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Hypoaldosteronism is an endocrinological disorder characterized decreased levels of the hormone aldosterone. Similarly, isolated hypoaldosteronism is the condition of having lowered aldosterone without corresponding changes in cortisol.^[1] (The two hormones are both produced by the adrenals.)

Causes

There are several causes for this condition, including adrenal insufficiency, congenital adrenal hyperplasia, and some medications such as certain diuretics, NSAIDs, and ACE inhibitors.

Primary aldosterone deficiency

Primary adrenal insufficiency
Congenital adrenal hyperplasia (21 and 11β but not 17)
Aldosterone synthase deficiency

Secondary aldosterone deficiency

Secondary adrenal insufficiency
Diseases of the pituitary or hypothalamus

Hyporeninemic hypoaldosteronism (due to decreased angiotensin 2 production as well as intra-adrenal dysfunction)^[2]

Renal dysfunction-most commonly diabetic nephropathy
NSAIDs
Ciclosporin

Diagnosis

Treatment

Aldosterone deficiency should be treated with a mineralocorticoid (such as fludrocortisone), as well as possibly a glucocorticoid for cortisol deficiency, if present.
Hyporeninemic hypoaldosteronism is amenable to fludrocortisone treatment,^[2] 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.^[3]

Effects

Hypoaldosteronism may result in hyperkalemia and is the cause of 'type 4 renal tubular acidosis', sometimes referred to as hyperkalemic RTA or tubular hyperkalemia. However, the acidosis, if present, is often mild. It can also cause urinary sodium wasting, leading to volume depletion and hypotension.

When adrenal insufficiency develops rapidly, the amount of Na+ lost from the extracellular fluid exceeds the amount excreted in the urine, indicating that Na+ also must be entering cells. When the posterior pituitary is intact, salt loss exceeds water loss, and the plasma Na+ falls. However, the plasma volume also is reduced, resulting in hypotension, circulatory insufficiency, and, eventually, fatal shock. These changes can be prevented to a degree by increasing the dietary NaCl intake. 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.^{[citation needed]}

References

^ Becker, Kenneth L. (2001). Principles and practice of endocrinology and metabolism. Lippincott Williams & Wilkins. pp. 785–. ISBN 978-0-7817-1750-2. Retrieved 15 July 2011.
^ ^a ^b DeFronzo RA (1980). "Hyperkalemia and hyporeninemic hypoaldosteronism". Kidney Int. 17 (1): 118–34. doi:10.1038/ki.1980.14. PMID 6990088.
^ Sebastian A, Schambelan M, Sutton JM (1984). "Amelioration of hyperchloremic acidosis with furosemide therapy in patients with chronic renal insufficiency and type 4 renal tubular acidosis". Am. J. Nephrol. 4 (5): 287–300. doi:10.1159/000166827. PMID 6524600.

External links

UpToDate Contents

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1. 低アルドステロン症（4型RTA）の病因、診断、および治療 etiology diagnosis and treatment of hypoaldosteronism type 4 rta
2. 腎尿細管性アシドーシスの概要と病態生理、およびカリウムバランスへの影響 overview and pathophysiology of renal tubular acidosis and the effect on potassium balance
3. まれな先天性副腎過形成 uncommon congenital adrenal hyperplasias
4. 幼児および小児における尿細管性アシドーシスの病因および臨床症状 etiology and clinical manifestations of renal tubular acidosis in infants and children
5. 成人における高カリウム血症の原因および評価 causes and evaluation of hyperkalemia in adults

English Journal

Relative hypoaldosteronism in a patient with Wolcott-Rallison syndrome.

Uçar A1, Aydemir Y2, Doğan A3, Tunçez E4.
Diabetic medicine : a journal of the British Diabetic Association.Diabet Med.2016 Mar;33(3):e13-6. doi: 10.1111/dme.12968.
BACKGROUND: Wolcott-Rallison syndrome is an autosomal recessive, multisystem disorder with onset of diabetes in the neonatal period or early infancy.CASE REPORT: A 9-year-old girl with diabetes and growth failure from 2 months of age presented with ketoacidosis and multiple organ failure. Evaluatio
PMID 26433138

Renal tubular acidosis type IV as a complication of lupus nephritis.

