Recessive mutations in a gene called phospholipase C epsilon or PLCE1 could result in a to a severe, early-onset form of kidney disease and renal failure in children, a previously unknown cause. This finding was disclosed by scientists at the University of Michigan Medical School.

Identification of the mutual gene is of interest to the scientists as PLCE1 affects the development of podocytes that are specialized cells playing a vital role in ability of the kidney when it comes to removing waste products from blood and retaining important blood proteins.

From Sciencedaily.com:

Some types of nephrotic syndrome can be treated with steroids or other drugs, but steroid-resistant forms of the disease as a rule do not respond to treatment. Untreated nephrotic syndrome often causes severe scarring and a condition called focal segmental glomerulosclerosis (FSGS), which progresses about 50 percent of the time to end-stage kidney disease and renal failure.

PLCE1 is the seventh gene scientists have found to be involved in different types of steroid-resistant nephrotic syndrome, and the second gene that is expressed in podocytes — specialized cells with octopus-like tentacles surrounding the glomerulus.

Podocytes are currently under intensive study, because scientists believe they play a vital role in the blood filtration process. Identifying genes and proteins that are active in podocytes will help scientists understand how they work.

“We found that PLCE1 is expressed in developing and mature podocytes,” says Hildebrandt. “Most of the PLCE1 mutations we identified apparently prevented podocytes from developing normally in the embryo, so defects were present at birth. A milder mutation seemed to interfere with repair mechanisms in the glomerulus, so defects didn’t show up until later in life.”

Friedhelm Hildebrandt, M.D., the U-M’s Frederick G L Huetwell Professor for the Cure and Prevention of Birth Defects, remarked that this is the first report of infants with two mutuations in recessive gene for steroid-resistant nephrotic syndrome that nevertheless responded to treatment with steroids.

Posted in Steroids | Comments Off

According to a study by neurologists at the Jefferson Headache Center at Thomas Jefferson University Hospital in Philadelphia, there are no additional benefits with steroids being combined with local anesthetics for treating daily headaches.

Findings of this study were disclosed after examining 29 men and women with chronic daily headaches who were divided into two groups. While one group was administered with GONB (greater occipital nerve block) with lidocaine and bupivicaine alone, the other group was administered with the two drugs with steroids.

From News-Medical.Net:

According to Avi Ashkenazi, M.D., assistant professor of neurology at Jefferson Medical College of Thomas Jefferson University, greater occipital nerve block (GONB) is a technique to treat acute headaches by locally injecting anesthetics such as lidocaine just under the skin to provide acute pain relief for acute headache attacks and migraines. Such treatments can work quickly, perhaps in seconds or by five to 10 minutes, and its effectiveness could last from hours to two or three days to several weeks. Treatment can be repeated if needed, he notes, and it has few side effects.

There are two ways to block the occipital nerves: by injecting a local anesthetic alone or by adding an anti-inflammatory steroid along with the anesthetic. No data exist whether one is better than the other, but the use of corticosteroids is controversial because of their potential side effects, such as hair loss at the site of injection. He notes that there is no consensus among headache experts about steroid use for headache.

It was concluded by Dr. Ashkenazi that he and his team will find steroids lengthening the anti-inflammatory effects of injections in the near future.

Posted in Steroids | Comments Off

Researchers from Children’s Hospital Boston and Brigham and Women’s Hospital have been able to discover an important genetic cause of a devastating kidney disease that is the second leading cause of kidney failure in children, as per The NephCure Foundation.

The study published online on December 20, 2009, by Nature Genetics is considered to provide new insights on development of treatment mechanisms for this complication and FSGS (focal segmental glomerulosclerosis).

NephCure that helped fund the study noted that there are nearly 26 million chronic kidney disease patients in the United States alone, of which FSGS is one of the most common forms.

From Sciencedaily.com:

Patients with FSGS are often treated with steroids, which are only partially effective and have very harsh side effects. In addition, they often face several trips a week to the hospital for dialysis, and many require a kidney transplant, along with lifelong treatment with powerful immunosuppressants to prevent organ rejection.

