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德朗什综合征:男性假两性畸形、肾炎和威尔姆斯瘤。

The Drash syndrome: male pseudohermaphroditism, nephritis, and Wilms tumor.

作者信息

Goldman S M, Garfinkel D J, Oh K S, Dorst J P

出版信息

Radiology. 1981 Oct;141(1):87-91. doi: 10.1148/radiology.141.1.6270728.

DOI:10.1148/radiology.141.1.6270728
PMID:6270728
Abstract

We report the radiographic and clinical findings of five infants and children who had the combination of male pseudohermaphroditism, progressive nephritis, and Wilms tumor. The components of this syndrome are so striking that the radiologist can often suggest the diagnosis months to years before all three components are apparent, if proteinuria, hypertension, or an abdominal mass develop in any child with male pseudohermaphroditism. Since the progressive renal failure probably can be managed by dialysis or renal transplantation in some cases, prompt recognition and treatment of the Wilms tumor may permit the child to live a long and relatively normal life.

摘要

我们报告了5例患有男性假两性畸形、进行性肾炎和肾母细胞瘤的婴幼儿及儿童的影像学和临床检查结果。该综合征的各组成部分非常显著,以至于如果患有男性假两性畸形的儿童出现蛋白尿、高血压或腹部肿块,放射科医生往往能在所有三个组成部分都明显出现之前数月至数年就提出诊断。由于在某些情况下,进行性肾衰竭或许可以通过透析或肾移植来处理,因此对肾母细胞瘤的及时识别和治疗可能使患儿过上长寿且相对正常的生活。

相似文献

1
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.
2
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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Renal tumors with pseudohermaphroditism and glomerular disease.伴有假两性畸形和肾小球疾病的肾肿瘤。
Acta Oncol. 1992;31(7):745-8. doi: 10.3109/02841869209083864.
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The Drash syndrome revisited: diagnosis and follow-up.再探德拉斯综合征:诊断与随访
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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
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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.
7
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.
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The association of Wilms' tumor, male pseudohermaphroditism and diffuse glomerular disease (Drash syndrome): report of eight cases with clinical and morphologic findings and review of the literature.
Pediatr Pathol. 1987;7(2):175-89. doi: 10.1080/15513818709177840.
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The association of Wilms' tumor with nephrologic disease.肾母细胞瘤与肾脏疾病的关联。
J Pediatr Surg. 1990 Jul;25(7):724-5. doi: 10.1016/s0022-3468(05)80005-3.
10
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.

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
Nonrandom loss of maternal chromosome 11 alleles in Wilms tumors.肾母细胞瘤中母源11号染色体等位基因的非随机丢失。
Am J Hum Genet. 1987 May;40(5):413-20.
4
Wilms' and associated renal tumors of childhood.儿童肾母细胞瘤及相关肾肿瘤
Pediatr Radiol. 1991;21(2):81-8. doi: 10.1007/BF02015609.
5
Etiopathogenesis, classification, investigations and diagnosis in intersex disorders.两性畸形的病因发病机制、分类、检查及诊断
Indian J Pediatr. 1992 Jul-Aug;59(4):475-85. doi: 10.1007/BF02751565.