DiseasesChemicals and Drugs
HypoaldosteronismHyperkalemiaFludrocortisoneAldosteroneRenin
Hypoaldosteronism
A congenital or acquired condition of insufficient production of ALDOSTERONE by the ADRENAL CORTEX leading to diminished aldosterone-mediated synthesis of Na(+)-K(+)-EXCHANGING ATPASE in renal tubular cells. Clinical symptoms include HYPERKALEMIA, sodium-wasting, HYPOTENSION, and sometimes metabolic ACIDOSIS.
Hyperkalemia
Abnormally high potassium concentration in the blood, most often due to defective renal excretion. It is characterized clinically by electrocardiographic abnormalities (elevated T waves and depressed P waves, and eventually by atrial asystole). In severe cases, weakness and flaccid paralysis may occur. (Dorland, 27th ed)
Fludrocortisone
A synthetic mineralocorticoid with anti-inflammatory activity.
Aldosterone
A hormone secreted by the ADRENAL CORTEX that regulates electrolyte and water balance by increasing the renal retention of sodium and the excretion of potassium.
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.

Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy. (1/22)

Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.  (+info)

Functional expression of a pseudohypoaldosteronism type I mutated epithelial Na+ channel lacking the pore-forming region of its alpha subunit. (2/22)

The autosomal recessive form of type I pseudohypoaldosteronism (PHA-I) is an inherited salt-losing syndrome resulting from diminution-of-function mutations in the 3 subunits of the epithelial Na+ channel (ENaC). A PHA-I stop mutation (alpha(R508stop)) of the ENaC alpha subunit is predicted to lack the second transmembrane domain and the intracellular COOH-terminus, regions of the protein involved in pore function. Nonetheless, we observed a measurable Na+ current in Xenopus laevis oocytes that coexpress the beta and gamma subunits with the truncated alpha subunit. The mutant alpha was coassembled with beta and gamma subunits and was present at the cell surface at a lower density, consistent with the lower Na+ current seen in oocytes with the truncated alpha subunit. The single-channel Na+ conductance for the mutant channel was only slightly decreased, and the appearance of the macroscopic currents was delayed by 48 hours with respect to wild-type. Our data suggest novel roles for the alpha subunit in the assembly and targeting of an active channel to the cell surface, and suggest that channel pores consisting of only the beta and gamma subunits can provide significant residual activity. This activity may be sufficient to explain the absence of a severe pulmonary phenotype in patients with PHA-I.  (+info)

Prerenal azotemia in a diabetic patient with hyporeninemic hypoaldosteronism and autonomic neuropathy. (3/22)

Patients with hyporeninemic hypoaldosteronism show mild to moderate renal insufficiency, with a creatinine clearance of 20-75 ml/min, and asymptomatic hyperkalemia. A low degree of sodium wasting and mild hyperchloremic metabolic acidosis are also usually present. However, severe sodium wasting and volume depletion are not typically seen unless the patient is placed on severe sodium restriction or has some other cause of extrarenal sodium loss. In fact, acute renal failure has not been reported in such patients. We describe a diabetic patient with hyporeninemic hypoaldosteronism and autonomic neuropathy who developed recurrent episodes of acute renal failure due to prerenal azotemia during acute exacerbations of diarrhoea. In our case, despite significant hypovolemia, the renin-aldosterone axis was markedly suppressed, implying that sympathetic tone played a decisive role in renin regulation.  (+info)

Isolated adrenocorticotropin deficiency presenting with impaired renin-angiotensin-aldosterone system and suppressed parathyroid hormone-vitamin D axis. (4/22)

We report here a 47-year-old woman with isolated adrenocorticotropin (ACTH) deficiency (IAD). She presented impaired renin-angiotensin-aldosterone (R-A-A) system and suppressed parathyroid hormone (PTH)-vitamin D system. She showed severe hyponatremia due to secondary adrenocortical insufficiency, which was deteriorated by hypoaldosteronism. She also showed hyperphosphatemia and relative hypercalcemia with suppressed PTH-vitamin D axis. Moreover, she showed hypothyroidism, which was thought to be important to maintain normal Ca levels under secondary hypoadrenalism via decrease in bone resorption by T3. Replacement with glucocorticoid completely normalized PTH-vitamin D axis and R-A-A system. Thus, the present case implicates that severe adrenocortical deficiency due to IAD might affect both R-A-A system and PTH-vitamin D axis. These findings suggest that the ACTH-cortisol axis has an important role in mineral metabolism in vivo.  (+info)

