Wednesday, January 31, 2007

Kidney stones in children on rise

Physicians suspect increased frequency of diagnosed children is due to diets with too much salt and not enough water
By Jonathan Bor
BALTIMORE SUN
Doctors at Johns Hopkins in Baltimore and other medical institutions are beginning to report a curious increase in children with kidney stones -- another possible consequence of America's dependence on processed foods.
Bad eating habits have already fueled twin epidemics of childhood obesity and Type 2 diabetes. Although doctors have blamed fats and sugar for those ills, they say the culprit in this case may be two other features of the fast-food culture: too much salt and not enough water.
Though kidney stones remain uncommon among children, specialists who once treated only a few cases a year are trying to figure out why they are now seeing many times that number.
"Five years ago, we used to see maybe a handful of children a year, maybe five or six," said Dr. Yegappan Lakshmanan, a pediatric urologist at the Johns Hopkins Children's Center. "Now, it's five or six a month. Some are repeat patients, but it's definitely a trend."
Kidney stones, once found almost exclusively in adults, are tiny mineral deposits that can cause excruciating pain when they lodge in the urinary tract.
Three years ago, Hopkins established a pediatric kidney stone clinic after noticing an increase in cases.
More recently, the Vanderbilt Children's Hospital in Nashville opened a stone clinic, and the Harvard-affiliated Children's Hospital in Boston is doing the same.
"We feel like we're seeing the same trend," said Dr. Caleb Nelson, a pediatric urologist at Harvard. "Whereas five or 10 years ago, you could go several months between (cases), now we see a couple a week."
Improved methods of diagnosing stones may be one reason for the sudden uptick of cases. They might account for larger numbers of children with stones too small to cause symptoms, doctors say, but not what many believe is an increase in children who arrive doubled over in pain.
"In children, we think diet has an important part to play -- a lot of fast food with high salt content," said Lakshmanan.
Eating too much salt causes people to excrete excessive amounts of calcium, which can crystallize in the urine in the form of stones.
That problem, Lakshmanan said, is compounded by the tendency of children to skimp on water, which in sufficient quantities can dilute the minerals that cause stones to form.
Quantifying the problem is difficult because childhood kidney stones have never been carefully tracked.
Pediatricians have long known that certain congenital problems -- including blockages in the kidney or bladder -- can cause stones to form. Additionally, babies treated in intensive care units sometimes get medications that can predispose them to stones.
Those factors produced a small caseload, but nothing like the numbers doctors have seen more recently.
Nelson agrees that it's too soon to declare diet the chief culprit, but he believes it's a leading candidate.
At Harvard and Hopkins, dietitians are not only advising families about healthy eating habits, but also compiling dietary histories that could help solve the riddle.
"It does make sense that the fatter the country, the more stones you'll see," Nelson said.
That doesn't necessarily mean that processed foods are causing kidney stones, he said. Children with kidney stones are more likely to be overweight than youngsters in years past -- but youngsters in general are heavier than they used to be.
Nonetheless, there's general agreement that too much salt and too little water can lead to kidney stones.
Similarly, panels convened recently by the American Medical Association and the National Academies of Science raised alarms about the salt content of the American diet.
"Keeping track of things like intake of salt and water is extremely difficult," Nelson said. "You go to grocery stores, and the intake of high-salt processed foods is through the roof."
Doctors recommend no more than one teaspoon of salt -- or 2.4 grams of sodium -- per day, said Dr. Alicia Neu of the Johns Hopkins clinic. But many Americans, and particularly children, consume much more than that from processed foods.
Youngsters should also drink two liters of water a day, doctors say.
Source

Tuesday, January 30, 2007

Incidence Of Benign Lesions For Clinically Localized Renal Masses Smaller Than 7 Cm In Radiological Diameter: Influence Of Sex

UroToday.com- In patients with small renal masses, the likelihood of benign histology in contemporary series has ranged between 15 and 20%. Despite this measurable frequency of benign tumors, percutaneous renal biopsies are not routinely performed since the incidence of a non-diagnostic result has ranged between 15 and 20%.In the December issue of the Journal of Urology, Snyder and colleagues from Memorial Sloan-Kettering Cancer Center report on a retrospective institutional review of 815 consecutive patients with surgically treated masses measuring less than 7 cm. Of these patients treated with partial or radical nephrectomy, 16.4% exhibited benign histology. The most common benign histologies were oncocytoma (10.7%), angiomyolipoma (2%), simple or multilocular cysts (1.2%), metanephric adenoma (1%), and cystic nephroma (0.6%). Ten patients (1.2%) were found to have secondary malignancies with a metastasis to the kidney.As previously reported in the literature, women were twice as likely to exhibit benign histology with a likelihood of 21.4% compared with 13.3% in men (p < 0.002). Interestingly, of all variables evaluated in multivariate analysis including age, presentation, race, and the presence of a cystic component, only gender persisted as an independent predictor of benign histology (hazard ratio = 1.81, 95% CI 1.24 to 2.64, p = 0.002). There was a trend for smaller tumors to be benign (25% of those 1 cm or smaller compared with 10% of those measuring 4-5 cm.), but this was not statistically significant (p = 0.013).These data suggest that women presenting with renal tumors measuring < 7 cm have double the likelihood of benign histology compared with men, irrespective of tumor size. While the diagnostic accuracy of percutaneous biopsies is insufficient to recommend them in all women presenting with small masses, this study certainly underscores the importance of performing a partial nephrectomy in women whenever technically feasible.
Source and references

Horseshoe Kidney

What is a horseshoe kidney?

Horseshoe kidney occurs in about one in 500 children. It occurs during fetal development as the kidneys move into their normal position in the flank area (area around the side, just above the waist). With horseshoe kidney, however, as the kidneys of the fetus rise from the pelvic area, they fuse together at the lower end or base. By fusing, they form a "U" shape, which gives it the name "horseshoe."
One-third of individuals with horseshoe kidney will have at least one other anomaly or complication involving the cardiovascular system, the central nervous system, or the genitourinary system, such as the following:

kidney stones - crystals and proteins that form stones in the kidney that may lead to a urinary tract obstruction.
hydronephrosis - enlargement of the kidneys that usually results from a urinary tract obstruction.
Wilm's tumor - an embryonic tumor of the kidneys that usually occurs during early childhood.
renal cancer, or polycystic kidney disease
hydrocephaly and/or spina bifida
various cardiovascular, gastrointestinal findings (i.e., anorectal malformations, malrotated bowel), or skeletal problems (i.e., cleft lip/palate, clubfoot, polydactyly)
Horseshoe kidney can occur alone or in combination with other disorders. The following are the most common disorders seen with horseshoe kidney:

Turner syndrome - Turner syndrome is a genetic disorder seen in girls that causes them to be shorter than others and to not mature sexually as they grow into adulthood. The severity of these problems varies among affected individuals. Other health problems may also be present involving the heart or renal system (kidneys, etc.). Turner syndrome is caused by the presence of a single X sex chromosome, rather than a pair. Sixty percent of girls with Turner syndrome have horseshoe kidneys.
Trisomy 18 - Trisomy 18 is caused by the presence of three #18 chromosomes, rather than a pair. Trisomy 18 is a serious, usually fatal, chromosome abnormality involving defects in nearly all organ systems, including horseshoe kidney in 20 percent of children affected.
What are the symptoms of horseshoe kidney?

About one-third of children will have no symptoms. The following are the most common symptoms of horseshoe kidney. However, each individual may experience symptoms differently. Symptoms may include:

urinary tract infection - urinary tract infections are usually uncommon in children under five years and unlikely in boys at any age.
kidney stones - if the stones remain in the kidney, your child may have no symptoms. If the stones pass through the urinary tract, the following symptoms may be present:
agonizing flank (around the side, just above the waist) pain
restlessness
sweating
nausea and/or vomiting
blood in urine
urinary frequency
chills
fever
cloudy urine
hydronephrosis - hydronephrosis occurs when there is a urinary tract obstruction and the kidney(s) become enlarged and potentially damaged. Symptoms of hydronephrosis may include the following:
abdominal mass
poor weight gain
decreased urination
urinary tract infection
The symptoms of horseshoe kidney may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is horseshoe kidney diagnosed?

In a child without symptoms, diagnosis or treatment may not be necessary. If your child is having any of the mentioned complications, your child's physician may order one or more of the following diagnostic tests:

renal ultrasound - a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
voiding cystourethrogram (VCUG) - a specific x-ray that examines the urinary tract. A catheter (hollow tube) is placed in the urethra (tube that drains urine from the bladder to the outside of the body) and the bladder is filled with a liquid dye. X-ray images will be taken as the bladder fills and empties. The images will show if there is any reverse flow of urine into the ureters and kidneys.
intravenous pyelogram (IVP) - a diagnostic imaging technique which uses an x-ray to view the structures of the urinary tract. An intravenous contrast of dye is given so that the structures can be seen on film. An IVP also reveals the rate and path of urine flow through the urinary tract.
blood tests (to determine how well the kidneys may be functioning)
urine tests (including a culture)
Treatment for horseshoe kidney:

In a child without symptoms, treatment may not be necessary. If your child has any of the mentioned complications, he/she may require supportive treatment, which means his/her symptoms will be treated, but there is no cure for the condition.
Specific treatment for horseshoe kidney will be determined by your child's physician based on:

your child's age, overall health, and medical history
the extent of the disease
your child's tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
your opinion or preference
Your child may be referred to an urologist (a physician who specializes in disorders and care of the urinary tract and the male genital tract) and/or a nephrologist (a physician who specializes in disorders or diseases of the kidney) for evaluation.
Treatment may include:

antibiotics (to treat an underlying infection)
antibiotics (to treat an underlying infection)
surgical intervention (for symptomatic kidney stones)
If your child has hydronephrosis, multiple treatment options may be discussed including non-surgical or surgical options.

