文献詳細
特集 副腎疾患の基礎と臨床─最前線を知る
〈臨床〉
文献概要
▶ポイント
・代表的な副腎疾患である褐色細胞腫・クッシング症候群・原発性アルドステロン症には,両側副腎疾患となる一型がそれぞれに存在し,近年それらの病態が明らかになってきている.
・両側副腎摘除術後はステロイド補充療法が必須になり,致死的な合併症である急性副腎不全症を起こす可能性がある.
・再生医療につながる幹細胞の研究が盛んに行われているが,副腎皮質は他臓器と比較すると遅れをとっており,今後の研究成果が期待される.
・代表的な副腎疾患である褐色細胞腫・クッシング症候群・原発性アルドステロン症には,両側副腎疾患となる一型がそれぞれに存在し,近年それらの病態が明らかになってきている.
・両側副腎摘除術後はステロイド補充療法が必須になり,致死的な合併症である急性副腎不全症を起こす可能性がある.
・再生医療につながる幹細胞の研究が盛んに行われているが,副腎皮質は他臓器と比較すると遅れをとっており,今後の研究成果が期待される.
参考文献
1) Fishbein L, Leshchiner I, Walter V, et al : Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma. Cancer Cell 31 : 181─193, 2017
2) Rossitti HM, Söderkvist P and Gimm O : Extent of surgery for phaeochromocytomas in the genomic era. Br J Surg 105 : e84─e98, 2018
3) Castinetti F, Taieb D, Henry JF, et al : MANAGEMENT OF ENDOCRINE DISEASE : Outcome of adrenal sparing surgery in heritable pheochromocytoma. Eur J Endocrinol 174 : R9─18, 2016
4) Lenders JW, Duh QY, Eisenhofer G, et al : Pheochromocytoma and paraganglioma : an endocrine society clinical practice guideline. J Clin Endocrinol Metab 99 : 1915─1942, 2014
5) Gruber LM, Erickson D, Babovic-Vuksanovic D, et al : Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1. Clin Endorinol (Oxf) 86 : 141─149, 2017
6) Fragoso MC, Alencar GA, Lerario AM, et al : Genetics of primary macronodular adrenal hyperplasia. J Endocrinol 224 : R31─43, 2015
7) Carney JA, Young WF and Stratakis CA : Primary bimorphic adrenocortical disease : cause of hypercortisolism in McCune-Albright syndrome. Am J Surg Pathol 35 : 1311─1326, 2011
8) Assié G, Libé R, Espiard S, et al : ARMC5 mutations in macronodular adrenal hyperplasia with Cushing's syndrome. N Engl J Med 369 : 2105─2114, 2013
9) Iacobone M, Albiger N, Scaroni C, et al : The role of unilateral adrenalectomy in ACTH-independent macronodular adrenal hyperplasia (AIMAH). World J Surg 32 : 882─889, 2008
10) 池田純一, 六車光英, 井上貴昭, 他 : 片側副腎摘除にて高血糖が改善したACTH非依存性大結節性副腎皮質過形成 (AIMAH) の1例. 泌紀63 : 7─10, 2017
11) Geller DS, Zhang J, Wisgerhof MV, et al : A novel form of human mendelian hypertension featuring nonglucocorticoid-remediable aldosteronism. J Clin Endocrinol Metab 93 : 3117─3123, 2008
12) Choi M, Scholl UI, Yue P, et al : K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension. Science 331 : 768─772, 2011
13) Fernandes-Rosa FL, Boulkroun and Zennaro MC : Somatic and inherited mutations in primary aldosteronism. J Mol Endocrinol 59 : R47─R63, 2017
14) 柳瀬敏彦 : 副腎ホルモン補充療法の現状と問題点. 日内会誌97 : 772─776, 2008
15) Erichsen MM, Løvås K, Fougner KJ, et al : Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death. Eur J Endocrinol 160 : 233─237, 2009
16) Hahner S, Spinnler C, Fassnacht M, et al : High incidence of adrenal crisis in educated patients with chronic adrenal insufficiency : a prospective study. J Clin Endocrinol Metab 100 : 407─416, 2015
17) Jung C and Inder WJ : Management of adrenal insufficiency during the stress of medical illness and surgery. Med J Aust 188 : 409─413, 2008
18) 柳瀬敏彦 : 急性副腎不全 (副腎クリーゼ). 日内会誌105 : 640─646, 2016
19) Johannsson G, Nilsson AG, Bergthorsdottir R, et al : Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency : a prospective randomized trial of a novel hydrocortisone dual-release formulation. J Clin Endocrinol Metab 97 : 473─481, 2012
20) Isidori AM, Venneri MA, Graziadio C, et al : Effect of once-daily, modified-release hydrocortisone versus standard glucocorticoid therapy on metabolism and innate immunity in patients with adrenal insufficiency (DREAM) : a single-blind, randomised controlled trial. Lancet Diabetes Endocrinol 6 : 173─185, 2018
21) O'Connor TP and Crystal RG : Genetic medicines : treatment strategies for hereditary disorders. Nat Rev Genet 7 : 261─276, 2006
22) Barker N, van Es JH, Kuipers J, et al : Identification of stem cells in small intestine and colon by marker gene Lgr5. Nature 449 : 1003─1007, 2007
23) Sato T, Vries RG, Snippert HJ, et al : Single Lgr5 stem cells build crypt-villus structures in vitro without a mesenchymal niche. Nature 459 : 262─265, 2009
24) Shimokawa M, Ohta Y, Nishikori S, et al : Visualization and targeting of LGR5+ human colon cancer stem cells. Nature 545 : 187─192, 2017
25) Walczak EM and Hammer GD : Regulation of the adrenocortical stem cell niche : implications for disease. Nat Rev Endocrinol 11, 14─28, 2015
26) Kim AC, Reuter AL, Zubair M, et al : Targeted disruption of beta-catenin in Sf1-expressing cells impairs development and maintenance of the adrenal cortex. Development 135 : 2593─2602, 2008
27) King P, Paul A and Laufer E : Shh signaling regulates adrenocortical development and identifies progenitors of steroidogenic lineages. Proc Natl Acad USA 106 : 21185─21190, 2009
28) Freedman BD, Kempna PB, Carlone DL, et al : Adrenocortical zonation results from lineage conversion of differentiated zona glomerulosa cells. Dev Cell 26 : 666─673, 2013
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