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A 68-year-old woman with a history of hepatitis C (contracted from a blood transfusion in 1974) complicated by cirrhosis and portal hypertension came to the Mayo Clinic in Rochester, Minn, for evaluation for possible liver transplantation. Her symptomatic ascites had been treated initially with furosemide and spironolactone, but this treatment regimen was limited because of an increase in her creatinine level. During evaluation, hypertension (an average blood pressure of 180/90 mm Hg on 6-hour ambulatory monitoring) and abnormal renal function were noted. She was referred to our institution for further evaluation of her blood pressure and abnormal urinalysis results. [ABSTRACT FROM AUTHOR]

Describing risk factors and outcomes in kidney transplant recipients with oxalate nephropathy (ON) may help elucidate the pathogenesis and guide treatment strategies. We used a large single-center database to identify patients with ON and categorized them into delayed graft function with ON (DGF-ON) and late ON. Incidence density sampling was used to select controls. A total of 37 ON cases were diagnosed between 1/2011 and 1/2021. DGF-ON (n = 13) was diagnosed in 1.05% of the DGF population. Pancreatic atrophy on imaging (36.4% vs. 2.9%, p = 0.002) and gastric bypass history (7.7% vs. 0%; p = 0.06) were more common in DGF-ON than with controls with DGF requiring biopsy but without evidence of ON. DGF-ON was not associated with worse graft survival (p = 0.98) or death-censored graft survival (p = 0.48). Late ON (n = 24) was diagnosed after a mean of 78.2 months. Late ON patients were older (mean age 55.1 vs. 48.4 years; p = 0.02), more likely to be women (61.7% vs. 37.5%; p = 0.03), have gastric bypass history (8.3% vs. 0.8%; p = 0.02) and pancreatic atrophy on imaging (38.9% vs. 13.3%; p = 0.02). Late ON was associated with an increased risk of graft failure (HR 2.0; p = 0.07) and death-censored graft loss (HR 2.5; p = 0.10). We describe two phenotypes of ON after kidney transplantation: DGF-ON and late ON. Our study is the first to our knowledge to evaluate DGF-ON with DGF controls without ON. Although limited by small sample size, DGF-ON was not associated with adverse outcomes when compared with controls. Late ON predicted worse allograft outcomes., (© 2024 The Author(s). Clinical Transplantation published by John Wiley & Sons Ltd.)

Background: Telenephrology has become an important health care delivery modality during the COVID-19 pandemic. However, little is known about patient perspectives on the quality of care provided via telenephrology compared to face-to-face visits. We aimed to use objective data to study patients' perspectives on outpatient nephrology care received via telenephrology (phone and video) versus face-to-face visits., Methods: We retrospectively studied adults who received care in the outpatient Nephrology & Hypertension division at Mayo Clinic, Rochester, from March to July 2020. We used a standardized survey methodology to evaluate patient satisfaction. The primary outcome was the percent of patients who responded with a score of good (4) or very good (5) on a 5-point Likert scale on survey questions that asked their perspectives on access to their nephrologist, relationship with care provider, their opinions on the telenephrology technology, and their overall assessment of the care received. Wilcoxon rank sum tests and chi-square tests were used as appropriate to compare telenephrology versus face-to-face visits., Results: 3,486 of the patient encounters were face-to-face, 808 phone and 317 video visits. 443 patients responded to satisfaction surveys, and 21% of these had telenephrology encounters. Established patients made up 79.6% of telenephrology visits and 60.9% of face-to-face visits. There was no significant difference in patient perceived access to health care, satisfaction with their care provider, or overall quality of care between patients cared for via telenephrology versus face-to-face. Patient satisfaction was also equally high., Conclusions: Patient satisfaction was equally high amongst those patients seen face-to-face or via telenephrology., Competing Interests: The authors have declared that no competing interests exist.