Source
http://www.childrenshospital.org/az/Site1052/mainpageS1052P0.html

Friday, January 26, 2007

Md. medical examiner issues warning on dialysis patient deaths—

BALTIMORE (AP) -
The state medical examiner's office is alerting kidney dialysis patients about two dozen deaths statewide since 2000 that may have been preventable.
The patients bled to death from the site where blood is taken from and returned to their bodies, dying in most cases at home and alone. Doctor David Fowler, the state's chief medical examiner, says most died after the access sites weakened from repeated use.
Nancy Armistead, director of Mid-Atlantic Renal Coalition, says some of the deaths may have been preventable, but noted the number was small compared to the 12-thousand dialysis patients who died between 2000 and 2006 in Maryland.
An advisory sent to dialysis patients reminds them they should have "repeated educational sessions" about the proper care of their access sites and the signs of trouble.
The advisories were sent to dialysis centers that treat up to 20-thousand Marylanders.
Source

American Association Of Kidney Patients Seeks Individuals To Serve On Its Speakers Bureau For 2007 Annual Convention

The American Association of Kidney Patients (AAKP) is seeking individuals to serve on its Speakers Bureau for the 2007 Annual Convention and the Kidney Beginnings: Live educational program. Candidates may include patients, caregivers and healthcare professionals.Patients interested in telling their stories about living with kidney disease (end-stage renal disease and chronic kidney disease) are encouraged to apply. This includes recently diagnosed patients, those on dialysis (hemodialysis and peritoneal dialysis) and transplant recipients. Selected patients will serve on discussion panels to talk about issues and experiences following diagnosis and how they are actively involved in their healthcare.In addition to patients and healthcare professionals, AAKP is also seeking family members and caregivers who are interested in sharing their experiences related to having a loved one with kidney disease. Their unique viewpoints and knowledge serve as an ideal focal point for panel discussions."Serving on AAKP's Speakers Bureau is extremely rewarding. Being able, as a patient or family member, to share your story with others empowers fellow patients," stated Kris Robinson, AAKP Executive Director/CEO.Individuals interested in applying for the AAKP Speakers Bureau should complete the Speakers Bureau Profile Form, available on AAKP's Web site, and return it to AAKP no later than February 26, 2007.The American Association of Kidney Patients (AAKP) is the voluntary, patient organization, which for more than 35 years, has been dedicated to improving the lives of fellow kidney patients and their families by helping them deal with the physical, emotional and social impact of kidney disease. The programs offered by AAKP inform and inspire patients and their families to better understand their condition, adjust more readily to their circumstances, and assume more normal, productive lives in their communities.
American Association of Kidney Patients (AAKP)

Thursday, January 25, 2007

A real life lesson

January 24, 2007
By Patrick Ferrell Staff Writer
NEW LENOX -- Brandon Shafer has grown 2½ inches since May.
For the average 11-year-old, that doesn't seem like too big a deal.
But for Brandon it is important. He checks his vertical progress weekly on his bedroom's closet door.
"He's maybe just as tall as most of the students in his class," his former teacher, Patsy Donahue, said.
Donahue probably notices Brandon's growth these days more than she does with other students.
Last May, Donahue, who's in her mid-20s, and Brandon made national headlines when the Oster-Oakview Elementary School teacher donated one of her kidneys to Brandon.
The boy suffered from polycystic kidney disease, a genetic disorder that causes cysts to grow on the kidneys, thus reducing their function. As a result, Brandon's growth had slowed, and he had to take daily injections of growth hormones.
Following the transplant, doctors warned he may grow seven inches in the first year. While he hasn't reached that, Brandon fits in better with his classmates these days.
It's just one of many signs the fifth-grader's life is returning to normal.
Read More

Do You Know Me?

From a blogger......
Do You Know Me?
Did you know I have a kidney disease? It's called Polycystic Kidney Disease and it is a hereditary disease. My cousin Monica has it. My Aunt had it when she died 13 years ago. My mom has it and is very close to starting dialysis.So, the world would say that my destiny is either dialysis or kidney transplant.I only tell you this for one reason and only one reason. For prayer. Cause I'm going to admit it - I'm praying for healing. Complete. Healing. That might be hard one for some of you to believe for - even the Christians among you. That's okay - I understand. But as for me and my house...we're praying for complete healing. This is not my end. God has a plan for me.Yes, me. At 40 years old. Still waaaay young!A plan for hope and a future.The best is yet to come!!Thanks for praying!!
Read More
http://blog.myspace.com/index.cfm?fuseaction=blog.view&friendID=62924848&blogID=218928991

Antenatal oligohydramnios of renal origin: long-term outcome

Abstract;
Background. Prognosis of fetuses with renal oligohydramnios (ROH) is often still regarded as poor. Neonatal complications and the long-term follow-up of fetuses with ROH in two pediatric centres are described.
Method. 23 fetuses (16 males, 7 females) were included as patients. Primary diseases included congenital anomalies of the kidney and urinary tract (n = 16), autosomal recessive polycystic kidney disease (n = 4) and renal tubular dysgenesis (n = 3). The analysis includes retrospective chart review.
Results. Seven children died (30%), the majority (n = 4, 17%) within the neonatal period due to pulmonary hypoplasia and renal insufficiency. Fourteen patients (61%) required postnatal mechanical ventilation for a median of 4 (range 1–60) days; 11 infants had an associated pneumothorax. All 16 surviving children have chronic kidney disease (CKD) at a current median age of 5.7 years (range 0.5–14.5), managed conservatively in eight patients [median glomerular filtration rate 51 (range 20–78) ml/min/1.73 m2]. Eight patients reached end-stage renal disease at a median age of 0.3 years (range 2 days to 8.3 years), including one patient with pre-emptive kidney transplantation. Five of the patients requiring dialysis underwent successful renal transplantation at a median age of 3.5 years (range 2.5–4). Growth was impaired in seven children requiring growth hormone treatment. Cognitive and motor development was normal in 12 (75%) of the 16 patients and showed a delay in four children, including two with associated syndromal features.
Conclusion. ROH is not always associated with a poor prognosis and long-term outcome in survivors is encouraging. The high incidence of neonatal complications and long-term morbidity due to CKD requires a multidisciplinary management of these children.
Keywords: chronic kidney disease; congenital; dialysis; pulmonary hypoplasia; renal oligohydramnios; renal transplant
Source

Wednesday, January 24, 2007

Oral and salivary changes in patients with end stage renal disease (ESRD): a two year follow-up study

C. P. Bots1, H. S. Brand2, J. H. G. Poorterman3, B. M. van Amerongen4, M. Valentijn-Benz5, E. C. I. Veerman6, P. M. ter Wee7 and A. V. Nieuw Amerongen8
Provides the first longitudinal study on oral health in ESRD patients.
Increases our knowledge concerning the importance of maintained oral health investigations in patients awaiting a renal transplant.
Salivary flow rates are only temporary suppressed and increase after renal transplantation.
After renal transplantation xerostomia and thirst return to normal, suggesting an important contribution to quality of life.
Abstract
Objectives To compare oral health, salivary flow rate, xerostomia and thirst in end stage renal disease (ESRD) patients remaining on dialysis treatment and after renal transplantation.
Design Longitudinal observation.
Setting ESRD patients recruited from dialysis centres in Amsterdam, The Hague and Utrecht, The Netherlands.
Method At baseline and after two years, salivary flow rates, xerostomia and thirst were determined in 43 ESRD patients. The number of decayed missing filled teeth/surfaces (DMFT/DMFS) was recorded, and periodontal status assessed.
Results After renal transplantation (n = 20), the salivary flow rate increased significantly from UWS = 0.30 0.21 ml/min to 0.44 0.29 ml/min (p <0.001) n =" 23)" href="http://www.nature.com/bdj/journal/vaop/ncurrent/full/bdj.2007.47.html#B1" minmax_bound="true">1 Due to improvements in medical care and prolonged life expectancy, patients with renal disorders are increasingly encountered in the dental practice.2
A broad variety of oral manifestations have been reported in ESRD patients including gingivitis, xerostomia, ammonia-like smell, mucosal pallor and lesions, tooth mobility, malocclusion and an increased risk of dental erosion due to frequent regurgitation.3, 4, 5, 6, 7 Systemic and salivary changes due to chronic renal failure, the use of multiple medication, vomiting and reduced oral self care could all potentially affect oral health in these patients.8, 9
Oral and salivary changes in patients with end stage renal disease (ESRD): a two year follow-up study
C. P. Bots1, H. S. Brand2, J. H. G. Poorterman3, B. M. van Amerongen4, M. Valentijn-Benz5, E. C. I. Veerman6, P. M. ter Wee7 and A. V. Nieuw Amerongen8
Provides the first longitudinal study on oral health in ESRD patients.
Increases our knowledge concerning the importance of maintained oral health investigations in patients awaiting a renal transplant.
Salivary flow rates are only temporary suppressed and increase after renal transplantation.
After renal transplantation xerostomia and thirst return to normal, suggesting an important contribution to quality of life.
Abstract
Objectives To compare oral health, salivary flow rate, xerostomia and thirst in end stage renal disease (ESRD) patients remaining on dialysis treatment and after renal transplantation.
Design Longitudinal observation.
Setting ESRD patients recruited from dialysis centres in Amsterdam, The Hague and Utrecht, The Netherlands.
Method At baseline and after two years, salivary flow rates, xerostomia and thirst were determined in 43 ESRD patients. The number of decayed missing filled teeth/surfaces (DMFT/DMFS) was recorded, and periodontal status assessed.
Results After renal transplantation (n = 20), the salivary flow rate increased significantly from UWS = 0.30 0.21 ml/min to 0.44 0.29 ml/min (p <0.001) and the level of xerostomia and thirst decreased. After two years, the percentage of bleeding on probing in dialysis patients (n = 23) decreased from 29.5 25.4% to 10.3 12.3%, (p <0.05). No differences in DMFT and DMFS were observed between dialysis and renal transplant patients. Conclusions DMFT, dental plaque, gingival bleeding and periodontal indices did not change remarkably after two years, comparing dialysis and renal transplant patients. Renal transplantation enhances salivary flow and decreases symptoms of xerostomia and thirst, and hence enhances the potential to improve the quality of life of affected individuals.
Read More

