Jersey Shore University Medical Center Kidney Expert Discovers New Class of Potentially Fatal Electrolyte Disorder, Treatable through Low-Potassium Diet

Mehandru Syndrome, one of only two new nephrology syndromes identified in last 35 years, affects regulation of potassium levels in the blood. Condition named for Dr. Sushil K. Mehandru, whose research led to its identification and treatment.

A New Jersey nephrologist and his colleagues at Hackensack Meridian Jersey Shore University Medical Center have identified for the first time a likely genetic disorder that is marked by hyperkalemia, an abnormally high level of potassium in the blood, along with a protocol to treat it. An article describing the condition, "Mehandru Syndrome, Newly Discovered Class of Pseudohypoaldosteronism (PHA-M), Role of Medication in Addition to Low Potassium Diet," was published recently in the International Journal of Clinical and Medical Cases.1  An article describing the condition was also published in the Saudi Journal of Kidney Diseases and Transplantation.2

Mehandru Syndrome is a potentially life-threatening electrolyte disorder that occurs in the absence of indicators or conditions that can cause high potassium levels, such as kidney disease, diabetes or high potassium diets or supplements. Potassium is the second most abundant molecule in the body and performs several important functions, including cellular metabolism, sugar and protein synthesis, and maintenance of electrical signals across cell membranes, especially in the heart. Mehandru Syndrome is named after renowned Nephrologist and Professor of Medicine Sushil K. Mehandru, M.D., whose research led to its discovery.

"This condition of high potassium is caused by a presumed genetic metabolic resistance to the hormone that regulates sodium, potassium and acid levels in the blood," said Dr. Mehandru, professor, Hackensack Meridian School of Medicine and chief, Division of Nephrology and Hypertension, Jersey Shore University Medical Center. "High potassium often has no symptoms, and if left untreated, it can lead to potentially fatal disturbances in heart rhythm. That's why it's so important that physicians learn about this syndrome. There may be millions of people with it in the U.S. and abroad who are unaware they have it, this could be the result of possible misdiagnosis. "

Mehandru Syndrome appears to affect individuals with an average age of 53 years, irrespective of gender, and predominantly affecting Caucasians. It has also been diagnosed in African American and Jewish populations. Adherence to a low-potassium diet is key to successful management of Mehandru syndrome, along with routine, sometimes weekly monitoring until the potassium levels decrease and stabilize. Foods that are high in potassium include bananas, oranges, dried fruits such as dates, potatoes, and certain green vegetables such as broccoli and spinach, among other foods.

Dr. Mehandru and his team have treated several families where several members were found to have high potassium without any known cause, and, after testing, they were diagnosed with Mehandru Syndrome. Through strict low-potassium diets, these patients were able to lower their high levels of potassium and avoid potential cardiac complications.

A patient foundation started by Dr. Mehandru covers ongoing care and laboratory tests for patients treated in his office who are uninsured or underinsured.

Dr. Mehandru has studied kidney disease and electrolyte abnormalities for many years, and is Director of The Mehandru Center for Innovation in Nephrology at Jersey Shore University Medical Center, recently named by Hackensack Meridian Health to recognize and honor Dr. Mehandru's ongoing contributions to kidney research and improving the health of patients with kidney disease and related conditions. Dr. Mehandru is working with Professor of Medicine, Cleveland Clinic Tushar Vaccharajani, M.D., and researchers at Columbia University and The New York Genome Center to identify the genetic mutation or mutations that cause resistance to aldosterone, the hormone which helps metabolize potassium, leading to Mehandru Syndrome.

"The advanced medical research being conducted by Dr. Mehandru, and other highly specialized physicians at our academic medical center, greatly benefits our patients and creates a culture of clinical excellence at Jersey Shore," said Kenneth N. Sable, M.D., MBA, FACEP, Hackensack Meridian Health, regional president, southern market.

About 15 years ago, Barbara Lombardo was busy raising her three children in Howell, NJ, with her husband Charlie and working in the office of an oral surgeon, and did not have much time to go to doctors' offices for diagnostic tests, let alone be seriously ill. But blood work that was part of an annual physical exam with her primary care doctor revealed "sky high" potassium levels with no known cause. This left her confused, especially when her primary care doctor did not think the results meant anything serious at the time.

To be on the safe side, she pursued a work up and advice from Dr. Mehandru at Jersey Shore University Medical Center, who painstakingly assessed her kidney function with a battery of tests but did not find that she had kidney failure. Her blood pressure was high, and he treated her with a medication for that, and with a medication called fludrocortisone to help get rid of excess potassium. He followed her year after year and did not find a decrease in kidney function, but theorized that she might have a failure of the parathyroid glands, which proved not to be the case.

Eventually, Dr. Mehandru determined that Barbara's profile fit the clinical group of patients he had been following who had high potassium but no other risks except for high blood pressure, and no kidney disease. He put her on a low potassium diet of about 2000 mg a day, which Barbara says "is a challenge to achieve but I knew it was important so I stuck to the diet." She mentioned that there were certain foods that were not obviously high in potassium that she had to avoid, such as certain green vegetables, but she was determined to get to the root of her high potassium problem.

After two months on the diet, she was able to slowly come off her high blood pressure medicine and the medication to lower her potassium, and her potassium level dropped to a nearly normal level. "Going through the clinical testing for kidney disease was stressful, and frankly it was frightening to not know why I had high potassium and to realize how it could have affected my heart," said Barbara, who is now retired. "Dr. Mehandru was the most qualified and experienced doctor to treat me, and his manner was so reassuring. He calmed me and told me that we could get through this together."

"He was right," Barbara says. "We got to the bottom of my condition, and now years later, I am in control of my health, and my potassium is normal, because I am so vigilant about my diet. I am so pleased not to have to take medications for my condition.  My advice for everyone is to get regular medical screenings and blood work, and if you have ongoing high potassium or any abnormal test result, go see a specialist. There is no need to risk losing your health. Expert medical care is there for you."

Dr. Mehandru adds that he and his colleagues "want to disseminate our findings about this syndrome of high potassium levels to the larger medical community with the goal of finding and helping to treat undiagnosed or misdiagnosed patients with high levels of potassium in their blood and no known causes. We want to prevent suffering and extend life."

Dr. Mehandru and the nephrology team were also recently published for their work in reducing high blood potassium caused by metabolic acidosis and other conditions, successfully establishing a new method for battling lactic acidosis in lymphoma and establishing a new treatment protocol for bacterial meningitis.

For more information, visit www.hackensackmeridianhealth.org/info/nephrology/. To make an appointment, call 732-974-0100.

References

1Mehandru, Kaur, Yuh, et al. "Mehandru Syndrome, Newly Discovered Class of Pseudohypoaldosteronism (PHA-M), Role of Medication in Addition to Low Potassium Diet," International Journal of Clinical and Medical Cases, Volume: 3.8, Boffin Access, Open Access Journal, December 2020.

2 Mehandru SK, Haroon A, Masud A, et al. Saudi J Kidney Dis Transpl 2020;31(5);1134-1139