Pseudo-hypo aldosteronism Type II. (5/22)

A 50-day-old infant diagnosed as meningitis had persistently elevated serum potassium, low serum bicarbonate and normal serum sodium. She had metabolic acidosis with low TTKG, low serum renin and low normal serum aldosterone with no renal failure or extra renal causes of hyperkalemia. Hence a diagnosis of Type II pseudo-hypoaldosteronism was made. She was started on oral thiazide following which her serum electrolytes normalized.  (+info)

Treating proteinuria in a diabetic patient despite hyperkalaemia due to hyporeninaemic hypoaldosteronism. (6/22)

Diabetes mellitus is a common cause of hyporeninaemic hypoaldosteronism that might result in significant hyperkalaemia. We describe a patient with diabetic nephropathy and proteinuria who developed a remarkable hyperkalaemia on treatment with an angiotensin-receptor blocker. The management of hyperkalaemia and the pathophysiological background of hyporeninaemic hypoaldosteronism are discussed.  (+info)

Successful treatment of chyluria, glomerular and tubular abnormalities in a young lady with silver nitrate instillation. (7/22)

A 21- year-old lady presented with chyluria, severe malnutrition, secondary amenorrhea, profound hypoalbuminemia, heavy proteinuria and renal tubular abnormalities suggestive of Type IV renal tubular acidosis. No particular cause for chyluria could be ascertained. She was successfully treated with an injection of 2% silver nitrate into the left ureter and urinary bladder. She continues to be well after 15 years of follow-up.  (+info)

Pseudohypoaldosteronism: case report and discussion of the syndrome. (8/22)

A 41-year-old man, complaining of leg cramps, was found to have persistent hyperkalemia. Except for mild hypertension, his physical examination and laboratory values to exclude connective tissue diseases and diabetes mellitus were normal. Renal function testing revealed a normal glomerular filtration rate and tubular capacity to acidify and dilute, as well as near-normal ability to concentrate his urine. Hormonal evaluation revealed a normal cortisol, as well as normal resting and stimulated renin and aldosterone levels. A selective defect in tubular potassium secretion was demonstrated. In the absence of aldosterone deficiency or renal dysfunction, it was assumed that the patient had primary renal resistance to aldosterone, known as pseudohypoaldosteronism. Treatment with hydrochlorothiazide controlled his hyperkalemia and hypertension. His case emphasizes the diagnostic and therapeutic factors that should be considered in evaluating and treating a non-hospitalized patient with sustained hyperkalemia.  (+info)

Hypoaldosteronism is a condition characterized by decreased levels of the hormone aldosterone, leading to sodium depletion, potassium retention, and either normal or low blood pressure.

Hypoaldosteronism is a medical condition characterized by decreased levels or impaired function of the hormone aldosterone, which is produced by the adrenal gland. Aldosterone plays a crucial role in regulating electrolyte and fluid balance in the body by increasing the reabsorption of sodium and excretion of potassium in the kidneys.

Hypoaldosteronism can lead to low blood pressure, muscle weakness, and an imbalance of electrolytes, particularly low serum sodium levels and high serum potassium levels. This condition can be caused by various factors, including damage to the adrenal gland, impaired production or function of aldosterone, or decreased responsiveness of the kidneys to aldosterone.

Hypoaldosteronism can be primary or secondary. Primary hypoaldosteronism is caused by a problem with the adrenal glands themselves, such as damage to the gland or a genetic disorder that affects aldosterone production. Secondary hypoaldosteronism is caused by a problem outside of the adrenal glands, such as decreased production of renin (an enzyme produced by the kidneys) or certain medications that interfere with aldosterone production or function.

Treatment for hypoaldosteronism depends on the underlying cause and may include medication to replace missing aldosterone or correct electrolyte imbalances, as well as addressing any underlying conditions contributing to the development of the condition.

