Autosomal dominant ApoA4 mutations present as tubulointerstitial kidney disease with medullary amyloidosis.

Sporadic cases of apolipoprotein A-IV medullary amyloidosis have been reported. Here we describe five families found to have autosomal dominant medullary amyloidosis due to two different pathogenic APOA4 variants. A large family with autosomal dominant chronic kidney disease (CKD) and bland urinary sediment underwent whole genome sequencing with identification of a chr11:116692578 G>C (hg19) variant encoding the missense mutation p.L66V of the ApoA4 protein. We identified two other distantly related families from our registry with the same variant and two other distantly related families with a chr11:116693454 C>T (hg19) variant encoding the missense mutation p.D33N. Both mutations are unique to affected families, evolutionarily conserved and predicted to expand the amyloidogenic hotspot in the ApoA4 structure. Clinically affected individuals suffered from CKD with a bland urinary sediment and a mean age for kidney failure of 64.5 years. Genotyping identified 48 genetically affected individuals; 44 individuals had an estimated glomerular filtration rate (eGFR) under 60 ml/min/1.73 m2, including all 25 individuals with kidney failure. Significantly, 11 of 14 genetically unaffected individuals had an eGFR over 60 ml/min/1.73 m2. Fifteen genetically affected individuals presented with higher plasma ApoA4 concentrations. Kidney pathologic specimens from four individuals revealed amyloid deposits limited to the medulla, with the mutated ApoA4 identified by mass-spectrometry as the predominant amyloid constituent in all three available biopsies. Thus, ApoA4 mutations can cause autosomal dominant medullary amyloidosis, with marked amyloid deposition limited to the kidney medulla and presenting with autosomal dominant CKD with a bland urinary sediment. Diagnosis relies on a careful family history, APOA4 sequencing and pathologic studies.

Burnout and Emotional Well-Being among Nephrology Fellows: A National Online Survey.

BACKGROUND: Physician burnout and emotional distress are associated with work dissatisfaction and provision of suboptimal patient care. Little is known about burnout among nephrology fellows. METHODS: Validated items on burnout, depressive symptoms, and well being were included in the American Society of Nephrology annual survey emailed to US nephrology fellows in May to June 2018. Burnout was defined as an affirmative response to two single-item questions of experiencing emotional exhaustion or depersonalization. RESULTS: Responses from 347 of 808 eligible first- and second-year adult nephrology fellows were examined (response rate=42.9%). Most fellows were aged 30-34 years (56.8%), male (62.0%), married or partnered (72.6%), international medical graduates (62.5%), and pursuing a clinical nephrology fellowship (87.0%). Emotional exhaustion and depersonalization were reported by 28.0% and 14.4% of the fellows, respectively, with an overall burnout prevalence of 30.0%. Most fellows indicated having strong program leadership (75.2%), positive work-life balance (69.2%), presence of social support (89.3%), and career satisfaction (73.2%); 44.7% reported a disruptive work environment and 35.4% reported depressive symptoms. Multivariable logistic regression revealed a statistically significant association between female gender (odds ratio [OR], 1.90; 95% confidence interval [95% CI], 1.09 to 3.32), poor work-life balance (OR, 3.97; 95% CI, 2.22 to 7.07), or a disruptive work environment (OR, 2.63; 95% CI, 1.48 to 4.66) and burnout. CONCLUSIONS: About one third of US nephrology fellows surveyed reported experiencing burnout and depressive symptoms. Further exploration of burnout-especially that reported by female physicians, as well as burnout associated with poor work-life balance or a disruptive work environment-is warranted to develop targeted efforts that may enhance the educational experience and emotional well being of nephrology fellows.

Primary Care Physicians' Perceived Barriers to Nephrology Referral and Co-management of Patients with CKD: a Qualitative Study.

