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男性假两性畸形中的肾母细胞瘤和肾病综合征

Wilms tumor and nephrotic syndrome in male pseudohermaphroditism.

作者信息

Koyanagi T, Hirama M, Taniguchi K, Gotoh T, Inoue K, Maru A, Tsuji I

出版信息

Urology. 1984 Dec;24(6):595-600. doi: 10.1016/0090-4295(84)90110-9.

DOI:10.1016/0090-4295(84)90110-9
PMID:6095508
Abstract

A case is reported of a child with male pseudohermaphroditism in whom Wilms tumor developed at age twenty-two months. The tumor was treated accordingly, but the child subsequently died of nephrotic syndrome with renal failure at age thirty-two months. After reviewing the similar concurrence of these disorders described as a syndrome, it was suggested that they may have basic embryologic abnormalities in common and that all of them originate during embryogenesis. The importance of bearing this syndrome in mind in the management of a child with abnormal gonadal differentiation is stressed.

摘要

报告了一例患有男性假两性畸形的儿童,该儿童在22个月大时患上了威尔姆斯瘤。对该肿瘤进行了相应治疗,但该儿童随后在32个月大时死于伴有肾衰竭的肾病综合征。在回顾了被描述为一种综合征的这些疾病的类似并发情况后,有人提出它们可能有共同的基本胚胎学异常,并且所有这些异常都起源于胚胎发生过程。强调了在管理性腺分化异常的儿童时牢记这种综合征的重要性。

相似文献

1
Wilms tumor and nephrotic syndrome in male pseudohermaphroditism.男性假两性畸形中的肾母细胞瘤和肾病综合征
Urology. 1984 Dec;24(6):595-600. doi: 10.1016/0090-4295(84)90110-9.
2
Pseudohermaphroditism, glomerulonephritis with the nephrotic syndrome, and Wilms' tumor in infancy.假性两性畸形、伴有肾病综合征的肾小球肾炎以及婴儿期的肾母细胞瘤。
J Pediatr. 1971 Oct;79(4):677-81. doi: 10.1016/s0022-3476(71)80322-0.
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A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease.一种假性两性畸形、肾母细胞瘤、高血压和退行性肾病综合征。
J Pediatr. 1970 Apr;76(4):585-93. doi: 10.1016/s0022-3476(70)80409-7.
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Nephroblastoma associated with mesangioproliferative glomerulonephritis.
Eur Urol. 1982;8(2):125-6. doi: 10.1159/000473495.
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Wilms' tumor, nephron disorder and ambiguous genitalia.肾母细胞瘤、肾单位疾病与生殖器模糊。
Z Kinderchir. 1983 Oct;38(5):345-9. doi: 10.1055/s-2008-1060003.
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The Drash syndrome: male pseudohermaphroditism, nephritis, and Wilms tumor.德朗什综合征:男性假两性畸形、肾炎和威尔姆斯瘤。
Radiology. 1981 Oct;141(1):87-91. doi: 10.1148/radiology.141.1.6270728.
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[Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome].[肾母细胞瘤、假性两性畸形与肾小球肾病的关联。该综合征的部分及完全形式]
Bol Med Hosp Infant Mex. 1979 Nov-Dec;36(6):1203-12.
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[Association of an anatomo-pathological syndrome of male pseudohermaphroditism, Wilms' tumor, parenchymatous nephropathy and XX/XY mosaicism].
Arch Fr Pediatr. 1967 Aug-Sep;24(7):729-39.
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A report of 4 patients with the Drash syndrome and a review of the literature.4例德朗综合征患者的报告及文献综述。
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Denys-Drash syndrome: relating a clinical disorder to genetic alterations in the tumor suppressor gene WT1.迪尼-德拉斯综合征:将一种临床病症与肿瘤抑制基因WT1的基因改变联系起来。
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引用本文的文献

1
Nephrotic syndrome in the 1st year of life.1岁儿童的肾病综合征。
Pediatr Nephrol. 1993 Aug;7(4):347-53. doi: 10.1007/BF00857534.
2
The Denys-Drash syndrome.迪尼-德拉斯综合征
J Med Genet. 1994 Jun;31(6):471-7. doi: 10.1136/jmg.31.6.471.