Abstract：
Autosomal recessive polycystic kidney disease (ARPKD) is a significant cause of morbidity and mortality in infants and children. In severe cases bilateral nephrectomies are considered but may be associated with significant neurological complications and life-threatening hypotension.
We describe a case of a 17 months old boy with genetically confirmed ARPKD who underwent sequential bilateral nephrectomies at the age of 4 and 10 months. Following the second nephrectomy the boy was started on continuous cycling peritoneal dialysis with blood pressure on the lower range. At the age of 12 months after a few days of poor feeding at home the boy experienced a severe episode of hypotension and coma of Glasgow Come Scale of three. Brain magnetic-resonance imaging (MRI) showed signs of hemorrhage, cytotoxic cerebral edema and diffuse cerebral atrophy. During the subsequent 72 h he developed seizures requiring anti-epileptic drug therapy, gradually regained consciousness but remained significantly hypotensive after discontinuation of vasopressors. Thus, he received high doses of sodium chloride orally and intraperitoneally as well as midodrine hydrochloride. His ultrafiltration (UF) was targeted to keep him in mild-to-moderate fluid overload. After two months of stable condition the patient started to develop hypertension requiring four antihypertensive medications. After optimizing peritoneal dialysis to avoid fluid overload and discontinuation of sodium chloride the antihypertensives were discontinued, but hyponatremia with hypotensive episodes reoccurred. Sodium chloride was reintroduced resulting in recurrent salt-dependent hypertension.
Our case report illustrates an unusual course of blood pressure changes following bilateral nephrectomies in an infant with ARPKD and the particular importance of tight regulation of sodium chloride supplementation. The case adds to the scarce literature about clinical sequences of bilateral nephrectomies in infants, and as well highlights the challenge of managing blood pressure in these patients. Further research on the mechanisms and management of blood pressure control is clearly needed.
We describe a case of a 17 months old boy with genetically confirmed ARPKD who underwent sequential bilateral nephrectomies at the age of 4 and 10 months. Following the second nephrectomy the boy was started on continuous cycling peritoneal dialysis with blood pressure on the lower range. At the age of 12 months after a few days of poor feeding at home the boy experienced a severe episode of hypotension and coma of Glasgow Come Scale of three. Brain magnetic-resonance imaging (MRI) showed signs of hemorrhage, cytotoxic cerebral edema and diffuse cerebral atrophy. During the subsequent 72 h he developed seizures requiring anti-epileptic drug therapy, gradually regained consciousness but remained significantly hypotensive after discontinuation of vasopressors. Thus, he received high doses of sodium chloride orally and intraperitoneally as well as midodrine hydrochloride. His ultrafiltration (UF) was targeted to keep him in mild-to-moderate fluid overload. After two months of stable condition the patient started to develop hypertension requiring four antihypertensive medications. After optimizing peritoneal dialysis to avoid fluid overload and discontinuation of sodium chloride the antihypertensives were discontinued, but hyponatremia with hypotensive episodes reoccurred. Sodium chloride was reintroduced resulting in recurrent salt-dependent hypertension.
Our case report illustrates an unusual course of blood pressure changes following bilateral nephrectomies in an infant with ARPKD and the particular importance of tight regulation of sodium chloride supplementation. The case adds to the scarce literature about clinical sequences of bilateral nephrectomies in infants, and as well highlights the challenge of managing blood pressure in these patients. Further research on the mechanisms and management of blood pressure control is clearly needed.
摘要：
背景：常染色体隐性遗传性多囊肾病（ARPKD）是婴儿和儿童发病和死亡的重要原因。在严重的情况下，考虑双侧肾切除术，但可能与严重的神经系统并发症和危及生命的低血压有关。
方法：我们描述了一个17个月大的经基因证实的ARPKD男孩，在4个月和10个月时接受了连续的双侧肾切除术。第二次肾切除术后，男孩开始进行连续循环腹膜透析，血压范围较低。在12个月大的时候，男孩在家里吃得不好几天后，经历了严重的低血压和格拉斯哥昏迷3级昏迷。脑部磁共振成像（MRI）显示出血迹象，细胞毒性脑水肿和弥漫性脑萎缩。在随后的72小时内，他出现了需要抗癫痫药物治疗的癫痫发作，逐渐恢复意识，但在停用血管加压剂后仍然明显低血压。因此，他口服和腹膜内接受了高剂量的氯化钠以及盐酸米多君。他的超滤（UF）旨在使他处于轻度至中度的液体超负荷状态。病情稳定两个月后，患者开始发展为高血压，需要四种抗高血压药物。在优化腹膜透析以避免液体超负荷和停用氯化钠后，停用抗高血压药物，但低钠血症伴低血压发作复发.重新引入氯化钠，导致盐依赖性高血压复发。
结论：我们的病例报告说明了ARPKD婴儿双侧肾切除术后血压异常变化的过程，以及严格控制氯化钠补充剂的重要性。该病例增加了有关婴儿双侧肾切除术临床序列的稀缺文献，并强调了控制这些患者血压的挑战。显然需要进一步研究血压控制的机制和管理。
方法：我们描述了一个17个月大的经基因证实的ARPKD男孩，在4个月和10个月时接受了连续的双侧肾切除术。第二次肾切除术后，男孩开始进行连续循环腹膜透析，血压范围较低。在12个月大的时候，男孩在家里吃得不好几天后，经历了严重的低血压和格拉斯哥昏迷3级昏迷。脑部磁共振成像（MRI）显示出血迹象，细胞毒性脑水肿和弥漫性脑萎缩。在随后的72小时内，他出现了需要抗癫痫药物治疗的癫痫发作，逐渐恢复意识，但在停用血管加压剂后仍然明显低血压。因此，他口服和腹膜内接受了高剂量的氯化钠以及盐酸米多君。他的超滤（UF）旨在使他处于轻度至中度的液体超负荷状态。病情稳定两个月后，患者开始发展为高血压，需要四种抗高血压药物。在优化腹膜透析以避免液体超负荷和停用氯化钠后，停用抗高血压药物，但低钠血症伴低血压发作复发.重新引入氯化钠，导致盐依赖性高血压复发。
结论：我们的病例报告说明了ARPKD婴儿双侧肾切除术后血压异常变化的过程，以及严格控制氯化钠补充剂的重要性。该病例增加了有关婴儿双侧肾切除术临床序列的稀缺文献，并强调了控制这些患者血压的挑战。显然需要进一步研究血压控制的机制和管理。
