Patient-provider communication in nephrology care for adolescents and young adults.

OBJECTIVE: To compare the relative quantity of talk between providers, caregivers, and adolescents and young adults (AYAs) with chronic kidney disease (CKD) and how communication differs by age. METHODS: During nephrology clinic visits, conversations between AYAs with CKD (N=99, ages 11-20, median=15), their caregivers, and providers (N=19) were audiotaped and coded using the Roter Interaction Analysis System. Linear mixed models tested AYA age differences in talk frequency by AYAs, caregivers, and providers. Post-hoc analyses tested differences in talk using AYA age groups. RESULTS: During clinic visits, providers spoke the most (63.7%), and caregivers spoke more (22.6%) than AYAs (13.7%). Overall talk differed by AYA age in AYAs (p<0.001) and caregivers (p<0.05), but not providers. Higher AYA age was associated with more AYA talk (biomedical information-giving, partnering, rapport-oriented) and less caregiver biomedical information-giving (ps<0.001-0.05). In post-hoc analyses, young adults talked more than adolescents; caregiver talk decreased in the middle-adolescent group. CONCLUSIONS: Increases in AYA talk occur primarily in young adulthood, whereas caregiver talk decreases in middle adolescence. This may indicate an appropriate developmental shift but raises concerns about conversational gaps during middle-adolescence. PRACTICE IMPLICATIONS: During transition-oriented treatment planning, providers should engage both AYAs and caregivers to avoid potential gaps in communication.

Predictors of arthritis in pediatric patients with lupus.

BACKGROUND: Arthritis is one of the most common manifestations of systemic lupus erythematosus (SLE). Although typically non-erosive and non-deforming, children with SLE arthritis can have significant morbidity with decreased quality of life. Our goal was to identify potential clinical and laboratory predictors of arthritis in a cohort of pediatric patients with SLE. METHODS: We performed a cohort study of incident and prevalent patients with SLE aged ≤ 19 years. In cross sectional analysis, we compared demographic and clinical characteristics at initial clinic presentation between patients with arthritis noted at any time during follow-up and those without arthritis. We performed time to event analysis using Cox proportional hazard ratios to identify predictors of arthritis, clustering for repeated measures. RESULTS: Forty seven children and adolescents with SLE were followed in the cohort, 91 % female and 68 % Black. In cross-sectional analyses, presence of malar rash was associated with arthritis. In longitudinal analyses, controlling for gender and race, increased age (HR: 1.4, 95 % CI: 1.1-1.7), malar rash (HR: 2.1, 95 % CI: 1.1-3.6), and presence of RNP antibodies (HR: 1.9, 95 % CI: 1.1-3.4) were predictive of arthritis. When controlling for gender, race, and medication use, anemia (HR: 8.5, 95 % CI: 2.9-24.2) and thrombocytopenia (HR: 6.1, 95 % CI: 2.4-15.6) were associated with increased risk of arthritis. CONCLUSIONS: We identified markers predictive of arthritis in a longitudinal cohort of children with SLE. The recognition of these markers may help clinicians identify patients at risk for arthritis before its onset thus improving quality of life in children with SLE.

Predictors of kidney disease in a cohort of pediatric patients with lupus.

