Hypoaldosteronism

Hypoaldosteronism is a hormonal disorder characterized by low levels of aldosterone. These low levels can be caused by decreased aldosterone production or a peripheral resistance to aldosterone. When hypoaldosteronism occurs as a result of an acquired decrease in renin production, the condition is more commonly referred to as renal tubular acidosis Renal Tubular Acidosis Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis ( RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis) type 4. Patients are usually older, with underlying renal disease (e.g., diabetic nephropathy). Hypoaldosteronism usually presents as hyperkalemia Hyperkalemia Hyperkalemia is defined as a serum potassium (K+) concentration >5.2 mEq/L. Homeostatic mechanisms maintain the serum K+ concentration between 3.5 and 5.2 mEq/L, despite marked variation in dietary intake. Hyperkalemia can be due to a variety of causes, which include transcellular shifts, tissue breakdown, inadequate renal excretion, and drugs. Hyperkalemia with a mild hyperchloremic metabolic acidosis Metabolic acidosis The renal system is responsible for eliminating the daily load of non-volatile acids, which is approximately 70 millimoles per day. Metabolic acidosis occurs when there is an increase in the levels of new non-volatile acids (e.g., lactic acid), renal loss of HCO3-, or ingestion of toxic alcohols. Metabolic Acidosis (normal anion gap). Most patients are asymptomatic and routine lab evaluation demonstrates hyperkalemia Hyperkalemia Hyperkalemia is defined as a serum potassium (K+) concentration >5.2 mEq/L. Homeostatic mechanisms maintain the serum K+ concentration between 3.5 and 5.2 mEq/L, despite marked variation in dietary intake. Hyperkalemia can be due to a variety of causes, which include transcellular shifts, tissue breakdown, inadequate renal excretion, and drugs. Hyperkalemia, prompting a further workup. The condition is diagnosed using serum and urine tests that demonstrate reduced aldosterone levels and a reduced transtubular potassium gradient, among other characteristic findings. Patients are managed based on their underlying etiology.

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Overview

Definition

Hypoaldosteronism is defined as decreased secretion of aldosterone from the zona glomerulosa of the adrenal cortex, which may be primary or secondary in nature.

Epidemiology

  • Exact incidence is unknown.
  • Age at diagnosis: 
    • Generally occurs in older adults (> 50 years of age)
    • May occur in children with underlying disorders (e.g., diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus, sickle cell anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview)
  • Male = female
  • Racial/ethnic bias Bias Epidemiological studies are designed to evaluate a hypothesized relationship between an exposure and an outcome; however, the existence and/or magnitude of these relationships may be erroneously affected by the design and execution of the study itself or by conscious or unconscious errors perpetrated by the investigators or the subjects. These systematic errors are called biases. Types of Biases: more common in Blacks, Native Americans, and Hispanics

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Etiology and Pathophysiology

Physiology review

  • Aldosterone is stimulated directly by:
    • Angiotensin II
    • Hyperkalemia
  • Renin-angiotensin-aldosterone system (RAAS):
    • Renin (secreted by the macula densa cells within the kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys) → converts angiotensinogen (secreted by hepatocytes) to angiotensin I
    • ACE (secreted by pulmonary vascular endothelium) → converts angiotensin I to angiotensin II
    • Angiotensin II → stimulates release of aldosterone (secreted by the zona glomerulosa in the adrenal cortex)
  • Factors that normally trigger RAAS (and thus ↑ aldosterone):
    • ↓ Blood pressure or blood volume
    • ↓ Sodium delivery to the kidney
    • ↑ Sympathetic tone
  • Effects of aldosterone: 
    • Stimulates production of the following proteins within the principle cells in the distal renal tubules:
      • Na+/K+ ATPase on the basolateral side
      • ENaC (epithelial sodium channels) on the lumen side: allows Na+ reabsorption from the lumen into the principal cells
      • ROMK (renal outer medullary potassium channel) on the lumen side: allows excretion of K+ into the urine
    • Stimulates Na+ reabsorption from the renal tubules:
      • Water follows the Na+.
      • Creates a negative electrical gradient across the lumen, promoting the secretion of K+ and H+ into the urine
Effects of aldosterone on the principal cells within the distal renal tubules

Effects of aldosterone on the principal cells within the distal renal tubules

Image by Lecturio.