BACKGROUND: Effective co-management of patients with chronic kidney disease (CKD) between primary care physicians (PCPs) and nephrologists is increasingly recognized as a key strategy to ensure the delivery of efficient and high-quality CKD care. However, the co-management of patients with CKD remains suboptimal. OBJECTIVE: We aimed to identify PCPs' perceptions of key barriers and facilitators to effective co-management of patients with CKD at the PCP-nephrology interface. STUDY DESIGN: Qualitative study SETTING AND PARTICIPANTS: Community-based PCPs in four US cities: Baltimore, MD; St. Louis, MO; Raleigh, NC; and San Francisco, CA APPROACH: We conducted four focus groups of PCPs. Two members of the research team coded transcribed audio-recorded interviews and identified major themes. KEY RESULTS: Most of the 32 PCPs (59% internists and 41% family physicians) had been in practice for > 10 years (97%), spent ≥ 80% of their time in clinical care (94%), and practiced in private (69%) or multispecialty group practice (16%) settings. PCPs most commonly identified barriers to effective co-management of patients with CKD focused on difficulty developing working partnerships with nephrologists, including (1) lack of timely adequate information exchange (e.g., consult note not received or CKD care plan unclear); (2) unclear roles and responsibilities between PCPs and nephrologists; and (3) limited access to nephrologists (e.g., unable to obtain timely consultations or easily contact nephrologists with concerns). PCPs expressed a desire for "better communication tools" (e.g., shared electronic medical record) and clear CKD care plans to facilitate improved PCP-nephrology collaboration. CONCLUSIONS: Interventions facilitating timely adequate information exchange, clear delineation of roles and responsibilities between PCPs and nephrologists, and greater access to specialist advice may improve the co-management of patients with CKD.

Bariatric surgery for obesity-associated decline in kidney function: filling the knowledge gap?

Chang et al. (2016) report a significantly lower risk of decline in estimated glomerular filtration rate among obese adults who underwent bariatric surgery compared with a matched nonsurgical cohort. In this propensity-matched analysis, data on confounding variables such as albuminuria, psychosocial, and medical conditions that precluded surgery in the comparator arm and health insurance are lacking. Furthermore, creatinine-based estimated glomerular filtration rate is not an accurate measure of kidney function after intentional weight loss. Although the study is interesting, physicians need to carefully weigh the risks versus benefits of bariatric surgery among obese adults at risk of kidney disease.

Impact of an Educational Program for Advanced Practice Nurses on Knowledge of Kidney Disease Outcomes Quality Initiative Guidelines.

Patients with chronic kidney disease (CKD) are often referred to a nephrologist late. Contributing factors include primary care providers' lack of awareness of practice guidelines for treating kidney disease and their uncertainty of timing for referral to a nephrologist. The purpose of this quasi-experimental study was to determine if advanced practice nurses working in primary care are knowledgeable about the National Kidney Foundations Kidney Disease Outcomes Quality Initiative Guidelines, if a CKD education program increases knowledge, and if knowledge is retained. Fourteen advanced practice nurses participated in the study. The knowledge outcome was measured using a CKD knowledge-based survey. The results showed a significant increase in knowledge post-intervention; moreover, knowledge gained was retained at the one-month follow-up intervaL This evidence-based practice project was developed to promote timely referral to a nephrologist, which may then slow the progression of kidney disease, decrease morbidity and mortality, and reduce healthcare cost.

Severe hypokalemia in a patient with subarachnoid hemorrhage.

Hypokalemia is a common electrolyte disorder in the intensive care unit. Its cause often is complex, involving both potassium losses from the body and shifts of potassium into cells. We present a case of severe hypokalemia of sudden onset in a patient being treated for subarachnoid hemorrhage in the surgical intensive care unit in order to illustrate the diagnosis and management of severe hypokalemia of unclear cause. Our patient received agents that promote renal potassium losses and treatments associated with a shift of potassium into cells. We outline the steps in diagnosis and management, focusing on the factors regulating the transcellular distribution of potassium in the body.

Update on role of direct renin inhibitor in diabetic kidney disease.

