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1. 原発性両側副腎皮質大結節性過形成によるクッシング症候群cushings syndrome due to primary bilateral macronodular adrenal hyperplasia [show details]
… macronodular adrenal hyperplasia (PMAH), massive macronodular adrenocortical disease (MMAD), autonomous macronodular adrenal hyperplasia (AMAH), ACTH-independent massive bilateral… rare types of nodular adrenocortical diseases that are usually bilateral: Corticotropin (ACTH)-dependent bilateral macronodular adrenal hyperplasia,…
2. クッシング症候群の原因および病態生理causes and pathophysiology of cushings syndrome [show details]
…biochemical feature of ACTH-dependent Cushing syndrome is a normal or elevated ACTH level, which reflects tumoral secretion. The tumor secretion of ACTH causes bilateral adrenocortical hyperplasia and hyperfunction …
3. クッシング症候群の治療の概要overview of the treatment of cushings syndrome [show details]
… There are two forms of ACTH-independent bilateral adrenal hyperplasia: primary pigmented nodular adrenocortical disease (PPNAD, also called micronodular adrenal hyperplasia); and bilateral macronodular …
4. 原発性色素沈着性結節性副腎皮質病変によるクッシング症候群cushings syndrome due to primary pigmented nodular adrenocortical disease [show details]
… pigmented nodular adrenocortical disease (PPNAD) is the most common form of corticotropin (ACTH)-independent micronodular hyperplasia or dysplasia. Other less frequent forms of bilateral hyperplasia in which… disease (pituitary ACTH-secreting tumor) or ectopic ACTH syndrome; Primary bilateral macronodular adrenal hyperplasia (BMAH) ACTH-independent micronodular hyperplasia or dysplasia…
5. 副腎アンドロゲン過剰症adrenal hyperandrogenism [show details]
… and bilateral macronodular adrenal hyperplasia ACTH-dependent causes include: Congenital adrenal hyperplasia (common forms such as 21-hydroxylase deficiency, as well as others) ACTH-dependent… treatment for patients with adrenocortical carcinoma and is technically feasible in most. Patients with congenital adrenal hyperplasia are treated with a glucocorticoid…

English Journal

Bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing's syndrome: A case report and review of the literature.

Gu YL, Gu WJ, Dou JT, Lv ZH, Li J, Zhang SC, Yang GQ, Guo QH, Ba JM, Zang L, Jin N, Du J, Pei Y, Mu YM.
World journal of clinical cases. 2019 Apr;7(8)961-971.
Adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome (CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease, ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortica
PMID 31119141

Two cases of Cushing's syndrome due to primary bilateral macronodular adrenal hyperplasia secondary to aberrant adrenal expression of hormone receptors.

Juliá-Sanchis MLL, Navarro-Téllez MDP, Falcones-Gracia KV, Ricart-Álvarez E, González-Bueno MV, Molina-Gasset R.
Clinical biochemistry. 2018 Sep;59()86-89.
Primary bilateral macronodular adrenal hyperplasia is an uncommon cause of endogenous Cushing's syndrome characterized by the presence of aberrant adrenal expression of ectopic receptors that regulate steroidogenesis by mimicking the events triggered by ACTH receptor activation. Receptors of this ty
PMID 29936051

Whole-genome sequencing revealed armadillo repeat containing 5 (ARMC5) mutation in a Chinese family with ACTH-independent macronodular adrenal hyperplasia.

Zhang Q, Cui L, Gao JP, Yan WH, Jin N, Chen K, Zang L, Du J, Wang XL, Guo QH, Yang GQ, Yang LJ, Ba JM, Gu WJ, Lv ZH, Dou JT, Mu YM, Lu JM.
Endocrine journal. 2018 Mar;65(3)269-279.
Primary macronodular adrenal hyperplasia (PMAH), also known in the past as bilateral macronodular adrenalhyperplasia or adrenocorticotropin (ACTH)-independent macronodular adrenal hyperplasia, is a rare type of Cushing's syndrome (CS) and is associated with bilateralenlargement of the adrenal glands
PMID 29279458

Japanese Journal

ACTH非依存性クッシング症候群の分子病態 (内分泌) -- (基礎分野での進歩)

小川誠司
Annual review. 糖尿病・代謝・内分泌, 167-173, 2016
NAID 40020739800

ACTH非依存性大結節性副腎皮質過形成に対し腹腔鏡下片側副腎摘除を行った2例

倉田研人,小野田尚佳,石川哲郎,川尻成美,高島勉,塩井淳,若狭研一,平川弘聖
日本臨床外科学会雑誌 = The journal of the Japan Surgical Association 73(2), 460-465, 2012-02-25
… ACTH非依存性大結節性副腎皮質過形成（ACTH-independent macronodular adrenocortical hyperplasia : AIMAH）に対し片側副腎摘除にて良好な経過を得た2例を報告する．症例1は51歳男性，治療抵抗性の高血圧精査にてAIMAHと診断．プレクリニカルクッシング症候群（PC）を伴っており，腹腔鏡下右副腎摘除術を施行した．症例2は61歳女性，40歳 …
NAID 10030462284