Hyperkalemia is a medical condition characterized by an abnormally elevated level of potassium (>5.5 mEq/L) in the blood serum, which can disrupt the normal functioning of the heart and other muscles, and may pose serious health risks if not promptly addressed.

Hyperkalemia is a medical condition characterized by an elevated level of potassium (K+) in the blood serum, specifically when the concentration exceeds 5.0-5.5 mEq/L (milliequivalents per liter). Potassium is a crucial intracellular ion that plays a significant role in various physiological processes, including nerve impulse transmission, muscle contraction, and heart rhythm regulation.

Mild to moderate hyperkalemia might not cause noticeable symptoms but can still have harmful effects on the body, particularly on the cardiovascular system. Severe cases of hyperkalemia (potassium levels > 6.5 mEq/L) can lead to potentially life-threatening arrhythmias and heart failure.

Hyperkalemia may result from various factors, such as kidney dysfunction, hormonal imbalances, medication side effects, trauma, or excessive potassium intake. Prompt identification and management of hyperkalemia are essential to prevent severe complications and ensure proper treatment.

Fludrocortisone is a synthetic mineralocorticoid hormone (fluorinated cortisol derivative) used primarily to treat adrenogenital syndrome, Addison's disease, and orthostatic hypotension, by its action in promoting sodium retention and increasing potassium excretion by the kidneys.

Fludrocortisone is a synthetic corticosteroid hormone, specifically a mineralocorticoid. It is often used to treat conditions associated with low levels of corticosteroids, such as Addison's disease. It works by helping the body retain sodium and lose potassium, which helps to maintain fluid balance and blood pressure.

In medical terms, fludrocortisone is defined as a synthetic mineralocorticoid with glucocorticoid activity used in the treatment of adrenogenital syndrome and Addison's disease, and as an adjunct in the treatment of rheumatoid arthritis. It is also used to treat orthostatic hypotension by helping the body retain sodium and water, thereby increasing blood volume and blood pressure.

It is important to note that fludrocortisone can have significant side effects, particularly if used in high doses or for long periods of time. These can include fluid retention, high blood pressure, increased risk of infection, and slowed growth in children. As with any medication, it should be used under the close supervision of a healthcare provider.

Aldosterone is a steroid hormone produced by the adrenal gland's outer zone (zona glomerulosa) that plays a crucial role in regulating electrolyte and fluid balance primarily through promoting sodium reabsorption and potassium excretion in the distal nephron of the kidney, thereby having significant impacts on blood pressure homeostasis.

Aldosterone is a hormone produced by the adrenal gland. It plays a key role in regulating sodium and potassium balance and maintaining blood pressure through its effects on the kidneys. Aldosterone promotes the reabsorption of sodium ions and the excretion of potassium ions in the distal tubules and collecting ducts of the nephrons in the kidneys. This increases the osmotic pressure in the blood, which in turn leads to water retention and an increase in blood volume and blood pressure.

Aldosterone is released from the adrenal gland in response to a variety of stimuli, including angiotensin II (a peptide hormone produced as part of the renin-angiotensin-aldosterone system), potassium ions, and adrenocorticotropic hormone (ACTH) from the pituitary gland. The production of aldosterone is regulated by a negative feedback mechanism involving sodium levels in the blood. High sodium levels inhibit the release of aldosterone, while low sodium levels stimulate its release.

In addition to its role in maintaining fluid and electrolyte balance and blood pressure, aldosterone has been implicated in various pathological conditions, including hypertension, heart failure, and primary hyperaldosteronism (a condition characterized by excessive production of aldosterone).

Renin is an enzyme generated primarily by the juxtaglomerular cells of the kidney that participates in the regulation of blood pressure and electrolyte balance through its role in the renin-angiotensin-aldosterone system (RAAS) by cleaving angiotensinogen to form angiotensin I.

Renin is a medically recognized term and it is defined as:

"A protein (enzyme) that is produced and released by specialized cells (juxtaglomerular cells) in the kidney. Renin is a key component of the renin-angiotensin-aldosterone system (RAAS), which helps regulate blood pressure and fluid balance in the body.

When the kidney detects a decrease in blood pressure or a reduction in sodium levels, it releases renin into the bloodstream. Renin then acts on a protein called angiotensinogen, converting it to angiotensin I. Angiotensin-converting enzyme (ACE) subsequently converts angiotensin I to angiotensin II, which is a potent vasoconstrictor that narrows blood vessels and increases blood pressure.