INTRODUCTION: Children with systemic lupus erythematosus (SLE) have an increased prevalence of kidney disease compared to their adult counterparts. Our goal was to identify potential clinical and laboratory predictors of renal disease. METHODS: We performed a cohort study of incident and prevalent patients with SLE aged ≤19 years. Retrospective data from initial presentation until study enrollment was also collected. Laboratory and clinic data were recorded from each clinic visit including disease activity indices, autoantibodies, urinalyses, blood counts, and metabolic profile. Kidney disease was defined as the presence of abnormal renal biopsy or by American College of Rheumatology case definition for lupus nephritis. Logistic regression analyses were used to determine the association between clinical and laboratory data with kidney disease in those who had renal involvement within 30 days of SLE diagnosis. We also performed a time to event analysis to identify antecedents of renal disease. RESULTS: Forty-seven children and adolescents with SLE were followed in the cohort, 91% female and 68% black. All of the males in the cohort developed renal disease, and all within one month of the diagnosis of SLE. In logistic regression, low serum albumin (odds ratio (OR): 4.8, 95% CI: 1.9-12.5) and positive dsDNA antibodies (OR: 3.2, 95% CI: 1.7-5.9) were associated with kidney disease. In longitudinal analyses, isolated sterile pyuria (hazard ratio (HR): 3, 95% CI: 1.1-6.4) and low serum albumin (HR: 3.4, 95% CI: 1.7-6.9) were predictors of future kidney disease. The presence of antibodies against Ro were protective against renal disease (HR: 0.2, 95% CI: 0.05-0.5). CONCLUSION: We identified variables associated with kidney disease, both at initial diagnosis of SLE and in longitudinal follow-up in a cohort of children with SLE. The recognition of these abnormal laboratory values may help clinicians identify patients at risk for kidney disease before its onset thus preventing long-term complications.

Increased hospitalizations and death in patients with ESRD secondary to lupus.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect almost any organ system, including the kidneys. Using a large national dataset, our goal was to compare the morbidity as measured by hospitalization and mortality rates between hemodialysis patients with end-stage renal disease (ESRD) secondary to SLE to those with ESRD due to other causes. METHODS: The risk of hospitalization was calculated by Poisson regression with clustering for repeated measures using the United States Renal Data System (USRDS) Hospitalization Analytic File in strata of pediatric and adult patients. Cox proportional hazard ratio was used to assess the mortality risk in hospitalized patients. Subjects were censored at transplantation or end of follow-up. RESULTS: Adult patients with ESRD secondary to SLE were hospitalized more frequently than other adults (incidence rate ratio (IRR): 1.43, 95% confidence interval (CI): 1.15-1.77) and had a higher risk of death (hazard ratio (HR): 1.89, 95% CI: 1.66-2.5). Mortality was higher in hospitalized pediatric patients with SLE compared to pediatric patients with other causes of ESRD (HR: 2.01, 95% CI: 1.75-2.31) and adults with SLE (HR: 2.05, 95% CI: 1.79-2.34). CONCLUSION: Our study demonstrates that there is a trend toward increased hospitalization rates in pediatric and adult patients with SLE. Among these hospitalized patients with SLE, there is an increased risk of death due to cardiovascular disease.

Assessing associations between medication adherence and potentially modifiable psychosocial variables in pediatric kidney transplant recipients and their families.

Post-transplant immunosuppressant (IS) medication adherence is essential for long-term graft survival and relatively little is known about psychosocial barriers that interfere with optimum medication adherence in pediatric kidney transplant patients. The objective of this prospective observational cohort study was to assess the impact of modifiable psychosocial variables on medication adherence. Our hypothesis was that parental stress, dysfunctional parent-child interactions and child behavior problems would be associated with poorer medication adherence. Thirteen pediatric kidney transplant patients and their caregivers were enrolled. Transplant recipients who were able to read and caregivers of all the transplant recipients completed behavioral and attitudinal surveys. A subgroup of seven families dispensed their primary IS medication from an electronic monitoring vial (MEMS Smart Cap). For these patients, medication adherence was calculated by computing a ratio of the medication taken divided by the prescribed dose. In addition, for the entire group, serial IS levels were reviewed by two board certified pediatric nephrologists who categorized all 13 transplant recipient families as either 'probably adherent (PA)' or 'possibly non-adherent (PNA)'. Pearson correlation coefficients and independent samples Student t-tests were used to assess the association between medication adherence and psychosocial variables measured by standardized questionnaires. In this study, elevated parental stress, dysfunctional parent-child interactions, and child behavior problems were associated with poorer medication adherence. In addition, we found evidence to support the relationship between subjective dissatisfaction with appearance and poorer medication adherence. These findings suggest that pre-transplant recipient evaluations of risk factors for poor adherence are warranted.

The impact of dialysis and transplantation on children.

