合并胡桃夹综合征和肾小球肾炎的儿科患者:单中心回顾性病例系列。

IF 2.9 3区 医学 Q1 UROLOGY & NEPHROLOGY Kidney Research and Clinical Practice Pub Date : 2024-11-29 DOI:10.23876/j.krcp.24.178
So Hyun Ki, Min Hwa Son, Hyung Eun Yim
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引用次数: 0

摘要

背景:据报道,胡桃夹子综合征(NCS)与各种肾小球肾炎(GN)共存。本研究调查了小儿病例系列中NCS合并GN的临床特征以及这两种情况之间可能的关系。方法:对15例NCS合并活检证实的GN患儿的临床及病理表现进行分析。NCS诊断为肾多普勒超声,腹部计算机断层扫描,和/或磁共振成像。肾小球病变分为轻度肾小球异常(MGAs)和明确肾小球异常(GN)两种病理类型。结果:所有患者平均年龄11±3.36岁,平均随访时间53.8±29.3个月。主诉为蛋白尿伴或不伴血尿。随访期间,5例患者出现左肾肿大。10例患者免疫检查结果异常。肾外症状包括性腺精索静脉曲张、脾囊肿、晕厥和贫血。肾活检中,7例患儿有MGAs, 8例患儿明确表现为GN(局灶性GN 1例,系膜增生性GN 1例,局灶节段性肾小球硬化1例,免疫球蛋白A [IgA]血管炎肾炎2例,IgA肾病3例)。虽然MGA组在最后一次就诊时表现出较高的分离性蛋白尿比例和较低的肾小球滤过率(eGFR),但两组之间在年龄、NCS临床特征、肾外症状、免疫检查和eGFR下降率方面没有差异。结论:NCS可能与多种GN的存在有关。NCS与GN之间的因果关系有待进一步研究。
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Combined nutcracker syndrome and glomerulonephritis in pediatric patients: a single-center retrospective case series.

Background: Nutcracker syndrome (NCS) has been reported to coexist with various glomerulonephritis (GN). This study investigated clinical features of NCS combined with GN in a pediatric case series and the possible relationship between these two conditions.

Methods: Clinical and pathologic findings of 15 children with NCS and biopsy-proven GN were analyzed. NCS was diagnosed with renal Doppler ultrasonography, abdominal computed tomography, and/or magnetic resonance imaging. Glomerular lesions were divided into two pathological categories: minor glomerular abnormalities (MGAs) and definite GN.

Results: Mean age of all patients was 11 ± 3.36 years and mean follow-up duration was 53.8 ± 29.3 months. Chief complaint was proteinuria with or without hematuria. During follow-up, five patients developed left kidney enlargement. Abnormal levels in immunological tests were revealed in 10 patients. Extrarenal symptoms including gonadal varicocele, splenic cyst, syncope, and anemia were found in seven patients. On kidney biopsy, seven patients had MGAs and eight children showed definite GN (one case of focal GN, one case of mesangial proliferative GN, one case of focal segmental glomerulosclerosis, two cases of immunoglobulin A [IgA] vasculitis nephritis, and three cases of IgA nephropathy). While the MGA group showed a higher proportion of isolated proteinuria and a lower estimated glomerular filtration rate (eGFR) at the last visit, there were no differences in age, clinical features of NCS, extrarenal symptoms, immunological tests, and eGFR decline rate between the two groups.

Conclusion: NCS may be associated with the presence of various GN. The causal relationship between NCS and GN should be further investigated.

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来源期刊
CiteScore
4.60
自引率
10.00%
发文量
77
审稿时长
10 weeks
期刊介绍: Kidney Research and Clinical Practice (formerly The Korean Journal of Nephrology; ISSN 1975-9460, launched in 1982), the official journal of the Korean Society of Nephrology, is an international, peer-reviewed journal published in English. Its ISO abbreviation is Kidney Res Clin Pract. To provide an efficient venue for dissemination of knowledge and discussion of topics related to basic renal science and clinical practice, the journal offers open access (free submission and free access) and considers articles on all aspects of clinical nephrology and hypertension as well as related molecular genetics, anatomy, pathology, physiology, pharmacology, and immunology. In particular, the journal focuses on translational renal research that helps bridging laboratory discovery with the diagnosis and treatment of human kidney disease. Topics covered include basic science with possible clinical applicability and papers on the pathophysiological basis of disease processes of the kidney. Original researches from areas of intervention nephrology or dialysis access are also welcomed. Major article types considered for publication include original research and reviews on current topics of interest. Accepted manuscripts are granted free online open-access immediately after publication, which permits its users to read, download, copy, distribute, print, search, or link to the full texts of its articles to facilitate access to a broad readership. Circulation number of print copies is 1,600.
期刊最新文献
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