BACKGROUND: Diabetic kidney disease (DKD) is a leading cause of end-stage renal disease (ESRD). Renin-angiotensin-aldosterone system (RAAS) plays a critical role in the development of DKD with angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) being the mainstay of treatment. Systemic RAAS activity has been implicated in the pathogenesis of DKD, but lately interest has shifted to intrarenal RAAS effect. With the discovery of the (pro)renin receptor and ACE independent pathways of angiotensin II production, our understanding of role of renin in end organ damage has improved significantly. SUMMARY: We summarize our current understanding of ACE dependent and independent pathways in the development of DKD and the preclinical models demonstrating renal effects of direct renin inhibitors (DRIs). We then review clinical studies and trials performed so far evaluating the efficacy of aliskiren on renal outcomes and safety in DKD. KEY MESSAGE: At present, there is little evidence for renal benefit of aliskiren in DKD beyond that offered by ACEIs or ARBs. Combining aliskiren with ACEI or ARB in DKD did not significantly improve renal outcomes in comparison with ACEI or ARB monotherapy in clinical trials. Slightly more adverse events including hyperkalemia, acute kidney injury and hypotension were observed in the combination therapy as compared to the monotherapy. Thus, current evidence suggests that aliskiren, because of its antihypertensive and antiproteinuric effects, maybe used as monotherapy in DKD and considered an equivalent alternative to ACEIs or ARBs. Careful monitoring for renal adverse effects would allow safe clinical use of DRI.

Clustering user preferences for personalized teleoperation control schemes via trajectory similarity analysis.

Successful operation of a teleoperated robot depends on a well-designed control scheme to translate human motion into robot motion; however, a single control scheme may not be suitable for all users. On the other hand, individual personalization of control schemes may be infeasible for designers to produce. In this paper, we present a method by which users may be classified into groups with mutually compatible control scheme preferences. Users are asked to demonstrate freehand motions to control a simulated robot in a virtual reality environment. Hand pose data is captured and compared with other users using SLAM trajectory similarity analysis techniques. The resulting pairwise trajectory error metrics are used to cluster participants based on their control motions, without foreknowledge of the number or types of control scheme preferences that may exist. The clusters identified for two different robots shows that a small number of clusters form stably for each case, each with its own control scheme paradigm. Survey data from participants validates that the clusters identified through this method correspond to the participants' control scheme rationales, and also identify nuances in participant control scheme descriptions that may not be obvious to designers relying only on participant explanations of their preferences.

Role of Physical Activity on Mental Health and Well-Being: A Review.

In addition to the apparent physical health benefits, physical activity also affects mental health positively. Physically inactive individuals have been reported to have higher rates of morbidity and healthcare expenditures. Commonly, exercise therapy is recommended to combat these challenges and preserve mental wellness. According to empirical investigations, physical activity is positively associated with certain mental health traits. In nonclinical investigations, the most significant effects of physical exercise have been on self-concept and body image. An attempt to review the current understanding of the physiological and psychological mechanisms by which exercise improves mental health is presented in this review article. Regular physical activity improves the functioning of the hypothalamus-pituitary-adrenal axis. Depression and anxiety appear to be influenced by physical exercise, but to a smaller extent in the population than in clinical patients. Numerous hypotheses attempt to explain the connection between physical fitness and mental wellness. Physical activity was shown to help with sleep and improve various psychiatric disorders. Exercise in general is associated with a better mood and improved quality of life. Physical exercise and yoga may help in the management of cravings for substances, especially in people who may not have access to other forms of therapy. Evidence suggests that increased physical activity can help attenuate some psychotic symptoms and treat medical comorbidities that accompany psychotic disorders. The dearth of literature in the Indian context also indicated that more research was needed to evaluate and implement interventions for physical activity tailored to the Indian context.

Learning curve for robotic assisted total knee arthroplasty: our experience with imageless hand-held Navio system.