Tuesday, January 23, 2007

Cilium-generated signaling and cilia-related disorders

Junmin Pan1, Qian Wang1 and William J Snell1
1Department of Cell Biology, University of Texas Southwestern Medical School, Dallas, TX, USA
Correspondence: Dr WJ Snell, PhD, Department of Cell Biology, University of Texas Southwestern Medical School, Rm. K2-226, 5323 Harry Hines Blvd., Dallas, TX 75390-9039, USA. E-mail: william.snell@utsouthwestern.edu
Received 16 December 2004; Accepted 16 December 2004; Published online 21 February 2005.
Biologists have long known that humans experience their environment through cilia. Light, odorant, and sound perception depend on these microtubule-filled, complex organelles present on cells in primary sensory tissues. Recently, discoveries on the mechanism of assembly of cilia (flagella) in the lowly, biflagellated, eucaryotic green alga Chlamydomonas have triggered a renaissance of interest in the organelles along with a recognition of their key sensory roles in nonsensory tissues. Chlamydomonas researchers uncovered an entirely new set of cellular machinery essential for transporting the protein components of cilia and flagella in all ciliated/flagellated eukaryotic cells between their site of synthesis in the cell body and their site of assembly at the tip of the flagellum (intraflagellar transport: IFT). Prompted by the surprising observations that disruption of IFT genes in mice led to polycystic kidney disease (PKD) and that PKD proteins are present on the sensory cilia of Caenorhabditis elegans, researchers have made a direct connection between PKD and cilia. At least five (and possibly all) of the seven identified human genes disrupted in PKD and a related disorder nephronophthisis encode proteins expressed in the primary cilia that project into the lumen from the epithelial cells that line renal tubules. Moreover, the renal cilia are flow sensors and at least two of the PKD genes encode ciliary transmembrane proteins essential for mechanosensation. Although their roles have not yet been as clearly identified, cilia also are at the center of a rare human disorder, Bardet–Biedl syndrome (BBS), in which patients exhibit phenotypes of common human diseases, including obesity and increased incidence of hypertension and diabetes. Five of the eight known BBS genes encode basal body or cilia proteins in mice or humans, and homologues of two of the remaining genes are present in basal bodies/cilia of model organisms. Here we briefly describe the biology of cilia and flagella, we outline how studies on model organisms have led to our current understanding of the roles of these organelles and their proteins in health and disease, and we highlight the notion that the primary cilia present on cells throughout the body, even those on brain neurons, may be essential for as yet undiscovered cilium-generated signaling functions.
Keywords:
Chlamydomonas, cilia, flagella, intraflagellar transport
Read More

Monday, January 22, 2007

Mitral Valve Prolapse: What You Should Know


What is the mitral valve? The mitral valve controls the flow of blood between 2 chambers (or "rooms") of your heart called the left atrium and the left ventricle. Normally, blood flows in one direction only, from the atrium to the ventricle. When the heart relaxes in between beats, the 2 flaps of the mitral valve swing open to let blood flow from the atrium to the ventricle. The flaps normally open only one way. (See the pictures below.)
What is mitral valve prolapse?
If you have mitral valve prolapse, the flaps of the valve don't work well. One of the flaps moves back into the atrium when the heart beats. This can let blood flow from the ventricle back into the atrium.
About 1 in 20 Americans has mitral valve prolapse. People are usually born with it. More women have it than men.
Mitral Valve Prolapse: What You Should Know
What is the mitral valve? The mitral valve controls the flow of blood between 2 chambers (or "rooms") of your heart called the left atrium and the left ventricle. Normally, blood flows in one direction only, from the atrium to the ventricle. When the heart relaxes in between beats, the 2 flaps of the mitral valve swing open to let blood flow from the atrium to the ventricle. The flaps normally open only one way. (See the pictures below.)
What is mitral valve prolapse?
If you have mitral valve prolapse, the flaps of the valve don't work well. One of the flaps moves back into the atrium when the heart beats. This can let blood flow from the ventricle back into the atrium.
About 1 in 20 Americans has mitral valve prolapse. People are usually born with it. More women have it than men.

How do I know that I have mitral valve prolapse?
Here are some of the possible symptoms of mitral valve prolapse:
Feeling like your heart is racing or is skipping beats
Chest pain that comes now and then
Shortness of breath
Dizziness
Anxiety or panic
Your doctor can find mitral valve prolapse during a regular exam. When listening to your heart with a stethoscope, your doctor may hear a clicking sound. The flap makes the click. If blood is flowing back into the atrium, your doctor will hear a "whooshing" sound. This sound is called a murmur.
To find out how well your valve is working, your doctor may have you get an echocardiogram. The echocardiogram provides a picture of your heart that shows your valve as the blood flows through it. This can help your doctor decide if you need treatment.
How is mitral valve prolapse treated?
Most people with mitral valve prolapse don't need any treatment. In fact, this condition usually has no effect on your health. About 2 of every 100 people with this condition have complications. If your valve is too leaky or if you are having lots of symptoms you may need surgery to fix the valve. You may also have to limit your participation in competitive sports. Ask your doctor if you are in this group.
If your mitral valve prolapse causes chest pain or other symptoms, your doctor might prescribe medicines such as beta blockers to make your symptoms better.
Some people get an infection in the leaky valve when they have surgery or dental work. You might need to take antibiotics before surgery or dental work to prevent this infection. Remind your dentists and doctors that you have mitral valve prolapse so that you can get a prescription for antibiotics before you have surgery or dental work.

Other Organizations
American Heart Associationhttp://www.americanheart.org

Source for diagrams

PKD Blog Kidney Bylines

American Association Of Kidney Patients Announces 2007 Kidney Beginnings: Live Program

Polyomavirus (BK) In Pediatric Renal Transplants: Evaluation Of Viremic Patients With And Without BK Associated Nephritis

No Evidence Of Disease After Radiofrequency Ablation In Delayed Nephrectomy Specimens

Pfizer's Sutent(reg) Is Granted Full Marketing Authorization For First-Line Use In Advanced Kidney Cancer In The EU

Friday, January 19, 2007

Soni running kidney scam since 2002, say police

Express News Service
Ahmedabad, January 18: Girish Soni, main accused in kidney transplantation racket busted by city Detection of Crime Branch (DCB), had been running the scam since 2002, police said. Soni had been arrested for a similar offence by Nadiad police in 2002 when he had ‘arranged’ for kidneys for two patients. For this, he had spent eight months in jail before getting bail. The case is pending in court.
Joint Commissioner of Police (DCB) Ashish Bhatia said that initially, Soni operated from outside the Muljibhai Patel Urological Hospital in Nadiad. “He used to sit at a tea-stall outside the hospital and act as an agent between recipients and donors. After some illegal transplantations, Soni came to be known among those who needed kidneys and were willing to pay a price for them,” said Bhatia.
Read More

Thursday, January 18, 2007

Transplant joy of kidney patient

By Reader's letter
It was good to read that kidney transplant patient Jackie Evans has got her life back (Kidney transplant patient looking forward to the new year, The Press, January 3). I offer her my sincere congratulations.
I know how she must be feeling as I too have been on dialysis for five years due to the condition, glomerularnephritis. I met Jackie twice while having holiday dialysis in my home city of York.
On January 1, I received a 5am call from the Klinikum Aachen, here in Germany, with the offer of a kidney transplant, which I undertook with success. What a wonderful start to the new year!
I too had an offer from a live donor, my daughter. God in his wisdom changed the plan, with a kidney from an unknown donor, from a most thoughtful family following bereavement. I will never know who, but my gratitude will be eternal.
I am a York man who has worked in my adopted country, Germany, for the past 26 years as a specialist trained nurse, having trained in York at the old County and City hospitals and also at St James's Hospital, Leeds, as a nurse tutor.
Read More

Tuesday, January 16, 2007

THE silent killer that goes undetected...

BY A STAFF REPORTER
Monday, January 15, 2007 12:0:36 IST

Renal failure is unnoticed until most of the damage is done
Over 1.5 million individuals around the world receive dialysis or have had a kidney transplant out of a total of 500 million sufferers Chronic non-communicable diseases - particularly cardiovascular disease, hypertension, diabetes mellitus and chronic kidney disease - have now replaced the communicable diseases as the leading threat to public health and health budgets worldwide. Deaths claimed by infectious diseases will decline by 3 per cent over the next decade. In marked contrast, chronic diseases that already account for 72 per cent of the total global burden of disease in people over 30 will increase by 17 per cent, much of this in developing countries The only feasible global response to this pending health and socioeconomic crisis is chronic disease prevention. The kidney, too often overlooked as part of global public health efforts, has now emerged as critical to such prevention efforts. Even more importantly, kidney disease is a “disease multiplier”. It causes death in many with diabetes and hypertension and predicts the development of a cardiovascular event. Abnormalities in kidney function often represent an early window into the state of the general vascular system. This window facilitates early disease detection before patients develop the more devastating problems such as stroke, peripheral vascular disease, coronary heart disease and kidney failure.
Read More

PKD Blog Kidney Headlines

Bayer And Onyx Announce Pivotal Nexavar Kidney Cancer Study Published In NEJM

JDRF Applauds Important Passage Of The Stem Cell Research Enhancement Act Of 2007

Kidney Disease Linked To Increased Risk Of Peripheral Artery Disease

Advanced Kidney Cancer - Median Progression-free Survival Doubled For Patients Treated With Nexavar (sorafenib), Phase III Data

American Association Of Kidney Patients Announces 2007 Medal Of Excellence Award Winner

