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皮质肾上腺腺瘤患者存在11β-羟化酶缺乏的证据。

Evidence of 11 beta-hydroxylase deficiency in a patient with cortical adrenal adenoma.

作者信息

Touitou Y, Lecomte P, Auzéby A, Bogdan A, Besnier Y

机构信息

Faculté de Médecine Pitié-Salpêtrière, Service de Biochimie Médicale, Paris, France.

出版信息

Horm Metab Res. 1989 May;21(5):272-4. doi: 10.1055/s-2007-1009210.

DOI:10.1055/s-2007-1009210
PMID:2789167
Abstract

We explored a 61 year old woman with mild hirsutism. An adrenal tumor was found in the left adrenal, which was held responsible for the androgen secretion. The in vitro incubation of the tumor tissue showed an impaired 11 beta-hydroxylation of 11-deoxycortisol. This is a rare and unusual case of adrenal pathology showing that a deficiency in 11 beta-hydroxylase activity does not rule out the presence of an adrenocortical adenoma.

摘要

我们对一名患有轻度多毛症的61岁女性进行了检查。在左肾上腺发现了一个肾上腺肿瘤,该肿瘤被认为是雄激素分泌的原因。肿瘤组织的体外培养显示11 - 脱氧皮质醇的11β - 羟化受损。这是一例罕见且不寻常的肾上腺病理病例,表明11β - 羟化酶活性缺乏并不排除肾上腺皮质腺瘤的存在。

相似文献

1
Evidence of 11 beta-hydroxylase deficiency in a patient with cortical adrenal adenoma.皮质肾上腺腺瘤患者存在11β-羟化酶缺乏的证据。
Horm Metab Res. 1989 May;21(5):272-4. doi: 10.1055/s-2007-1009210.
2
Combined 21- and 11 beta-hydroxylase deficiency in familial congenital adrenal hyperplasia.家族性先天性肾上腺皮质增生症中的21-羟化酶和11β-羟化酶联合缺乏症
J Clin Endocrinol Metab. 1985 Apr;60(4):631-8. doi: 10.1210/jcem-60-4-631.
3
Adrenocortical tumor in a patient with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症患者的肾上腺皮质肿瘤
Pediatrics. 1981 Aug;68(2):242-6.
4
Neonatal salt loss in the hypertensive form of congenital adrenal hyperplasia.先天性肾上腺皮质增生症高血压型的新生儿失盐
Pediatrics. 1980 Apr;65(4):777-81.
5
Dynamics of adrenal steroidogenesis in childhood: changes in the excretion of 16-oxygenated- and 11-oxygenated-steroids by 21-hydroxylase deficient children at various ages.儿童肾上腺类固醇生成的动力学:不同年龄的21-羟化酶缺乏儿童16-氧代类固醇和11-氧代类固醇排泄的变化。
Endokrinologie. 1982 Oct;80(2):151-7.
6
[Bilateral testicular hypertrophy and congenital adrenal hyperplasia caused by 11-beta-hydroxylase deficiency].
Arch Fr Pediatr. 1987 Aug-Sep;44(7):513-6.
7
[Hirsutism secondary to congenital adrenal hyperplasia caused by a 21-hydroxylase deficiency of late onset. A clinical case].
Rev Chil Obstet Ginecol. 1984;49(5):295-9.
8
Primary adrenal insufficiency with hypertension. A case report on partial primary adrenal insufficiency and partial 11-beta-hydroxylase deficiency.
Neth J Med. 1979;22(6):191-4.
9
Decreased levels of steroid 21-hydroxylase [P450(c21)] and its mRNA in an adrenocortical adenoma associated with 21-hydroxylase deficiency.与21-羟化酶缺乏相关的肾上腺皮质腺瘤中类固醇21-羟化酶[P450(c21)]及其mRNA水平降低。
Endocrinol Jpn. 1990 Dec;37(6):841-50. doi: 10.1507/endocrj1954.37.841.
10
Adrenal androgen excess and defective 11 beta-hydroxylation in women with idiopathic hirsutism.
Arch Intern Med. 1982 Apr;142(4):729-35.

引用本文的文献

1
Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency.11β-羟化酶缺乏所致先天性肾上腺皮质增生症的临床观点
Endocrine. 2017 Jan;55(1):19-36. doi: 10.1007/s12020-016-1189-x. Epub 2016 Dec 7.
2
Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11 beta-hydroxylase) deficiency.具有P450c11(11β-羟化酶)缺乏症、酷似先天性肾上腺皮质增生症的男性化肾上腺肿瘤。
Eur J Pediatr. 1994 Jun;153(6):411-5. doi: 10.1007/BF01983403.