Additionally, angiotensin II stimulates the adrenal glands to release aldosterone, a hormone that promotes sodium reabsorption in the kidneys and increases water retention, further raising blood pressure.

Therefore, renin plays a critical role in maintaining proper blood pressure and electrolyte balance in the body."

HyporeninemicDeficiencyAldosteroneHyperkalemiaMineralocorticoidHyporeninaemicDiagnosisHyporeninemic hypoaldosteronismPrimary hypoaldosteronismCongenitalAldosterone levelsEndocrinologicalFamilialFludrocortisoneKidneysCortisolSymptomsSecondaryIncludeClinicalConditions
Hyporeninemic7
  • citation needed] Hyporeninemic hypoaldosteronism is amenable to fludrocortisone treatment, but the accompanying hypertension and edema can prove a problem in these patients, so often a diuretic (such as the thiazide diuretic, bendrofluazide, or a loop diuretic, such as furosemide) is used to control the hyperkalemia. (wikipedia.org)
  • This article reviews some of the pathophysiologic aspects and the clinical picture of, as well as treatment strategies for, hyporeninemic hypoaldosteronism, a condition in which hyperkalemia results from a deficiency of renin and aldosterone. (medscape.com)
  • Strictly speaking, the term hyporeninemic hypoaldosteronism should be limited to cases in which testing reveals the cause of hyperkalemia to be, as stated above, a deficiency of renin and aldosterone. (medscape.com)
  • Rrbp1 - knockout (KO) mice had lower blood pressure and were more likely to die suddenly from severe hyperkalemia caused by phenotypically hyporeninemic hypoaldosteronism than wild-type controls. (biomedcentral.com)
  • RRBP1 deficiency in mice caused hyporeninemic hypoaldosteronism, resulting in lower blood pressure, severe hyperkalemia, and sudden cardiac death. (biomedcentral.com)
  • Consistently, we found that Rrbp1 - knockout (KO) mice had lower blood pressure and hyporeninemic hypoaldosteronism, which cause severe hyperkalemic cardiac arrhythmia-induced sudden death. (biomedcentral.com)
  • These results suggest (1) the renin-aldosterone system generally responds normally in diabetics without nephropathy but responds subnormally when nephropathy is present, (2) hyporeninemic hypoaldosteronism is frequent in diabetics with nephropathy but may occur in the absence of clinical nephropathy, and (3) hyperkalemia in some diabetic patients may be secondary to hypoaldosteronemia and hyperglycemia. (diabetesjournals.org)
Deficiency3
  • The causes of hypoaldosteronism include both acquired (secondary mineralocorticoid deficiency) and, less often, inherited disorders (primary mineralocorticoid deficiency), which can affect adrenal aldosterone synthesis or renal (and maybe adrenal) renin release. (studybuff.com)
  • Hypoaldosteronism in three sibs due to 18-dehydrogenase deficiency. (bmj.com)
  • Dr. Michael Kaback and Dr. David Rimoin, who developed the West Coast study, included HIBIM as well as three diseases common to the Persian community, which are relatively easy to treat: pseudocholinesterase deficiency (anesthesia sensitivity) congenital hypoaldosteronism (salt losing disorder), and polyglandular deficiency (multiple hormone deficiency). (jta.org)
Aldosterone6
  • Hypoaldosteronism is an endocrinological disorder characterized by decreased levels of the hormone aldosterone. (wikipedia.org)
  • Similarly, isolated hypoaldosteronism is the condition of having lowered aldosterone without corresponding changes in cortisol. (wikipedia.org)
  • Low aldosterone levels in the presence of high renin activity, often with low sodium, high potassium, is associated with primary hypoaldosteronism. (wikipedia.org)
  • The plasma aldosterone-to-renin ratio is calculated to determine if levels are sufficiently deranged to consider a diagnosis of hypoaldosteronism. (wikipedia.org)
  • Hypoaldosteronism can occur as part of the presentation of Addison's disease, resulting from destruction of both adrenal glands or due to selective injury to cells producing aldosterone. (studybuff.com)