Etiology

The 2 primary mechanisms that cause hypoaldosteronism are reduced aldosterone production (which can be hyporeninemic or hyperreninemic) and aldosterone resistance. Medications are often the cause of both mechanisms.

Mechanism Etiology
Reduced aldosterone synthesis due to ↓ stimulation of RAAS:
Hyporeninemic hypoaldosteronism (also referred to as type 4 renal tubular acidosis Renal Tubular Acidosis Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis ( RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis))
  • Renal disease:
    • Diabetic nephropathy (most common)
    • Chronic interstitial nephritis
    • Acute glomerulonephritis
  • Volume expansion
  • Autonomic neuropathy
  • Systemic disease:
    • Sickle cell disease Sickle cell disease Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Sickle Cell Disease
    • Systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus ( SLE SLE Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus)
    • HIV
  • NSAIDs
Reduced aldosterone synthesis due to direct adrenal issues:
Hyperreninemic hypoaldosteronism
  • Primary adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease (Addison disease)
  • Chronic heparin therapy
  • Severe illness
  • Discontinuation of mineralocorticoid drugs after prolonged use
  • Congenital or genetic anomalies:
    • Congenital adrenal hyperplasia Congenital adrenal hyperplasia Congenital adrenal hyperplasia (CAH) consists of a group of autosomal recessive disorders that cause a deficiency of an enzyme needed in cortisol, aldosterone, and androgen synthesis. The most common subform of CAH is 21-hydroxylase deficiency, followed by 11β-hydroxylase deficiency. Congenital Adrenal Hyperplasia (21-hydroxylase deficiency)
    • Isolated (primary) hypoaldosteronism (CYP11B2 defect)
    • Adrenal hypoplasia
Aldosterone resistance (psudohypoaldosteronism)
  • Genetic anomalies in the mineralocorticoid receptors
  • Obstructive uropathies
  • Calcineurin inhibitors

Pathophysiology

  • End result of ↓ aldosterone: 
    • ↑ Serum K+ (↓ urinary excretion of K+)
    • Metabolic acidosis (↓ urinary excretion of H+): renal tubular acidosis Renal Tubular Acidosis Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis type 4
    • ↓ Serum Na+ (↑ urinary excretion of Na+)
    • Volume depletion and hypotension Hypotension Hypotension is defined as low blood pressure, specifically < 90/60 mm Hg, and is most commonly a physiologic response. Hypotension may be mild, serious, or life threatening, depending on the cause. Hypotension
  • Type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis:
    • ↑ K+ → inhibits renal ammonia synthesis and urinary ammonia excretion
    • ↓ In urinary ammonia excretion → mild metabolic acidosis Metabolic acidosis The renal system is responsible for eliminating the daily load of non-volatile acids, which is approximately 70 millimoles per day. Metabolic acidosis occurs when there is an increase in the levels of new non-volatile acids (e.g., lactic acid), renal loss of HCO3-, or ingestion of toxic alcohols. Metabolic Acidosis (type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis)
  • Mechanisms of drug-induced hypoaldosteronism:
    • Drugs that cause a ↓ in aldosterone synthesis or release:
      • ACE inhibitors/ARBs
      • Heparin (direct toxic effect on the adrenal zona glomerulosa cells)
      • NSAIDs/COX-2 inhibitors
      • Beta-blockers
    • Inhibit aldosterone effects: 
      • Aldosterone receptor antagonists: spironolactone, eplerenone
      • ENaC inhibitors: amiloride, trimethoprim Trimethoprim The sulfonamides are a class of antimicrobial drugs inhibiting folic acid synthesize in pathogens. The prototypical drug in the class is sulfamethoxazole. Although not technically sulfonamides, trimethoprim, dapsone, and pyrimethamine are also important antimicrobial agents inhibiting folic acid synthesis. The agents are often combined with sulfonamides, resulting in a synergistic effect. Sulfonamides and Trimethoprim, pentamidine
    • Induce aldosterone resistance: calcineurin inhibitors cyclosporine and tacrolimus