The research team, led by Elizabeth Brown, MD of Children’s Division of Nephrology, working in the laboratory of Martin Pollak, MD of the Renal Division at Brigham and Women’s Hospital, identified the gene by performing a genetic linkage analysis in two large families with FSGS. Linkage analysis is a gene-finding technique that compares affected with unaffected family members, looking for a piece of DNA whose location is already known, and that is inherited only by affected members. Using that piece of DNA as a “signpost,” researchers can then look nearby to find the disease gene.

This study was supported by the National Institute of Diabetes and Digestive and Kidney Diseases, the Clinical Investigator Training Program: Beth Israel Deaconess Medical Center Harvard and Massachusetts Institute of Technology Health Sciences and Technology, Pfizer Inc., Merck and Co., The NephCure Foundation, and the Cole Pasqualucci Nephrotic Syndrome and FSGS Research fund.

Posted in Steroids | Comments Off

The use of anabolic steroids by patients suffering kidney diseases may prove to be effective to increase lean muscle mass besides minimizing the fatigue level to a significant extent, as per a new study.

Kidney disease patients tend to experience malnutrition and an accompanying loss of muscle mass and these side effects have been associated in the past with an increased mortality.

From Bio-Medicine.Org:

A team of University of California San Francisco researchers, led by Kirsten L. Johansen, M.D., UCSF assistant professor of medicine, epidemiology & biostatistics, studied a group of patients who were receiving dialysis and were suffering from malnutrition.

In a randomized, double blind trial, 29 patients were given either the anabolic steroid nandrolone decanote or a placebo (dummy medication) for a six-month period. All of the patients were receiving dialysis treatments at San Francisco General Hospital. At the end of the trial, patients receiving the anabolic steroid gained an average of 5.7 pounds more lean body mass than the patients who got the placebo. Also, the patients who received the steroid reported less fatigue and scored better on physical performance tests. The researchers’ results are published in the April 14 issue of the Journal of the American Medical Association (JAMA).

Hemodialysis, the most common form of dialysis in the United States, is a medical procedure employed when a patient’s kidneys cannot properly clean the blood of toxins and waste products. A patient’s blood is removed from the body via a tube inserted in a vein, filtered by a dialysis machine, and then replaced. Typically, this process must be repeated three times a week and takes three to four hours. In some cases, dialysis is considered a “bridge” while the patient is awaiting a kidney transplant – which can be up to a three year wait in the US.

This study was supported by grants from the National Center for Research Resources, the National Institute of Diabetes and Digestive and Kidney Diseases, and the Bay Area Nutrition Center.

Posted in Steroids | Comments Off

A novel immunosuppression minimization protocol has been developed by Transplant researchers at the University of Pittsburgh’s Thomas E. Starzl Transplantation Institute. This protocol can dramatically improve intestinal transplant graft survival besides reducing infection and rejection rates.

It was noted by the researchers that the protocol may prove effective for improving overall life quality of patients and help them make lesser use of many anti-rejection drugs that may result in serious infections and major complications.

From News-Medical.Net:

Following transplantation, the 103 patients who received thymoglobulin and the 20 patients who received campath received the standard anti-rejection drug tacrolimus and none of the patients received steroids. Tapering of tacrolimus was attempted after 120 days.

While 43 percent of patients experienced some level of rejection before initial weaning, none showed evidence of chronic rejection. Patients under the campath protocol did slightly better than those treated with thymoglobulin.

Of the 123 intestinal transplants, 55 involved children, while the other 68 were adult cases.

The current success of this novel anti-rejection protocol should allow major improvement in both the long-term efficacy and quality of life after intestinal and multivisceral transplants, according to the researchers.

Kareem Abu-Elmagd, M.D., Ph.D., F.A.C.S., professor of surgery at the University of Pittsburgh School of Medicine and director of the Intestinal Rehabilitation and Transplant Center at the University of Pittsburgh Medical Center’s (UPMC) Thomas E. Starzl Transplantation Institute and lead author of the study, remarked that the protocol is helpful in reducing the amount of anti-rejection drugs in patients for helping them to lead a full, productive life.

Posted in Steroids | Comments Off