Volume Progression in Autosomal Dominant Polycystic Kidney Disease

Abstract

Autosomal dominant polycystic kidney disease (PKD) is a hereditary condition characterized by the progressive enlargement of innumerable renal cysts that contribute to life-altering morbidity early in the course of the disease. Evidence indicates that the rate of increase in kidney volume can be reliably measured by magnetic resonance or computed tomography imaging, thus providing objective means to judge the effectiveness of therapies that are targeted to the aberrant growth of renal tubules. It is now possible, therefore, to monitor the effectiveness of potential therapies on the signature abnormality in autosomal dominant PKD before irreversible damage has been done by the cysts. Evidence accumulated from human cross-sectional and longitudinal studies and longitudinal studies of PKD models in animals provide strong support for the view that reducing the rate of kidney volume enlargement will ameliorate the late-stage development of renal insufficiency.

Quality of Life in Autosomal Dominant Polycystic Kidney Disease Patients not yet on Dialysis

“…Therefore, it is likely that the SF-36 questionnaire is not sensitive to detect changes in quality of life in ADPKD patients. Given the association between renal size and gross hematuria and pain, as well as the close relationship between kidney size and GFR (22), it was anticipated that renal volume would inversely relate to SF-36 scores, particularly the PCS component, in this study. However, no relationship between increasing renal volume or renal volumes Ͼ1000 ml and PCS, MCS, or individual component scores could be found.…”

Tradução, adaptação cultural e aplicação de um questionário de dor para pacientes com doença renal policística

“…6,15,22,32 The use of that questionnaire has also evidenced that the presence of a family Translation, cultural adaptation and aplication of a pain questionnaire for patients with polycystic kidney disease history of ADPKD was the finding that most frequently led to the diagnosis of ADPKD (55% of the cases). However, low back pain was the symptom most frequently reported by the patients (13%), and, when added to abdominal pain, it reached 22% of the patients, confirming that pain can contribute to the diagnosis of ADPKD.…”

“…4,5 Among the renal manifestations of ADPKD, the following are the most common: arterial hypertension; urinary infection; nephrolithiasis; low back pain; hematuria; increased kidney volume; and progressive loss of renal function. 6 Arterial hypertension is an early and frequent complication of ADPKD. It affects 60% of the patients, even before renal function impairment, probably due to stimulation of the renin-angiotensin--aldosterone system caused by the growth of the cysts.…”

“…The low back and/or abdominal pain in patients with ADPKD is multifactorial. It can occur acutely due to cyst rupture or infection, kidney stone elimination, and UTI, or can be chronic resulting from increased kidney or liver volume, due to cyst expansion, 6 or even from diverticulitis. As the cysts are associated with excessive angiogenesis, polycystic kidneys are specially susceptible to traumas, which can lead to hemorrhage or bleeding in the retroperitoneal space, usually accompanied by intense pain.…”

Two autosomal dominant polycystic kidney (ADPKD) cases with advanced renal dysfunction, effectively treated with tolvaptan

“…It is already known that tolvaptan can prevent vasopressin-mediated water reabsorption in the kidneys by competitively blocking the binding of vasopressin to its V2 receptors (V2Rs), resulting in aquaresis without changing electrolyte excretion [3,4]. The disease changes the kidney structure and function through massive growth of numerous fluid-filled cysts and cell proliferation [1,5]. Higher rates of kidney enlargement and larger kidney volume in Japanese ADPKD patients are associated with a more rapid decrease in kidney function [6].…”