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斯里兰卡原因不明的慢性肾脏病(CKDu)患者的形态学和临床发现:与中美洲肾病的相似之处和不同之处。

Morphological and clinical findings in Sri Lankan patients with chronic kidney disease of unknown cause (CKDu): Similarities and differences with Mesoamerican Nephropathy.

机构信息

Division of Renal Medicine, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.

Department of Pharmacology, Faculty of Medicine, Rajarata University of Sri Lanka, Saliyapura, Sri Lanka.

出版信息

PLoS One. 2018 Mar 7;13(3):e0193056. doi: 10.1371/journal.pone.0193056. eCollection 2018.

Abstract

In Sri Lanka, an endemic of chronic kidney disease of unknown origin (CKDu) is affecting rural communities. The endemic has similarities with Mesoamerican Nephropathy (MeN) in Central America, however it has not yet been clarified if the endemics are related diagnostic entities. We designed this study of kidney biopsies from patients with CKDu in Sri Lanka to compare with MeN morphology. Eleven patients with CKDu were recruited at the General Hospital, Polonnaruwa, using similar inclusion and exclusion criteria as our previous MeN studies. Inclusion criteria were 20-65 years of age and plasma creatinine 100-220 μmol/L. Exclusion criteria were diabetes mellitus, uncontrolled hypertension and albuminuria >1g/24h. Kidney biopsies, blood and urine samples were collected, and participants answered a questionnaire. Included participants were between 27-61 years of age and had a mean eGFR of 38±14 ml/min/1.73m2. Main findings in the biopsies were chronic glomerular and tubulointerstitial damage with glomerulosclerosis (8-75%), glomerular hypertrophy and mild to moderate tubulointerstitial changes. The morphology was more heterogeneous and interstitial inflammation and vascular changes were more common compared to our previous studies of MeN. In two patients the biopsies showed morphological signs of acute pyelonephritis but urine cultures were negative. Electrolyte disturbances with low levels of serum sodium, potassium, and/or magnesium were common. In the urine, only four patients displayed albuminuria, but many patients exhibited elevated α-1-microglobulin and magnesium levels. This is the first study reporting detailed biochemical and clinical data together with renal morphology, including electron microscopy, from Sri Lankan patients with CKDu. Our data show that there are many similarities in the biochemical and morphological profile of the CKDu endemics in Central America and Sri Lanka, supporting a common etiology. However, there are differences, such as a more mixed morphology, more interstitial inflammation and vascular changes in Sri Lankan patients.

摘要

在斯里兰卡,一种原因不明的慢性肾脏病(CKDu)地方性流行正在影响农村社区。这种地方性流行与中美洲的中美洲肾脏病(MeN)相似,但尚未明确这些地方性流行是否与相关的诊断实体有关。我们设计了这项对来自斯里兰卡 CKDu 患者的肾脏活检的研究,以与 MeN 形态学进行比较。在波隆纳鲁瓦总医院,我们使用与我们之前的 MeN 研究类似的纳入和排除标准,招募了 11 名 CKDu 患者。纳入标准为年龄 20-65 岁,血浆肌酐 100-220μmol/L。排除标准为糖尿病、未控制的高血压和蛋白尿 >1g/24h。收集了肾脏活检、血液和尿液样本,参与者回答了一份问卷。纳入的参与者年龄在 27-61 岁之间,平均 eGFR 为 38±14ml/min/1.73m2。活检的主要发现是慢性肾小球和肾小管间质损伤,伴有肾小球硬化(8-75%)、肾小球肥大和轻度至中度肾小管间质改变。与我们之前的 MeN 研究相比,这种形态学更为混杂,间质炎症和血管变化更为常见。在两名患者中,活检显示出急性肾盂肾炎的形态学迹象,但尿液培养为阴性。电解质紊乱,血清钠、钾和/或镁水平低很常见。在尿液中,只有 4 名患者显示出蛋白尿,但许多患者显示出α-1-微球蛋白和镁水平升高。这是第一项报告来自斯里兰卡 CKDu 患者的详细生化和临床数据以及肾脏形态学(包括电子显微镜)的研究。我们的数据表明,中美洲和斯里兰卡的 CKDu 地方性流行在生化和形态学特征上有许多相似之处,支持共同的病因。然而,也存在一些差异,如斯里兰卡患者的形态更为混杂,间质炎症和血管变化更为常见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d40a/5841753/b38189cf2f36/pone.0193056.g001.jpg

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