Sefer S, Trotić R, Lacmanović V, Degoricija V, Ratković-Gusić I, Kes P
Department of Nephrology and Dialysis, Sisters of Mercy University Hospital, Zagreb, Croatia.
Croat Med J. 2000 Jun;41(2):203-6.
To present a unique case of Alport's syndrome in monozygotic twins with two different treatment modalities - renal transplantation and hemodialysis, and to evaluate the effects of therapy on hearing and ophthalmological findings.
Pure-tone audiogram and ophthalmologic examinations were performed in both twins at the age of 30. At the age of 46, 4 years after renal transplantation in the first twin and after 6 years of hemodialysis in the second twin, both twins underwent control audiometric and ophthalmologic examinations.
Control audiometric measurements showed the progression of bilateral sensorineural hearing loss in the high-frequency range (>2,000 Hz) in both twins. The hearing threshold progressed from initial 50 dB in both twins at the time of the diagnosis to 55 dB in the twin on hemodialysis, and 85 dB in the twin with a transplanted kidney. Retinal blurry hyperpigmentations disappeared in the twin with a transplanted kidney.
In comparison with hemodialysis, renal transplantation in Alport's syndrome may have deleterious effect on hearing, when associated with plasma hyperviscosity and hyperlipidemia, but may lead to regression of retinal hyperpigmentation.
呈现一对单卵双胞胎患奥尔波特综合征采用两种不同治疗方式（肾移植和血液透析）的独特病例，并评估治疗对听力和眼科检查结果的影响。
在这对双胞胎30岁时进行纯音听力图和眼科检查。在46岁时，即第一个双胞胎肾移植4年后和第二个双胞胎血液透析6年后，两人均接受了听力和眼科对照检查。
对照听力测量显示，两个双胞胎在高频范围（>2000 Hz）均出现双侧感音神经性听力损失进展。听力阈值从诊断时两人最初的50分贝进展到血液透析的双胞胎为55分贝，肾移植双胞胎为85分贝。肾移植双胞胎的视网膜模糊色素沉着消失。
与血液透析相比，奥尔波特综合征患者进行肾移植时，若伴有血浆高黏滞血症和高脂血症，可能对听力产生有害影响，但可能导致视网膜色素沉着消退。