Thursday, January 11, 2007

AVI BioPharma and Eleos Announce Cross-License Agreement for p53 Therapeutics

Portland -
PORTLAND -- AVI BioPharma, Inc. (Nasdaq:AVII), and Eleos Inc. announced today a cross-license agreement for the development of antisense drugs targeting p53, a well-studied human protein that controls cellular response to genetic damage.
Under the terms of the agreement, AVI is granting Eleos an exclusive license to AVI’s NEUGENE® third-generation antisense chemistry to treat cancer with p53-related drugs. In return, Eleos is granting AVI an exclusive license to its patents for treatment of most viral diseases with drugs that target p53. The companies are sharing rights in other medical fields where targeting p53 may be therapeutically useful.
Each company will make milestone payments and royalty payments to the other on development and sales of products that utilize technology licensed under the agreement. In addition, Eleos is making an upfront payment of $500,000 to AVI.
“This agreement gives AVI access to an important human target that may help us develop even better antiviral therapies,” said Denis R. Burger, Ph.D., chief executive officer of AVI. “Targeting both the virus itself and host cell factors that contribute to pathogenesis may be the ultimate strategy for treating certain viral diseases.”
“AVI’s proprietary antisense chemistry has pharmaceutically important properties that are complementary to our existing technology,” said Larry J. Smith, Ph.D., chief executive officer of Eleos. “Having access to NEUGENE chemistry expands Eleos’ ability to develop commercial p53 cancer products with extended capabilities.”
p53 is a high-profile molecule both in experimental medicine literature and in the popular press. The normal function of p53 is to rid the body of cells that have sustained certain pathological changes in their DNA by causing such cells to either repair the damage or to commit suicide (undergo apoptosis). However, in many medical situations, such as cancer and some viral diseases, p53’s normal function has been subverted in a way that actually produces the disease or many of its symptoms. For example, once cancer has developed, blocking p53 increases the cancer’s vulnerability to the killing effects of radiation and chemotherapy while making normal cells more resistant to those treatments.
About AVI BioPharma AVI BioPharma develops therapeutic products for the treatment of life-threatening diseases using third-generation NEUGENE antisense drugs. AVI’s lead NEUGENE antisense compound is designed to target cell proliferation disorders, including cardiovascular restenosis, cancer and polycystic kidney disease. In addition to targeting specific genes in the body, AVI’s antiviral program uses NEUGENE antisense compounds to combat disease by targeting single-stranded RNA viruses, including West Nile virus, hepatitis C virus, dengue virus, Ebola virus and influenza A virus. AVI has introduced a NEUGENE-based exon-skipping technology called ESPRIT therapy. More information about AVI is available on the company’s Web site at www.avibio.com.
Source

Kidney transplantation is associated with a significant increase in the risk of cancer

SYDNEY, Australia, Dec. 19 -- Kidney transplantation is associated with a significant increase in the risk of cancer, including a tripling of the risk for cancer at 18 sites, researchers here reported.
Action Points
Explain to interested patients that the results reported here are based on an observational study.
Explain to interested patients that these results suggest an increased risk for cancers associated with viral infections, such as the human papilloma virus or hepatitis B or C.
A study of 28,855 end-stage renal disease patients who received kidney transplants found a slight but significant (P=0.002), increase in risk of cancer during dialysis (standardized incidence ratio 1.35; 95% CI, 1.27-1.45), but after transplant the SIR increased to 3.27 (95% CI, 3.09-3.46, P for trend <0.001).
Moreover, most of these cancers are those with known or suspected viral causes, reported epidemiologist Claire M.Vajdec, Ph.D., of the University of New South Wales, in the Dec. 20 issue of the Journal of the American Medical Association.
Following transplantation, there was a significant increase in risk of cancer at 25 sites, she said.

Continue Here

FDA Issues Alert on Gadolinium-Based Contrast Agent for Kidney Patients

ROCKVILLE, Md., Dec. 22 -- The FDA updated a public health advisory today about a serious new kidney disease that is apparently associated with a gadolinium-based contrast agent used with MRI or MR angiography.
The agency said that as of Dec. 21 it had received reports of 90 patients with moderate to end-stage kidney disease who developed the new disease, called nephrogenic systemic fibrosis or nephrogenic fibrosing dermopathy (NSF/NFD), after they had an MRI or MRA with a gadolinium-based contrast agent.
The FDA recommended that whenever possible patients with moderate to end-stage kidney disease who need an imaging study, be given imaging methods other than MRI or MRA with a gadolinium-based contrast agent.
NSF/NFD began from two days to 18 months after exposure to the contrast agent, said the FDA. Many, but not all of these patients, received a high dose of the contrast agent, yet some received only one dose.
The signs of NSF/NFD also include: burning, itching, swelling, hardening and tightening of the skin; red or dark patches on the skin; yellow spots on the whites of the eyes; stiffness in joints with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness.
The FDA notified physicians and patients that:
Patients with moderate to end-stage kidney disease who receive an MRI or MRA with a gadolinium-based contrast agent may get NSF/NFD which is debilitating and may cause death.
Patients who believe they may have NSF/NFD should get in touch with their doctor. Patients who develop NSF/NFD have areas of tight, rigid skin and may have scarring of their body organs.
If these patients must receive a gadolinium-based contrast agent, prompt dialysis following the MRI or MRA should be considered.
It is not clear why NSF/NFD occurs in patients with moderate to end-stage kidney disease who receive a gadolinium-based contrast agent.
There are five FDA-approved gadolinium-based contrast agents, Magnevist, MultiHance, Omniscan, OptiMARK, and ProHance. These contrast agents are FDA approved for use during an MRI scan, but not for use during an MRA scan. Although NSF/NFD has been reported for only three of the five gadolinium-based contrast agents, the FDA said it believes that there is a potential for NSF/NFD to occur with the use of any of the approved gadolinium-based contrast agents.
The advisory was an update to an alert last June when the FDA said it had learned of 25 cases of NSF/NFD in patients with kidney failure who received Omniscan, and took the MRA test. "These reports provide more evidence for a causal relationship between gadolinium-based contrast agents and the development of NSF/NFD," the FDA said.
Worldwide, the re have been about 215 patients with NSF/NFD reported, said the FDA. The medical histories of 75 of these patients were reviewed in detail, and all of the patients had received a gadolinium-based contrast agent for an MRI or MRA. Researchers, the agency said, have identified gadolinium in skin biopsies of patients with NSF/NFD.
Additional General Nephrology Coverage

Wednesday, January 10, 2007

Long-Term Outcome of Pediatric Renal Transplantation:

Follow-Up of 300 Children from 1973 to 2000
Written by Pasquale Casale, MD
Tuesday, 09 January 2007
BERKELEY, CA (UroToday.com) - Rees, et al from the Great Ormond Street Hospital for Children and the Royal Free Hospital in London report their experience of pediatric renal transplantation from the beginning of their program.
They retrospectively reviewed patient and transplant survival and influencing factors in the 300 children transplanted between 1973-2000.
The group found that 300 children have received a total of 354 transplantations. Fifty-six were living related donations. The median age at transplantation was 10.3 years. Forty-four percent had congenital structural abnormalities of the urinary tract. Forty-six children required a second and 8 required a third transplantation. The overall patients survival at 5, 10 and 20 years was 97, 94 and 72% respectively. In the overall cohort, the donor type did not affect mortality, nor did age at transplantation. However, those transplanted before 5 years of age had a significantly shorter post-transplant survival time.
Transplant survival after the first transplant for deceased and living-related donors was 66 and 87% at 5 years, 51 and 54% at 10 years, and 36% at 20 years (deceased-donor transplants only.) Although the overall transplant survival was inferior in children transplanted before 2 years of age, in the most recent cohort between 1990-2000, age did not affect the outcome. They performed a multiple regression analysis and found that the only predictor of transplant survival was the era of transplantation. The median final height was within the normal range for males and females. Seven patients required growth hormone after transplantation.
The group concluded that the outlook for successful transplantation is improving, especially in the last decade where age did not affect the success of transplantation. The group stated that survival of living related donor transplants is superior to cadaveric transplants for the first 5 years. They feel that they can predict that a 10 year old child receiving a transplant in 2000 also on cyclosporin-based immunosuppression can expect a transplant half-life of approximately 13.1 years from a living-related donor and one of 10.8 tears from cadaveric transplantation. These are very promising results which will require further investigation and warrant future updating.
Rees L, Shroff R, Hutchinson C, Fernando ON, Trompeter RS.
Nephron Clinical Practice 105(2): 68-76, 2007

Yup, still growing

I had an ultrasound today to check the growth of my kidney cysts. Yes, they are growing, as are my kidneys. My right kidney is bumping up against my uterus, whereas my left kidney is displacing all my intestines as it extends medially into my abdomen. I don't know why I feel discouraged by this news, or even all that surprised. PKD is a progressive disease. I'm progressing. I guess I just thought that it would stablilize, or something. I don't know why I thought that--wishful thinking? Denial?I had a 5cm cyst on my right kidney which is now a 7.5 cm cyst. My kidneys themselves are too big to measure completely accurately with ultrasound, as the transducer is not big enough to project the kidneys on the screen all at once. Great.A 24 hour urine test next week will tell me how things are going with my kidney functions. Hopefully that will bring good news.Hey, at least my BP is low. That's good.We just take it one day at a time, I guess. One day at a time.
http://livingwithpkd.blogspot.com/2007/01/yup-still-growing.html

