The Company's rare neuromuscular franchise includes KEVEYIS (dichlorphenamide), the first and only FDA-approved treatment for hyperkalemic
, hypokalemic, and related variants of primary periodic paralysis.
A potassium level greater than 7.0 mEq/L is generally considered a hyperkalemic
emergency and can be potentiated by ongoing tumor lysis, hypocalcemia, and/or renal impairment.
state is defined as potassium serum blood levels above 5.1 mmol/L.
PHA type II (PHA2) is also known as Gordon syndrome or familial hyperkalemic
SCN4A -associated disorders include hyperkalemic
periodic paralysis (hyperPP, OMIM: 170500), hypokalemic periodic paralysis Type 2 (OMIM: 613345), paramyotonia congenita (PMC, OMIM: 168300), sodium channel myotonias (SCM, OMIM: 608390), and congenital myasthenic syndrome (OMIM: 614198).
Karet, "Mechanisms in hyperkalemic
renal tubular acidosis," Journal of theAmerican Society of Nephrology, vol.
While primary hyperkalemic
paralysis is secondary to a defective sodium channel, a number of aetiologies have been reported as leading to secondary hyperkalemic
state, cardiac effects of potassium occur due to the cell membrane depolarization.
(2) RTA is suspected in the presence of hyperchloremic metabolic acidosis (HMA) and it is divided into three categories: proximal RTA, distal RTA (dRTA), and hyperkalemic
Updated, this edition has a new two-color format; redrawn and colorized line drawings; new coverage of recent developments in disorders of water homeostasis, genetic hypokalemic and hyperkalemic
disorders, calcium, phosphorus, vitamin D, parathyroid hormone activity, the mechanisms of vascular and epithelial kidney injury during ischemia, the physiology and pathophysiology of urinary tract obstruction, chronic kidney disease, proteinuric states, preeclampsia and eclampsia, and the glomerulopathies and vasculitides; updated discussion of the key role of the kidney in the pathogenesis of hypertensive states; updated information on acid-base disorders; and a new chapter on the genomic and nongenomic effects of angiotensin and aldosterone in renal and cardiovascular disease.
(18) In cases of superimposed hereditary or genetic channelopathy variants, such as HPP (hyperkalemic
periodic paralysis) where deficient potassium channels are dysfunctional and potassium levels rise.
Zhang et al., "Hyperkalemic
hypertension-associated cullin 3 promotes WNK signaling by degrading KLHL3," The Journal of Clinical Investigation, vol.