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Hypoaldosteronism

Hypoaldosteronism is a rare condition caused by the deficiency of the hormone aldosterone. It can either be congenital or acquired which, in turn, can be primary or secondary and can be associated with elevated or diminished levels of renin. The disorder is characterized by hyperkalemia and metabolic acidosis. Diagnosis is based on laboratory tests.


Presentation

Hypoaldosteronism is a rare disorder which is usually asymptomatic. It can either be congenital/primary [1] or can be secondary to renal dysfunction, multiple myeloma, diabetes, drugs like angiotensin-converting enzyme (ACE) inhibitors, debilitating diseases, lead toxicity and enzyme defects in aldosterone synthesis [2].

Patients with mild hyperkalemia are often asymptomatic but those with severe hyperkalemia may present with fatigue, asthenia, nausea, vomiting, dyspnea (Kussmaul respiration), palpitations, angina, myopathies, flaccid paralysis, paralytic ileus, and cardiac arrhythmias [3]. Clinical features of underlying conditions like edema or pathological fractures or autoimmune disorders can also be noticed.

Congenital hypoaldosteronism is due to the deficiency of the enzyme aldosterone synthase and it is inherited in an autosomal recessive pattern. Two types of biochemically different aldosterone deficiencies have been reported: corticosterone methyl oxidase (CMO) deficiency type I and type II [4]. Clinically both the disorders present with failure to thrive, growth restriction and depletion of sodium with elevated levels of serum potassium [5].

Fracture
  • Clinical features of underlying conditions like edema or pathological fractures or autoimmune disorders can also be noticed.[symptoma.com]

Workup

Hypoaldosteronism is often diagnosed during laboratory tests but it should be suspected in patients with end-stage or chronic renal disease. Physicians should inquire about the chronic use of analgesics or ACE inhibitors, occupational or recreational exposure to either lead or moonshine (alcohol) respectively, obstructive uropathy, and an abnormal appetite (pica) for paint/mud. History of diseases like diabetes or autoimmune disorders like systemic lupus erythematosus (SLE) should also be elicited. Physical examination may reveal non-specific findings such as hypertension and rarely Kussmaul type of respiration can be noted.

Laboratory tests are ordered based on history and physical examination focusing on complete blood count, platelet count, serum electrolytes, blood sugar levels, renal function tests, urinalysis and serology tests to determine underlying renal disorders like SLE, multiple myeloma, hepatitis and human immunodeficiency virus (HIV) infection. Fractional excretion of potassium and the gradient of transtubular potassium is calculated from a potassium, creatinine, and urine osmolar spot test. Extremely low levels in the presence of hyperkalemia are suggestive of hypoaldosteronism. Urinary pH analysis helps to differentiate between type I and type IV renal tubular acidosis and a cosyntropin test is indicated in cases suspected to have adrenal insufficiency. If hyporeninemic hypoaldosteronism is suspected then a trial of oral fludrocortisone is given to confirm the diagnosis. The potassium levels normalize within two days of administration of fludrocortisone in patients with hyporeninemic hypoaldosteronism [6].

An electrocardiogram is obtained in all patients with hyperkalemia and may show the classical features of a tall peaked T wave, absent P wave with wide QRS complexes [7] and ventricular arrhythmias.

Patients with suspected obstructive uropathy should undergo ultrasonography and a renal biopsy may be required for definitive diagnosis in patients with an early-stage chronic renal disease.

Hyperreninemia
  • Laboratory data revealed selective aldosterone deficiency with hyperreninemia. Biopsy documented replacement of the adrenal glands with metastatic hepatocellular carcinoma.[ncbi.nlm.nih.gov]
  • Case Report Abstract Author Information We report a child with primary renal tubular alkalosis who had hypokalemia, profound hyperreninemia, and hypoaldosteronism. A 14-year-old girl presented with short stature and delayed puberty.[journals.lww.com]
Potassium Increased
  • On the other hand, rapid ACTH administration in the presence of 4.4 mEq/l of serum potassium increased both plasma aldosterone and cortisol.[ncbi.nlm.nih.gov]
Wide QRS Complex
  • An electrocardiogram is obtained in all patients with hyperkalemia and may show the classical features of a tall peaked T wave, absent P wave with wide QRS complexes and ventricular arrhythmias.[symptoma.com]
Absent A-Waves
  • An electrocardiogram is obtained in all patients with hyperkalemia and may show the classical features of a tall peaked T wave, absent P wave with wide QRS complexes and ventricular arrhythmias.[symptoma.com]
Hepatocellular Carcinoma
  • A case of hyperreninemic hypoaldosteronism secondary to metastatic hepatocellular carcinoma is reported. Laboratory data revealed selective aldosterone deficiency with hyperreninemia.[ncbi.nlm.nih.gov]

Treatment

  • Abstract Two patients with insulin-dependent diabetes mellitus (Type I), developed severe, life-threatening hyperkalaemia, the first following treatment with spironolactone, the second during treatment for staphylococcal septicaemia when glucose-induced[ncbi.nlm.nih.gov]
  • Rats survive indefinitely on extra salt alone, but in dogs and most humans, the amount of supplementary salt needed is so large that it is almost impossible to prevent eventual collapse and death unless mineralocorticoid treatment is also instituted.[en.wikipedia.org]

