IR@PKUHSC  > 北京大学第一临床医学院  > 肾脏内科
其他题名Clinicopathological manifestations of nephronophthisis-medullary cystic kidney disease
王素霞1; 章友康1; 周福德1; 王仁贵1; 屈海云1; 邹万忠1
关键词肾单位 肾痨 肾髓质 囊肿 病理学 Nephron Nephrophthisis Kidney Medulla Cysts Pathology
收录类别中国科技核心期刊 ; 中文核心期刊 ; CSCD
文章类型Journal Article
摘要目的 报道两例.肾单位肾痨-髓质囊肿病(NPH-MCKD)的临床病理特点.方法 分析本院诊断的两例NPH-MCKD的临床资料及肾活检组织的病理改变,并结合文献复习,探讨NPH-MCKD的临床病理特点及其诊断方法.结果 两例均为青年患者,首发症状为烦渴、多尿,低相对密度尿,轻度蛋白尿,尿沉渣无明显异常,肾小管浓缩功能及酸化功能下降.2例均有轻度肾功能不全,无肾脏病家族史,无肾外损害表现.B超可见肾实质回声增强,皮髓质分界结构不清;其中1例CT可见双肾的多发小囊肿,主要分布于皮髓质交界处.肾活检病理检查:2例均以弥漫性肾小管间质损伤为特点,表现为肾小管基底膜破坏、肾小管萎缩及囊性扩张、肾间质纤维化的三联征;可见部分肾小球硬化.皮髓质交界处.肾小管扩张形成多发囊肿是NPH-MCKD的特征性病理改变.结论 以肾小管功能受损为突出表现的青少年患者要高度怀疑NPH-MCKD.B超或CT检查可提供重要线索.肾活检病理检查见到皮髓质交界处多发性小囊肿形成可确诊本病. Objective To report the clinicopathological features of 2 cases of nephronophthisis-medullary cystic kidney disease (NPH-MCKD). Methods The clinical data and pathological changes of renal biopsy in two patients of NPH-MCKD from our hospital were analyzed, and associated literatures were reviewed simultanously. The clinicopathological featuresand diagnosis of NPH-MCKD were discussed. Results Two adolescent patients were admitted to our hospital for indolent renal insufficiency, polyuria accompanied by polydipsia as first signs.Urine analysis showed low specific density urine, mild proteinuria, and few formed elements in urinary sediments. The ability of urine concentration and acidification was decreased. Familial history of renal disease and extra-renal lesions were not found. Renal ultrasound presented an increased echogenicity with diminished cortico-meduUary differentiation, and multiple small cysts in renal corticomedullary border were identified in one case by computed tomography. Pathological examination of renal biopsy revealed diffuse tubular interstitial lesion which was characterized by the triad of tubular basement disintegration, tubular atrophy with cyst development, and interstitial fibrosis. Some of glomerular sclerosis occurred. Cyst development at the corticomedullary border of the kidneys was the specific feature of NPH-MCKD. Conclusions Young patients with impaired tubular function should be suspected of NPH-MCKD. Renal ultrasound or computed tomography can provide an important clue. Multiple renal cysts at the corticomedullary border identified by renal biopsy can be a diagnostic indication for NPH-MCKD.
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GB/T 7714
王素霞,章友康,周福德,等. 肾单位肾痨-髓质囊肿病的临床病理[J]. 中华肾脏病杂志,2008,24(7):461-465.
APA 王素霞,章友康,周福德,王仁贵,屈海云,&邹万忠.(2008).肾单位肾痨-髓质囊肿病的临床病理.中华肾脏病杂志,24(7),461-465.
MLA 王素霞,et al."肾单位肾痨-髓质囊肿病的临床病理".中华肾脏病杂志 24.7(2008):461-465.
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