Newswise — Researchers at The Mehandru Center for Innovation in Nephrology at Hackensack Meridian Jersey Shore University Medical Center and other authors recently had their new case report article “Metabolic Acidosis, Hyperkalemia, and Renal Unresponsiveness to Aldosterone Syndrome: Response to Treatment with Low-Potassium Diet,” published in the Saudi Journal of Kidney Diseases and Transplantation.

Led by Nephrologist and Professor of Medicine Sushil K. Mehandru, M.D., the article describes three cases of Mehandru Syndrome, 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.  In each case, the high blood potassium was managed successfully with a low-potassium diet.

Mehandru Syndrome is named after renowned Nephrologist and Professor of Medicine Dr. Mehandru, whose research led to its discovery. “This condition of high potassium is caused by a presumed genetic metabolic resistance to  aldosterone, the hormone that helps break down potassium 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.”

The publication describes three cases of Mehandru Syndrome in patients who had metabolic acidosis, a condition where too much acid accumulates in the body; hyperkalemia, a high level of the electrolyte potassium in the blood; normal blood pressure; normal calcium excretion by the kidneys; and normal levels of renin, and aldosterone.

These patients did not respond to diuretics to attempt to reduce the high levels of potassium in their blood or to sodium restriction.  Rather, management of their care with a low-potassium diet resulted in the normalization of potassium blood levels and correction of the metabolic acidosis.

The authors ruled out the possible diagnosis of Gordon syndrome in these patients. It is an autosomal dominant disease that usually presents in children and adolescents. Classical presentation of Gordon syndrome includes high potassium, severe hypertension, metabolic acidosis, low renin and aldosterone levels in the presence of normal renal function. The treatment of Gordon syndrome has conventionally included aggressive salt restriction and low-dose diuretics. In the published article, the authors report that the three cases they described did not have severe hypertension or elevated calcium in the urine, and did not respond to salt restriction or diuretic therapy. 

The authors propose that Mehandru Syndrome be considered in the differential diagnosis of patients who present in the clinical setting with unexplained high levels of potassium and metabolic acidosis.  “The cases we presented in this paper have unusual clinical characteristics and as yet undefined genetic abnormalities,” said Dr. Mehandru.  “Further genetic mutations need to be defined to understand the changes at the molecular level.”

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 contributions to kidney research and improving the health of patients with kidney disease and related conditions.

Reference

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

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