11-beta-Hydroxysteroid Dehydrogenase Type 2/genetics* ; Humans ; Mineralocorticoid Excess Syndrome, Apparent/genetics* ; Pedigree

Chronic ingestion of licorice or licorice-like compounds induces a syndrome with typical findings of mineralocorticoid excess such as hypertension, hypokalemia, metabolic alkalosis, low plasma renin activity. The only unique feature is that plasma aldosterone concentration is decreased. We described a 79-year-old woman who, with a plasma K+ 1.75 mEq/L, showed a paralysis and severe rhabdomyolysis after the habitual comsumption of licorice in the form of a herbal medication. Following potassium replacement therapy and discontinuation of licorice ingestion, complete clinical recovery was observed within ten days. It is important for physicians to keep licorice consumption in mind as a cause for hypokalemic paralysis and rhabdomyolysis.
Aged ; Aldosterone ; Alkalosis ; Eating ; Female ; Glycyrrhiza ; Humans ; Hypertension ; Hypokalemia* ; Mineralocorticoid Excess Syndrome, Apparent ; Muscular Diseases* ; Paralysis ; Plasma ; Potassium ; Renin ; Rhabdomyolysis

Secondary hypertension can account for 15% of hypertension cases. The causes of secondary hypertension mostly come from renal diseases, such as renal parenchymal or renovascular disease, and endocrine diseases. The importance of diagnosing secondary hypertension lies in the fact that it may convert an incurable disease into a potentially curable disease. Even if the underlying disease may not be curable, being able to offer disease specific treatments may often make blood pressure control much easier. The causes of endocrine hypertension include primary aldosteronism, pheochromocytoma, Cushing's syndrome, acromegaly, hyper- or hypothyroidism, hyperparathyroidism and other mineralocorticoid hypertension (e.g. apparent mineralocorticoid excess syndrome, Liddle's syndrome). Primary aldosteronsim, pheochromocytoma, and Cushing's syndrome are among the common causes of endocrine hypertension. The first step in evaluating a patient with suspected endocrine-related hypertension is to exclude other secondary causes, particularly renal disorders. An accurate diagnosis of endocrine hypertension provides the clinician a unique treatment opportunity. This topic review will summarize rare causes of endocrine hypertension except primary aldosteronism and pheochromocytoma.
Acromegaly ; Blood Pressure ; Cushing Syndrome ; Endocrine System Diseases ; Humans ; Hyperaldosteronism ; Hyperparathyroidism ; Hypertension ; Hypothyroidism ; Mineralocorticoid Excess Syndrome, Apparent ; Mineralocorticoids ; Pheochromocytoma ; Resin Cements

Mutations in genes encoding ion channels, transporters, exchangers, and pumps in human tissues have been increasingly reported to cause hypokalemia. Assessment of history and blood pressure as well as the K+ excretion rate and blood acid-base status can help differentiate between acquired and inherited causes of hypokalemia. Familial periodic paralysis, Andersen's syndrome, congenital chloride-losing diarrhea, and cystic fibrosis are genetic causes of hypokalemia with low urine K+ excretion. With respect to a high rate of K+ excretion associated with faster Na+ disorders (mineralocorticoid excess states), glucoricoid-remediable aldosteronism and congenital adrenal hyperplasia due to either 11beta-hydroxylase and 17alpha-hydroxylase deficiencies in the adrenal gland, and Liddle's syndrome and apparent mineralocorticoid excess in the kidney form the genetic causes. Among slow Cl- disorders (normal blood pressure, low extracellular fluid volume), Bartter's and Gitelman's syndrome are most common with hypochloremic metabolic alkalosis. Renal tubular acidosis caused by mutations in the basolateral Na+/HCO3 - cotransporter (NBC1) in the proximal tubules, apical H+-ATPase pump, and basolateral Cl-/HCO3 - exchanger (anion exchanger 1, AE1) in the distal tubules and carbonic anhydroase II in both are genetic causes with hyperchloremic metabolic acidosis. Further work on genetic causes of hypokalemia will not only provide a much better understanding of the underlying mechanisms, but also set the stage for development of novel therapies in the future.
Acid-Base Equilibrium ; Acidosis ; Acidosis, Renal Tubular ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Aldosterone ; Alkalosis ; Blood Pressure ; Carbon ; Cystic Fibrosis ; Diarrhea ; Extracellular Fluid ; Humans ; Hyperaldosteronism ; Hypokalemia ; Hypotension ; Ion Channels ; Kidney ; Mineralocorticoid Excess Syndrome, Apparent ; Paralyses, Familial Periodic ; Renin