eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Hyperaldosteronism: Follow-up

Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London), Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece
Coauthor(s): Antony Lafferty, MB ChB, FRACP, Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia
Contributor Information and Disclosures

Updated: Mar 4, 2009

Follow-up

Further Outpatient Care

  • Frequency and requirement for follow-up depends on the cause of the hyperaldosteronism.
  • Patients who are treated medically need regular follow-up to ensure adequacy of blood pressure control and treatment of hypokalemia.
  • In children, doses must be adjusted as patients grow.
  • In cases with familial hyperaldosteronism, genetic counseling is also important at an age-appropriate level.

Inpatient & Outpatient Medications

  • Severe hypokalemia may require intravenous correction if the potassium is less than 2.5 mmol/L or the patient is clinically symptomatic. Once stable, sodium restriction and oral potassium supplements may be used as effectively as or in addition to potassium-sparing diuretics.
  • Spironolactone is the most effective drug for controlling the effects of hyperaldosteronism, although it may interfere with the progression of puberty. Newer drugs with greater specificity for the mineralocorticoid receptor than spironolactone are becoming available.
  • Alternative medications for patients in whom aldosterone antagonists are contraindicated include amiloride and triamterene as well as calcium channel antagonists (see Medication), alpha-adrenergic antagonists (especially alpha1-specific agents, eg, prazosin, doxazosin); in patients with angiotensin II–responsive disease, angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists are indicated.

Transfer

  • Patients receiving medical treatment for hyperaldosteronism must be transferred to a physician with experience managing such cases. This may be an endocrinologist, a cardiologist, or a nephrologist.

Complications

Complications of primary hyperaldosteronism can be divided into those due to the primary disease, such as hypertension and hypokalemia, and those arising from treatment.

  • Hypertension
    • Hypertension due to hyperaldosteronism can cause damage to many organs and organ systems, including the heart (hypertrophy and myocardial fibrosis), the blood vessels (vascular remodeling with medial and intimal hypertrophy and acceleration of atherogenesis), the eyes (arterial narrowing, retinal ischemia, and papilledema), the kidneys (progressive deterioration with nephrosclerosis), and the brain (hemorrhage).
    • Aggressive blood pressure control and early diagnosis and treatment of the underlying hyperaldosteronism minimize the risk of these complications.
  • Hypokalemia
    • Hypokalemia initially results in weakness, constipation, polyuria, and, if more severe, may cause cardiac arrhythmias.
    • Patients on cardiac drugs are at greater risk of this complication.
  • Adrenal venous sampling
    • Adrenal venous sampling should be performed in centers with appropriate expertise. Adrenal veins are often small, and the right vein is difficult to cannulate.
    • Performance of adrenal venography is not recommended because this may cause bleeding into the gland.
  • Specific treatment-related complications
    • The use of laparoscopic adrenalectomy considerably reduces postoperative recovery time and complication risk. The risk of operative complications is related directly to the experience of the surgeon.
    • In addition to these complications, following surgical removal of aldosteronoma, a period of hypoadrenalism can occur. If not recognized, this can result in clinically significant hyponatremia and hyperkalemia.

Prognosis

  • The age of the patient and the duration of disease before diagnosis are the 2 most important prognostic factors.
  • Adult studies have shown that hypertension is improved significantly in approximately 70% of cases (see Treatment). This figure is likely to be higher in children because disease duration is shorter and the prevalence of other causes of hypertension is lower.

Patient Education

  • Patients with mild hyperaldosteronism must learn how to avoid foods that are high in sodium because this exacerbates their hypertension and increases their tendency to develop hypokalemia.
  • Patients also need to know that medication can lead to hyperkalemia and hypotension, particularly in the presence of intercurrent illness, and they should be advised to see their pediatrician in these circumstances.

Miscellaneous

Medicolegal Pitfalls

  • Potential medicolegal problems may arise in the diagnosis and treatment of hyperaldosteronism. A delay in diagnosis of hypertension can lead to prolonged exposure to hypertension and secondary damage as well as permanent remodeling of the blood vessels.
  • The differentiation of primary hyperaldosteronism from more common secondary causes is another area where potential medicolegal problems may arise. This may arise because of failure to discontinue medications, failure to appreciate factors that may confound testing results, or failure to control blood pressure when the relevant medications are stopped.
  • Adrenal venous sampling is not without risk and can lead to damage of the adrenal if not performed correctly. Similarly, failure to cannulate the right adrenal vein can lead to an incorrect diagnosis of unilateral disease when, in fact, it is bilateral.
  • The presence of a solitary adrenal mass on CT scan in a child or young adult with hyperaldosteronism is very likely to be the cause of the hyperaldosteronism because the prevalence of nonfunctioning adrenal adenomas is very low in childhood.

