Elmaghrabi Ayah, Brown Elizabeth, Khin Ei, Hassler Jared, Hendricks Allen R
Children's Medical Center, Dallas, Texas, USA.
UT Southwestern Medical Center, Dallas, Texas, USA.
Case Rep Nephrol Dial. 2017 Jun 23;7(2):91-101. doi: 10.1159/000477661. eCollection 2017 May-Aug.
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children. We report 3 pediatric cases in which the renal biopsy showed TRIs on electron microscopy without underlying SLE or HIV infection. The first patient presented at 2 years of age with nephrotic syndrome and renal failure. His renal biopsy revealed focal segmental glomerulosclerosis and TRIs. The second patient presented at 6 months of age with infantile nephrotic syndrome, and his renal biopsy revealed membranous glomerulopathy and TRIs. The last patient presented at 4 years of age with acute kidney injury of unclear etiology leading to chronic kidney disease. Her biopsy revealed acute and chronic tubulointerstitial nephritis with TRIs. Despite extensive evaluation in all 3 patients, including testing for HIV infection and SLE, we could not identify an underlying etiology to explain the presence of TRIs. In conclusion, renal biopsy with TRIs in the absence of underling SLE and HIV remains obscure. We propose a possible role for excess IFN triggered by an abnormal immune response to common viral infections in the formation of TRIs and renal injury.
管网状包涵体（TRIs）是位于内质网池内的亚细胞结构。TRIs的形成与内源性或外源性来源的过量干扰素（IFN）暴露有关。在肾脏疾病中，TRIs最常与系统性红斑狼疮（SLE）和人类免疫缺陷病毒相关性肾病（HIVAN）相关。在成人中，肾活检显示有TRIs但无潜在SLE或HIV的患者病例报告并不常见，据我们所知，儿童中尚未有此类报告。我们报告3例儿科病例，其肾活检在电子显微镜下显示有TRIs，但无潜在的SLE或HIV感染。首例患者2岁时出现肾病综合征和肾衰竭。其肾活检显示局灶节段性肾小球硬化和TRIs。第二例患者6个月大时出现婴儿肾病综合征，其肾活检显示膜性肾小球病和TRIs。最后一例患者4岁时出现病因不明的急性肾损伤并导致慢性肾病。她的活检显示急性和慢性肾小管间质性肾炎伴TRIs。尽管对所有3例患者进行了广泛评估，包括检测HIV感染和SLE，但我们仍无法确定解释TRIs存在的潜在病因。总之，在无潜在SLE和HIV情况下肾活检发现TRIs的病因仍不明确。我们提出，对常见病毒感染的异常免疫反应触发的过量IFN可能在TRIs形成和肾损伤中起作用。
