Longitudinal Assessment of Left Ventricular Mass in Autosomal Dominant Polycystic Kidney Disease.

INTRODUCTION: The high burden of cardiovascular morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD) is related to development of hypertension and left ventricular hypertrophy. Blood pressure reduction has been shown to reduce left ventricular mass in ADPKD; however, moderators and predictors of response to lower blood pressure are unknown. METHODS: This was a post hoc cohort analysis of HALT PKD study A, a randomized placebo controlled trial examining the effect of low blood pressure and single versus dual renin-angiotensin blockade in early ADPKD. Participants were hypertensive ADPKD patients 15 to 49 years of age with estimated glomerular filtration rate (eGFR) > 60 ml/min per 1.73 m2 across 7 centers in the United States. Predictors included age, sex, baseline eGFR, systolic blood pressure, total kidney volume, serum potassium, and urine sodium, potassium, albumin, and aldosterone. Outcome was left ventricular mass index (LVMI) measured using 1.5-T magnetic resonance imaging at months 0, 24, 48, and 60. RESULTS: Reduction in LVMI was associated with higher baseline systolic blood pressure and larger kidney volume regardless of blood pressure control group assignment (P < 0.001 for both). Male sex and baseline eGFR were associated with a positive annual slope in LVMI (P < 0.001 and P = 0.07, respectively). CONCLUSION: Characteristics associated with higher risk of progression in ADPKD, including higher systolic blood pressure, larger kidney volume, and lower eGFR are associated with improvement in LVMI with intensive blood pressure control, whereas male sex is associated with a smaller slope of reduction in LVMI.

Dialysis at a crossroads--Part II: A call for action.

A previous commentary pointed out that the renal community has led American healthcare in the development and continuous improvement of quality outcomes. However, survival, hospitalization, and quality of life for US dialysis patients is still not optimal. This follow-up commentary examines the obstacles, gaps, and metrics that characterize this unfortunate state of affairs. It posits that current paradigms are essential contributors to quality outcomes but are no longer sufficient to improve quality. New strategies are needed that arise from a preponderance of evidence, in addition to beyond a reasonable doubt standard. This work offers an action plan that consists of new pathways of care that will lead to improved survival, fewer hospitalizations and rehospitalizations, and better quality of life for patients undergoing dialysis therapy. Nephrologists in collaboration with large and small dialysis organizations and other stakeholders, including the Centers for Medicare and Medicaid Services, can implement these proposed new pathways of care and closely monitor their effectiveness. We suggest that our patients deserve nothing less and must receive even more.

Cardiac magnetic resonance assessment of left ventricular mass in autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is associated with a substantial cardiovascular disease burden including early onset hypertension, intracranial aneurysms, and left ventricular hypertrophy (LVH). A 41% prevalence of LVH has been reported in ADPKD, using echocardiographic assessment of LV mass (LVM). The HALT PKD study was designed to assess the effect of intensive angiotensin blockade on progression of total kidney volume and LVM. Measurements of LVM were performed using cardiac magnetic resonance (MR). DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Five hundred forty-three hypertensive patients with GFR >60 ml/min per 1.73 m(2) underwent MR assessment of LVM at baseline. LVM was adjusted for body surface area and expressed as LVM index (LVMI; g/m(2)). RESULTS: Baseline BP was 125.1 ± 14.5/79.3 ± 11.6 mmHg. Average duration of hypertension was 5.79 years. Prior use of angiotensin-converting enzyme inhibitors or angiotensin receptor blockers was present in 59.5% of patients. The prevalence of LVH assessed using nonindexed LVM (g) was 3.9% (n = 21, eight men and 13 women) and 0.93% (n = 5, one man and four women) using LVMI (g/m(2)). In exploratory analyses, the prevalence of LVH using LVM indexed to H(2.7), and the allometric index ppLVmass(HW), ranged from 0.74% to 2.23% (n = 4 to 12). Multivariate regression showed significant direct associations of LVMI with systolic BP, serum creatinine, and albuminuria; significant inverse associations with LVMI were found with age and female gender. CONCLUSIONS: The prevalence of LVH in hypertensive ADPKD patients <50 years of age with short duration of hypertension, and prior use of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers is low. Early BP intervention in ADPKD may have decreased LVH and may potentially decrease cardiovascular mortality.

Medicare reimbursement policies and hemodialysis vascular access outcomes: a need for change.

In March 2010, the Center for Medicare and Medicaid Services (CMS) convened several clinical technical expert panels (C-TEP) to provide recommendations for improving various aspects of hemodialysis management. One of the C-TEPs was tasked with recommending measures to decrease vascular access-related infections. The members of this C-TEP, who are the authors of this manuscript, concluded unanimously that the single most important measure would be to remove financial and regulatory barriers to timely placement and revision of hemodialysis fistulas and the concurrent avoidance of catheter use. The following position paper outlines the financial barriers to improved vascular access outcomes and our proposals for a future CMS demonstration project.

