[Ambulatory 24-hour blood pressure monitoring of children and young adults with autosome dominant polycystic kidney degeneration].
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder leading to terminal renal failure. About 8% of the dialysis patients suffer from ADPKD, the gene frequency in the general population being about 1:1000. Many facts contribute to the hypothesis that arterial hypertension plays a major role in the pathophysiology of ADPKD. We observed a prevalence of 30% of hypertension in patients with ADPKD and normal serum-creatinine, and of 80% in patients with terminal renal failure. The time of onset of abnormalities of blood pressure regulation is of great interest, since an increase of blood pressure, even in the normotensive range, accelerates the rate of progression. To answer this question, we examined the time of onset of abnormalities in blood-pressure regulation in 23 probands and 23 control patients in a cross-sectional study. The results document abnormal circadian blood-pressure changes and higher blood pressures, although still within the normotensive range, in asymptomatic carriers of the ADPKD-trait, even before and more definitely after onset of puberty. Even at an age when circadian blood pressure is not significantly different, there is an increased LVM as evidence of target organ damage. The findings suggest that (intermittent) increases in blood pressure and blood-pressure-dependent target organ damage precede overt hypertension and renal failure by years or decades.
Geberth
S
S
Sektion Nephrologie, Medizinischen Universitätsklinik, Heidelberg.
Zeier
M
M
Schmidt
K G
KG
Mandelbaum
A
A
Ritz
E
E
D000293:Adolescent; D000328:Adult; D001022:Aortic Valve Insufficiency / Q000235:genetics / Q000503:physiopathology; D001794:Blood Pressure / Q000502:physiology*; D015924:Blood Pressure Monitors; D002648:Child; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D002940:Circadian Rhythm / Q000502:physiology*; D005260:Female; D005799:Genes, Dominant / Q000235:genetics*; D006801:Humans; D006977:Hypertension, Renal / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D007690:Polycystic Kidney Diseases / Q000235:genetics* / Q000503:physiopathology; D016277:Ventricular Function, Left / Q000502:physiology
Ambulante 24-Stunden-Blutdruckmessung bei Kindern und jugendlichen Erwachsenen mit autosomal dominanter polyzystischer Nierendegeneration.