The main purpose of this study was to determine the learning curve of Robotic assisted Total Knee Arthroplasty surgery through assessment of operative time and comparison with that of conventional jig based Total Knee Arthroplasty. The study included our first 75 Robotic assisted Total Knee Arthroplasty and 25 randomly selected conventional jig-based Total knee arthroplasty from June 2017 to December 2017. The 75 cases were divided into 3 groups of 25 consecutive cases. The mean of operative time for each phase and total time was compared between the 3 groups and with the mean of total time for conventional jig based group. In our experience, Robotic assisted Total Knee arthroplasty was associated with a learning curve of approximately 25 cases. The mean for Registration phase of Group A (1st set of 25 cases) was 6.12 min (SD 1.8 min), group B (2nd set of 25 cases) was 4.46 min (SD 0.79 min) and group C (3rd set of 25 cases) was 4.17 min (SD 0.59 min). The mean for Planning phase of group A was 5.08 min (SD 1.01 min), group B was 4.04 min (SD 0.37 min) and group C was 4.01 min (SD 0.35 min). The mean for Cutting Phase of group A was 28.22 min (SD 6.24 min), group B was 22.49 min (SD 0.79 min) and group C was 22.36 min (SD 0.88 min). The mean for total time of group A was 39.42 min (SD 8.02), group B was 31 min (SD 1.22 min), group C was 30.53 min (SD 1.14 min) and conventional group was 30.54 min (SD 1.14 min). On comparing the Registration phase (Group A vs B, p < 0.001; Group B vs C, p 0.14; Group A vs C, p < 0.001), Planning phase (Group A vs B, p < 0.001; Group B vs C, p 0.75; Group A vs C, p < 0.001), Cutting phase (Group A vs B, p < 0.001; Group B vs C, p 0.58; Group A vs C, p < 0.001) and Total time (Group A vs B, p < 0.001; Group B vs C, p 0.74; Group A vs C, p < 0.001; Group A vs Conventional, p < 0.001; Group B vs Conventional, p 0.17, Group C vs Conventional, p 0.99), the results showed that the inflection point for learning curve in our hands was 25 cases. The learning curve and increased operation theatre time are likely to be major barrier in widespread acceptance of robotic technology amongst arthroplasty surgeons. We, in our experience can say that the learning curve was approximately 25 cases. The results of this study will help the arthroplasty surgeons in accepting this technology and achieve better outcomes.

Breast Cancer and the Brain: A Comprehensive Review of Neurological Complications.

Breast cancer, one of the most prevalent malignancies globally, poses a substantial health burden with its diverse neurological complications. This comprehensive review examines the intricate landscape of breast cancer's neurological effects, encompassing brain metastases, non-metastatic complications, and their profound influence on the quality of life, prognosis, and survival of affected individuals. The mechanisms, clinical manifestations, and treatment modalities of brain metastasis and the critical role of interdisciplinary collaboration in their management are explored. Additionally, we address non-metastatic neurological complications, including paraneoplastic syndromes, treatment-related side effects, leptomeningeal carcinomatosis, and radiation-induced neurotoxicity, shedding light on the challenges they present and the importance of cognitive and emotional well-being. Prognostic factors and survival rates are discussed, emphasizing the complexity of variables impacting patient outcomes. Lastly, we underscore the vital role of collaborative care in addressing these multifaceted challenges, highlighting future research directions and the ongoing quest to enhance the quality of life for breast cancer patients.

Exploring the Risks, Benefits, Advances, and Challenges in Internet Integration in Medicine With the Advent of 5G Technology: A Comprehensive Review.

The integration of 5G technology in the healthcare sector is poised to bring about transformative changes, offering numerous advantages such as enhanced telemedicine services, expedited data transfer for medical records, improved remote surgery capabilities, real-time monitoring and diagnostics, advancements in wearable medical devices, and the potential for precision medicine. However, this technological shift is not without its concerns, including potential health implications related to 5G radiation exposure, heightened cybersecurity risks for medical devices and data systems, potential system failures due to technology dependence, and privacy issues linked to data breaches in healthcare. We are striking a balance between harnessing these benefits and addressing the associated risks. Achieving this equilibrium requires the establishment of a robust regulatory framework, ongoing research into the health impacts of 5G radiation, the implementation of stringent cybersecurity measures, education and training for healthcare professionals, and the development of ethical standards. The future of 5G in the medical field holds immense promise, but success depends on our ability to navigate this evolving landscape while prioritizing patient safety, privacy, and ethical practice.

Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis.