Clinical Presentation

  • Usually asymptomatic (discovered on routine lab evaluation)
  • Hyperkalemia (> 5 mEq/L) can present with:
    • Cardiac arrhythmias
    • Muscle weakness
  • Hyponatremia Hyponatremia Hyponatremia is defined as a decreased serum sodium (sNa+) concentration less than 135 mmol/L. Serum sodium is the greatest contributor to plasma osmolality, which is very tightly controlled via antidiuretic hormone (ADH) release from the hypothalamus and by the thirst mechanism. Hyponatremia (< 135 mEq/L):
    • More common in children and patients with adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease
    • Can present with: 
      • Polyuria
      • Dehydration
      • Nausea and vomiting
      • Neurologic symptoms: short-term memory loss, confusion, lethargy, seizures Seizures A seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized. Seizures
  • Clinical features suggestive of associated conditions (e.g., sickle cell anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview, SLE SLE Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus, adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease)

Diagnosis

The diagnosis of hypoaldosteronism should be considered in any patient with persistent hyperkalemia Hyperkalemia Hyperkalemia is defined as a serum potassium (K+) concentration >5.2 mEq/L. Homeostatic mechanisms maintain the serum K+ concentration between 3.5 and 5.2 mEq/L, despite marked variation in dietary intake. Hyperkalemia can be due to a variety of causes, which include transcellular shifts, tissue breakdown, inadequate renal excretion, and drugs. Hyperkalemia without an obvious cause, such as renal failure or potassium-sparing diuretics Potassium-sparing diuretics Potassium-sparing diuretics are medications that act in the principal cells in the collecting ducts to induce diuresis that does not result in excretion of potassium. These diuretics include 2 subclasses: sodium channel blockers and aldosterone antagonists. Potassium-sparing Diuretics.

  • Aldosterone-specific tests:
    • Serum aldosterone:
      • ↓ In all cases of true hypoaldosteronism
      • ↑ In cases of pseudohypoaldosteronism (aldosterone resistance)
    • Plasma renin activity (PRA):
      • In type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis (hyporeninemic hypoaldosteronism)
      • ↑ In primary adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease
    • Transtubular potassium gradient (TTKG): estimates the action of aldosterone on cortical collecting tubules
      • TTKG = [urine K ÷ (urine osmolality/serum osmolality)] ÷ serum K
      • ↓ TTKG (< 7) in the presence of hyperkalemia Hyperkalemia Hyperkalemia is defined as a serum potassium (K+) concentration >5.2 mEq/L. Homeostatic mechanisms maintain the serum K+ concentration between 3.5 and 5.2 mEq/L, despite marked variation in dietary intake. Hyperkalemia can be due to a variety of causes, which include transcellular shifts, tissue breakdown, inadequate renal excretion, and drugs. Hyperkalemia: Hypoaldosteronism is likely.
    • Serum cortisol: to screen for adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease
      • ↓ In adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease
      • Normal in isolated hypoaldosteronism
  • In type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis (hyporeninemic hypoaldosteronism), laboratory tests will show:
    • Aldosterone-specific test findings:
      • ↓ Serum aldosterone
      • ↓ PRA
      • ↓ TTKG
    • Basic metabolic panel (BMP) findings:
      • Non-anion gap metabolic acidosis Metabolic acidosis The renal system is responsible for eliminating the daily load of non-volatile acids, which is approximately 70 millimoles per day. Metabolic acidosis occurs when there is an increase in the levels of new non-volatile acids (e.g., lactic acid), renal loss of HCO3-, or ingestion of toxic alcohols. Metabolic Acidosis
      • ↓ Serum HCO3 (usually mild, 18–22 mEq/L)
      • ↑ K+
      • ↑ Cl
      • Markers of ↓ renal function are common (e.g., ↑ creatinine)
    • Urine findings:
      • ↓ Urinary K+
      • Normal urinary phosphate
      • Normal or ↓ urinary calcium
      • Urine pH < 5.3‒5.5
  • Electrocardiogram Electrocardiogram An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Normal Electrocardiogram (ECG) (due to ↑ cardiac risks associated with hyperkalemia Hyperkalemia Hyperkalemia is defined as a serum potassium (K+) concentration >5.2 mEq/L. Homeostatic mechanisms maintain the serum K+ concentration between 3.5 and 5.2 mEq/L, despite marked variation in dietary intake. Hyperkalemia can be due to a variety of causes, which include transcellular shifts, tissue breakdown, inadequate renal excretion, and drugs. Hyperkalemia)
  • Testing to rule out other associated conditions, if suspected:
    • 17-hydroxyprogesterone (congenital adrenal hyperplasia)
    • HIV test

