Autosomal recessive polycystic kidney disease: Long-term outcome of neonatal survivors
Autosomal recessive polycystic kidney disease: Long-term outcome of neonatal survivors
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DOI：
10.1007/s004670050281
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发表时间：
1997-06-01
影响因子：
3
通讯作者：
Barratt, TM
中科院分区：
文献类型：
--
作者：
Roy, S;Dillon, MJ;Barratt, TM
常染色体隐性遗传性多囊肾病会导致儿童时期的肾和肝功能障碍。我们描述了 1950 年至 1993 年间出生的 52 名患有此诊断的儿童的临床结果。目前，23 名儿童存活，24 名死亡，5 名失访； 1例已透析，7例已移植。对生命第一个月幸存的患者的生命表分析显示，精算肾存活率在 1 年时为 86%，在 15 年时为 67%。 1 岁时需要抗高血压治疗的概率为 39%，15 岁时为 60%。 8 名患者出现胃食管静脉曲张出血，平均年龄 12.5 岁，其中 6 名患者出现脾功能亢进的血液学证据。该研究表明，在新生儿期存活下来的这种疾病患者的预后相对较好，并强调了早期发现和适当治疗全身性和门静脉高压的重要性。
Autosomal recessive polycystic kidney disease causes renal and hepatic dysfunction in childhood. We describe the clinical outcome of 52 children with this diagnosis born between 1950 and 1993. Currently 23 are alive, 24 dead and 5 have been lost to follow-up; 1 has been dialysed and 7 transplanted. Life-table analysis of the patients surviving the Ist month of life revealed an actuarial renal survival of 86% at 1 year and 67% at 15 years. The probability of requiring anti-hypertensive treatment was 39% at 1 year and 60% at 15 years of age. Bleeding from gastro-oesophageal varices occurred in 8 patients at a mean age of 12.5 years, and was preceded by haematological evidence of hypersplenism in 6 of them. The study indicates a relatively good prognosis for patients with this condition who survive the neonatal period and emphasises the importance of early detection and appropriate management of systemic and portal hypertension.