儿童无尿的遥远原因

急性肾损伤是特发性肾病综合征合并mesPGN的罕见并发症。我们报告一位两岁男性病患，有4天无尿、全身水肿及高血容量病史。除了蛋白尿和肾衰竭，其他任何证据都无法通过实验室检查和多普勒超声检查确定。无尿表现被认为与迅速进展的肾小球肾炎、弥漫性系膜硬化或急性肾小管坏死有关。然而，肾活检显示系膜增生性肾小球肾炎(mesPGN)。强的松龙2 mg/kg/天。逐渐开始利尿，发病第10天无尿消失，急性肾损伤恢复，无任何后遗症。这个病例是由于临床表现和组织病理学诊断不一致而提出的。结论:无尿和急性肾损伤可能是儿童期特发性肾病综合征的主要表现。

急性肾损伤合并无尿是儿童特发性肾病综合征的一种特殊而令人担忧的并发症。肾衰竭在没有肾小球炎症的情况下很少发生，也可能在出现肾小球炎时发生。评估应排除严重血管内容量衰竭、急性肾小管坏死、双侧肾静脉血栓形成、急性肾盂肾炎和急性间质肾炎[2-4]。我们在此报告一个以无尿及肾损伤为表现的肾盂肾炎病例。我们发现这个病例很有趣，因为临床表现和组织病理学诊断不一致。与系膜增生(mesPGN)相关的常见症状在这个病人中没有出现。

2岁男性患者无尿4天，全身性水肿。无感染、脱水、有毒物质或用药史。父母没有血缘关系。他体重20公斤(> 97p)，身高102厘米(> 97p)。生命体征:血压90/60 mmHg (90p)，脉搏86/min，呼吸22/min，体温36.5℃。体检正常，全身水肿除外。化验结果如下:白细胞计数(WBC) 10800 / mm3,血红蛋白水平的10.5 gr / dl 176000 / mm3血小板计数,血尿素氮水平的77 mg / dl,血清肌酐水平的3.27 mg / dl,血清钙水平的8.5 mg / dl,血清钠水平的139毫克当量/ L,血清钾水平的4.5毫克当量/ L,血清尿酸水平的9.5 mg / dl,血清白蛋白2.8 g/dl，血清总蛋白5 g/dl，血清磷7 mg/dl，血清胆固醇137 mg/dl，血清甘油三酯133 mg/dl。血pH为7.41,HCO3为23 mEq/L，网红计数为1.4%(0.39-7.54)，结合珠蛋白水平为189 (> 30mg/dl)，血清补体C3水平为97 mg/dl(90-180)，血清补体C4水平为24 mgr/dl(10-40)。ASO水平为29I U/ml。 Estimated GFR (calculated by Schwartz formula) was 34 ml/min/1.73 m2 and urine volume was 2 cc/day. Urinalysis showed PH 6.5, density 1045, protein 4+, blood negative, microscopy 3 RBC and 4 WBC/high power field. Urine protein/creatinine ratio was 30mg/mg. Serum creatinine was increased up to 5.03 mg/dl. Renal sonogram and doppler were normal. There was no response to adequate hydration and IV furosemide. He was totally anuric and hemodialysis was performed for every other day because of severe hypervolemia. Following clinical and laboratory evaluation, any evidence other than proteinuria and renal failure could not be identified. Anuric presentation was thought to be related with acute tubular necrosis, diffuse mesangial sclerosis or rapidly progressive glomerulonephritis. Therefore renal biopsy was performed on the 5th day of anuria. Renal biopsy revealed 38 glomeruli. Contradictory to clinical presentation many of the glomeruli seemed normal and normocellular at first glance with hematoxylin and eosin. Tubules, interstitial and vascular structures were also normal (Figure 1). Mesangial proliferation was more conspicuous when Periodic Acid Schift staining was performed (Figure 2). Immunofluorescence microscopy revealed mesangial deposits of IgM. The histopathologic diagnosis was mesangial proliferative glomerulonephritis (mesPGN).

在第7天获得组织病理学诊断后，给予强的松龙2 mg/kg/天。逐渐开始利尿，发病第10天无尿消失，急性肾损伤恢复，无任何后遗症。类固醇治疗第10天缓解。

急性肾损伤和无尿是儿童期特发性肾病综合征的罕见临床表现。Pstrusinka等人的[5]发现1006例6 - 17.5岁的特发性肾病综合征患儿中有8例(0.8%)患有急性肾ınjury。据报道，明显的低蛋白血症、感染和肾病综合征的严重病程是发展急性肾损伤的危险因素。Bohman等人的[6]显示足突融合程度与GFR和过滤分数密切相关，提示足突融合可导致肾小球过滤面积和/或对水和小溶质的渗透性降低。这种下降是暂时的，在缓解后会迅速恢复正常。Van de Walle等[7]发现肾小球通透性的改变可能在急性肾衰竭中起重要作用。此外，meespgn在特发性肾病综合征中是一种罕见的形式。Mubarak等人的[8]和Gulati等人的[9]报告的儿童mesPGN发生率分别为4.81%和7.6%。MesPGN的特点是系膜细胞增殖，系膜基质增加，并在系膜区沉积。男性受感染的可能性是女性的2.5倍。 Usually patients with mesPGN present with clinical findings of nephrotic syndrome like edema, hypoalbuminemia, proteinuria and hypercholesterolemia [3]. The normal cholesterol level, together with the slightly decreased albumin level in our patient gave the impression that proteinuria would not be long-lasting. This patient was deemed an interesting case because of the incompatibility between clinical findings and histopathologic diagnosis. Even though he presented with anuria, the final diagnosis was idiopathic nephrotic syndrome with MesPGN. With the history of the patient and findings of renal histopathology, we decided that the severe and sudden course of mesangial proliferation was the cause of anuria in our patient. Treatment was aimed at correcting both the nephrotic syndrome and the renal injury. Corticosteroids are generally the main stay of therapy and rapid recovery is an expected result [10].

meespgn是一种罕见的儿童特发性肾病综合征。无尿和急性肾损伤也是罕见的症状。在这种情况下，应通过肾活检进行鉴别诊断。为改善急性肾损伤患者的临床表现，皮质类固醇治疗应成为首选治疗方法。

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