Management

The management depends on the underlying etiology.

  • Medication induced: Adjust/remove medications that may be contributing.
  • Type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis (hyporeninemic hypoaldosteronism):
    • Low-potassium diet
    • Loop or thiazide diuretics Thiazide diuretics Thiazide and thiazide-like diuretics make up a group of highly important antihypertensive agents, with some drugs being 1st-line agents. The class includes hydrochlorothiazide, chlorothiazide, chlorthalidone, indapamide, and metolazone. Thiazide Diuretics
    • Alkali supplementation (NaHCO3) if acidosis does not improve
    • Mineralocorticoid replacement is not recommended, as many patients have underlying conditions (e.g., edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema, hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension) that would be exacerbated.
  • Primary adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease and congenital adrenal hyperplasia:
    • Mineralocorticoid replacement: fludrocortisone
    • Glucocorticoid replacement: hydrocortisone or prednisone
  • Hyperkalemia with ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Normal Electrocardiogram (ECG) changes: emergency management to ↓ K+

Clinical Relevance

  • Congenital adrenal hyperplasia Congenital adrenal hyperplasia Congenital adrenal hyperplasia (CAH) consists of a group of autosomal recessive disorders that cause a deficiency of an enzyme needed in cortisol, aldosterone, and androgen synthesis. The most common subform of CAH is 21-hydroxylase deficiency, followed by 11β-hydroxylase deficiency. Congenital Adrenal Hyperplasia (CAH): consists of a group of autosomal recessive Autosomal recessive Autosomal inheritance, both dominant and recessive, refers to the transmission of genes from the 22 autosomal chromosomes. Autosomal recessive diseases are only expressed when 2 copies of the recessive allele are inherited. Autosomal Recessive and Autosomal Dominant Inheritancedisorders involving a genetic mutation Mutation Genetic mutations are errors in DNA that can cause protein misfolding and dysfunction. There are various types of mutations, including chromosomal, point, frameshift, and expansion mutations. Types of Mutations that results in an enzyme deficiency required for cortisol and/or aldosterone synthesis.
  • Primary Addison disease: also called primary adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease. Defined as the inadequate production of adrenocortical hormones Hormones Hormones are messenger molecules that are synthesized in one part of the body and move through the bloodstream to exert specific regulatory effects on another part of the body. Hormones play critical roles in coordinating cellular activities throughout the body in response to the constant changes in both the internal and external environments. Hormones: Overview, including glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids, mineralocorticoids Mineralocorticoids Mineralocorticoids are a drug class within the corticosteroid family and fludrocortisone is the primary medication within this class. Fludrocortisone is a fluorinated analog of cortisone. The fluorine moiety protects the drug from isoenzyme inactivation in the kidney, allowing it to exert its mineralocorticoid effect. Mineralocorticoids, and adrenal androgens Androgens Androgens are naturally occurring steroid hormones responsible for development and maintenance of the male sex characteristics, including penile, scrotal, and clitoral growth, development of sexual hair, deepening of the voice, and musculoskeletal growth. Androgens and Antiandrogens. Primary adrenal