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肾母细胞瘤、肾单位疾病与生殖器模糊。

Wilms' tumor, nephron disorder and ambiguous genitalia.

作者信息

Kaneko M, Saito S, Tsuchida Y, Nakajo T, Akiyama H

出版信息

Z Kinderchir. 1983 Oct;38(5):345-9. doi: 10.1055/s-2008-1060003.

DOI:10.1055/s-2008-1060003
PMID:6139927
Abstract

Two male cases with Wilms' tumor in association with ambiguous genitalia and nephrotic syndrome are described. Seventeen similar cases reported in the literature are also analyzed. In these patients, nephrotic syndrome was noticed at younger ages, and progressed more rapidly than usual. Renal damage was so serious that at least 11 out of 9 patients died of renal failure, though not of Wilms' tumor itself. It should be stressed that the clinical course and associated anomalies were quite similar among these patients. We suggest that Wilms' tumor, ambiguous genitalia and this type of nephrotic syndrome may have some etiologic connection.

摘要

本文描述了两例患有肾母细胞瘤并伴有生殖器模糊和肾病综合征的男性病例。同时对文献中报道的17例类似病例进行了分析。在这些患者中,肾病综合征在较年轻时就被发现,且进展比通常情况更快。肾脏损害非常严重,尽管9例患者中至少有11例死于肾衰竭,但并非死于肾母细胞瘤本身。应当强调的是,这些患者的临床病程及相关异常情况非常相似。我们认为肾母细胞瘤、生殖器模糊和这种类型的肾病综合征可能存在某种病因学联系。

相似文献

1
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.
2
[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.
3
Adult Wilms' tumor with hypospadias and cryptorchidism: a case report.成人肾母细胞瘤合并尿道下裂和隐睾症:一例报告
Kaohsiung J Med Sci. 2007 Nov;23(11):584-9. doi: 10.1111/j.2410-8650.2007.tb00002.x.
4
[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.
5
The nephropathy associated with male pseudohermaphroditism and Wilms' tumor (Drash syndrome): a distinctive glomerular lesion--report of 10 cases.与男性假两性畸形和肾母细胞瘤相关的肾病(德拉斯综合征):一种独特的肾小球病变——10例报告
Clin Nephrol. 1985 Dec;24(6):269-78.
6
[Diagnosis and treatment of Wilms' tumor].[肾母细胞瘤的诊断与治疗]
Gan To Kagaku Ryoho. 1983 Oct;10(10):2117-24.
7
[Causes of ambiguous external genitalia in neonates].[新生儿外生殖器模糊的原因]
Srp Arh Celok Lek. 2001 Mar-Apr;129(3-4):57-60.
8
[Wilms' tumor and aniridia--a genetically determined syndrome?].
Klin Padiatr. 1972 Jul;184(4):312-7.
9
[Bilateral Wilms' tumor associated with multiple developmental defects in a newborn infant].
Vopr Okhr Materin Det. 1975 Jun;20(6):80-3.
10
Wilms' tumor and glomerular disease. Occurrence with features of membranoproliferative glomerulonephritis and secondary focal, segmental glomerulosclerosis.
Arch Pathol Lab Med. 1984 Feb;108(2):141-6.

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.
3
Nephropathy with Wilms tumour or gonadal dysgenesis: incomplete Denys-Drash syndrome or separate diseases?伴有威尔姆斯瘤或性腺发育不全的肾病:不完全型迪尼-德拉斯综合征还是独立疾病?
Eur J Pediatr. 1995 Jul;154(7):577-81. doi: 10.1007/BF02074838.