  • Pseudohypoaldosteronism type I resembles other forms of hypoaldosteronism except that aldosterone levels are high. (msdmanuals.com)
Hyperkalemia2
  • Hypoaldosteronism causes low sodium (hyponatremia), high potassium (hyperkalemia), and metabolic acidosis, a condition in which the body produces excess acid. (wikipedia.org)
  • The survival of Rrbp1 -KO mice significantly decreased under high potassium intake due to lethal hyperkalemia-induced arrhythmia and persistent hypoaldosteronism, which could be rescued by fludrocortisone. (biomedcentral.com)
Mineralocorticoid1
  • The syndrome of apparent mineralocorticoid excess (AME) is an autosomal recessive disorder characterized by hypertension, hypokalemia, low renin, and hypoaldosteronism. (richardvigilantebooks.com)
Hyporeninaemic2
Diagnosis2
  • citation needed] Patients with a suspected diagnosis of hypoaldosteronism are often screened with simple blood tests. (wikipedia.org)
  • 1 Diagnosis of, and differentiation between, primary and secondary causes of hyper- or hypoaldosteronism. (sas-centre.org)
Hyporeninemic hypoaldosteronism3
  • citation needed] Hyporeninemic hypoaldosteronism is amenable to fludrocortisone treatment, but the accompanying hypertension and edema can prove a problem in these patients, so often a diuretic (such as the thiazide diuretic, bendrofluazide, or a loop diuretic, such as furosemide) is used to control the hyperkalemia. (wikipedia.org)
  • Hyporeninemic hypoaldosteronism and diabetes mellitus: Pathophysiology assumptions, clinical aspects and implications for management. (medscape.com)
  • Morbidity and mortality rates associated with hyporeninemic hypoaldosteronism. (albionfoundation.org)
Primary hypoaldosteronism1
  • Low aldosterone levels in the presence of high renin activity, often with low sodium, high potassium, is associated with primary hypoaldosteronism. (wikipedia.org)
Congenital1
  • There are rare forms of congenital Hypoaldosteronism that can be inherited in families. (nih.gov)
Aldosterone levels1
  • Pseudohypoaldosteronism type I resembles other forms of hypoaldosteronism except that aldosterone levels are high. (msdmanuals.com)
Endocrinological1
  • Hypoaldosteronism is an endocrinological disorder characterized by decreased levels of the hormone aldosterone. (wikipedia.org)
Familial2
  • A rare type of familial hypoaldosteronism characterized by early infantile onset of vomiting diarrhea severe dehydration and failure to thrive. (globalgenes.org)
  • Newly diagnosed with Early-onset familial hypoaldosteronism? (globalgenes.org)
Fludrocortisone1
  • No CYP 21 mutation was identified, fludrocortisone was continued for presumed hypoaldosteronism, hydrocortisone. (eurospe.org)
Kidneys1
  • Hypoaldosteronism may be described as hyporeninemic (low renin level) or hyperreninemic (high renin level) based on the amount of another chemical produced in the kidneys called renin. (nih.gov)
Cortisol2
  • This condition is diagnosed based on the symptoms and confirmed by various blood tests (plasma renin activity, serum aldosterone, and serum cortisol) The exact incidence of Hypoaldosteronism is unknown. (nih.gov)
  • Similarly, isolated hypoaldosteronism is the condition of having lowered aldosterone without corresponding changes in cortisol. (wikipedia.org)
Symptoms1
  • When Do Symptoms of Hypoaldosteronism Begin? (nih.gov)
Secondary1
  • Diagnosis of, and differentiation between, primary and secondary causes of hyper- or hypoaldosteronism, management of renal artery stenosis, diagnosis and location of renin secreting tumours & monitoring mineralocorticoid replacement therapy. (southtees.nhs.uk)
Include1
  • Associated with complications include hyporeninaemic hypoaldosteronism. (shilpaotc.com)
Clinical1
  • https://en.wikipedia.org/w/index.php?title=Clinical_urine_tests&oldid=990832435, Creative Commons Attribution-ShareAlike License, The presence of nitrites in urine, termed, A urinalysis is frequently ordered during the workup of. (ryosiri.com)
Conditions1
  • Hypoaldosteronism can be caused by other health conditions or medications. (nih.gov)