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髓质海绵肾的肾酸化缺陷

Renal acidification defects in medullary sponge kidney.

作者信息

Osther P J, Hansen A B, Røhl H F

机构信息

Department of Urology, Odense University Hospital, Denmark.

出版信息

Br J Urol. 1988 May;61(5):392-4. doi: 10.1111/j.1464-410x.1988.tb06581.x.

DOI:10.1111/j.1464-410x.1988.tb06581.x
PMID:3395796
Abstract

Thirteen patients with medullary sponge kidney underwent a short ammonium chloride loading test to investigate their renal acidification capacity. All but 1 presented with a history of recurrent renal calculi and showed bilateral widespread renal medullary calcification on X-ray examination. Nine patients had some form of renal acidification defect; 8 had the distal type of renal tubular acidosis, 2 the complete and 6 the incomplete form. One patient had proximal renal tubular acidosis. These findings, which suggest that renal acidification defects play an important role in the pathogenesis of renal calculi in medullary sponge kidney, have considerable therapeutic implications.

摘要

13例髓质海绵肾患者接受了短程氯化铵负荷试验,以研究其肾脏酸化能力。除1例患者外,其余患者均有复发性肾结石病史,X线检查显示双侧肾髓质广泛钙化。9例患者存在某种形式的肾脏酸化缺陷;8例为远端肾小管酸中毒,其中2例为完全型,6例为不完全型。1例患者为近端肾小管酸中毒。这些发现提示肾脏酸化缺陷在髓质海绵肾结石的发病机制中起重要作用,具有重要的治疗意义。

相似文献

1
Renal acidification defects in medullary sponge kidney.髓质海绵肾的肾酸化缺陷
Br J Urol. 1988 May;61(5):392-4. doi: 10.1111/j.1464-410x.1988.tb06581.x.
2
Urine acidification capacity in renal stone formers.肾结石患者的尿液酸化能力。
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Growth failure associated with medullary sponge kidney, due to incomplete renal tubular acidosis type 1.
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Distal renal tubular acidosis in recurrent renal stone formers.复发性肾结石患者的远端肾小管酸中毒
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A case of incomplete renal tubular acidosis (type 1) associated with medullary sponge kidney followed by nephrocalcinosis.1例与髓质海绵肾相关的不完全性1型肾小管酸中毒继发肾钙质沉着症。
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Unilateral and segmental medullary sponge kidney: renal function and calcium excretion.单侧及节段性髓质海绵肾:肾功能与钙排泄
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Renal calculi associated with incomplete distal renal tubular acidosis.与远端肾小管酸中毒不完全相关的肾结石
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Ehlers-Danlos syndrome with renal tubular acidosis and medullary sponge kidneys. A report of a case and studies of renal acidification in other patients with the Ehlers-Danlos syndrome.伴有肾小管酸中毒和髓质海绵肾的埃勒斯-当洛综合征。1例报告及对其他埃勒斯-当洛综合征患者的肾酸化研究。
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引用本文的文献

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Medullary Sponge Kidney and Its Relationship with Primary Distal Renal Tubular Acidosis: Case Reports and a Comprehensive Genetics-First Approach.海绵肾髓质及其与原发性远端肾小管酸中毒的关系:病例报告和全面的遗传优先方法。
Nephron. 2024;148(8):569-577. doi: 10.1159/000538037. Epub 2024 Mar 8.
2
The pathophysiology of distal renal tubular acidosis.远端肾小管性酸中毒的病理生理学。
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3
Evaluation of urinary acidification in children: Clinical utility.
儿童尿酸化评估:临床应用价值。
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Proteomic Analysis of Urinary Extracellular Vesicles Reveals a Role for the Complement System in Medullary Sponge Kidney Disease.尿细胞外囊泡的蛋白质组学分析揭示补体系统在髓质海绵肾疾病中的作用。
Int J Mol Sci. 2019 Nov 5;20(21):5517. doi: 10.3390/ijms20215517.
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Incomplete distal renal tubular acidosis from a heterozygous mutation of the V-ATPase B1 subunit.V-ATPase B1 亚基杂合突变导致不完全远端肾小管性酸中毒。
Am J Physiol Renal Physiol. 2014 Nov 1;307(9):F1063-71. doi: 10.1152/ajprenal.00408.2014. Epub 2014 Aug 27.
6
Medullary sponge kidney associated with distal renal tubular acidosis in a 5-year-old girl.一名5岁女孩患髓质海绵肾并伴有远端肾小管酸中毒。
Eur J Pediatr. 2006 Sep;165(9):648-51. doi: 10.1007/s00431-006-0125-0. Epub 2006 Apr 7.
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Pathophysiology of incomplete renal tubular acidosis in recurrent renal stone formers: evidence of disturbed calcium, bone and citrate metabolism.复发性肾结石患者不完全性肾小管酸中毒的病理生理学:钙、骨和柠檬酸盐代谢紊乱的证据。
Urol Res. 1993 May;21(3):169-73. doi: 10.1007/BF00590032.
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A double-blind placebo-controlled trial of omeprazole on urinary pH in healthy subjects.奥美拉唑对健康受试者尿液pH值影响的双盲安慰剂对照试验。
Int Urol Nephrol. 1992;24(3):229-31. doi: 10.1007/BF02549529.