儿童阿努里亚的偏远原因

急性肾损伤是特发性肾病综合征与MESPGN的罕见并发症。在这里，我们提出了一名2岁的男性患者，患有4天的Anuria，广义水肿和高血症病史。除蛋白尿和肾衰竭以外的任何证据都无法通过实验室测试和多普勒超声检查来识别。高声介绍被认为与快速进行性肾小球肾炎，弥漫性肾小球硬化或急性管状坏死有关。然而，肾脏活检显示肾小球增殖性肾小球肾炎（MESPGN）。开处方2 mg/kg/天的泼尼松龙。利列他人逐渐开始，在疾病的第10天开始，消除了Anuria，并且没有任何续集就回收了急性肾脏损伤。由于临床发现与组织病理学诊断之间的不相容性，因此出现了这种情况。可以得出结论，尽管很少见，但阿努里亚和急性肾损伤可能是儿童特发性肾病综合征的症状。

急性肾脏损伤与Anuria是童年时期特发性肾病综合征的异常并发症[1]。在没有肾小球炎症的情况下，肾衰竭可能很少发生，并且可能在表现时发生。应进行评估，以排除严重的血管内量耗竭，急性管状坏死，双侧肾静脉血栓形成，急性肾盂肾炎和急性间质肾炎[2-4]。在这里，我们提出了一个MESPGN的案例，该案件出现了Anuria和肾脏损伤。我们发现该病例很有趣，因为临床发现与组织病理学诊断之间存在不相容性。该患者没有出现与肾小球增殖（MESPGN）相关的常见症状。

一名2岁的男性患者有4天的Anuria病史和广义水肿。没有病史，例如感染，脱水，有毒物质或使用药物。父母不近亲。他的体重为20公斤（> 97便士），身高为102厘米（> 97p）。生命体征如下：血压为90/60 mmHg（90p），脉搏率为86/min，呼吸频率为22/min，体温为36.5°C。身体检查正常浮肿。实验室研究如下：白细胞计数为（WBC）10,800 /mm3，血红蛋白水平为10.5 gr /dl，血小板计数为176,000 /mm3，血尿素氮水平为77 mg /dl，一种血清肌酐3.27 mg/dL的水平，血清钙水平8.5 mg/dl，血清钠水平139 mEq/L，血清钾水平为4.5 mEq/L，血清尿酸水平为9.5 mg/dl，一种白蛋白水平为2.8 g/dL，血清总蛋白质水平为5 g/dL，血清磷水平为7 mg/dL，血清胆固醇水平为137 mg/dl，血清甘油三酸酯水平为133 mg/dl。血液pH值为7.41，HCO3为23 mEq/L，网状细胞计数为1.4％（0.39-7.54），触蛋白水平为189（> 30mg/dl），血清补体C3水平为97 mg/dl（90-180）和血清（90-180）和血清 complement C4 level was 24 mgr/dl (10-40). ASO level was 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，开了2 mg/kg/天的泼尼松龙。利列他人逐渐开始，在疾病的第10天开始，消除了Anuria，并且没有任何续集就回收了急性肾脏损伤。在类固醇治疗的第10天进行缓解。

急性肾脏损伤和Anuria是特发性肾病综合征与MESPGN的罕见诊所表现。Pstrusinka等。[5]注意到1006名儿童中有8名（0.8％）中有急性肾脏肾脏，特发性肾病综合征在6-17.5岁之间。据报道，明显的低藻症，感染和严重的肾病综合征病程是急性肾损伤发展的危险因素。Bohman等。[6]显示了脚步融合程度与GFR和过滤分数之间的密切关系，这表明脚步过程的融合可能导致肾小球滤波区域的降低以及/或对水和小溶质的渗透性。这种减少是暂时的，缓解后迅速恢复正常。范·沃尔（Van de Walle）等。[7]发现肾小球通透性的变化可能在急性肾衰竭中具有重要作用。此外，MESPGN是特发性肾病综合征的一种罕见形式。 Mubarak, et al. [8] reported the incidence of mesPGN as 4.81% and Gulati, et al. [9] as 7.6% in children. MesPGN is characterized by the proliferation of mesangial cells with an increase in the mesangial matrix and deposits in the mesangial region. Males are 2.5 times more likely to be affected than females [10]. 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].

MESPGN是儿童特发性肾病综合征的一种罕见形式。Anuria和急性肾脏受伤也是罕见的迹象。在这种情况下，应通过肾脏活检进行鉴别诊断。皮质类固醇疗法应成为MESPGN急性肾脏损伤的首选，以改善临床。

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