Effective methods to treat end stage renal disease (ESRD) in children have become available in the United States during the last 3 decades. Since the United States Congress created the Medicare ESRD Program in 1972, most children with ESRD have the option of Medicare insurance. Medicare expenditures for children with ESRD range from $14,000 for transplant recipients to $43,000 for dialysis patients per year. The tremendous expense of ESRD treatment has led to research to determine which treatment options are associated with the best health outcomes and the best value (quality/cost) for the money spent treating ESRD. The National Kidney Foundation's Dialysis Outcomes Quality Initiative recommends the use of quality of life and health status measures to gauge the impact of renal replacement therapy on quality of life in the ESRD population. In adult patients with renal failure, several generic and disease-specific quality of life measures have been validated and tested for reliability. In contrast, little research using validated and reliable health status measures has been performed in pediatric patients to measure the impact of ESRD. This article summarizes existing literature on how we currently measure the impact of dialysis and transplantation on children, discusses existing health status measures for children and adolescents, and describes how these measures might be used to improve our care of patients and long-term outcomes for children with kidney failure.

Does growth retardation indicate suboptimal clinical care in children with chronic renal disease and those undergoing dialysis?

Growth failure is an important problem for children with end-stage renal disease (ESRD). Patients receiving replacement therapy for longstanding renal failure since childhood are likely to report dissatisfaction with certain aspects of their lives, especially with final adult height. Additionally, recent data suggest that growth failure in children with ESRD is associated with adverse clinical outcomes, including more frequent hospitalizations, and increased mortality. Although poor growth is unlikely to be the cause of this increased morbidity, growth failure may be a marker for a group of patients at high risk of adverse events. In this review, the authors describe the prevalence of growth retardation in children in the US with chronic renal disease, and present recent data on morbidity associated with growth failure. After reviewing published reports documenting available strategies to optimize growth, the authors conclude that despite vigilance and aggressive clinical management, a subset of children with long-term renal insufficiency and ESRD may still have poor linear growth. A discussion of "optimal care" leads one to consider evidence of current variability in the management of growth retardation in ESRD, and the strengths and limitations of developing practice guidelines to optimize growth in this population.

Validation of PD Adequest 2.0 for pediatric dialysis patients.

Kinetic modeling has proven to be a valuable tool for peritoneal dialysis (PD) prescription in adult PD patients. The clinical application of this procedure has rarely been studied in children. We therefore evaluated the PD Adequest 2.0 for Windows program (Baxter Healthcare Co., Deerfield, IL) as a prescription aid for the management of pediatric PD patients by comparing the measured and predicted PD clearances, total drain volumes, and net ultrafiltration in 34 children (15 males) (mean age 10.9 +/- 6.0 years) receiving long-term PD. In each case, a 4-h peritoneal equilibration test was conducted with a standardized test exchange volume of 1,100 ml/m2 BSA. A total of 43 24-h dialysate (plus urine in 12) collections were analyzed. The levels of agreement between measured and predicted values for weekly peritoneal and total urea Kt/V, weekly peritoneal and total creatinine clearance, daily drain volume, net ultrafiltration and daily peritoneal urea and creatinine mass removal were assessed with correlation coefficients (re) and Bland-Altman limits of agreement. The study revealed that there is a basic level of agreement between measured and modeled values for solute removal and total drain volume, with correlation coefficients ranging from 0.75 to 0.98. In contrast, the rc for net ultrafiltration was only 0.34. The majority (75%) of patients had modeled urea and creatinine clearances that were within 20% of their measured values. These data suggest that the PD Adequest 2.0 for Windows program can predict urea and creatinine clearances with reasonable accuracy in pediatric PD patients, making it a valuable resource in prescription management.

Relation between pediatric experience and treatment recommendations for children and adolescents with kidney failure.

CONTEXT: Children and adolescent patients with renal failure are frequently cared for by adult subspecialists. While peritoneal dialysis is used in less than 17% of adults with kidney failure, it is the preferred dialysis treatment for children. National data show that 45% of children receiving dialysis are treated with peritoneal dialysis and pediatric nephrologists report its use in 65% of patients receiving dialysis. Whether differences in peritoneal dialysis use among children are due to the pediatric experience of the clinician has not been examined. OBJECTIVE: To assess whether the pediatric experience of nephrologists directly affects treatment recommendations for children with kidney failure. DESIGN: Cross-sectional survey using 10 case vignettes per survey based on random combinations of 8 patient characteristics (age, sex, race, distance from facility, cause of renal failure, family structure, education, and compliance). SETTING AND PARTICIPANTS: National random sample of office-, hospital-, and academic medical center-based adult and pediatric nephrologists, stratified by geographic region and conducted June to November 1999. Of 519 eligible physicians, 316 (61%) responded, including 191 adult and 125 pediatric nephrologists. MAIN OUTCOME MEASURE: Treatment recommendations for peritoneal dialysis vs hemodialysis, compared based on nephrologists' pediatric experience. RESULTS: After controlling for patient characteristics, pediatric nephrologists were 60% more likely than adult nephrologists to recommend peritoneal dialysis for identical patients (odds ratio, 1.61; 95% confidence interval, 1.35-1.92). This was true regardless of dialysis training, years in practice, practice setting, geography, or patient characteristics. CONCLUSIONS: Our data indicate that pediatric specialization of clinicians influences treatment recommendations for children and adolescents with end-stage renal disease. Referring children to adult subspecialists may lead to differences in treatment choices and processes of care.

Risk factors for urolithiasis in children on the ketogenic diet.

Kidney stones have been associated with use of the ketogenic diet in children with refractory seizure disorders. We performed a case-control study examining risk factors for the development of stones on the ketogenic diet, and prospectively followed children initiating the ketogenic diet to evaluate the incidence of urolithiasis. Clinical characteristics of 18 children presenting with stones (8 uric acid stones, 6 mixed calcium/uric acid stones, 1 calcium oxalate/phosphate stone, 3 stones not evaluated) were compared with characteristics of non-stone-forming children initiating the ketogenic diet at Johns Hopkins since July 1996. Since July 1996, 112 children initiating the ketogenic diet have been followed for development of stones. Follow-up times on the diet range from 2 months to 2.5 years. Of 112 children, 6 have developed stones (3 uric acid, 3 mixed calcium/uric acid stones) (0.8 children developing stones/ 100 patient-months at risk). Comparisons of children presenting with stones on the ketogenic diet with characteristics of the entire cohort initiating the ketogenic diet suggest younger age at diet initiation and hypercalciuria are risk factors for the development of stones. Prospective evaluation of children initiating the ketogenic diet revealed that almost 40% of patients had elevated fasting urine calcium: creatinine ratios at baseline; this increased to 75% after 6 months on the diet. Median urine pH was 5.5 at diet initiation, and remained at 6.0 thereafter. In a subset of patients tested, urinary citrate excretion fell from a mean of 252 mg/24 h pre diet initiation to 52 mg/24 h while on the diet. Uric acid excretion remained normal. Patients maintained on the ketogenic diet often have evidence of hypercalciuria, acid urine, and low urinary citrate excretion. In conjunction with low fluid intake, these patients are at high risk for both uric acid and calcium stone formation.

Racial differences in access to the kidney transplant waiting list for children and adolescents with end-stage renal disease.

CONTEXT: Renal transplantation is the treatment of choice for pediatric patients with end-stage renal disease (ESRD). Black patients wait longer for kidney transplants than do white patients. OBJECTIVE: To determine whether the increased time to transplantation for black pediatric patients is attributable not only to a shortage of suitable donor organs, but also to racial differences in the time from a child's first treatment for ESRD until activation on the cadaveric kidney transplant waitlist. DESIGN: National longitudinal cohort study. SETTING: US Medicare-eligible, pediatric ESRD population. PATIENTS: Children and adolescents

Immunizations for pediatric dialysis patients.

Children maintained on chronic dialysis are at high risk for infection, and although the burden of vaccine-preventable disease in this population has not been fully documented, primary care of these patients should include careful compliance with the routine childhood immunization schedule. There have been considerable changes in this schedule in recent years, and an update is provided. In addition the supplemental vaccines for pneumococcal and influenza vaccines are discussed. Where available, data regarding vaccine response in children on dialysis are presented.

For-profit versus not-for-profit dialysis care for children with end stage renal disease.

OBJECTIVE: Over the last 2 decades, for-profit dialysis units have become the most common providers of renal replacement therapy for adults with end stage renal disease (ESRD) and have had an increasing role in the dialysis of children. We undertook a study to determine whether dialysis facility profit status influences the choice of dialysis therapy in the pediatric population. DESIGN: Cross-sectional study of national data from the Health Care Financing Administration. SETTING: Free-standing and hospital-based outpatient dialysis facilities in the United States. PATIENTS: A total of 1568 children and adolescents (

Longitudinal evaluation of transport kinetics in children receiving peritoneal dialysis.

Functional stability of the peritoneal membrane is necessary for maintenance of peritoneal dialysis (PD) as a therapeutic option. Few studies have investigated this issue in children. We evaluated the peritoneal membrane solute transport capacity longitudinally in 26 children (mean age 11.0+/-5.5 years) receiving long-term PD. Each patient underwent a standardized peritoneal equilibration test on two occasions (mean interval between studies 19.8+/-5.9 months) to determine solute dialysate to plasma (D/P) ratios, dialysate glucose to initial dialysate glucose (D/D(0)) ratios, and mass transfer area coefficients (MTAC). The correlation of transport capacity with peritonitis history was also assessed. No significant change in MTAC, D/P, or D/D(0) values were found when comparing original and follow-up data of the group overall. However, transport of creatinine and glucose was significantly (P<0.05) greater in the peritonitis group compared with the group without peritonitis, and differences in the change over time between the peritonitis groups was significant for MTAC creatinine (P=0.035) and D/D(0) glucose (P=0.020). In summary, this experience demonstrates functional stability of the peritoneal membrane in pediatric patients receiving PD. However, follow-up assessments of peritoneal solute kinetics may be necessary in patients with a history of peritonitis in order to permit early identification of those who may be at risk for ultrafiltration failure and sclerosing peritonitis.

Plasmapheresis, intravenous cytomegalovirus-specific immunoglobulin and reversal of antibody-mediated rejection in a pediatric renal transplant recipient: a case report.

This is a pediatric case report illustrating the development of antibody (Ab)-mediated rejection in a patient with low levels of pretransplant anti-human leucocyte antigen (HLA) panel reactive antibodies (PRA). The clinical course of this patient suggests that aggressive use of a combination of plasmapheresis, monoclonal anti-T-lymphocyte antibody therapy, and intravenous immunoglobulin (IVIG) therapy can reverse Ab-mediated rejection in previously allosensitized pediatric transplant recipients.

Chronic renal insufficiency in children and adolescents: the 1996 annual report of NAPRTCS. North American Pediatric Renal Transplant Cooperative Study.

The 1996 annual report of the Chronic Renal Insufficiency Arm of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) summarizes descriptive data and highlights important features on 1,725 patients from 130 centers. This database contains information on patients with an estimated glomerular filtration rate (GFR) < or = 75 ml/min per 1.73 m2 as calculated by the Schwartz formula, who were treated on or after 1 January 1994. Thus this report reflects 2 years of data entry. Analysis of the data revealed that nearly two-thirds of patients registered had a structural anomaly. On average, patients were 1.5 standard deviations below age- and sex-specific norms for height, and 0.6 standard deviations below weight norms. Mean serum creatinine for the entire group was 2.4 mg/dl and 68% of patients had a baseline GFR of at least 25 ml/min per 1.73 m2. The mean hematocrit for all children at registration was 33.3 +/- 6.3%, and did not vary among age groups. Overall, 30.9% of patients had a hematocrit < 30%. Only 12.8% of patients were receiving Epoetin therapy. Although still in infancy, the Chronic Renal Insufficiency Arm of the NAPRTCS database in providing important insights into this disorder.

Report of the American Society of Nephrology meetings.

PURPOSE: We summarize presentations on topics of interest to the pediatric urologist from the annual meeting of the American Society of Nephrology, November 1996. MATERIALS AND METHODS: We reviewed all abstracts submitted for presentation and subsequently published in abstract form. Only those abstracts containing information pertinent to the field of pediatric urology were summarized for this report. RESULTS: A total of 24 abstracts addressing a variety of topics were summarized. Obstructive uropathy was represented by the greatest number of papers and reflects the multiple investigative efforts currently evaluating the cellular aspects of this disorder. The genetic basis of hypercalciuria and the impact of water metabolism on nephroliathisis were also discussed. CONCLUSIONS: Clinical and basic research activities that are of mutual interest to pediatric urologists and nephrologists are being conducted. The topic of obstructive uropathy has received the greatest attention during the last year. However, additional research, potentially collaborative in nature, on topics such as nephroliathisis and reflux nephropathy should be encouraged.

Immunization guidelines for pediatric renal disease.

It is imperative that pediatric nephrologists monitor the immunization status of pediatric chronic renal insufficiency, dialysis and transplantation patients closely to reduce the risk of vaccine-preventable disease. Pediatric patients with chronic renal insufficiency and those on dialysis should receive all the standard immunizations according to the schedule as deliniated by the Red Book. In addition to these standard vaccines, these patients will also benefit from influenza and pneumococcal vaccine. Pediatric renal transplant recipients should also be immunized with standard and special vaccines; however, all live viral vaccines should be avoided in this population. Because patients with renal disease may not respond optimally to all immunizations, it is important to study antibody response to MMR and varicella in patients before transplantation. If these patients are unprotected, they should be immunized before transplantation. It seems that pediatric dialysis and transplantation patients may not respond optimally to hepatitis B vaccine. Therefore, if at all possible, this vaccine should be administered before these therapies. Doubling the recommended dose of hepatitis B vaccine may improve response. Antibody levels to hepatitis B should be monitored every other year, and this vaccine should be readministered when the antibody level decreases to less than 10 mIU/mL. Hopefully the morbidity and mortality associated with vaccine-preventable disease can be reduced in this population by ensuring that pediatric patients with chronic renal disease are adequately immunized.

Renal disease in Down syndrome: autopsy study with emphasis on glomerular lesions.

A range of renal diseases have been previously described in patients with Down syndrome. With increased survival, it appears that a growing number of these patients present with chronic renal failure. Definition of underlying causes of renal failure could potentially lead to prevention of progressive renal dysfunction in this population. We report two index cases of teenaged Down patients who presented with proteinuria and focal segmental glomerulosclerosis with hyalinosis, not previously described in this population. In addition, autopsy files were reviewed at the Johns Hopkins Hospital to assess renal and especially glomerular pathology in Down patients. Additional cases, including acute glomerulonephritis with early crescents and vasculitis, minimal change disease, and membranous nephropathy, were identified; the latter two diseases had not been previously reported in patients with Down syndrome. Semiquantitative studies on glomerular changes in all cases examined through autopsy also were performed. The only pathological finding that was significantly more common in the Down syndrome group, compared with age-matched cases from the autopsy files, was cystic dilation of Bowman's space. Histological findings described as increased in the Down population in previously published autopsy studies were also present in the control population, highlighting the need to adequately control such studies. The cases of acquired glomerular disease reported here were seen largely after the first decade of life. Monitoring of Down patients for renal and especially glomerular disease should be done regularly as patients age into the second and third decades.

Recommended immunization practices for pediatric renal transplant recipients.

To reduce the risk of morbidity and mortality from vaccine preventable disease, it is imperative that physicians caring for pediatric renal transplant recipients monitor the immunization status of their patients and keep abreast of changes in the recommended immunization guidelines. An update of the standard immunization guidelines of the AAP, ACIP and AAFP is provided, as well as the necessary modifications for and additional vaccines recommended for immunocompromised individuals. Where available, data regarding the safety of and response to the various vaccines in pediatric transplant patients are provided.