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冈比亚/西非地区蛋白尿性肾病的病因

Etiology of Kidney Diseases With Proteinuria in the Gambia/West Africa.

作者信息

Vester Udo, Fombah Augustin, Hölscher Maite, Garba Danlami, Tapgun Mary, N'Jai Pamela Collier, Mendy Philipp, Bass Gibril, Muhammad Abdul K, Anderson Suzanne T, Sanneh Abdoulie, Onyeama Charles, Helmchen Udo, Bojang Khalifa, Hoyer Peter F, Corrah Tumani

机构信息

Helios Klinikum St Johannes, Duisburg, Germany.

Medical Research Council, Fajara, Gambia.

出版信息

Front Pediatr. 2022 Mar 21;10:854719. doi: 10.3389/fped.2022.854719. eCollection 2022.

Abstract

UNLABELLED

In West Africa, kidney diseases are frequently seen, but diagnostic and therapeutic options are poor due to limited access to specialized facilities. To unravel the etiology and develop clinical guidelines, we collected clinical data and results of kidney biopsies in 121 pediatric and mostly young adult patients with edema and proteinuria in The Gambia. Workup included clinical examination, urine and serum analysis, and kidney biopsy findings. Selected cases were treated with steroids.

RESULTS

The median age was 14.9 years (range 1.8-52.0) at presentation. The most frequent underlying histologies were post-infectious glomerulonephritis (PIGN) in 38%, focal-segmental glomerulosclerosis (FSGS) in 30%, minimal change nephrotic syndrome (MCNS) in 15%, and membranous glomerulonephritis (MGN) in 10% of cases. Patients with PIGN were significantly younger and had less proteinuria and higher serum albumin levels than the other three. Infected scabies was seen more often in cases with PIGN. Clinical parameters could not distinguish patients with FSGS, MCNS, and MGN. Steroid response was prompt in patients with MCNS (remission in 10/10 cases) compared to FSGS (4/19) and MGN (0/4). In summary, the clinical histopathological correlation allows a better approach to therapy and can be the basis for urgently needed interventional studies in steroid-resistant cases.

摘要

未标注

在西非,肾脏疾病很常见,但由于获得专业设施的机会有限,诊断和治疗选择不佳。为了阐明病因并制定临床指南,我们收集了冈比亚121例患有水肿和蛋白尿的儿科及大多数年轻成年患者的临床数据和肾脏活检结果。检查包括临床检查、尿液和血清分析以及肾脏活检结果。选定的病例接受了类固醇治疗。

结果

就诊时的中位年龄为14.9岁(范围1.8 - 52.0岁)。最常见的潜在组织学类型为感染后肾小球肾炎(PIGN),占38%;局灶节段性肾小球硬化(FSGS),占30%;微小病变肾病综合征(MCNS),占15%;膜性肾小球肾炎(MGN),占10%。与其他三种类型相比,PIGN患者明显更年轻,蛋白尿更少,血清白蛋白水平更高。PIGN病例中疥疮感染更为常见。临床参数无法区分FSGS、MCNS和MGN患者。与FSGS(4/19)和MGN(0/4)相比,MCNS患者对类固醇反应迅速(10/10例缓解)。总之,临床组织病理学相关性有助于更好地进行治疗,并且可以成为类固醇抵抗病例急需的干预性研究的基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc45/8978824/509c19166e5d/fped-10-854719-g0001.jpg

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