We report a case of systemic oxalosis involving the eyes and joints due to long-term use of high-dose vitamin C in a patient receiving maintenance peritoneal dialysis (PD). This 76-year-old woman with autosomal dominant polycystic kidney disease underwent living unrelated kidney transplantation 10 years earlier. The transplant failed 6 months before presentation, and she initiated hemodialysis therapy before transitioning to PD therapy 4 months later. During the month before presentation, the patient noted worsening arthralgias and decreased vision. Ophthalmologic examination revealed proliferative retinopathy and calcium oxalate crystals. Plasma oxalate level was markedly elevated at 187 (reference range, <1.7) μmol/L, and urine oxalate-creatinine ratio was high (0.18mg/mg). The patient reported taking up to 4g of vitamin C per day for several years. Workup for causes of primary and secondary hyperoxaluria was otherwise negative. Vitamin C use was discontinued, and the patient transitioned to daily hemodialysis for 2 weeks. Plasma oxalate level before the dialysis session decreased but remained higher (30-53μmol/L) than typical for dialysis patients. Upon discharge, the patient remained on thrice-weekly hemodialysis therapy with stabilized vision and improved joint symptoms. This case highlights the risk of high-dose vitamin C use in patients with advanced chronic kidney disease, especially when maintained on PD therapy., (Copyright © 2019 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Withdrawal from maintenance hemodialysis before death has become more common because of high disease and treatment burden. The study objective was to identify patient factors and examine the terminal course associated with hemodialysis withdrawal, and assess patterns of palliative care involvement before death among patients on maintenance hemodialysis., Design, Setting, Participants, & Measurements: We designed an observational cohort study of adult patients on incident hemodialysis in a midwestern United States tertiary center, from January 2001 to November 2013, with death events through to November 2015. Logistic regression models evaluated associations between patient characteristics and withdrawal status and palliative care service utilization., Results: Among 1226 patients, 536 died and 262 (49% of 536) withdrew. A random sample (10%; 52 out of 536) review of Death Notification Forms revealed 73% sensitivity for withdrawal. Risk factors for withdrawal before death included older age, white race, palliative care consultation within 6 months, hospitalization within 30 days, cerebrovascular disease, and no coronary artery disease. Most withdrawal decisions were made by patients (60%) or a family member (33%; surrogates). The majority withdrew either because of acute medical complications (51%) or failure to thrive/frailty (22%). After withdrawal, median time to death was 7 days (interquartile range, 4-11). In-hospital deaths were less common in the withdrawal group (34% versus 46% nonwithdrawal, P =0.003). A third (34%; 90 out of 262) of those that withdrew received palliative care services. Palliative care consultation in the withdrawal group was associated with longer hemodialysis duration (odds ratio, 1.19 per year; 95% confidence interval, 1.10 to 1.3; P <0.001), hospitalization within 30 days of death (odds ratio, 5.78; 95% confidence interval, 2.62 to 12.73; P <0.001), and death in hospital (odds ratio, 1.92; 95% confidence interval, 1.13 to 3.27; P =0.02)., Conclusions: In this single-center study, the rate of hemodialysis withdrawals were twice the frequency previously described. Acute medical complications and frailty appeared to be driving factors. However, palliative care services were used in only a minority of patients., (Copyright © 2018 by the American Society of Nephrology.)

Background and Objectives: Medical specialty and subspecialty fellowship programs administer subject-specific in-training examinations to provide feedback about level of medical knowledge to fellows preparing for subsequent board certification. This study evaluated the association between the American Society of Nephrology In-Training Examination and the American Board of Internal Medicine Nephrology Certification Examination in terms of scores and passing status., Design, Setting, Participants, & Measurements: The study included 1684 nephrology fellows who completed the American Society of Nephrology In-Training Examination in their second year of fellowship training between 2009 and 2014. Regression analysis examined the association between In-Training Examination and first-time Nephrology Certification Examination scores as well as passing status relative to other standardized assessments., Results: This cohort included primarily men (62%) and international medical school graduates (62%), and fellows had an average age of 32 years old at the time of first completing the Nephrology Certification Examination. An overwhelming majority (89%) passed the Nephrology Certification on their first attempt. In-Training Examination scores showed the strongest association with first-time Nephrology Certification Examination scores, accounting for approximately 50% of the total explained variance in the model. Each SD increase in In-Training Examination scores was associated with a difference of 30 U (95% confidence interval, 27 to 33) in certification performance. In-Training Examination scores also were significantly associated with passing status on the Nephrology Certification Examination on the first attempt (odds ratio, 3.46 per SD difference in the In-Training Examination; 95% confidence interval, 2.68 to 4.54). An In-Training Examination threshold of 375, approximately 1 SD below the mean, yielded a positive predictive value of 0.92 and a negative predictive value of 0.50., Conclusions: American Society of Nephrology In-Training Examination performance is significantly associated with American Board of Internal Medicine Nephrology Certification Examination score and passing status., (Copyright © 2018 by the American Society of Nephrology.)