Tuesday, January 09, 2007

Your kidneys,your health

Ohnn Nahm, M.D.
Healthy kidneys function to remove extra water and wastes, help control blood pressure, keep body electrolytes in balance, keep bones strong and tell your body to make red blood cells. Chronic kidney disease occurs when kidneys are no longer able to clean toxins and waste products from the blood and perform their functions to full capacity. This can happen suddenly or over time.Diabetes is the number one cause of kidney disease, responsible for about 30 percent to 40 percent of all kidney failure in the United States and in Europe. High blood pressure is the second cause, responsible for about 25 percent. Another form of kidney disease is glomerulonephritis, a general term for different types of kidney inflammation. Genetic diseases such as polycystic kidney disease, autoimmune diseases, birth defects and other problems can also cause kidney disease.
Most people do not have any severe symptoms until their kidney disease gets worse. However, kidney disease symptoms include:
• Less energy• Poor appetite• Trouble sleeping• Dry, itchy skin• Muscle cramping at night• Swollen feet and ankles• Puffiness around your eyes, especially in the morning• Need to urinate more often, especially at nightKidney disease can be diagnosed through lab tests or by symptoms. High blood levels of creatinine or high levels of protein in your urine suggest a presence of kidney disease. Diabetic patients should have a yearly urine test for microalbumin, small amounts of protein that don’t show up on the standard urine protein test.An early referral to a kidney specialist, called a nephrologist, may improve survival of patients with chronic kidney disease. The role of the nephrologist includes:• Slowing or preventing progression of kidney disease.• Identifying and correcting complications associated with chronic kidney disease such as high blood pressure, anemia, bone disease and other electrolyte problems.• Preparing for kidney replacement therapy such as dialysis or kidney transplantation if necessary.
http://www.dhonline.com/articles/2007/01/06/lifestyles/healthy_living/fit01.txt

Monday, January 08, 2007

PKD Blog News

American Association Of Kidney Patients Announces 2007 Kidney Beginnings: Live Program

Sensitization Of Pelvic Nerve Afferents And Mast Cell Infiltration In The Urinary Bladder Following Chronic Colonic Irritation Is Mediated By Neuropep

Ways To Make Kidney Transplant Allocation More Efficient And Equitable

Renal Cell Carcinoma In Children: Experience Of A Single Center

ELAD(R) Bioartificial Liver Trial In China Achieves Significance At Halfway Point

Endoscopic Management of Seminal-Vesical Cyst with Right Renal Agenesis Causing Acute Urinary Retention: Case Report

A.S. Gözen, M.D., et al. - Seminal-vesicle cysts associated with renal agenesis are rare congenital or acquired lesions. We report a case of seminal-vesicle cyst causing urinary retention in a young patient with right renal agenesis who was treated successfully with transurethral unroofing. Magnetic resonance imaging aided us in making the conclusive diagnosis without the need for any invasive investigations...
Journal of Endourology, 01/08/07

End-Stage Renal Disease Use in Hurricane-Prone Areas: Should Nephrologists Increase the Utilization of Peritoneal Dialysis?

Myra A. Kleinpeter - Hurricane Katrina resulted in massive destruction of the gulf coast of the United States in 2005. In the immediate aftermath, displaced dialysis patients required urgent hemodialysis or additional peritoneal dialysis (PD) supplies. Massive damage to the health care infrastructure in these communities disrupted dialysis services for several months...
Advances In Chronic Kidney Disease , 01/02/07

A transplant surgeon has warned against selling body parts, after a report suggested organs are for sale online.

The Sun newspaper claims people are selling organs such as kidneys, parts of their liver, and the corneas from their eyes, online to raise money.
Although there is a shortage of organs available for transplantation, the sale of organs in the UK is illegal.
Mr Keith Rigg condemned the practice and warned of a definite risk of death for transplant donors.
Read More:
http://news.bbc.co.uk/1/hi/health/6240307.stm

Friday, January 05, 2007

Stepwise Shock Wave Lithotripsy: Results Of Initial Study For The Treatment Of Urinary Stones In Childhood

UroToday.com- A study by Demirci, et al assessed the effectiveness of Stepwise extracorporeal shockwave lithotripsy in the treatment of upper urinary stones in the pediatric population. Between August 1998 and August 2003, a total of 31 patients were treated for renal or ureteral stones. All treatments were performed with a Dornier Compact Delta lithotripter. The number of shock waves was limited to a maximum of 3000 shock waves per session. The voltage was increased in a stepwise fashion beginning at 10 kV and increased to 12.75 kV. Stone clearance rates were assessed at 3 months. The stone free status was defined as the absence of stone fragments.The group found that a total of 31 stones (24 renal and 7 ureteral stones) were treated. The median age of the patients was 8 years with a range of 9 months to 12 years. The average stone burden was 1 cm for renal stones and 5 mm for ureteral stones. One patient required an open pyelolithotomy for residual stone burden. The overall stone free rates for renal and ureteral stones were 79% and 100%, respectively. They noted that gross hematuria was observed in all cases but was not clinically significant.
http://www.medicalnewstoday.com/medicalnews.php?newsid=60089

Read new labels on OTC painkillers

By Richard Harkness
McClatchy Newspapers
(MCT)
Q: Last week you wrote about FDA's new product labeling that warns about liver toxicity with excessive doses of acetaminophen (e.g., Tylenol). What about the new labeling for OTC NSAID products?
A: The proposed new NSAID labeling calls attention to the risk of NSAID-related stomach bleeding.
As with acetaminophen, the FDA wants product labels to use bold or fluorescent type for further emphasis.
OTC NSAIDs help relieve pain, inflammation, and fever. Products include aspirin, ibuprofen (e.g., Advil, Motrin IB, Midol IB, Nuprin), naproxen (Aleve), and ketoprofen (Actron, Orudis KT).
The upcoming label will highlight the potential for NSAID-related stomach bleeding in those who:
_are over age 60
_have had prior stomach or duodenal ulcers or bleeding
_take a blood thinner such as warfarin (Coumadin)
_take more than one product containing an NSAID
_drink moderate amounts of alcohol
_take the product for a longer time than directed
Between 1998 and 2001, the FDA's Adverse Event Reporting System turned up a total of 279 cases of stomach bleeding associated with OTC NSAID products: 197 cases for ibuprofen, naproxen, and ketoprofen, and 82 cases for aspirin.
That's a tiny number considering the widespread use of these products, and it's likely that many more cases went unreported.
OTC NSAIDs, as well as most prescription NSAIDs, are non-selective. That means they block both COX-1 and COX-2 enzymes.
Blocking COX-2 helps relieve pain, inflammation, and fever. This is the therapeutic effect produced by NSAIDs and the reason they are taken.
However, COX-1 and COX-2 lead to a number of benefits, and blocking them may cause some detrimental effects.
For one thing, COX-1 is involved in protecting the stomach lining against damage from the strong acid it secretes. Blocking it paves the way for stomach injury and bleeding, and in some cases, ulcers. (Blocking COX-1 thins the blood, which increases this bleeding risk.)
That's what prompted FDA's new NSAID label proposal.
Both COX-1 and COX-2 are involved in kidney-protective effects. Blocking either one may compromise kidney function, promote fluid retention (edema), raise blood pressure, and boost cardiovascular risk.
That's the reason for the long-standing recommendation to use NSAIDs cautiously in those with high blood pressure or congestive heart failure.
According to the FDA, NSAID-related acute kidney failure is rare. The risk is higher in those with chronic kidney disease, congestive heart failure, liver cirrhosis with ascites (fluid in the abdomen), or low blood volume such as that associated with dehydration.
Consumer groups are urging FDA to include label statements about these risks in addition to that for stomach bleeding, but some OTC manufacturers are resistant to further label warnings.
My view: These other potential adverse effects have been known for decades, and the new labeling should also address them.
That said, OTC NSAIDs appear to be safe for most people when taken as directed and with proper cautions in mind.
http://www.belleville.com/mld/belleville/living/16390557.htm

Thursday, January 04, 2007

Bayer Yakuhin Ltd. Has Filed Fosrenol(R) For The Treatment Of Hyperphosphatemia In Japan

Today, January 04, 2007, 4 hours ago
Bayer Yakuhin Ltd. has submitted a marketing application for Fosrenol(R) (lanthanum carbonate) for the treatment of hyperphosphatemia in end stage renal disease (ESRD) patients in Japan. Bayer Yakuhin received an exclusive license from Shire International Licensing BV in December, 2003 to develop and sell Fosrenol(R) in Japan and completed Phase II and Phase III clinical trials in Japan. [click link for full article]

National Kidney Foundation Calls Chronic Kidney Disease Growing Public Health Problem

