Primary aldosteronism in childhood due to primary adrenal hyperplasia. 1988

Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
Department of Pediatrics, Kenritsu Shinjo Hospital.

We present an unusual case of primary aldosteronism in childhood. A 9-year-old boy had hypertension, hypokalemia, hyporeninemia and hyperaldosteronism. Dexamethasone administration decreased plasma aldosterone transiently but failed to correct the hyperaldosteronism, excluding dexamethasone-suppressible hyperaldosteronism. Plasma aldosterone decreased with upright posture and showed a circadian rhythm. Spironolactone treatment normalized blood pressure and serum potassium and lowered aldosterone secretion. During the studies, plasma aldosterone correlated with serum cortisol but not with plasma renin. Preoperative results indicated that this patient presented the functional features of aldosteronoma. Adrenal computed tomography, scintigraphy and left venography were not diagnostic of adrenal lesions. The left adrenal venous sampling showed hypersecretion of aldosterone from the left adrenal gland. The left adrenalectomy revealed micronodular hyperplasia but resulted in a prompt and sustained reversal of hypertension and hyperaldosteronism. These findings suggest that primary aldosteronism in this patient resulted from primary adrenal hyperplasia. Thus, adrenal hyperplasia is a heterogenous group of disorders and carefully selected studies allow prospective selection of appropriate treatment.

UI MeSH Term Description Entries
D006973 Hypertension Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more. Blood Pressure, High,Blood Pressures, High,High Blood Pressure,High Blood Pressures
D007008 Hypokalemia Abnormally low potassium concentration in the blood. It may result from potassium loss by renal secretion or by the gastrointestinal route, as by vomiting or diarrhea. It may be manifested clinically by neuromuscular disorders ranging from weakness to paralysis, by electrocardiographic abnormalities (depression of the T wave and elevation of the U wave), by renal disease, and by gastrointestinal disorders. (Dorland, 27th ed) Hypopotassemia,Hypokalemias,Hypopotassemias
D007019 Hypoproteinemia A condition in which total serum protein level is below the normal range. Hypoproteinemia can be caused by protein malabsorption in the gastrointestinal tract, EDEMA, or PROTEINURIA. Hypoproteinemias
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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
D000312 Adrenal Hyperplasia, Congenital A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders. Congenital Adrenal Hyperplasia,Hyperplasia, Congenital Adrenal,Adrenal Hyperplasias, Congenital,Congenital Adrenal Hyperplasias,Hyperplasias, Congenital Adrenal
D000315 Adrenalectomy Excision of one or both adrenal glands. (From Dorland, 28th ed) Adrenalectomies

Related Publications

Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
November 1980, The Journal of clinical endocrinology and metabolism,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
May 2006, Nihon rinsho. Japanese journal of clinical medicine,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
September 1998, Journal of endocrinological investigation,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
April 2005, Hypertension research : official journal of the Japanese Society of Hypertension,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
February 1997, Zhonghua yi xue za zhi = Chinese medical journal; Free China ed,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
August 1956, Lancet (London, England),
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
May 1984, Klinische Wochenschrift,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
April 2022, Cureus,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
July 1959, A.M.A. journal of diseases of children,
Y Kikuta, and K Sanjo, and K Nakajima, and I Ashizawa, and M Ojima
March 1980, Pediatrics,
Copied contents to your clipboard!