Purpose: To identify approaches to operationalizing the development of competence in Accreditation Council for Graduate Medical Education (ACGME) milestones., Method: The authors reviewed all 25 "Milestone Project" documents available on the ACGME Web site on September 11, 2013, using an iterative process to identify approaches to operationalizing the development of competence in the milestones associated with each of 601 subcompetencies., Results: Fifteen approaches were identified. Ten focused on attributes and activities of the learner, such as their ability to perform different, increasingly difficult tasks (304/601; 51%), perform a task better and faster (171/601; 45%), or perform a task more consistently (123/601; 20%). Two approaches focused on context, inferring competence from performing a task in increasingly difficult situations (236/601; 29%) or an expanding scope of engagement (169/601; 28%). Two used socially defined indicators of competence such as progression from "learning" to "teaching," "leading," or "role modeling" (271/601; 45%). One approach focused on the supervisor's role, inferring competence from a decreasing need for supervision or assistance (151/601; 25%). Multiple approaches were often combined within a single set of milestones (mean 3.9, SD 1.6)., Conclusions: Initial ACGME milestones operationalize the development of competence in many ways. These findings offer insights into how physicians understand and assess the developmental progression of competence and an opportunity to consider how different approaches may affect the validity of milestone-based assessments. The results of this analysis can inform the work of educators developing or revising milestones, interpreting milestone data, or creating assessment tools to inform milestone-based performance measures.

Background: The changing healthcare landscape requires physicians to develop new knowledge and skills such as high-value care, systems improvement, population health, and team-based care, which together may be referred to as the Science of Health Care Delivery (SHCD). To engender public trust and confidence, educators must be able to meaningfully assess physicians' abilities in SHCD. We aimed to develop a novel set of SHCD milestones based on published Accreditation Council for Graduate Medical Education (ACGME) milestones that can be used by medical schools to assess medical students' competence in SHCD., Methods: We reviewed all ACGME milestones for 25 specialties available in September 2013. We used an iterative, qualitative process to group the ACGME milestones into SHCD content domains, from which SHCD milestones were derived. The SHCD milestones were categorized within the current ACGME core competencies and were also mapped to Association of American Medical Colleges' Entrustable Professional Activities (AAMC EPAs)., Results: Fifteen SHCD sub-competencies and corresponding milestones are provided, grouped within ACGME core competencies and mapped to multiple AAMC EPAs., Conclusions: This novel set of milestones, grounded within the existing ACGME competencies, defines fundamental expectations within SHCD that can be used and adapted by medical schools in the assessment of medical students in this emerging curricular area. These milestones provide a blueprint for SHCD content and assessment as ongoing revisions to milestones and curricula occur.

Purpose: To understand the pregnancy, childbirth, and parental leave plans and experiences of trainees in multiple graduate medical education (GME) programs at a single institution., Method: In 2013, the authors developed and deployed a voluntary, Internet-based survey of trainees in 269 residency and fellowship programs across the three sites of the Mayo School of Graduate Medical Education. The survey assessed pregnancy-related issues, including use of relevant institutional policies, changes in work due to pregnancy, and activities during pregnancy and parental leave. The authors analyzed the responses to make comparisons across groups., Results: Forty-two percent (644/1,516) of trainees responded. Less than half (264; 41%) had children, and 46 (7%) were currently pregnant (themselves or their partners). Among parents, 24 (of 73; 33%) women and 28 (of 81; 35%) men planned to have another child during their current training program, and 13 (18%) women and 14 (17%) men planned to do so during their next training program. Among nonparents, 40 (of 135; 30%) women and 36 (of 111; 32%) men planned pregnancies during their current training program, and 25 (19%) women and 14 (13%) men planned pregnancies during their next training program. Of respondents eligible for parental leave, 81 (of 83; 98%) women and 89 (of 101; 88%) men had used it., Conclusions: Approximately 40% of respondents planned to have children during their GME training; most will require family leave and institutional support. GME programs should pursue policies and practices to minimize the effects of these leaves on their workforce.

