ABSTRACT
Background: The clinical manifestations of autosomal dominant polycystic kidney disease (ADPKD) usually appear in adulthood, however pediatric series report a high morbidity. The objective of the study was to analyze the clinical characteristics of ADPKD in young adults. Methods: Family history, hypertension, albuminuria, estimated glomerular filtration rate (eGFR) and imaging tests were examined in 346 young adults (18-30 years old) out of 2521 patients in the Spanish ADPKD registry (REPQRAD). A literature review searched for reports on hypertension in series with more than 50 young (age <30 years) ADPKD patients. Results: The mean age of this young adult cohort was 25.24 (SD 3.72) years. The mean age at diagnosis of hypertension was 21.15 (SD 4.62) years, while in the overall REPQRAD population was aged 37.6 years. The prevalence of hypertension was 28.03% and increased with age (18-24 years, 16.8%; 25-30 years, 36.8%). Although prevalence was lower in women than in men, the age at onset of hypertension (21 years) was similar in both sexes. Mean eGFR was 108 (SD 21) mL/min/1.73 m2, 38.0% had liver cysts and 3.45% of those studied had intracranial aneurysms. In multivariate analyses, hematuria episodes and kidney length were independent predictors of hypertension (area under the curve 0.75). The prevalence of hypertension in 22 pediatric cohorts was 20%-40%, but no literature reports on hypertension in young ADPKD adults were found. Conclusions: Young adults present non-negligible ADPKD-related morbidity. This supports the need for a thorough assessment of young adults at risk of ADPKD that allows early diagnosis and treatment of hypertension.
ABSTRACT
Membrane bioincompatibility was demonstrated by successive white blood cell counts and C3a generation. Pulse wave analysis was obtained by applanation tonometry (SphygmoCor) in a sequential way: basal, after 30 minutes with nul ultrafiltration, and after a complete dialysis with ultrafiltration. At 15 minutes of haemodialysis, significant decrease in leukocyte count occurred: 6801 ± 1186 versus 4412 ± 1333 (P < 0.001), while C3a levels sharply increased from 427 ± 269 to 3501 ± 1638 ng/mL (P < 0.000). No changes were observed in augmentation index without ultrafiltration: 26.1 ± 11.1 versus 26.6 ± 12.4. Only aortic systolic blood pressure was lower at 15 minutes: 120.1 ± 17.7 versus 110.4 ± 25.8 mmHg (P = 0.009), in agreement with a reduction in brachial systolic blood pressure: 135.1 ± 18.1 versus 122.7 ± 27.4 mmHg (P = 0.01), without changes in aortic or brachial diastolic blood pressure. Important changes in pulse wave analysis were observed after a complete haemodialysis session: augmentation index 29.9 ± 10.1 versus 18.6 ± 15.0, aortic systolic blood pressure 139.8 ± 25.5 versus 119.4 ± 28.5 mmHg (P < 0.00), without changes in aortic diastolic blood pressure. In summary, haemodialysis with cellulose diacetate acutely induced a transient state of immunoactivation due to bioincompatibility, this phenomenon was nondetectable by pulse wave analysis. Complete haemodialysis session led to important changes in pulse wave analysis.
ABSTRACT
Disturbances of mineral metabolism occur during the early stages of chronic kidney disease. As renal function worsens, excess dietary phosphorus accumulates and blood levels increase, that can be clearly seen when the glomerular filtration rate has fallen below 30 ml/min/1.73 m2. In patients with end stage renal disease, standard dialysis (three times/week) falls far short of removing adequate amounts of absorbed phosphorus; therefore, hyperphosphataemia is found in the majority of these patients. Hyperphosphataemia has long been associated with progression of secondary hyperparathyroidism and renal osteodystrophy, it can also lead to soft-tissue and vascular calcification. Recent observational data have associated hyperphosphataemia with increased cardiovascular mortality among dialysis patients. Adequate control of serum phosphorus remains a cornerstone in the clinical management and, despite the growing amount of available therapeutic options, achievement of NFK/KDOQI targets for mineral metabolism remain poor. Several reasons may explain the failure to adequately treat hyperphosphataemia: poor compliance with diet and phosphate binder prescriptions are common causes. Also, factors related with cost, tolerance, palatability, safety and efficacy are important. In this article, the authors review the advantages and drawbacks of conventional and emerging therapies in phosphorous binding.
