the two youngest sons, Nate and Mike, in Shorewood swim club starting in about the seventh grade. In more recent years, they were friends mostly on social media.

"I used to time her at swim meets," Paul said, never dreaming that this child would someday provide him with a lifesaving kidney.

Cassie was troubled when she read of the difficult decision faced by Paul's sons.

"So I thought, you know what, let me at least try. If I'm not a match, then I'm not a match. I knew we both had the same blood type, so I thought there would be a really good chance," she said.

Cassie, who lives in Walker's Point, discussed it with her fiancé, Juan Antonio Rodriguez. They plan to marry on Oct. 14 in Milwaukee. That's right, she jumped into the process of donating a kidney while planning a wedding.

She and Juan met working together at Miller Park on the Brewers crew that creates fan experiences, and they continue in that job. Both have donned the costumes and run their share of sausage races, Cassie usually as the bratwurst in hot pink shoes. [Read more]

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