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Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy.

作者信息

Mattoo T K, al-Sowailem A M, al-Harbi M S, Mahmood M A, Katawee Y, Hassab M H

机构信息

Maternity and Children's Hospital, Riyadh, Saudi Arabia.

出版信息

Pediatr Nephrol. 1992 Jan;6(1):16-8. doi: 10.1007/BF00856821.

DOI:10.1007/BF00856821
PMID:1536734
Abstract

The study includes 17 infants with nephrotic syndrome. Their ages at the onset of disease ranged from 10 days to 10 months (median 2 months), and included 10 males and 7 females. Ten patients had consanguineous parents and 4 had a history of similar illness in the sibling. Renal biopsy revealed diffuse mesangial proliferation in 10 patients, diffuse mesangial sclerosis in 3, Finnish microcystic disease in 2, and focal segmental glomerulosclerosis and atypical glomerulopathy in 1 each. Unilateral nephrectomy was performed in 8 patients with intractable proteinuria associated with multiple complications. Five are well after 4-36 months follow-up, 1 died of septicaemia soon after the procedure, 1 needed dialysis and is stable at 26 months and 1 showed no reduction in the degree of proteinuria. We conclude that unilateral nephrectomy is a better alternative to bilateral nephrectomy in some infants with severe nephrotic syndrome.

摘要

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Home Albumin Infusion Therapy, Another Alternative Treatment in Patients With Congenital Nephrotic Syndrome of the Finnish Type.

本文引用的文献

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家庭白蛋白输注疗法,芬兰型先天性肾病综合征患者的另一种替代治疗方法。
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A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan.日本先天性和婴儿期肾病综合征的全国横断面调查。
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Diagnostic and Management Challenges in Congenital Nephrotic Syndrome.先天性肾病综合征的诊断与管理挑战
Pediatric Health Med Ther. 2019 Dec 17;10:157-167. doi: 10.2147/PHMT.S193684. eCollection 2019.
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Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry.肾脏替代治疗的时机不影响由NPHS1基因突变引起的先天性肾病综合征患儿的生存和生长:来自ESPN/ERA-EDTA注册中心的数据。
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Management of congenital nephrotic syndrome.先天性肾病综合征的管理
Pediatr Nephrol. 2003 May;18(5):426-30. doi: 10.1007/s00467-003-1131-3. Epub 2003 Apr 8.
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Glomerular basement membrane abnormalities in infants with heavy proteinuria.
Pediatr Nephrol. 1993 Aug;7(4):401-3. doi: 10.1007/BF00857552.
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Management of congenital nephrotic syndrome of the Finnish type.芬兰型先天性肾病综合征的管理
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10
Hypothyroidism in infants with nephrotic syndrome.肾病综合征患儿的甲状腺功能减退症
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4
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J Pediatr. 1974 Nov;85(5):615-21. doi: 10.1016/s0022-3476(74)80502-0.
5
A clinicopathologic study of forty-eight infants with nephrotic syndrome.
Kidney Int. 1985 Mar;27(3):544-52. doi: 10.1038/ki.1985.45.
6
Management of Finnish congenital nephrotic syndrome by unilateral nephrectomy.
Pediatr Nephrol. 1989 Oct;3(4):451-3. doi: 10.1007/BF00850226.
7
Cystic lymphangiectasia of the kidneys in an infant with nephrotic syndrome.一名患有肾病综合征的婴儿的肾囊性淋巴管扩张症。
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