Warns that Doctors Often Miss “Silent Killer,” Endorses Tests for Early Diagnosis Offers Six-Step Health Primer, Announces Free Screenings on March 8
New York, NY November 14, 2006
Most Americans know that heart disease and cancer can be silent killers and understand that monitoring blood pressure and cholesterol and having regular mammograms are critical to protecting their health. Too few adults—and not enough doctors—realize, however, that Chronic Kidney Disease (CKD) is another common, life-threatening illness that often goes undetected until very advanced when it could be diagnosed early through simple tests.
Twenty million Americans (1 in 9 adults) suffer from CKD—an increase of more than five-fold since 1980—and another 20 million are at risk. Worse, today’s epidemics of diabetes and obesity could contribute to even higher rates of CKD in the future. Undiagnosed and untreated, CKD can lead to serious health problems including kidney failure (end-stage renal disease). Caught early, it can often be managed, and kidney damage can be slowed or stopped. That’s why early testing for people at risk is so important.
In preparation for National Kidney Month (March 2007) and World Kidney Day (March 8), the National Kidney Foundation (NKF) offers this 6-step primer for protecting health.
Step 1: Know These Facts
6 Things Healthy Kidneys Do:
Regulate the body’s water levels
Activate Vitamin D to maintain healthy bones
Filter wastes and toxins from the blood
Release the hormone that directs production of red blood cells
Release the hormone that regulates blood pressure
Keep blood minerals in balance (sodium, phosphorus, potassium)
8 Problems CKD Can Cause:
Cardiovascular disease
weak bones
Heart attack and stroke
nerve damage (neuropathy)
high blood pressure
Kidney failure (end-stage renal disease, or ESRD)
death
anemia
Step 2: Assess Your Risk
4 Main Risk Factors:
Diabetes (self or family)
High blood pressure (self or family)
Cardiovascular disease (self or family)
Family history of kidney disease
10 Additional Risk Factors:
African-American heritage
Prolonged use of NSAIDs, a type of painkillers
Native American heritage
Obesity
Hispanic, Asian, Pacific Islander heritage
Chronic urinary tract infections
Age 60 or older
Kidney stones
Lupus, other autoimmune disorders
Low birth weight
Step 3: Recognize Symptoms
Most people with early CKD have no symptoms, which is why testing is critical. By the time symptoms appear, CKD may be advanced, and symptoms can be misleading. Pay attention to these:
8 Possible Trouble Signs:
Fatigue, weakness
Puffy eyes
Difficult, painful urination
Swollen face, hands, abdomen, ankles, feet
Foamy urine
Increased thirst
Pink, dark urine (blood in urine)
Increased need to urinate (especially at night)
Step 4: Get Tested
If you or a loved one belong to a high-risk group, ask your primary-care physician about these tests—and be especially insistent about the last one. Your doctor may want to perform other tests as well.
4 Simple, Life-Saving Tests :
What:
Blood Pressure
Why:
High blood pressure can damage small blood vessels (glomeruli) in the kidneys. It is the second-leading cause of kidney failure after diabetes.
Good Score:
Below 140/90 is good for most people. Below 130/80 is better if you have chronic kidney disease. Below 120/80 is best.
What:
Protein in Urine
Why:
Traces of albumin in urine (microalbuminuria) is an early sign of CKD. Persistent amounts of albumin and other proteins in the urine (proteinuria) indicate kidney damage.
Good Score:
Less than 30 mg of albumin per gram of urinary creatinine (a normal waste product)
What:
Creatinine in Blood (Serum Creatinine)
Why:
Healthy kidneys filter creatinine (a waste product from muscle activity) out of the blood. When kidney function is reduced, creatinine levels rise.
Good Score:
0.6 to 1.2 mg per deciliter of blood, depending on other variables
What:
Glomular Filtration Rate (GFR)
Why:
This is the most sensitive and accurate gauge of kidney function. Doctors measure blood creatinine levels and perform a calculation based on age, race, gender and body size.
Good Score:
Over 90 is good. 60-89 should be monitored. Less than 60 for 3 months indicates CKD.
Step 5: Stay Healthy
7 Things People with CKD Should Do:
Lower blood pressure
Reduce salt intake
Reduce potassium
Keep blood-sugar levels under
Avoid NSAIDs , a type of
Moderate protein consumption control if diabetic painkillers
Get an annual flu shot
9 Things Everyone Should Do:
Exercise regularly • Quit smoking • Monitor cholesterol levels
• Control weight • Drink only in moderation • Get an annual physical
• Follow a balanced diet • Stay hydrated • Know the family medical history
Step 6: Learn More
The National Kidney Foundation will offer free kidney screenings through its Kidney Early Evaluation Program (KEEP) for people at risk for CKD in at least 20 cities across the country on World Kidney Day, March 8. For locations and schedules, visit www.keeponline.org.
To learn more about CKD risk factors, prevention and treatment, visit www.kidney.org.
The National Kidney Foundation works to prevent kidney and urinary tract diseases, improve the health and well being of people and families affected, influence public policy to support the kidney community and promote organ transplantation.
http://www.kidney.org/news/newsroom/newsitem.cfm?id=358

Learn More About PKD Clinical Trials

(Updated October 11, 2006)Tolvaptan and HALT PKD clinical trials are enrolling patients, while promising new research on drugs such as rapamycin are already leading to additional studies. Read below for more information or, to visit the Current Research section of the website, click here.
* NOTE: Each trial is different and demands on patients vary widely. Before signing up for any clinical trials, talk to your doctor or health care team. To learn more about the clinical trial process and how drug therapies are approved through the U.S. Food and Drug Administration, please click here.
Current Clinical Trials
Rapamune (Sirolimus/Rapamycin) This is a 24-month prospective, controlled open label study at the University of Zurich in Switzerland, investigating whether Rapamycin retards cyst growth and slows renal functional deterioration in patients with PKD. Renal volume will be measured by magnetic resonance imaging. More information about the study and eligibility criteria can be obtained by clicking here, or you may directly email Dr. Andreas Serra, Principal Investigator, at andreas.serra@usz.ch
CRISP IIThe CRISP study has been selected for extended funding which should begin in the next three months. This continuation of the original CRISP study, now called CRISP II, will follow the original patient cohort for an additional four years to study the relationship between kidney size and disease progression. Some patients may be in other studies as well but will continue to be monitored here. To read about the original CRISP study, click here.Tolvaptan (Otsuka Maryland Research Institute): This study, which recently received “fast track” designation by the Food and Drug Administration because of its promise as a treatment for PKD, is looking at the effectiveness of a vasopressin antagonist drug to slow or stop cyst growth in PKD patients. Phase II clinical trials are finishing up. Enrollment has not yet begun for Phase III clinical trials. Phase III trials are larger studies looking at drug effectiveness in PKD patients living in the Americas, Asia, and Europe. Patients and physicians interested in participating in these trials should contact Otsuka at 866-712-5837. Information on Otsuka can be obtained from their public website at http://www.otsuka.com/ .
HALT PKD Treatment Network (NIDDK): The HALT study is a nationwide research initiative to investigate the effectiveness of anti-hypertensive drugs on PKD progression and its cardiovascular complications. ACE (angiotensin converting enzyme) inhibitors in combination with or without ARB (angiotensin receptor binding) inhibitors will be evaluated. The study will involve travel to selected sites for periodic examinations, home blood pressure monitoring and laboratory testing. Costs for medications, home blood pressure equipment and patient evaluations will be fully covered. Travel costs will be totally or partially covered, depending on how far the participant lives from the study site. Enrolment begins Spring 2006. For more information about the study, go to www.clinicaltrials.gov/ct/ and type in “polycystic kidney disease” and select “HALT Progression of PKD” or call the study sites listed below:
- Tufts-New England Medical Center in Boston, MA: 1-866-846-2735 or 1-617-636-8783- Beth Israel Deaconess Medical Center in Boston, MA: 1-617-636-8783 or 1-866-650-1815 - Emory University in Atlanta, GA: 1-404-686-8280.- Mayo Clinic in Rochester, MN: 1-507-284-0944 or 1-888-630-2616- University of Colorado in Denver, CO: 1-877-765-9297- Kansas University Medical Center in Kansas City, KS: 1-913-588-7609- Cleveland Clinic Foundation in Cleveland, OH: 1-800-223-2273, ext 44680Heart Disease and PKD: The aim of this non-invasive study is to examine possible mechanisms leading to heart disease in PKD. The study is located at the Tufts-New England Medical Center and is directed to PKD patients in the Boston area. Those interested in participating, call Vandana Menon, M.D., Ph.D. at (617) 636-8791 or (617) 636-5895.ARPKD and Congenital Hepatic Fibrosis: The National Human Genome Research Institute at the National Institutes of Health wants to collect comprehensive date on kidney and liver disease in ARPKD/CHF and follow patients over time to provide the groundwork for more focused studies and novel therapeutic interventions. The protocol includes children and adults with a clinical diagnosis of ARPKD/CHF. Patients who have received a kidney or liver transplant and have stable graft function without severe complications are eligible. The study requires admission to the NIH Clinical Center for 4-5 days, with follow-up visits every 1-2 years. There are no medical expenses for the patients, and travel can be provided. For more information, contact Dr. Meral Gunay at 1-301-594-4181 or by e-mail at mgaygun@nih.gov or go to www.clinicaltrials.gov/ct/ and type in “polycystic kidney disease” and select “Evaluation of ARPKD and Congenital Hepatic Fibrosis.”

Future Clinical Trials
Rapamune (Sirolimus/Rapamycin) This study is a prospective, randomized, placebo-controlled clinical trial sponsored by the Cleveland Clinic and designed to evaluate the effects of an agent (rapamycin) that has antiproliferative, antiangiogenesis and tumor-progression blocking capabilities. This study is not yet open for patient recruitment. For more information, go to www.clinicaltrials.gov/ct/ and type in “polycystic kidney disease” and select “Pilot Study of Rapamycin as Treatment for Autosomal Dominant PKD.” The person to contact for more information is Susan Wirth, BSN, at wirths@ccf.org.
Statin Therapy and PKD Disease Progression in Children and Young AdultsThe purpose of this clinical trial is to determine the effect of blood pressure control with angiotensin converting enzyme (ACE) inhibition on renal cyst growth over a five-year study period in children and young adults aged 4-21 years with ADPKD. Results of this study could significantly impact the standard of care for management of ADPKD in this population. The study will take place at The Children’s Hospital in Denver, CO. All expenses including study-related medications, a home blood pressure machine, travel costs to and from Denver (including one parent to accompany minor children) and lab and radiology testing will be provided. For more information, call Dr. Melissa Cadnapaphornchai toll-free at 877.765.9297 or send an e-mail to pkd.nurse@uchsc.edu .Observational StudyChronic Kidney Disease in Children Prospective Cohort Study (CKiD)This is a prospective epidemiological study of children with chronic kidney disease and includes children with ARPKD and ADPKD. The primary goals of the study are to determine the risk factors for decline in kidney function and define how a progressive decline in kidney function impacts neurocognitive function and behavior; the risk factors for cardiovascular disease; and growth failure and its associated morbidity. Enrollment has begun. For more information go to http://www.clinicaltrials.gov/ct/show/NCT00327860.
Study sites and contact information are as follows: 1. Johns Hopkins University, Baltimore, MD: Susan Furth, M.D., Ph.D., sfurth@jhmi.edu or 410-502-7964 2. Children’s Mercy Hospital, Kansas City, MO: Brad Warady, M.D., bwarady@cmh.edu or 816-234-3812Somatostatin This general aim of this study is to compare the effects on disease progression of three year treatment regimen using long-acting somatostatin or placebo in patients with ADPKD and normal renal function or mild to moderate renal insufficiency. This study is sponsored by the Mario Negri Institute for Pharmacological Research in Bergamo, Italy. This study is not yet open for patient recruitment. For more information, go to www.clinicaltrials.gov/ct/ and type in “polycystic kidney disease”. Then select “Somatostatin in Polycystic Kidney: A Long-Term Three Year Follow-up Study” or contact Norberto Perico, M.D., at perico@marionegri.it . PKD Gene Modifier Mapping Study (Canadian Institutes of Health Research): This is an international observational study designed to map modifier genes for renal disease progression in type I ADPKD. There are 10 different ADPKD research centers in North America and Europe.
http://www.pkdcure.org/site/PageServer?pagename=mn_Aprilclinicaltrials

