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目的探讨儿童脂蛋白肾病(LPG)的临床及预后。方法回顾性分析1例儿童LPG的临床资料,归纳总结国内外报道的儿童LPG的临床特点及预后。结果患儿,女,9岁,以尿频起病,初次尿检提示菌尿、血尿、蛋白尿,规律抗感染治疗1周后,仍有血尿、蛋白尿,血清白蛋白轻度降低,高脂血症,轻度贫血;肾脏组织活检,镜下可见肥大肾小球,扩张的肾小球毛细血管管腔,其内充以脂蛋白栓子,油红O染色阳性;电镜下多见层状或簇状“栓子”内含颗粒状脂质空泡。基因检测APOE Tokyo(Leu141-Lys143→0)。诊断为LPG,给予降脂治疗后病情明显缓解。结论儿童LPG罕见,血脂水平显著增高,激素治疗无效,肾脏穿刺活检是确诊的主要依据,基因检测提示其遗传背景;降脂治疗可缓解病情进展。
Objective To investigate the clinical and prognosis of children with lipoprotein nephropathy (LPG). Methods The clinical data of one child with LPG were retrospectively analyzed. The clinical features and prognosis of children with LPG were summarized. The results of children, 9-year-old children, onset of urinary frequency, the first urine test showed bacteriuria, hematuria, proteinuria, regular anti-infection treatment after 1 week, there is still hematuria, proteinuria, serum albumin mildly decreased, hyperlipidemia Symptoms, mild anemia; Kidney biopsy, microscopic hypertrophy glomerular, dilated glomerular capillary lumen filled with lipoprotein embolus, oil red O staining; electron microscopy more layered or Clustered “emboli ” contains granular lipid vacuoles. Gene assay APOE Tokyo (Leu141-Lys143 → 0). Diagnosis of LPG, given lipid-lowering therapy significantly alleviated the condition. Conclusions Children with LPG are rare, blood lipid levels are significantly increased, and hormone therapy is ineffective. Kidney biopsy is the main basis for diagnosis. Genetic testing indicates the genetic background. Lipid-lowering therapy may alleviate the disease progression.