Background: Cardiovascular disease among hemodialysis (HD) patients is linked to poor outcomes. The Acute Dialysis Quality Initiative Workgroup proposed echocardiographic (ECHO) criteria for structural heart disease (SHD) in dialysis patients. The association of SHD with important patient outcomes is not well defined., Objectives: This study sought to determine prevalence of ECHO-determined SHD and its association with survival among incident HD patients., Methods: We analyzed patients who began chronic HD from 2001 to 2013 who underwent ECHO ≤1 month prior to or ≤3 months following initiation of HD (n = 654)., Results: Mean patient age was 66 ± 16 years, and 60% of patients were male. ECHO findings that met 1 or more and ≥3 of the new criteria were discovered in 87% and 54% of patients, respectively. Over a median of 2.4 years, 415 patients died: 108 (26%) died within 6 months. Five-year mortality was 62%. Age- and sex-adjusted structural heart disease variables associated with death were left ventricular ejection fraction (LVEF) ≤45% (hazard ratio [HR]: 1.48; confidence interval [CI]: 1.20 to 1.83) and right ventricular (RV) systolic dysfunction (HR: 1.68; CI: 1.35 to 2.07). An additive of higher death risk included LVEF ≤45% and RV systolic dysfunction rather than neither (HR: 2.04; CI: 1.57 to 2.67; p = 0.53 for test for interaction). Following adjustment for age, sex, race, diabetic kidney disease, and dialysis access, RV dysfunction was independently associated with death (HR: 1.66; CI 1.34 to 2.06; p < 0.001)., Conclusions: SHD was common in our HD study population, and RV systolic dysfunction independently predicted mortality., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Recent policy clarifications by the Centers for Medicare & Medicaid Services have changed access to outpatient dialysis care at end-stage renal disease (ESRD) facilities for individuals with acute kidney injury in the United States. Tools to predict "ESRD" and "acute" status in terms of kidney function recovery among patients who previously initiated dialysis therapy in the hospital could help inform patient management decisions., Study Design: Historical cohort study., Setting & Participants: Incident hemodialysis patients in the Mayo Clinic Health System who initiated in-hospital renal replacement therapy (RRT) and continued outpatient dialysis following hospital dismissal (2006 through 2009)., Predictor: Baseline estimated glomerular filtration rate (eGFR), acute tubular necrosis from sepsis or surgery, heart failure, intensive care unit, and dialysis access., Outcomes: Kidney function recovery defined as sufficient kidney function for outpatient hemodialysis therapy discontinuation., Results: Cohort consisted of 281 patients with a mean age of 64 years, 63% men, 45% with heart failure, and baseline eGFR≥30mL/min/1.73m(2) in 46%. During a median of 8 months, 52 (19%) recovered, most (94%) within 6 months. Higher baseline eGFR (HR per 10-mL/min/1.73m(2) increase eGFR, 1.27; 95% CI, 1.16-1.39; P<0.001), acute tubular necrosis from sepsis or surgery (HR, 3.34; 95% CI, 1.83-6.24; P<0.001), and heart failure (HR, 0.40; 95% CI, 0.19-0.78, P=0.007) were independent predictors of recovery within 6 months, whereas first RRT in the intensive care unit and catheter dialysis access were not. There was a positive interaction between absence of heart failure and eGFR≥30mL/min/1.73m(2) for predicting kidney function recovery (P<0.001)., Limitations: Sample size., Conclusions: Kidney function recovery in the outpatient hemodialysis unit following in-hospital RRT initiation is not rare. As expected, higher baseline eGFR is an important determinant of recovery. However, patients with heart failure are less likely to recover even with a higher baseline eGFR. Consideration of these factors at hospital discharge informs decisions on ESRD status designation and long-term hemodialysis care., (Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Clinical disease due to human T cell lymphotropic virus type 1 (HTLV-1), a retrovirus endemic in certain regions of the world, is rarely reported after solid organ transplantation. In 2009, universal deceased donor organ screening for HTLV-1 was discontinued in the United States. We report the first case of donor-derived HTLV-1-associated myelopathy in a kidney transplant recipient from the United States. The patient, who was HTLV-1-seronegative prior to transplantation, likely acquired HTLV-1 infection from a seropositive organ donor. In this era when screening of donors and recipients for HTLV infection is not mandatory, clinicians should be vigilant in recognizing the risk and potential occurrence of this donor-derived infection in recipients with epidemiologic exposures., (© Copyright 2014 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Objective: To report 3 cases of reversible hypothyroidism-induced kidney dysfunction and review the interaction between these commonly encountered, yet seemingly disparate, conditions., Methods: We describe the clinical course and laboratory and physical findings of 3 patients who presented with kidney dysfunction that improved after initiating thyroid hormone replacement therapy. We also review similar cases in the literature and discuss the pathophysiologic mechanisms., Results: A 68-year-old male presented with classical signs and symptoms of hypothyroidism, including fatigue, confusion, and gait imbalance. Physical exam showed bradycardia, thyromegaly, slow mentation, and cracked, thin skin; he was found to have decreased kidney function. Second, a 42-year-old previously healthy female presented with bilateral hand swelling and elevated serum creatinine with an otherwise unremarkable physical exam. The third patient was a 72-year-old male with advanced heart failure on amiodarone and stage 3 chronic kidney disease who presented with fatigue, acute kidney injury, and lower extremity edema. In all cases, serum creatinine and thyroid-stimulating hormone (TSH) were elevated at presentation (1.4-3.0 mg/dL and 94.1-184 mIU/L respectively), and free thyroxine (T4) was low (undetectable-0.4 ng/dL). The initiation or increased dose of levothyroxine normalized serum creatinine to baseline within 2 to 10 months., Conclusion: Hypothyroidism and kidney dysfunction are both commonly encountered clinical entities, but the interplay between the thyroid gland and kidneys may be infrequently recalled, causing the reversible relationship between these 2 disorders to be missed.