Special Concerns

  • Secondary hyperaldosteronism
    • Secondary hyperaldosteronism may be due to a physiologic attempt of the organism to maintain an adequate blood volume.
    • The patient may be normotensive and have edema or may have hypertension and no edema. Secondary hyperaldosteronism may be secondary to renal ischemia.
    • Secondary hyperaldosteronism can be distinguished clinically and biochemically from primary hyperaldosteronism.
  • Syndrome of apparent mineralocorticoid excess
    • The syndrome of apparent mineralocorticoid excess is a rare cause of juvenile hypertension that was first described in 1979 with an additional 25-30 cases reported since. Patients present with severe hypokalemia and metabolic alkalosis and suppressed plasma renin activity (PRA) and aldosterone.
    • Two types of apparent mineralocorticoid excess have been described. Type I apparent mineralocorticoid excess has impaired 11bHSD activity with impaired conversion of cortisol to cortisone and impaired 5b-reductase activity. These patients have markedly elevated urinary ratio of cortisol (F), tetrahydrocortisone-F (THF), and allo-THF to cortisone (E), THE, and allo-THE. Many of these patients have molecular defects of 11bHSD type 2. Type II apparent mineralocorticoid excess is characterized by a decreased rate of cortisol clearance and turnover but a normal urinary ratio of THF to THE.
    • Treatment of apparent mineralocorticoid excess is often difficult. A low-sodium diet and spironolactone (1-4 mg/kg/d) is often effective but may not be long-lasting.
    • Patients with type II apparent mineralocorticoid excess respond to suppression of cortisol production with dexamethasone, a steroid with little mineralocorticoid activity. The problem is that dexamethasone is not suitable for growing children because of its significant growth-suppressing properties.
  • Liddle syndrome
    • Liddle syndrome is an autosomal dominant disorder that can partially mimic hyperaldosteronism. Patients present at a young age with hypertension and hypokalemia.
    • Both PRA and aldosterone levels are suppressed. It is caused by mutations of the carboxy terminus of the beta-subunits or gamma-subunits of the renal epithelial sodium channel that result in a constitutively open channel. Treatment with the potassium-sparing diuretic triamterene is often effective.
  • Glucocorticoid resistance
    • Glucocorticoid resistance is a rare disorder that has been identified in several patients or members of kindreds. When familial, it is transmitted in both an autosomal recessive and dominant fashion. Point mutations and microdeletions of the glucocorticoid receptor have been described.
    • Affected patients have an absence of cushingoid features, increased cortisol and adrenocorticotropic hormone (ACTH) levels (compensating for reduced glucocorticoid receptor function), and resistance to dexamethasone suppression of cortisol levels. The clinical manifestations are highly variable, although increased production of adrenal steroidogenic precursors, such as deoxycorticosterone and adrenal androgens (eg, delta-4-androstenedione and dehydroepiandrostenedione), can produce hypertension in both sexes and hyperandrogenism in children and women.
    • Treatment is with high-dose synthetic glucocorticoids with minimal mineralocorticoid activity, such as dexamethasone (1-3 mg/d), to suppress plasma levels of ACTH and, ultimately, the secretion of adrenal steroids with androgenic and mineralocorticoid activity.
  • Congenital adrenal hyperplasia
    • 11-beta-hydroxylase deficiency is the second most common form of congenital adrenal hyperplasia (accounting for about 5% of all cases of congenital adrenal hyperplasia), with a frequency of 1 in 100,000 live births. Because conversion of 11-deoxycortisol to cortisol and 11-deoxycorticosterone to aldosterone are both reduced, ACTH hypersecretion leads to excessive production of adrenal androgens as well as these precursors. 11-deoxycorticosterone has mineralocorticoid activity and can produce hypertension and sometimes hypokalemia.
    • The extent of virilization widely varies, ranging from newborn female infants with ambiguous genitalia to early male virilization to hirsutism and infertility in adult women.
    • The diagnosis should be considered in patients with features of hyperandrogenism and hypertension of mineralocorticoid excess type. The age at presentation correlates with the severity of the defect. Treatment in younger children is with hydrocortisone or cortisone acetate. Those who have finished growing may be treated with dexamethasone. Care must be taken; this may precipitate a salt-losing state because this synthetic steroid has no mineralocorticoid activity and suppresses levels of 11-deoxycorticosterone by inhibiting ACTH release. Patients with 11-beta-hydroxylase deficiencies who are treated with glucocorticoids may need treatment with mineralocorticoid during acute intercurrent illness.
    • Various mutations of the P-450c11 gene have been described. The diagnosis can be made on the basis of elevated levels of 11-deoxycorticosterone after ACTH stimulation, although basal levels are often diagnostic in affected neonates and infants. Treatment involves glucocorticoid replacement at physiologic doses.
    • Lyase and 17alpha-hydroxylase deficiencies are very rare. P-450c17 mutations produce a block in production of a single enzyme with both 17alpha-hydroxylase and 17,20-lyase activities (see Media file 1).