insufficiency Adrenal Insufficiency Adrenal insufficiency (AI) is the inadequate production of adrenocortical hormones: glucocorticoids, mineralocorticoids, and adrenal androgens. Primary AI, also called Addison’s disease, is caused by autoimmune disease, infections, and malignancy, among others. Adrenal insufficiency can also occur because of decreased production of adrenocorticotropic hormone (ACTH) from disease in the pituitary gland (secondary) or hypothalamic disorders and prolonged glucocorticoid therapy (tertiary). Adrenal Insufficiency and Addison’s Disease is caused by afflictions in the gland itself. Classic findings include mucosal and cutaneous hyperpigmentation, hypotension Hypotension Hypotension is defined as low blood pressure, specifically < 90/60 mm Hg, and is most commonly a physiologic response. Hypotension may be mild, serious, or life threatening, depending on the cause. Hypotension, and GI symptoms.
  • Diabetic nephropathy: Diabetes mellitus describes a group of metabolic diseases that are characterized by chronic hyperglycemia and insulin Insulin Insulin is a peptide hormone that is produced by the beta cells of the pancreas. Insulin plays a role in metabolic functions such as glucose uptake, glycolysis, glycogenesis, lipogenesis, and protein synthesis. Exogenous insulin may be needed for individuals with diabetes mellitus, in whom there is a deficiency in endogenous insulin or increased insulin resistance. Insulin resistance. Long-standing diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus causes vascular changes that eventually result in cardiovascular, renal, retinal, and neurological complications.
  • Hyperkalemia: defined as an elevated serum potassium level (> approximately 5.5mEq/L). The condition is usually an incidental finding in the general population but affects 10% of hospitalized patients. There are many causes, including transcellular shifts, tissue breakdown, inadequate excretion, and drug induced. Patients are usually asymptomatic.
  • Hyponatremia Hyponatremia Hyponatremia is defined as a decreased serum sodium (sNa+) concentration less than 135 mmol/L. Serum sodium is the greatest contributor to plasma osmolality, which is very tightly controlled via antidiuretic hormone (ADH) release from the hypothalamus and by the thirst mechanism. Hyponatremia: defined as a decreased plasma sodium concentration (> approximately 135 mEq/L). This condition occurs from either loss of sodium or excess water in the extracellular fluid.
  • RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis: an accumulation of acid in the body due to a failure of the kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys to appropriately acidify the urine. The condition is classified into 4 types.

References

  1. Williams, G. H., Dluhy, R.G. (2008). Disorders of the adrenal cortex. In Fauci, A. S., Braunwald, E., Kasper, D.L., et al. (Eds.). Harrison’s Internal Medicine. 17th Ed., p. 2266.
  2. Rajkumar, V. (2021). Hypoaldosteronism. In Waseem, M. (Ed.). StatPearls. Retrieved March 10, 2021, from https://www.statpearls.com/ArticleLibrary/viewarticle/23254
  3. Sondheimer, J. H. (2020). Hyporeninemic hypoaldosteronism. In Batuman, V. (Ed.). Medscape. Retrieved March 10, 2021, from https://emedicine.medscape.com/article/242494-overview
  4. Young, W. F. (2019). Etiology, diagnosis, and treatment of hypoaldosteronism (type 4 RTA RTA Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis). In Forman, J. P. (Ed.). UpToDate. Retrieved March 10, 2021, from https://www.uptodate.com/contents/etiology-diagnosis-and-treatment-of-hypoaldosteronism-type-4-rta 

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