Ask-Upmark Kidney and Tubulointerstitial Nephritis in a Woman with Severe Renal Failure

Abstract

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered.     

Acute tubulointerstitial nephritis,treatment with steroid and impact on renal outcomes

Background: The use and timing of steroids in the management of acute tubulointerstitial nephritis (ATIN) remains debatable. Aims: To determine the incidence and aetiology of ATIN in our unit, and to examine trends in the use of steroids and their impact on renal outcomes. Methods: Patients with a histological diagnosis of ATIN over a 9‐year period were identified and divided into steroid‐treated (StG) and steroid‐naïve groups (SnG). Mean change in estimated glomerular filtration rate (eGFR) was determined. Results: Forty‐nine patients had ATIN as their main diagnosis, 67% of cases were drug‐induced, and proton pump inhibitors (PPI) were the second commonest implicated drug category. Majority (75%) of patients received steroids, and eGFR improved to a significantly greater degree in these steroid‐treated patients (3.4‐fold improvement vs 2.0‐fold in SnG; P < 0.05, unpaired t‐test). Despite comparable eGFR at presentation (StG: 11.7; SnG: 15.4), steroid‐treated patients were less likely to receive dialysis, although not significantly so (OR 0.27; 95% CI 0.06–1.15, P = 0.066, chi‐squared test). However, there was no significant relation between the degree of eGFR improvement and delay in starting steroids (Pearson r = ?0.25, P > 0.45), and no difference in eGFR at the time of last follow‐up (StG: 33 ± 3; SnG: 32 ± 7; P > 0.9, unpaired t‐test). Conclusion: StG patients had a greater degree of improvement in renal function, but with no correlation between degree of improvement in eGFR and delay in starting steroids, and similar eGFR values at final follow‐up. PPI were the second commonest drug category among drug‐induced cases.     

帕立骨化醇抑制糖尿病肾病大鼠肾小管间质纤维化

目的:探讨帕立骨化醇(paricalcitol,P)对糖尿病肾病(DN)肾小管间质纤维化的干预作用及可能机制。方法:大鼠禁食后,采用单次无菌腹腔注射链脲佐菌素建立DN模型。将DN大鼠随机分为:(1)帕立骨化醇干预组(P组):帕立骨化醇溶于丙二醇中,于造模成功后第2天以0.4μg/kg的剂量腹腔注射,每周3次;(2)糖尿病肾病组(D组):给予等体积的丙二醇腹腔注射。设置正常对照组(C组)。帕立骨化醇连续干预12周后,测血、尿生化指标;进行肾脏病理学检查;利用免疫组化及Western blotting检测肾组织TGF-β1、Wnt-4、β-catenin及Klotho蛋白的表达;并进行指标间的相关分析。结果:(1)与C组比较,D组大鼠SCr、BUN及24 h尿蛋白水平均升高,而P组均较D组降低(P0.05)。(2)与C组比较,D组大鼠肾小管间质纤维化面积增加,而P组较D组减小(P0.05)。(3)D组大鼠肾组织Klotho蛋白表达低于C组,而P组高于D组(P0.05);与C组比较,D组大鼠肾组织TGF-β1、Wnt-4及β-catenin蛋白表达增加,而P组表达均较D组减少(P0.05)。(4)Klotho与纤维化面积、TGF-β1、Wnt-4及β-catenin均呈负相关(P0.05)。结论:帕立骨化醇可抑制DN大鼠肾小管间质纤维化,其作用可能与增加肾组织Klotho表达,抑制Wnt/β-catenin信号通路激活,同时减少TGF-β1合成相关。     

IgA肾病合并急性肾盂肾炎

46岁女性患者,临床表现为寒战、发热、腰痛、尿频、尿急、尿痛,解茶色小便,伴血清肌酐升高,实验室检查发现尿白细胞阳性,中段尿培养大肠埃希菌阳性。经抗感染治疗后症状明显缓解,但仍存在较多蛋白尿和镜下血尿,肾活检组织学为IgA肾病,伴肾小管间质性肾炎,结合临床,考虑IgA肾病合并肾盂肾炎。     

IgG4相关系统性疾病的肾脏损害

IgG4相关系统性疾病(IgG4-related systemic disease,IgG4-RSD)是一组最近认识的临床综合征,可累及多个器官,胰腺最常受累,主要表现为血清IgG4升高,受累组织弥漫IgG4阳性浆细胞浸润。本文主要介绍IgG4-RSD累及肾脏时的临床表现、影像学、实验室检查、病理特点及治疗情况。     

Renal Microvascular Disease in an Aging Population: A Reversible Process?

Renal microvascular disease and tubulointerstitial fibrosis are usually demonstrated in aging in humans and animals. It has recently been proposed that renal microvascular disease is the crucial determinant of tubulointerstitial disease or fibrosis. Enhanced circulating endothelial cell loss is a biomarker that reflects glomerular endothelial injury or renal microvascular disease, and fractional excretion of magnesium (FE Mg) is a sensitive biomarker that reflects an early stage of tubulointerstitial fibrosis. In aging in humans, both of these biomarkers are abnormally elevated. In addition, a glomerular endothelial dysfunction determined by altered hemodynamics associated with peritubular capillary flow reduction is substantiated. A correction of such hemodynamic alteration with vasodilators can effectively improve renal perfusion and restore renal function. Thus, anti-aging therapy can reverse the renal microvascular disease and dysfunction associated with the aging process.