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[高血容量对常染色体显性遗传性多囊肾病患者电解质水平及容量调节激素水平的影响]

[The effect of hypervolemia on electrolyte level and and level of volume regulating hormones in patients with autosomal dominant polycystic kidney disease].

作者信息

Michalski A, Grzeszczak W

机构信息

Katedra i Klinika Chorób Wewnetrznych i Zawodowych Slaskiej Akademii Medycznej w Zabrzu.

出版信息

Pol Arch Med Wewn. 1996 Oct;96(4):329-43.

PMID:9082344
Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most frequent autosomal dominant diseases. Apart from kidneys it also includes alimentary system, nervous system, cardiovascular system. ADPKD is associated with endocrinal disorders of the hormones regulating volume, arterial blood pressure and water and electrolyte balance such as: ARO, AVP, Aldo. 24 patients with ADPKD (12 with normal renal function-gr. III, 12 with advanced renal insufficiency-gr. IV) and 15 healthy subjects to compare with-gr. I and 16 patients with advanced renal insufficiency of other origin than ADPKD-gr. II were examined. In all groups the levels of Aldo, AVP and ARO in blood and Na+, K+ and creatinine concentrations in blood serum were examined Also an excretion of Na, K, creatinine with urine and clearances: CNa, CK, CKrea and filtration fractions: FENa and FEK were determined. Arterial blood pressure was measured in all groups. The above described parameters were studied in standard conditions in patients in supine position and fasting-survey I; directly after intravenous infusion of 1000 ml 0.16 M NaCl at 16.7 ml/min for 1 h-survey II and two hours after intravenous infusion-survey III. Isotonic intravenous infusion of natrium chloride increased central blood volume in the examined patients (induced hypervolemia). The received results were compared among groups in standard conditions (before infusion) as well as reaction of all groups to increased central volemia was compared. On the basis of the received results it was observed that the patients with ADPKD with normal renal function (gr III) show a significant increase of ARO, AVP, arterial blood pressure what differs them from healthy individuals (gr. I). The increased values of the above mentioned parameters were typical for the patients with chronic renal insufficiency regardless to a cause of the failure (gr. II). Consequently, patients with ADPKD showed some hormonal disorders typical for patients with advanced renal insufficiency despite fairly big difference in creatinine level and renal function condition among groups. Comparing the groups with advanced renal insufficiency (gr. II and gr. IV) it was shown that despite the same creatinine and electrolytes level in blood serum they exhibited different renine activity of serum and arterial blood pressure. These parameters were higher in the group with ADPKD. After volume expansion by 1000 ml 0.16 M NaCl infusion no significant differences between renal response to induced hypervolemia in patients with ADPKD and control groups were observed. The received results show that the patients with ADPKD exhibit disorders in hormonal regulation, water and electrolyte balance and in value of arterial blood pressure regardless to a degree of renal function. Thus genetic disease alone predisposes to the above mentioned disorder. Nevertheless, patients with ADPKD show normal mechanisms of renal volemic regulation in volume expansion conditions that can be compared to control groups. It also means that renal response to induced hypervolemia is similar in all the examined groups and is independent of renal function degree.

摘要

常染色体显性多囊肾病(ADPKD)是最常见的常染色体显性疾病之一。除了肾脏外,它还累及消化系统、神经系统和心血管系统。ADPKD与调节血容量、动脉血压以及水和电解质平衡的激素的内分泌紊乱有关,如:促红细胞生成素(ARO)、抗利尿激素(AVP)、醛固酮(Aldo)。研究了24例ADPKD患者(12例肾功能正常——III级,12例晚期肾功能不全——IV级)以及15名作为对照的健康受试者——I级,还有16例非ADPKD所致晚期肾功能不全的患者——II级。检测了所有组血液中醛固酮、抗利尿激素和促红细胞生成素的水平以及血清中钠、钾和肌酐的浓度。还测定了尿中钠、钾、肌酐的排泄量以及清除率:钠清除率(CNa)、钾清除率(CK)、肌酐清除率(CKrea)和滤过分数:滤过钠分数(FENa)和滤过钾分数(FEK)。测量了所有组的动脉血压。在标准条件下对患者进行上述参数的研究,患者仰卧位且禁食——调查I;以16.7 ml/分钟的速度静脉输注1000 ml 0.16 M氯化钠1小时后——调查II;静脉输注两小时后——调查III。等渗氯化钠静脉输注增加了受试患者的中心血容量(诱发高血容量)。将标准条件下(输注前)各组的所得结果进行比较,同时比较所有组对中心血容量增加的反应。根据所得结果观察到,肾功能正常的ADPKD患者(III级)促红细胞生成素、抗利尿激素、动脉血压显著升高,这使其有别于健康个体(I级)。无论肾衰竭的病因如何(II级),上述参数升高的值对于慢性肾功能不全患者来说是典型的。因此,ADPKD患者尽管各组间肌酐水平和肾功能状况差异很大,但仍表现出一些晚期肾功能不全患者典型的激素紊乱。比较晚期肾功能不全组(II级和IV级)发现,尽管血清肌酐和电解质水平相同,但它们的血清肾素活性和动脉血压不同。ADPKD组的这些参数更高。在输注1000 ml 0.16 M氯化钠使血容量增加后,未观察到ADPKD患者与对照组对诱发高血容量的肾脏反应有显著差异。所得结果表明,无论肾功能程度如何,ADPKD患者在激素调节、水和电解质平衡以及动脉血压值方面均存在紊乱。因此,仅这种遗传性疾病就易导致上述紊乱。然而,ADPKD患者在血容量增加的情况下表现出正常的肾脏血容量调节机制,这可与对照组相比较。这也意味着在所有受试组中,对诱发高血容量的肾脏反应相似,且与肾功能程度无关。

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