Aims: The number of elderly persons with end-stage renal disease is increasing with many requiring hospitalizations. This study examines the causes and predictors of hospitalization in older haemodialysis patients., Methods: We reviewed hospitalizations of older (≥65 years) incident chronic haemodialysis patients initiating therapy between 1 January 2007 and 31 December 2009 under the care of a single Midwestern United States dialysis provider., Results: Of 125 patients, the mean age was 76 ± 7 years and 72% were male. At first dialysis, 68% used a central venous catheter (CVC) and 51% were in the hospital. Mean follow-up was 1.8 ± 1.0 years. At least one hospitalization occurred in 89 (71%) patients and half of all patients were hospitalized once within the first 223 days. Total hospital admission rate was 1.48 per patient year with hospital days totalling 8.54 days per patient year. The three most common reasons for first admission were cardiac (33%), infection (18%) and gastrointestinal (12%). Predictors of future hospitalization included the first dialysis occurring in hospital (hazard ratios (HR) 2.1, 95% CI 1.4-3.3, P = 0.0005) and the use of a CVC at first haemodialysis (HR 2.6, CI 1.6-4.4, P < 0.0001)., Conclusion: Hospitalizations are common in older incident haemodialysis patients. Access preparation and overall burden of illness leading to the initial hospitalization appear to play a role. Identification of additional factors associated with hospitalization will allow for focused interventions to reduce hospitalization rates and increase the value of care., (© 2013 Asian Pacific Society of Nephrology.)

Thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and scleroderma renal crisis (SRC) all present with features of thrombotic microangiopathy. Distinguishing among these entities is critical, however, as treatments differ and may be mutually exclusive. We describe the case of a 25-year-old woman with an undefined mixed connective tissue disease who presented 6 weeks post-partum with fever, transient aphasia, thrombocytopenia, hemolytic anemia, and acute kidney injury eventually requiring initiation of hemodialysis. Renal biopsy revealed thrombotic microangiopathy. Renal function did not improve despite immediate initiation of plasma exchange, and an angiotensin-converting enzyme (ACE) inhibitor was initiated following discontinuation of plasma exchange. At last follow up, she remained dialysis dependent. Due to the myriad causes of thrombotic microangiopathy and potential for diagnostic uncertainty, the patient's response to therapy should be closely monitored and used to guide modification of therapy.

We present the case of a 62-year-old man with a history of Wegener granulomatosis who developed chronic thrombotic microangiopathy attributed to gemcitabine chemotherapy. Wegener granulomatosis had been diagnosed 15 years earlier, and the patient was treated using cyclophosphamide and prednisone, then maintained on mycophenolate mofetil and prednisone. Four years previously, he had been treated with mitomycin C for urothelial carcinoma and at the time of presentation had developed significant anemia and thrombocytopenia after a course of gemcitabine and carboplatin due to metastasis of the carcinoma. He was managed using red blood cell and platelet transfusions but then developed acute kidney injury, along with progressive dyspnea and pulmonary infiltrates. Imaging studies showed bilateral ureteral obstruction requiring placement of nephrostomy tubes. Because of concern about a flare of Wegener granulomatosis after withdrawing maintenance immunosuppression in the context of the malignancy, a kidney biopsy was performed that showed chronic thrombotic microangiopathy, likely secondary to gemcitabine chemotherapy. Clinical, laboratory, and pathologic findings of this case are discussed to illustrate the natural history of thrombotic microangiopathy associated with gemcitabine chemotherapy., (Copyright © 2011 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Studies of the impact of systemic lupus erythematosus (SLE) and its pregnancy complications have yielded conflicting results. Major limitations of these studies relate to their small numbers of patients and retrospective designs. The aim of this study was to perform a systematic literature review of pregnancy outcomes in women with SLE and a meta-analysis of the association of lupus nephritis with adverse pregnancy outcomes., Design, Setting, Participants, & Measurements: We searched electronic databases from 1980 to 2009 and reviewed papers with validity criteria. Random-effects analytical methods were used to evaluate pregnancy complications rates., Results: Thirty-seven studies with 1842 patients and 2751 pregnancies were included. Maternal complications included lupus flare (25.6%), hypertension (16.3%), nephritis (16.1%), pre-eclampsia (7.6%), and eclampsia (0.8%). The induced abortion rate was 5.9%, and when excluded, fetal complications included spontaneous abortion (16.0%), stillbirth (3.6%), neonatal deaths (2.5%), and intrauterine growth retardation (12.7%). The unsuccessful pregnancy rate was 23.4%, and the premature birth rate was 39.4%. Meta-regression analysis showed statistically significant positive associations between premature birth rate and active nephritis and increased hypertension rates in subjects with active nephritis or a history of nephritis. History of nephritis was also associated with pre-eclampsia. Anti-phospholipid antibodies were associated with hypertension, premature birth, and an increased rate of induced abortion., Conclusions: In patients with SLE, both lupus nephritis and anti-phospholipid antibodies increase the risks for maternal hypertension and premature births. The presented evidence further supports timing of pregnancy relative to SLE activity and multispecialty care of these patients.

Background: The benefits of mentoring residents have been studied, but there is no research about mentoring new program directors. Program directors' responsibilities have become increasingly complicated, and they may not be adequately prepared for their role at the time of appointment without the benefit of mentoring that is specific to their new role., Objective: To assess whether nephrology subspecialty program directors were specifically mentored and whether they felt prepared for the educational and administrative aspects of this role., Methods: We conducted an electronic needs assessment survey of the nephrology subspecialty program directors through the American Society of Nephrology listserv for program directors., Results: The survey response rate was 42% (58 of 139). Of the respondents, 58% did not feel adequately prepared when they first became subspecialty program directors, and only 32% reported having formal or informal mentoring for the role. Individuals who had served as associate program directors (34%) were more likely to report mentoring than those who had not (P  =  .02). Eighty percent of respondents reported that mentoring from another nephrology program director would have been beneficial during their first several years., Conclusions: This appears to be the first study specifically evaluating mentoring experiences relevant to the role of nephrology program director. As a result of this survey, the American Society of Nephrology Training Program Director Executive Committee recognized the need to provide opportunities for mentoring new nephrology program directors and formed a New Training Program Director Training and Mentoring Work Group to recommend initiatives for mentoring and training new program directors. Further investigation is needed to assess whether mentoring benefits subspecialty program directors and whether these findings can be generalized to other specialties and subspecialties.

Transient episodes of pain are common in autosomal dominant polycystic kidney disease (ADPKD). A small fraction of patients have disabling chronic pain. In this review, we discuss the etiologies of pain in ADPKD; review how ADPKD patients should be assessed; and discuss medical, surgical, and other management options., (Copyright 2010 National Kidney Foundation, Inc. All rights reserved.)

Membranous nephropathy (MN) recurs posttransplant in 42% of patients. We compared MN recurrence rates in a historical cohort transplanted between 1990 and 1999 and in a current cohort diagnosed by protocol biopsies, we analyzed the progression of the disease and we assessed the effects of anti-CD20 antibodies (Rituximab) on recurrent MN. The incidence of recurrent MN was similar in the historical (53%) and the current cohorts (41%), although in the later the diagnosis was made earlier (median, 4[2-21] months vs. 83[6-149], p = 0.002) and the disease was clinically milder. Twelve out of 14 patients (86%) with recurrent MN in the current cohort had progressive increases in proteinuria. Eight recipients were treated with Rituximab after their proteinuria increased from median, 211 mg/day (64-4898) at diagnosis to 4489 (898-13 855) (p = 0.038). Twelve months post-Rituximab, 75% of patients had either partial (PR) or complete remission (CR). After 24 months 6/7 (86%) had PR/CR and one patient relapsed. Posttreatment biopsies showed resorption of electron dense immune deposits in 6/7 cases and were negative for C3 (4/7) and IgG (3/7). Protocol biopsies allow early diagnosis of subclinical recurrent MN, which is often progressive. Treatment of recurrent MN with Rituximab is promising and should be evaluated in a prospective randomized controlled trial.

Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults. MN can recur after kidney transplantation causing proteinuria, allograft dysfunction and graft failure. In this study we assessed the incidence of MN recurrence utilizing surveillance graft biopsies. The study included 1310 renal allograft recipients from 2000 to 2006. Glomerular diseases were the cause of kidney failure in 28% of patients and 23 (2%) had idiopathic MN. Recurrent MN was diagnosed in eight of 19 patients included in this analysis (42%) 13 +/- 20 months (median = 4; range 2-61 months) after transplant. The initial clinical manifestations of recurrent MN were mild or absent. Urine protein excretion was 825 +/- 959 (64-2286) mg/day and three patients had no proteinuria. Five of seven patients who did not receive additional immunosuppression for MN had significant increases in proteinuria during follow up and three became nephrotic. At diagnosis, light microscopic changes were subtle or absent. All patients had granular glomerular basement membrane deposits of IgG but little or absent C3 by immunofluorescence. Subepithelial deposits were observed in all cases by electron microscopy. In conclusion, idiopathic MN recurred in 42% of patients after transplantation. The initial clinical and histologic manifestations are subtle but the disease is progressive.

A 68-year-old woman with a history of hepatitis C (contracted from a blood transfusion in 1974) complicated by cirrhosis and portal hypertension came to the Mayo Clinic in Rochester, Minn, for evaluation for possible liver transplantation. Her symptomatic ascites had been treated initially with furosemide and spironolactone, but this treatment regimen was limited because of an increase in her creatinine level. During evaluation, hypertension (an average blood pressure of 180/90 mm Hg on 6-hour ambulatory monitoring) and abnormal renal function were noted. She was referred to our institution for further evaluation of her blood pressure and abnormal urinalysis results.

Background: Up-regulation of heme oxygenase-1 (HO-1) occurs in, and often confers protection to, the injured kidney. Up-regulation of monocyte chemoattractant protein-1 (MCP-1) promotes not only acute and chronic nephritides but also acute ischemic and nephrotoxic injury. The present study was stimulated by the hypothesis that expression of MCP-1 is suppressed by HO-1, and analyzed the effect of HO-1 on the expression of MCP-1 in stressed and unstressed conditions., Methods: Expression of MCP-1 and pathophysiologic correlates were examined in HO-1 knockout (HO-1-/-) and wild-type (HO-1+/+) mice in the unstressed state in young and aged mice, and following nephrotoxic and ischemic insults., Results: In unstressed HO-1-/- mice, plasma levels of MCP-1 protein were elevated, and MCP-1 mRNA expression was increased in circulating leukocytes and in the kidney. Such early and heightened up-regulation of MCP-1 was eventually accompanied by phenotypic changes in the aged kidney consistent with MCP-1, namely, proliferative changes in glomeruli, tubulointerstitial disease, and up-regulation of transforming growth factor-beta1 (TGF-beta1) and collagens I, III, and IV. In response to a nephrotoxic insult such as hemoglobin, MCP-1 mRNA was up-regulated in a markedly sustained manner in HO-1-/- mice. In response to a duration of ischemia that exerted little effect in HO-1+/+ mice, HO-1-/- mice exhibited higher expression of MCP-1 mRNA, enhanced activation of nuclear factor-kappaB (NF-kappaB) (the transcription factor that regulates MCP-1), markedly greater functional and structural renal injury, increased caspase-3 expression, and increased mortality., Conclusion: In the absence of HO-1, expression of MCP-1 is significantly and consistently enhanced in unstressed and stressed conditions. We speculate that the protective effects of HO-1 in injured tissue may involve, at least in part, the capacity of HO-1 to restrain up-regulation of MCP-1.

Many reports confirm that thin basement membrane nephropathy (TBMN) commonly occurs together with other glomerular diseases such as minimal change glomerulonephritis, diabetes, membranous nephropathy, immunoglobulin (Ig)A glomerulonephritis, and focal segmental glomerulosclerosis. We postulate 3 explanations for these observations. The association of minimal change glomerulonephritis with TBMN probably is artifactual whereas the association with diabetes and membranous glomerulonephritis probably is coincidental. However, the link between TBMN and IgA disease and focal segmental glomerulosclerosis may be pathogenetic. Clinical evidence indicates that the presence of an associated glomerulopathy significantly worsens the prognosis of TBMN. Thus, patients with TBMN and another glomerular lesion usually have more marked proteinuria, and are more likely to have hypertension and renal insufficiency. The frequency of another glomerulopathy in patients with TBMN means that all patients in whom TBMN is suspected but who have heavy proteinuria or renal insufficiency should undergo a renal biopsy examination. However, there is no evidence that TBMN alters the prognosis of another glomerulopathy, and, in particular, patients with TBMN and IgA disease do not have different clinical features or a worse prognosis than those with IgA disease alone.

Background: Information about pregnancy in females with primary hyperoxaluria types I and II (PH-I, PH-II) is limited to isolated case reports. This study sought to determine the number and outcomes of pregnancies in a series of women with PH-I and PH-II, to assess the incidence of complications during pregnancy, and to characterize infant outcomes., Methods: From a database of patients with PH followed at the Mayo Clinic, we identified 16 females who had been pregnant. A retrospective medical record review and telephone survey were performed., Results: Forty pregnancies occurring between 1961 and 1998 were identified: 26 pregnancies in 11 patients with PH-I and 14 pregnancies in 5 patients with PH-II. Thirty (75%) of the pregnancies were carried to term, and 33 infants were born. Four miscarriages, 4 preterm births, and 2 elective abortions occurred. No maternal complications were reported in half of the pregnancies. In the remaining pregnancies, the most common complications were hypertension, urinary tract infection, and urolithiasis-associated symptoms. One PH-I patient developed pre-eclampsia resulting in a stillborn infant, and another PH-1 patient developed hyperemesis gravidarum with a decline in renal function. Approximately 75% of the infants had no perinatal problems. All of the PH-I patients eventually required initiation of renal replacement therapy at a mean of 17.5 years following their first pregnancy. No PH-II patients have required renal replacement therapy., Conclusion: Overall, pregnancy appeared to be well tolerated in this series of women with primary hyperoxaluria, with 1 of 16 women experiencing loss of renal function during 1 of 40 pregnancies.

Hypertension is an asymptomatic chronic disease that contributes to the development of serious health problems including coronary artery disease, chronic renal failure, and stroke. Despite published guidelines addressing goals for the treatment of hypertension, control rates (defined as a blood pressure <140/90 mm Hg) have not increased in recent years, and uncontrolled hypertension remains a serious public health issue. Both patient- and provider-related factors contribute to these poor control rates, and new approaches to the management of hypertension must be sought. In this review, we describe unique physician-nurse team approaches to improve the control of hypertension both in a subspecialty hypertension practice and in a primary care clinic setting. By implementing practice models that result in sustainable improvements in blood pressure control rates, the morbidity and mortality resulting from target organ damage and ensuing costs to society may be expected to decline as well.

Acute renal failure is commonly due to acute tubular necrosis (ATN), the latter representing an acute, usually reversible loss of renal function incurred from ischemic or nephrotoxic insults occurring singly or in combination. Such insults instigate a number of processes-hemodynamic alterations, aberrant vascular responses, sublethal and lethal cell damage, inflammatory responses, and nephron obstruction-that initiate and maintain ATN. Eventually, reparative and regenerative processes facilitate the resolution of renal injury and the recovery of renal function. Focusing mainly on ischemic ATN, this article reviews evidence indicating that the inordinate or aberrant generation of reactive oxygen species (ROS) may contribute to the initiation and maintenance of ATN. This review also discusses the possibility that ROS may instigate adaptive as well as maladaptive responses in the kidney with ATN, and raises the possibility that ROS may participate in the recovery phase of ATN.