Quality of life in autosomal dominant polycystic kidney disease patients not yet on dialysis.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited progressive disorder associated with significant pain and discomfort affecting quality of life. This study determined the impact of pain medication use and other clinical, biochemical and genetic characteristics on the physical and mental well being of predialysis ADPKD patients using the Short Form 36 (SF-36) questionnaire. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The authors prospectively evaluated ADPKD patients in the Cohort Study, funded by the Polycystic Kidney Disease Foundation. Data on clinical, biochemical, and radiologic variables were collected in patients who were given the Short Form-36 questionnaire. Variables independently associated with the Physical Component Summary (PCS) and the Mental Component Summary (MCS) scores were identified. RESULTS: One hundred fifty-two patients had a mean PCS and MCS of 46.9 +/- 11.3 and 51.0 +/- 9.0, similar to the general population and better than the ESRD population. Eleven (7%) reported pain medication intake within 1 mo of evaluation and demonstrated lower PCS than those not taking pain medications. Patients with GFR >or= 80 ml/min/1.73 m(2) had greater PCS than those with GFR < 80 ml/min/1.73 m(2). Age, BMI, pulse pressure, pain medication use, and education level independently associate with PCS and account for 32% of the variability of the measurement. Pulse pressure correlated with MCS. CONCLUSIONS: Predialysis ADPKD patients assess their quality of life similar to the general population. Age, BMI, pulse pressure, pain medication intake, and education level link to their physical well-being.

L-carnitine use in dialysis patients: is national coverage for supplementation justified? What were CMS regulators thinking--or were they?

Careful review of all available clinical trials of L-carnitine leads to the conclusion that there is insufficient evidence to support the routine use of L-carnitine for any indication in dialysis patients. The literature suffers from a lack of adequately designed studies, and many of the studies which supposedly justify payment for L-carnitine supplementation are more than 10 years old. While some studies support a subjective improvement in symptoms after a few months of L-carnitine treatment, there is little confirming objective data. Biochemical parameters show minimal, if any, improvements. A major criticism is that many of the reported symptoms could be attributable to anemia, which at the time the L-carnitine studies were taking place, was generally being corrected with EPO. On the other hand, there is little data to support the hypothesis that L-carnitine enhances the response to EPO or overcomes EPO resistance. The decrease in the use of L-carnitine in the past several years may be related in part to difficulty with reimbursement. The decrease also suggests that practitioners have abandoned the hypothesis that L-carnitine supplementation provides substantial clinical benefits, and therefore no longer prescribe it for dialysis patients. For those physicians who plan to prescribe L-carnitine based on the recent CMS reimbursement decision, it must be remembered that the laboratory measurement of free carnitine may be difficult and inaccurate. For those patients with private insurance, payment for the lab test is out of pocket. If the free carnitine level is measured once dialysis starts, a value in the CMS "deficient" range can occur since carnitine drops early in the dialysis procedure and slowly rebounds after the treatment. Therefore, it is critical that the measurement be done pre-dialysis after a three-day interdialytic interval to obtain the most accurate value. If strict guidelines for use of L-carnitine are adhered to (i.e., the patient has true EPO-resistant anemia unexplained by any identifiable factor and true unexplained hypotension), then the use of L-carnitine in ESRD patients should be very uncommon. In conclusion, the clinical value of L-carnitine supplementation in hemodialysis patients remains to be documented by credible evidence from rigorous scientific studies. While "proof beyond a reasonable doubt" need not always be the requirement for reimbursement from payers, at a minimum "a preponderance of the evidence" should be documented in the literature. L-carnitine may prove to be a beneficial supplement. However, before justifying a national coverage policy, a new randomized, prospective controlled trial should be conducted to determine the utility of i.v. L-carnitine supplementation for anemia management and refractory dialysis-associated hypotension. Cost-benefit analysis is a critical aspect of such a study because it is important to determine the total cost (no matter who pays) of L-carnitine supplementation as compared to money saved by a reduction in EPO and iron administration. When reimbursement policies are developed, they need to be rational and based on the best evidence that is available. An NKF Carnitine Consensus Conference concluded that current literature and clinical experience leave unanswered questions regarding the use of L-carnitine in dialysis patients. Until there is scientific evidence to support use of L-carnitine supplementation, and it proves to be cost-effective, reimbursement is not justified. Therefore, the current CMS reimbursement decision for L-carnitine appears to be flawed.

ESRD in the geriatric population: the crisis of managed care and the opportunity of disease management.

The geriatric population with end-stage renal disease (ESRD) is placed at risk with regards to the quality and extent of medical coverage because of the rapidly changing financial environment. Managed care organizations (MCOs) are generally for-profit companies that must focus on the bottom line. While the verbal commitment to quality care is voiced, the financial pressures on MCOs have led to a decrease in coverage of many services and outright denial for some necessary treatments. While denying services, the MCOs have also reduced payments to providers for services rendered. The coverage crisis is compounded by health maintenance organizations (HMOs) quitting Medicare because the reimbursement from the Health Care Financing Administration (HCFA) is less than their costs. Because of the above issues which can potentially impact on the quality of care delivered to the ESRD geriatric population, a new approach to disease management has created the opportunity to improve total patient care to a level not yet achieved in the United States. Disease management encompasses integrated care across all disciplines. Every component of care can be tracked by a dedicated information system. Improvement in outcomes has far exceeded the U.S. Renal Data System (USRDS) benchmark performance measurements with a disease management model approach. The key to success is the health service coordinator (HSC), a senior nurse with many years of ESRD experience. This individual coordinates care across all disciplines and expedites necessary referrals. With rapid attention to patient needs there has been a significant reduction in hospital admissions, hospital length of stay, and emergency room visits. Patient care will steadily improve as the disease management system matures as a consequence of understanding the patients total physical and psychosocial needs.