Biomaterial Database

Familial, dexamethasone-suppressible, normokalemic hyperaldosteronism. 1980

C E Grim, and M H Weinberger

An 8-year-old boy was found to be hypertensive on routine exam (144/88). His brother (age 6) and father (age 31) were also found to have elevated blood pressure. Detailed investigations first revealed a low renin level without hypokalemia. Further study revealed that all three patients had low plasma renin activity and nonsuppresible plasma aldosterone levels after saline infusion. Serum potassium was almost always normal. A trial of dexamethasone therapy normalized blood pressure, and plasma and urinary aldosterone decreased to low levels and renin levels increased. Therapy with spironolactone and prednisone also normalized blood pressure. However, the amount of prednisone required to maintain normotension resulted in Cushingoid features and has been discontinued. Studies in the father suggest that the aldosterone production by his adrenals is hyperresponsive to adrenocorticotropic hormone (ACTH). Renin levels should be determined in all hypertensive children and their hypertensive parents. If renin is low and plasma aldosterone fails to be suppressed by saline infusion, a trial of dexamethasone would seem indicated before other investigations are carried out.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D011188	Potassium	An element in the alkali group of metals with an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte that plays a significant role in the regulation of fluid volume and maintenance of the WATER-ELECTROLYTE BALANCE.
D012083	Renin	A highly specific (Leu-Leu) endopeptidase that generates ANGIOTENSIN I from its precursor ANGIOTENSINOGEN, leading to a cascade of reactions which elevate BLOOD PRESSURE and increase sodium retention by the kidney in the RENIN-ANGIOTENSIN SYSTEM. The enzyme was formerly listed as EC 3.4.99.19.	Angiotensin-Forming Enzyme,Angiotensinogenase,Big Renin,Cryorenin,Inactive Renin,Pre-Prorenin,Preprorenin,Prorenin,Angiotensin Forming Enzyme,Pre Prorenin,Renin, Big,Renin, Inactive
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002940	Circadian Rhythm	The regular recurrence, in cycles of about 24 hours, of biological processes or activities, such as sensitivity to drugs or environmental and physiological stimuli.	Diurnal Rhythm,Nyctohemeral Rhythm,Twenty-Four Hour Rhythm,Nycthemeral Rhythm,Circadian Rhythms,Diurnal Rhythms,Nycthemeral Rhythms,Nyctohemeral Rhythms,Rhythm, Circadian,Rhythm, Diurnal,Rhythm, Nycthemeral,Rhythm, Nyctohemeral,Rhythm, Twenty-Four Hour,Rhythms, Circadian,Rhythms, Diurnal,Rhythms, Nycthemeral,Rhythms, Nyctohemeral,Rhythms, Twenty-Four Hour,Twenty Four Hour Rhythm,Twenty-Four Hour Rhythms
D003907	Dexamethasone	An anti-inflammatory 9-fluoro-glucocorticoid.	Hexadecadrol,Decaject,Decaject-L.A.,Decameth,Decaspray,Dexasone,Dexpak,Hexadrol,Maxidex,Methylfluorprednisolone,Millicorten,Oradexon,Decaject L.A.
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006889	Hydroxycorticosteroids	A group of corticosteroids carrying hydroxy groups, usually in the 11- or 17-positions. They comprise the bulk of the corticosteroids used systemically. As they are relatively insoluble in water, salts of various esterified forms are often used for injections or solutions.
D006929	Hyperaldosteronism	A condition caused by the overproduction of ALDOSTERONE. It is characterized by sodium retention and potassium excretion with resultant HYPERTENSION and HYPOKALEMIA.	Aldosteronism,Conn Syndrome,Conn's Syndrome,Primary Hyperaldosteronism,Conns Syndrome,Hyperaldosteronism, Primary,Syndrome, Conn,Syndrome, Conn's
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults