脂蛋白肾病1例报道及文献复习

Lipoprotein Nephropathy: A Case Report and Literature Review

摘要: 目的：加深对脂蛋白肾病的临床认识。方法：回顾性分析2017年6月川北医学院附属医院肾内科收治1例脂蛋白肾病患者的临床特征、实验室检查指标、诊疗经过及治疗转归，并结合相关文献复习展开讨论。结果：患者，50岁，男性，以反复颜面及双下肢浮肿为主要临床表现，考虑为肾病综合症，给予激素及免疫抑制剂治疗后症状缓解不明显，实验室指标加重，行肾穿刺活检术诊断为脂蛋白肾病，给予调脂、减少尿蛋白、保肾等治疗后，患者浮肿消退，白蛋白、血脂恢复正常范围，肾功能无明显恶化，进入临床随访观察阶段。结论：脂蛋白肾病是一种罕见的遗传性肾脏疾病，发病率低，以不同程度蛋白尿为主要表现，对激素、细胞毒性药物治疗无效，后期多进展为慢性肾功能衰竭，预后差。需引起广大临床医师的重视。

Abstract: Objective: To deepen the clinical understanding of lipoprotein nephropathy. Methods: A case of lipoprotein nephropathy admitted to the Department of Nephrology of the Affiliated Hospital of North Sichuan Medical College in June 2017 was selected. The clinical characteristics, laboratory examination indicators, diagnosis, treatment and transformation and development of the disease were retrospectively analyzed, and discussed with related literature review. Results: The patient was a 50 years old male. Repeated facial and lower extremity edema was the main clinical mani-festation. Nephrotic syndrome was diagnosed. Symptoms were not relieved after treatment with hormones and immunosuppressants. Laboratory indicators were aggravated. Renal biopsy was performed to diagnose lipoprotein nephropathy. After treatment with lipid lowering, urinary protein lowering and kidney protection, edema subsided, albumin and blood lipid returned to normal range, renal function did not deteriorate significantly. He then went into the follow-up ob-servation stage clinically. Conclusion: Lipoprotein nephropathy is a rare hereditary kidney disease with low incidence. It is mainly manifested by proteinuria of different degrees. It has no effect on hormones and cytotoxic drugs. It usually develops into chronic renal failure in later stages and poor prognosis. It needs to attract the attention of clinicians.