Prognosis

  • Hyperreninemic hypoaldosteronism syndrome was related to a greater pro-inflammatory status and degree of acute organ failure, and was independently associated with a worse prognosis.[ncbi.nlm.nih.gov]
  • Hyperaldosteronism Prognosis With early diagnosis and cure, the prognosis for Primary Hyperaldosteronism is found to be quite good. In case of Secondary Hyperaldosteronism, the outcome depends on the underlying cause of the disease.[hxbenefit.com]
  • This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional.[hubpages.com]
  • […] filtration rate may also contribute to the development of hyperkalemia Intrinsic Renal Disease Acute Glomerulonephritis with Volume Expansion (see Acute Glomerulonephritis, [[Acute Glomerulonephritis]]) Treatment: responds to mineralocorticoid replacement Prognosis[mdnxs.com]

Etiology

  • The objective of this review is to highlight what is known about the epidemiology, pathophysiology, etiology, presentation, diagnosis, and treatment of SH.[ncbi.nlm.nih.gov]
  • Etiology It is due to mutations of the /CYP11B2/ (aldosterone synthase) gene. Management and treatment Increased sodium intake and mineralocorticoid supplementation provides effective treatment.[orpha.net]
  • (See "Etiology and treatment of hypoaldosteronism (type 4 RTA)", section on Congenital adrenal hyperplasia and isolated hypoaldosteronism, which reviews the biosynthetic defects in this disorder).[enotes.tripod.com]
  • Etiology Decreased Aldosterone Synthesis Inherited Disorders Congenital Isolated Hypoaldosteronism 21 Hydroxylase Deficiency Other Defects Pseudohypoaldosteronism Type 2 (Gordon’s Syndrome) Physiology : defects in WKNK1 or WNK4 kinases Clinical Familial[mdnxs.com]

Epidemiology

  • The objective of this review is to highlight what is known about the epidemiology, pathophysiology, etiology, presentation, diagnosis, and treatment of SH.[ncbi.nlm.nih.gov]
  • […] typically within 1-2 wks) leads to resolution of hyperkalemia Chronic Kidney Disease (CKD) (see Chronic Kidney Disease, [[Chronic Kidney Disease]]): with chronic interstitial nephritis Diabetic Nephropathy (see Diabetes Mellitus, [[Diabetes Mellitus]]) Epidemiology[mdnxs.com]
  • […] collaborate analysis of diabetic criteria in Asia DECODA研究 Diabetes epidemiology collaborate analysis of diabetic criteria in Europe DECODE研究 Diabetes epidemiology research international DERI研究 Diabetes prevention program DPP試験 Diabetes prevention trial[jds.or.jp]
  • Goitre epidemiology: Thyroid volume, iodine excretion, thyroglobulin and thyrotropin in Germany and Sweden. Acta Endocrinol 1 12:494501. ‎[books.google.es]
Sex distribution
Age distribution

Pathophysiology

  • The management of hyperkalaemia and the pathophysiological background of hyporeninaemic hypoaldosteronism are discussed.[ncbi.nlm.nih.gov]
  • This article reviews some of the pathophysiologic aspects, the clinical picture, and the treatment strategies of hyporeninemic hypoaldosteronism from the standpoint of clinical presentation, evaluation, and treatment.[emedicine.medscape.com]

Prevention

  • These changes can be prevented to a degree by increasing the dietary NaCl intake.[en.wikipedia.org]
  • Both patients eventually received long-term FF treatment to prevent impairment of longitudinal growth caused by chronic salt-loss.[ncbi.nlm.nih.gov]
  • […] program DPP試験 Diabetes prevention trial-1 DPT-1試験 Diabetes prevention trial-Type 1 DPT-1試験 Diabetic nephropathy remission and regression team trial in Japan DNETT-Japan Diabetic retinopathy candesartan trials DIRECT試験 Duloxetine デュロキセチン Dupuytren contracture[jds.or.jp]
  • Previous reports showed beneficial effects of CoQ10 on some neuromuscular symptoms, prevention of progressive insulin secretory defect, exercise intolerance, hearing loss, myocardial dysfunction, and intestinal pseudo-obstruction in MIDD patients (13,16[scielo.br]

References

Article

  1. Kokko JP. Primary acquired hypoaldosteronism. Kidney International. 1985; 27 (4): 690-702
  2. Ferri FF. Ferri's Clinical Advisor 2010. Philadelphia. Mosby; 2009;
  3. Lehnhardt A, Kemper MJ. Pathogenesis, diagnosis, and management of hyperkalemia. Pediatr Nephrol. 2011 Mar; 26(3):377-84.
  4. Ulick S. Diagnosis and nomenclature of the disorders of the terminal portion of the aldosterone biosynthetic pathway. Journal of Clinical Endocrinology and Metabolism 1976; 43: 92 –96.
  5. Peter M, Bunger K, Drop SLS, Sippell WG. Molecular genetic study in two patients with congenital hypoaldosteronism (type I and II) in relation to previously published hormonal studies. European Journal of Endocrinology. 1998;139:96-100
  6. Watanabe T, Nitta K. Transient hyporeninemic hypoaldosteronism in acute glomerulonephritis. Pediatr Nephrol. 2002;17:959–63.
  7. Slovis C, Jenkins R. ABC of clinical electrocardiography: conditions not primarily affecting the heart. BMJ. 2002;324:1320–3.

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Last updated: 2019-07-11 21:31