Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
Pediatr Nephrol. 2018 Apr;33(4):595-604. doi: 10.1007/s00467-017-3686-4. Epub 2017 May 29.
Life with a solitary functioning kidney (SFK) may be different from that when born with two kidneys. Based on the hyperfiltration hypothesis, a SFK may lead to glomerular damage with hypertension, albuminuria and progression towards end-stage renal disease. As the prognosis of kidney donors was considered to be very good, having a SFK has been considered to be a benign condition. In contrast, our research group has demonstrated that being born with or acquiring a SFK in childhood results in renal injury before adulthood in over 50% of those affected. Most congenital cases will be detected during antenatal ultrasound screening, but up to 38% of cases of unilateral renal agenesis are missed. In about 25-50% of cases of antenatally detected SFK there will be signs of hypertrophy, which could indicate additional nephron formation and is associated with a somewhat reduced risk of renal injury. Additional renal and extrarenal anomalies are frequently detected and may denote a genetic cause for the SFK, even though for the majority of cases no explanation can (yet) be found. The ongoing glomerular hyperfiltration results in renal injury, for which early markers are lacking. Individuals with SFK should avoid obesity and excessive salt intake to limit additional hyperfiltration. As conditions like hypertension, albuminuria and a mildly reduced glomerular filtration rate generally do not result in specific complaints but may pose a threat to long-term health, screening for renal injury in any individual with a SFK would appear to be imperative, starting from infancy. With early treatment, secondary consequences may be diminished, thereby providing the optimal life for anyone born with a SFK.
患有孤立功能肾(SFK)的生活可能与天生拥有两个肾脏的生活不同。基于超滤假说,SFK 可能导致肾小球损伤,进而导致高血压、蛋白尿和进展为终末期肾病。由于肾供体的预后被认为非常好,因此 SFK 被认为是一种良性疾病。相比之下,我们的研究小组已经证明,在儿童时期患有或获得 SFK 会导致超过 50%的受影响者在成年前发生肾脏损伤。大多数先天性病例将在产前超声筛查中检测到,但多达 38%的单侧肾发育不全病例会被遗漏。在大约 25-50%的产前检测到的 SFK 病例中,会出现肥大的迹象,这可能表明额外的肾单位形成,并且与肾脏损伤的风险略有降低相关。经常会检测到额外的肾脏和肾外异常,并可能表示 SFK 的遗传原因,尽管对于大多数病例,目前还无法找到(确切)解释。持续的肾小球超滤导致肾脏损伤,但缺乏早期标志物。SFK 患者应避免肥胖和过度盐摄入,以限制额外的超滤。由于高血压、蛋白尿和肾小球滤过率轻度降低等情况通常不会导致特定的症状,但可能对长期健康构成威胁,因此对任何患有 SFK 的个体进行肾脏损伤筛查似乎是必要的,应从婴儿期开始。通过早期治疗,可以减轻继发性后果,从而为任何患有 SFK 的人提供最佳生活。