Autosomal-dominant polycystic kidney disease in infancy and childhood: Progression and outcome
Autosomal-dominant polycystic kidney disease in infancy and childhood: Progression and outcome
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DOI：
10.1111/j.1523-1755.2005.00678.x
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发表时间：
2005-11-01
影响因子：
19.6
通讯作者：
Schrier, RW
中科院分区：
文献类型：
--
作者：
Shamshirsaz, A;Bekheirnia, RM;Schrier, RW
常染色体显性多囊肾病在婴儿和儿童：进展和结果。儿童和婴儿常染色体显性遗传性多囊肾病（ADPKD）的自然史尚未得到很好的描述。对46例出生前18个月（VEO）诊断为ADPKD的儿童和153例出生后18个月至18岁（非VEO）诊断为ADPKD的儿童的临床特征进行分析。当调整年龄时，VEO儿童比非VEO儿童有更多的囊肿和更大的肾脏体积。在VEO和非VEO儿童中，诊断时存在体征或症状以及研究访视时存在血尿或蛋白尿均与较大的肾体积相关。早期诊断（VEO）或由于体征或症状而诊断的儿童也更有可能患有高血压。在随访期间，2例VEO患儿和0例非VEO患儿达到终末期肾病。与许多已发表的病例报告表明VEO儿童发生早期终末期肾病相比，本研究的结果更为乐观。超过90%的VEO儿童在童年时期保持了良好的肾功能。
Autosomal-dominant polycystic kidney disease in infancy and childhood: Progression and outcome.Background. The natural history of autosomal-dominant polycystic kidney disease (ADPKD) has not been well described in children and infants.Methods. The present study analyzed the characteristics of 46 ADPKD children diagnosed before 18 months of life (VEO) and 153 children diagnosed between 18 months of age and 18 years of age (non-VEO).Results. VEO children had more cysts and larger renal volumes than non-VEO children when adjusted for age. In both VEO and non-VEO children, the presence of signs or symptoms at the time of diagnosis as well as the presence of hematuria or proteinuria at the study visit were associated with larger renal volumes. Children diagnosed early (VEO) or diagnosed due to signs or symptoms were also more likely to have high blood pressure. Two VEO children and no non-VEO children reached end-stage renal disease during follow-up.Conclusion. In contrast to many published case reports suggesting the occurrence of early end-stage renal disease in VEO children, the results of the present study were much more optimistic. Over 90% of the VEO children maintained preserved renal function well into childhood.