Wednesday, January 03, 2007

Gadolinium-containing Contrast Agents

Gadolinium-containing Contrast Agents - Omniscan, OptiMARK, Magnevist, ProHance, and MultiHance - [UPDATED] Reports provide evidence for a causal relationship between gadolinium-based contrast agents and the development of Nephrogenic Systemic Fibrosis/Nephrogenic Fibrosing Dermopathy.(Posted 12/22/2006)

Clubbing

From Wikipedia, the free encyclopedia
Jump to: navigation, search
Name of Symptom/Sign:ClubbingClassifications and external resources
ICD-10
R68.3
ICD-9
781.5
Clubbing is also used to refer to the activity of gathering socially at nightclubs. Clubbing can also refer to an act of violence in which the primary weapon of attack is a blunt object. A Clubbed thumb is an unrelated congenital abnormality.
In medicine, clubbing (or digital clubbing) is a deformity of the fingers and fingernails that is associated with a number of diseases, mostly of the heart and lungs. Idiopathic clubbing can also occur. Hippocrates was probably the first to document clubbing as a sign of disease, and the phenomenon is therefore occasionally called Hippocratic fingers.
Contents[hide]
1 Signs and diagnosis
2 Disease associations
2.1 Isolated clubbing
2.2 HPOA
2.3 Primary HOA
3 Pathophysiology
4 References
//

Signs and diagnosis

Clubbing of the fingernail. The red line shows the outline of a clubbed nail
Clubbing develops in five steps:[1]
Fluctuation and softening of the nail bed (increased ballotability)
Loss of the normal <165° title="Distal" href="http://en.wikipedia.org/wiki/Distal">distal finger (resembling a drumstick)
Shiny aspect and striation of the nail and skin
Schamroth's test or Schamroth's window test (originally demonstrated by South African cardiologist Dr Leo Schamroth on himself[2]) is a popular test for clubbing. When the distal phalanges of corresponding digits of opposite hands are directly apposed, fingernail to fingernail, a small diamond-shaped "window" is apparent between the nailbeds. If this window is obliterated, the test is positive and clubbing is present.
When clubbing is encountered in patients, doctors will seek to identify its cause. They usually accomplish this by obtaining a medical history— particular attention is paid to lung, heart, and gastrointestinal conditions —and conducting a clinical examination, which may disclose associated features relevant to a diagnosis. Additional studies such as a chest x-ray may also be performed Disease associations

Isolated clubbing
Clubbing is associated with:
Lung disease:
Lung cancer, mainly large-cell (35% of all cases), not seen frequently in small cell lung cancer[3]
Interstitial lung disease
Tuberculosis
Bronchiectasis
Suppurative lung disease: lung abscess, empyema
Cystic fibrosis
Pulmonary hypertension
Mesothelioma
Heart disease:
Any disease featuring chronic hypoxia
Congenital cyanotic heart disease (most common cardiac cause)
Subacute bacterial endocarditis
Atrial myxoma (benign tumor)
Others:
Crohn's disease and ulcerative colitis
Hyperthyroidism (thyroid acropachy)
Liver diseases (in the "hepatopulmonary syndrome", a complication of cirrhosis)[4]
Malabsorption
Vascular anomalies of the affected arm (in unilateral clubbing)
Although many of these associations are recognised (such as the link with lung cancer), some are based on a few observations and might be false. Prospective studies of patients presenting with clubbing have not been performed, and hence there are no reliable numbers as to the distribution of the causes and the prognosis.
It is also worth noting that clubbing is not associated with chronic obstructive pulmonary disease (COPD). Indeed, the presence of clubbing in a patient with COPD should prompt a search for an underlying (lung) malignancy
.
HPOA
A special form of clubbing is hypertrophic pulmonary osteo-arthropathy, known in continental Europe as Pierre Marie-Bamberger syndrome. This is the combination of clubbing and thickening of periosteum (connective tissue lining of the bones) and synovium (lining of joints), and is often initially diagnosed as arthritis. It is associated almost exclusively with lung cancer. In dogs the condition is known as hypertrophic osteopathy.

Primary HOA
Primary hypertrophic osteo-arthropathy is HPOA without signs of pulmonary disease. This form has a hereditary component, although subtle cardiac abnormalties can occasionally be found. It is known in continental Europe as the Touraine-Solente-Golé syndrome.

Pathophysiology
Even though clubbing is a widely recognized symptom of many diseases the physiological mechanism that actually causes clubbing is not well understood. Current understanding is that these diseases cause vasodilation in the distal circulation which leads to hypertrophy of the tissue of the nailbeds and thus to the clubbed fingernails.
Other factors that have been implicated are the local effects of growth factors (such as platelet-derived growth factor and hepatocyte growth factor) that are usually sequestrated in the pulmonary capillary bed. Many of the conditions associated with clubbing result in shunting across some of the capillary beds in the pulmonary circulation.

References
^ Myers KA, Farquhar DR. The rational clinical examination: does this patient have clubbing? JAMA. 2001;286:341-7. PMID 11466101.
^ Schamroth L. Personal experience. S Afr Med J 1976;50:297-300. PMID 1265563.
^ Sridhar KS, Lobo CF, Altman RD. Digital clubbing and lung cancer. Chest 1998;114:1535-37. PMID 9872183
^ Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. Swiss Med Wkly. 2003;133:163-9. PMID 12715285.
Retrieved from "http://en.wikipedia.org/wiki/Clubbing"