      Steroid biosynthetic pathway.

      Steroid biosynthetic pathway.

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      Steroid biosynthetic pathway.

      Steroid biosynthetic pathway.

    • Blockade of production of sex steroids can produce failure of female pubertal development and variable degrees of incomplete virilization with ambiguous genitalia in males. Deficient cortisol production results in ACTH hypersecretion with increased production of aldosterone precursors, including 11-deoxycorticosterone. Plasma renin activity and aldosterone are low. Treatment involves glucocorticoid treatment similar to 11-beta-hydroxylase. Males respond to testosterone in the neonatal period with phallic growth that may improve the outcome of corrective surgery. Both sexes also need pubertal induction.
  • Drug-induced apparent mineralocorticoid excess
    • Some drugs can cause a clinical and biochemical picture consistent with hyperaldosteronism. They include carbenoxolone, a synthetic derivative of glycyrrhizinic acid that is used to treat peptic and oral ulcers and gastroesophageal reflux. It causes fluid and sodium retention and may cause hypokalemia, headaches, and myopathy.
    • Excessive ingestion of licorice produces a picture similar to apparent mineralocorticoid excess because of its content of glycyrrhetinic acid that blocks the enzyme 11b-HSD2 at the distal tubule, allowing access of circulating glucocorticoid to the mineralocorticoid receptor.
    • Biochemically, the features of this disorder include suppression of both aldosterone and renin.
 


More on Hyperaldosteronism

Overview: Hyperaldosteronism
Differential Diagnoses & Workup: Hyperaldosteronism
Treatment & Medication: Hyperaldosteronism
Follow-up: Hyperaldosteronism
Multimedia: Hyperaldosteronism
References
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Further Reading

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Keywords

hyperaldosteronism, aldosteronism, primary aldosteronism, primary hyperaldosteronism, familial hyperaldosteronism type I, FH-I, glucocorticoid remediable aldosteronism, GRA, familial hyperaldosteronism type II, FH-II, secondary hyperaldosteronism, idiopathic hyperaldosteronism, IHA, hyperkalemia, hypertension, hypoalbuminemia, cardiac output states, nephrotic syndrome, bilateral adrenal hyperplasia, adenoma, diabetes mellitus, cardiac fibrosis, headaches, facial flushing, constipation, polyuria, polydipsia, Wilms tumor, anxiety attacks, neurofibromatosis, multiple endocrine neoplasia type 2, MEN, MEN 2A, MEN 2B, von Hippel-Lindau syndrome, Wilms tumor, hepatomegaly, splenomegaly, ascites

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Contributor Information and Disclosures

Author

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London), Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece
George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Antony Lafferty, MB ChB, FRACP, Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia
Antony Lafferty, MB ChB, FRACP is a member of the following medical societies: Endocrine Society
Disclosure: Nothing to disclose.

Medical Editor

Thomas A Wilson, MD, Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook
Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Lynne Lipton Levitsky, MD, Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor, Department of Pediatrics, Harvard University Medical School
Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds PI, also occasional consultant

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfizer, Inc. Honoraria Consulting

 
 
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