Vergesslich K A, Ponhold W, Balzar E, Syrè G, Ulrich W
Wien Klin Wochenschr. 1986 May 30;98(11):351-5.
Familial juvenile nephronophthisis (FJN) represents an important cause of chronic renal insufficiency in the first two decades of life. Its frequency is reported to vary between 7 and 20% of all cases of terminal renal failure in childhood. Usually the onset is insidious, with polyuria, polydipsia and anaemia being the main clinical features. The diagnosis is based on clinical, laboratory and pathological findings. The purpose of our report is to emphasize the importance of this pathological entity with respect to the clinical symptoms and signs and diagnostic approach on the basis of the case reports of four patients.
家族性青少年肾单位肾痨（FJN）是儿童期前二十年慢性肾功能不全的一个重要病因。据报道，其在儿童终末期肾衰竭所有病例中的发生率在7%至20%之间。通常起病隐匿，多尿、烦渴和贫血是主要临床特征。诊断基于临床、实验室和病理检查结果。我们报告的目的是根据4例患者的病例报告，强调这一病理实体在临床症状体征及诊断方法方面的重要性。