Polycystic Kidney Disease

On this page:
Autosomal Dominant PKD
Autosomal Recessive PKD
Acquired Cystic Kidney Disease
Hope Through Research
Points to Remember
For More Information
Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts in the kidneys. The cysts are filled with fluid. PKD cysts can slowly replace much of the mass of the kidneys, reducing kidney function and leading to kidney failure.
The kidneys are two organs, each about the size of a fist, located in the upper part of a person's abdomen, toward the back. The kidneys filter wastes from the blood to form urine. They also regulate amounts of certain vital substances in the body.
When PKD causes kidneys to fail—which usually happens after many years—the patient requires dialysis or kidney transplantation. About one-half of people with the major type of PKD progress to kidney failure, also called end-stage renal disease (ESRD).
PKD can cause cysts in the liver and problems in other organs, such as the heart and blood vessels in the brain. These complications help doctors distinguish PKD from the usually harmless "simple" cysts that often form in the kidneys in later years of life.
In the United States, about 500,000 people have PKD, and it is the fourth leading cause of kidney failure. Medical professionals describe two major inherited forms of PKD and a noninherited form:
Autosomal dominant PKD is the most common inherited form. Symptoms usually develop between the ages of 30 and 40, but they can begin earlier, even in childhood. About 90 percent of all PKD cases are autosomal dominant PKD.
Autosomal recessive PKD is a rare inherited form. Symptoms of autosomal recessive PKD begin in the earliest months of life, even in the womb.
Acquired cystic kidney disease (ACKD) develops in association with long-term kidney problems, especially in patients who have kidney failure and who have been on dialysis for a long time. Therefore it tends to occur in later years of life. It is not an inherited form of PKD.
[Top]
Autosomal Dominant PKD
What is autosomal dominant PKD?
Autosomal dominant PKD is one of the most common inherited disorders. The phrase "autosomal dominant" means that if one parent has the disease, there is a 50-percent chance that the disease will pass to a child (see Genetic Diseases). At least one parent must have the disease for a child to inherit it. Either the mother or father can pass it along, but new mutations may account for one-fourth of new cases. In some rare cases, the cause of autosomal dominant PKD occurs spontaneously in the child soon after conception—in these cases the parents are not the source of this disease.
Many people with autosomal dominant PKD live for decades without developing symptoms. For this reason, autosomal dominant PKD is often called "adult polycystic kidney disease." Yet, in some cases, cysts may form earlier, even in the first years of life.
The disease is thought to occur equally in men and women and equally in people of all races. However, some studies suggest that it occurs more often in whites than in blacks and more often in females than in males.
The cysts grow out of nephrons, the tiny filtering units inside the kidneys. The cysts eventually separate from the nephrons and continue to enlarge. The kidneys enlarge along with the cysts (which can number in the thousands), while retaining roughly their kidney shape. In fully developed PKD, a cyst-filled kidney can weigh as much as 22 pounds. High blood pressure occurs early in the disease, often before cysts appear.
What are the symptoms of autosomal dominant PKD?
The most common symptoms are pain in the back and the sides (between the ribs and hips), and headaches. The dull pain can be temporary or persistent, mild or severe.
People with autosomal dominant PKD also can experience the following:
urinary tract infections
hematuria (blood in the urine)
liver and pancreatic cysts
abnormal heart valves
high blood pressure
kidney stones
aneurysms (bulges in the walls of blood vessels) in the brain
diverticulosis (small sacs on the colon)
How is autosomal dominant PKD diagnosed?
To diagnose autosomal dominant PKD, a doctor typically observes three or more kidney cysts using ultrasound imaging. The diagnosis is strengthened by a family history of autosomal dominant PKD and the presence of cysts in other organs.
An ultrasound imaging device passes harmless sound waves through the body to detect possible kidney cysts.
In most cases of autosomal dominant PKD, the person's physical condition appears normal for many years, even decades, so the disease can go unnoticed. Physical checkups and blood and urine tests may not lead to diagnosis. The slow, undetected progression is why some people live for many years without knowing they have autosomal dominant PKD.
Once cysts have formed, however, diagnosis is possible with imaging technology. Ultrasound, which passes sound waves through the body to create a picture of the kidneys, is used most often. Ultrasound imaging employs no injected dyes or radiation and is safe for all patients, including pregnant women. It can also detect cysts in the kidneys of a fetus.
More powerful and expensive imaging procedures such as computed tomography (CT scan) and magnetic resonance imaging (MRI) also can detect cysts, but they usually are not required for diagnosis because ultrasound provides adequate information. CT scans require x rays and sometimes injected dyes.
A genetic test can detect mutations in the PKD1 and PKD2 genes. Although this test can detect the presence of the autosomal dominant PKD mutations before cysts develop, its usefulness is limited by two factors; it cannot predict the onset or ultimate severity of the disease, and no absolute cure is available to prevent the onset of the disease. On the other hand, a young person who knows of a PKD gene mutation may be able to forestall the disease through diet and blood pressure control. The test may also be used to determine whether a young member of a PKD family can safely donate a kidney to a parent. Anyone considering genetic testing should receive counseling to understand all the implications of the test.
How is autosomal dominant PKD treated?
Although a cure for autosomal dominant PKD is not available, treatment can ease the symptoms and prolong life.
Pain. A doctor will first suggest over-the-counter pain medications, such as aspirin or Tylenol. For most but not all cases of severe pain, surgery to shrink cysts can relieve pain in the back and flanks. However, surgery provides only temporary relief and usually does not slow the disease's progression toward kidney failure.
Headaches that are severe or that seem to feel different from other headaches might be caused by aneurysms, or swollen blood vessels, in the brain. Headaches also can be caused by high blood pressure. People with autosomal dominant PKD should see a doctor if they have severe or recurring headaches—even before considering over-the-counter pain medications.
Urinary tract infections. Patients with autosomal dominant PKD tend to have frequent urinary tract infections, which can be treated with antibiotics. People with the disease should seek treatment for urinary tract infections immediately, because infection can spread from the urinary tract to the cysts in the kidneys. Cyst infections are difficult to treat because many antibiotics do not penetrate into the cysts. However, some antibiotics are effective.
High blood pressure. Keeping blood pressure under control can slow the effects of autosomal dominant PKD. Lifestyle changes and various medications can lower high blood pressure. Patients should ask their doctors about such treatments. Sometimes proper diet and exercise are enough to keep blood pressure low.
End-stage renal disease. Because kidneys are essential for life, people with ESRD must seek one of two options for replacing kidney functions: dialysis or transplantation. In hemodialysis, blood is circulated into an external machine, where it is cleaned before reentering the body; in peritoneal dialysis, a fluid is introduced into the abdomen, where it absorbs wastes, and it is then removed. Transplantation of healthy kidneys into ESRD patients has become a common and successful procedure. Healthy (non-PKD) kidneys transplanted into PKD patients do not develop cysts.
[Top]
Autosomal Recessive PKD
What is autosomal recessive PKD?
Autosomal recessive PKD is caused by a particular genetic flaw that is different from the genetic flaw that causes autosomal dominant PKD. Parents who do not have PKD can have a child with the disease if both parents carry the abnormal gene and both pass the gene to their baby. The chance of this happening (when both parents carry the abnormal gene) is one in four. If only one parent carries the abnormal gene, the baby cannot get the disease.
The symptoms of autosomal recessive PKD can begin before birth, so it is often called "infantile PKD." Children born with autosomal recessive PKD usually develop kidney failure within a few years. Severity of the disease varies. Babies with the worst cases die hours or days after birth. Children with an infantile version may have sufficient renal function for normal activities for a few years. People with the juvenile version may live into their teens and twenties and usually will have liver problems as well.
What are the symptoms of autosomal recessive PKD?
Children with autosomal recessive PKD experience high blood pressure, urinary tract infections, and frequent urination. The disease usually affects the liver, spleen, and pancreas, resulting in low blood-cell counts, varicose veins, and hemorrhoids. Because kidney function is crucial for early physical development, children with autosomal recessive PKD are usually smaller than average size.
How is autosomal recessive PKD diagnosed?
Ultrasound imaging of the fetus or newborn baby reveals cysts in the kidneys but does not distinguish between the cysts of autosomal recessive and autosomal dominant PKD. Ultrasound examination of kidneys of relatives can be helpful; for example, a parent or grandparent with autosomal dominant PKD cysts could help confirm diagnosis of autosomal dominant PKD in a fetus or child. (It is extremely rare, although not impossible, for a person with autosomal recessive PKD to become a parent.) Because autosomal recessive PKD tends to scar the liver, ultrasound imaging of the liver also aids in diagnosis.
How is autosomal recessive PKD treated?
Medicines can control high blood pressure in autosomal recessive PKD, and antibiotics can control urinary tract infections. Eating increased amounts of nutritious food improves growth in children with autosomal recessive PKD. In some cases, growth hormones are used. In response to kidney failure, autosomal recessive PKD patients must receive dialysis or transplantation (see End-stage renal disease).
Genetic Diseases
Genes are segments of DNA, the long molecules that reside in the nuclei of your body's cells. The genes, through complex processes, cause chemical activities that lead to growth and maintenance of the body. At conception, DNA (and therefore genes) from both parents are passed to the child.
A genetic disease occurs when one or both parents pass abnormal genes to a child at conception. If receiving an abnormal gene from just one parent is enough to produce a disease in the child, the disease is said to have dominant inheritance. If receiving abnormal genes from both parents is needed to produce disease in the child, the disease is said to be recessive.
The chance of acquiring a dominant disease (one gene copy is enough) is higher than the chance of acquiring a recessive disease (two gene copies are needed). A child who receives only one gene copy for a recessive disease at conception will not develop the genetic disease (such as autosomal recessive PKD), but could pass the gene to the following generation.
[Top]
Acquired Cystic Kidney Disease
What is ACKD?
ACKD develops in kidneys with long-term damage and bad scarring, so it often is associated with dialysis and end-stage renal disease. About 90 percent of people on dialysis for 5 years develop ACKD. People with ACKD can have any underlying kidney disease, such as glomerulonephritis or kidney disease of diabetes.
The cysts of ACKD may bleed. Kidney tumors, including kidney (renal) cancer, can develop in people with ACKD. Renal cancer is rare yet occurs at least twice as often in ACKD patients as in the general population.
How is ACKD diagnosed?
Patients with ACKD usually seek help because they notice blood in their urine (hematuria). The cysts bleed into the urinary system, which discolors urine. Diagnosis is confirmed using ultrasound, CT scan, or MRI of the kidneys.
How is ACKD treated?
Most ACKD patients are already receiving treatment for kidney problems. In rare cases, surgery is used to stop bleeding of cysts and to remove tumors or suspected tumors.
[Top]
Hope Through Research
Scientists have begun to identify the processes that trigger formation of PKD cysts. Advances in the field of genetics have increased our understanding of the abnormal genes responsible for autosomal dominant and autosomal recessive PKD. Scientists have located two genes associated with autosomal dominant PKD. The first was located in 1985 on chromosome 16 and labeled PKD1. PKD2 was localized to chromosome 4 in 1993. Within 3 years, the scientists had isolated the proteins these two genes produce—polycystin-1 and polycystin-2.
When both of these genes are normal, the proteins they produce work together to foster normal kidney development and inhibit cyst formation. A mutation in either PKD1 or PKD2 can lead to cyst formation, but evidence suggests that the disease development also requires other factors, in addition to the mutation in one of the PKD genes.
Genetic analyses of most families with PKD confirm mutations in either the PKD1 or PKD2 gene. In rare cases, however, families with PKD have been found to have normal PKD1 and PKD2 genes. As a result, researchers theorize that a PKD3 gene exists, but that gene has not been mapped or identified.
Researchers recently identified the autosomal recessive PKD gene (called PKHD1) on chromosome 6. No genetic test kit is yet available to detect mutations in PKHD1.
Researchers have bred mice with a genetic disease that parallels both inherited forms of human PKD. Studying these mice will lead to greater understanding of the genetic and nongenetic mechanisms involved in cyst formation. In 2000, scientists reported that a cancer drug was successful in inhibiting cyst formation in mice with the PKD gene. In 2003, scientists also demonstrated that another compound, one that blocks function of a kidney receptor, inhibits cyst formation in mice with the ADPKD or ARPKD gene. The scientists hope that further testing will lead to safe and effective treatments for humans.
[Top]
Points to Remember
The three types of PKD are
two inherited forms:
a common form that usually causes symptoms in midlife
a rare form that usually causes symptoms in early childhood
a noninherited form associated with long-term kidney problems, dialysis, and old age
The signs of PKD include
pain in the back and lower sides
headaches
urinary tract infections
blood in the urine
cysts in the kidneys and other organs
Diagnosis of PKD is obtained by
ultrasound imaging of kidney cysts
ultrasound imaging of cysts in other organs
family medical history (genetic testing)
PKD has no cure. Treatments include
medicine and surgery to reduce pain
antibiotics to resolve infections
dialysis to replace functions of failed kidneys
kidney transplantation
[Top]
For More Information
Polycystic Kidney Disease Foundation9221 Ward Parkway, Suite 400Kansas City, MO 64114–3367Phone: 1–800–PKD–CURE (753–2873) or 816–931–2600Email: pkdcure@pkdcure.orgInternet: www.pkdcure.org
American Association of Kidney Patients3505 East Frontage Road, Suite 315Tampa, FL 33607Phone: 1–800–749–2257 or 813–636–8100Email: info@aakp.orgInternet: www.aakp.org
National Kidney Foundation30 East 33rd StreetNew York, NY 10016Phone: 1–800–622–9010 or 212–889–2210Email: info@kidney.orgInternet: www.kidney.org
[Top]
The U.S. Government does not endorse or favor any specific commercial product or company. Trade, proprietary, or company names appearing in this document are used only because they are considered necessary in the context of the information provided. If a product is not mentioned, the omission does not mean or imply that the product is unsatisfactory.