Sánchez-Marcos C1, Hoffman V2, Prieto-González S1, Hernández-Rodríguez J1, Espinosa G3.
Lupus.Lupus.2016 Mar;25(3):307-9. doi: 10.1177/0961203315603143. Epub 2015 Sep 7.
Renal tubular acidosis (RTA) is a rare complication of renal involvement of systemic lupus erythematosus (SLE). We describe a 24-year-old male with type IV lupus nephropathy as a presenting manifestation of SLE. He presented with improvement of renal function following induction therapy with three p
PMID 26345674

Apparent mineralocorticoid excess and the long term treatment of genetic hypertension.

Razzaghy-Azar M1, Yau M2, Khattab A3, New MI3.
The Journal of steroid biochemistry and molecular biology.J Steroid Biochem Mol Biol.2016 Feb 15. pii: S0960-0760(16)30028-0. doi: 10.1016/j.jsbmb.2016.02.014. [Epub ahead of print]
Apparent Mineralocorticoid Excess (AME) is a genetic disorder causing severe hypertension, hypokalemia, and hyporeninemic hypoaldosteronism owing to deficient 11 beta-hydroxysteroid dehydrogenase type-2 (11βHSD2) enzyme activity. The 11βHSD2 enzyme confers mineralocorticoid receptor specificity fo
PMID 26892095

Japanese Journal

Two novel mutations of the CYP11B2 gene in a Japanese patient with aldosterone deficiency type 1

, , , , , , , , ,
Endocrine Journal 60(1), 51-55, 2013
… Isolated hypoaldosteronism is a rare and occasionally life-threatening cause of salt wasting in infancy. …
NAID 130004770381

4型腎尿細管性アシドーシス

石橋正太,竹林晃三,麻生好正 [他]
内分泌・糖尿病・代謝内科 32(1), 116-122, 2011-01
NAID 40018735848

抑肝散による著明な低Ｋ血症で緊急入院となった８１歳女性認知症患者の１例

西山直樹,竹下雅子,田中健一郎,宮尾益理子,水野有三
日本老年医学会雑誌 48(5), 553-557, 2011
症例は81歳女性．高血圧でA病院，アルツハイマー型認知症でB病院に通院し，6カ月前より昼夜逆転，不穏等の周辺症状（behavioral and psychological symptoms of dementia：BPSD）に対し抑肝散7.5 gの投与が開始された．2009年某日，デイケア中に気分不快，嘔気，高血圧（SBP 200 mmHg以上）を認め，著明な低K血症（1.29 mEq/l）と心電 …
NAID 130004485780

Related Pictures

Arthur S. Schneider, M.D. Department of Pathology - ppt video online download Normal Anion Gap Acidoses Renal Tubular Acidosis - ppt video online download Hyporeninemic hypoaldosteronism and diabetes mellitus: Pathophysiology assumptions QAP Case Presentation ppt video online download Hyperkalemia Diagnostic Approach - Renal Failure primary hypoaldosteronism vs Addison's disease - Google Search | MLT Stuff | Pinterest Non-anion gap Metabolic Acidosis (NAGMA) Hypoaldosteronism; Hypoaldosteronism, Hyporeninemic; Acidosis, Renal Tubular Type IV

★リンクテーブル★

リンク元	「アルドステロン」「低アルドステロン症」「アルドステロン症」「type IV renal tubular acidosis」
拡張検索	「hyporeninemic hypoaldosteronism」「secondary hypoaldosteronism」「pseudohypoaldosteronism」

「アルドステロン」

Hypoaldosteronism
	Aldosterone
Specialty	Endocrinology

　　[★]

英: aldosterone
関: 尿細管

基準値

血漿濃度は35-240 pg/ml, EDTA加血漿安静臥位　30-160 pg/ml
30-160 pg/ml (LAB.715)
35.7-240 pg/ml (随時), 29.9-159pg/ml (臥位), 38.9-307pg/ml (立位) (SRL)

分類

副腎皮質ホルモン

電解質コルチコイド

性状

ステロイド

産生組織

副腎皮質球状層

標的組織

主に集合管(SP.792)

生理作用

1. 腎の接合尿細管と集合管、唾液腺、乳腺、汗腺等に働いてNa⁺の再吸収を促進し、K⁺の排出(分泌)を促進する (SP.791,792 によれば、腎接合尿細管を含む)
2. 腎集合管でH⁺の排出(分泌)を促進する。

Na+/K+-ATPase活性↑＠遠位尿細管・皮質集合管　→　管腔側K↑　→　K再吸収/H+分泌 (QB CBT vol2 p.360)　←　成書での裏付けがないが、確かにアルドステロン↑によりK+分泌が↑となれば、管腔側にK+があふれるのでα間在細胞上の管腔側にあるK+/H+交換輸送体担体によりH+管腔側にくみ出されるな。