Renal, cardiovascular and hormonal characteristics of young adults with autosomal dominant polycystic kidney disease.
We studied young adults with autosomal dominant polycystic kidney disease (ADPKD) to determine the characteristics that precede renal impairment. Nineteen affected (A) and 20 unaffected (U) offspring from families with ADPKD showed no significant differences in basal glomerular filtration rate (A: mean 97, SD 19; U: 100, SD 23 ml/min/1.73 m2) or renal functional reserve, but effective renal plasma flow was significantly lower in affected offspring (A: 532, SD 86; U: 605, SD 118 ml/min/1.73 m2, P less than 0.01). Plasma renin activity [A: median 26 (95% CI: 15 to 37); U: 14 (11 to 27) microU/ml, P less than 0.05, one-tailed test] and aldosterone [A: 2.5 (2.0 to 3.0), U: 1.0 (1.5 to 2.0) micrograms/100 ml, P less than 0.04, one-tailed test] were increased in affected offspring despite the higher systolic blood pressure (A: mean 123, SD 5; U: 115, SD 3 mm Hg, P less than 0.02) and significant expansion of total exchangeable sodium (A: 40.8, SD 2.3; U: 38.0, SD 3.5 mmol/kg, P less than 0.01). The ouabain-sensitive component of red cell sodium efflux was less in affected offspring (A: 0.258; SD 0.040; U: 0.288, SD 0.042 hr-1, P less than 0.04) and in both groups was correlated inversely with total exchangeable sodium. Echocardiography revealed no difference in left ventricular mass index nor prevalence of mitral valve prolapse. Potential cyst growth factors such as the glucocorticoids and somatomedin C were similar in both affected and unaffected groups.(ABSTRACT TRUNCATED AT 250 WORDS)
Harrap
S B
SB
MRC Blood Pressure Unit, Western Infirmary, Glasgow, Scotland, United Kingdom.
Davies
D L
DL
Macnicol
A M
AM
Dominiczak
A F
AF
Fraser
R
R
Wright
A F
AF
Watson
M L
ML
Briggs
J D
JD
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000097:blood; D000328:Adult; D000450:Aldosterone / Q000097:blood*; D009320:Atrial Natriuretic Factor / Q000097:blood; D004044:Dietary Proteins; D005799:Genes, Dominant; D005919:Glomerular Filtration Rate; D006439:Hemodynamics; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D007668:Kidney / Q000503:physiopathology*; D016891:Polycystic Kidney, Autosomal Dominant / Q000097:blood / Q000235:genetics / Q000503:physiopathology*; D011188:Potassium / Q000493:pharmacokinetics; D012079:Renal Circulation; D012083:Renin / Q000097:blood*; D012964:Sodium / Q000493:pharmacokinetics
D000305:Adrenal Cortex Hormones; D004044:Dietary Proteins; D000450:Aldosterone; D007334:Insulin-Like Growth Factor I; D009320:Atrial Natriuretic Factor; D012964:Sodium; D012083:Renin; D011188:Potassium