Among the various renal manifestations of sarcoidosis, granulomatous inflammation confined to the tubulointerstitial compartment is the most commonly reported finding. We present the case of a 66-year-old man with acute kidney injury, hypercalcemia, mild restrictive pulmonary disease, and neurologic signs of parietal lobe dysfunction. Kidney biopsy showed diffuse interstitial inflammation with noncaseating granulomas that exhibited the unusual feature of infiltrating the walls of small arteries with destruction of the elastic lamina, consistent with granulomatous vasculitis. The findings of granulomatous interstitial nephritis on kidney biopsy, hypercalcemia, and possible cerebral and pulmonary involvement in the absence of other infectious, drug-induced, or autoimmune causes of granulomatous disease established the diagnosis of sarcoidosis. Pulse methylprednisolone followed by maintenance prednisone therapy led to improvement in kidney function, hypercalcemia, and neurologic symptoms. Vasculocentric granulomatous interstitial nephritis with granulomatous vasculitis is a rare and under-recognized manifestation of renal sarcoidosis.

Access to health care among adults evaluated for CKD: findings from the Kidney Early Evaluation Program (KEEP).

BACKGROUND: Data are scant regarding access to health care in patients with chronic kidney disease (CKD). We performed descriptive analyses using data from the National Kidney Foundation's Kidney Early Evaluation Program (KEEP), a nationwide health screening program for adults at high risk of CKD. METHODS: From 2000-2010, a total of 122,502 adults without end-stage renal disease completed KEEP screenings; 27,927 (22.8%) met criteria for CKD (10,082, stages 1-2; 16,684, stage 3; and 1,161, stages 4-5). CKD awareness, self-rated health status, frequency of physician visits, difficulty obtaining medical care, types of caregivers, insurance status, and medication coverage and estimated costs were assessed. RESULTS: Participants with CKD were more likely to report fair/poor health status than those without CKD. Health care utilization increased at later CKD stages; ~95% of participants at stages 3-5 had visited a physician during the preceding year compared with 83.7% of participants without CKD. More Hispanic and African American than white participants at all CKD stages reported not having a physician. Approximately 40% of participants younger than 65 years reported fair/poor health status at stages 4-5 compared with ~30% who were 65 years and older. Younger participants at all stages were more likely to report extreme or somewhat/moderate difficulty obtaining medical care. Comorbid conditions (diabetes, hypertension, and prior cardiovascular events) were associated with increased utilization of care. Utilization of nephrology care was poor at all CKD stages; <6% of participants at stage 3 and <30% at stages 4-5 reported ever seeing a nephrologist. CONCLUSIONS: Lack of health insurance and perceived difficulty obtaining medical care with lower health care utilization, both of which are consistent with inadequate access to health care, are more likely for KEEP participants who are younger than 65 years, nonwhite, and without previously diagnosed comorbid conditions. Nephrology care is infrequent in elderly participants with advanced CKD who are nonwhite, have comorbid disease, and have high-risk states for cardiovascular disease.

Does robotic technology successfully restore the joint line after total knee arthroplasty? A retrospective analysis.

BACKGROUND: Our study aims to determine the effectiveness of robotic technology for total knee arthroplasty in the successful restoration of the joint line of the knee with respect to that of a normal human anatomical knee. The restoration of the joint line is an important technical goal on which the postoperative outcomes and the success of the surgery depend. METHODS: Sixty-four postoperative plain anteroposterior radiographs of 60 patients, who received total knee arthroplasty by using the robotic technology were analyzed and compared with 66 similar radiographs of 60 patients who received the conventional method. The distances of the lateral epicondyle to the joint line (LEJL) and proximal tibiofibular joint to the joint line (PTFJJL) were calculated and analyzed. RESULTS: We found that the mean value of LEJL minus PTFJJL in the robotic group was 0.334 ± 0.115 (mean ± SD), while in the conventional group, it was 2.304 ± 0.308. The difference between the two groups was statistically significant. The mean ratio (LEJL:PTFJJL) in the robotic group was also equal to 1.017 ± 0.042. CONCLUSION: From these findings it could be concluded that the robotic technology significantly increases the accuracy of the total knee arthroplasty and, compared to the conventional method, achieves an almost anatomical position of the joint line.

Burnout Among Nephrologists in the United States: A Survey Study.

Rationale & Objective: Burnout decreases job satisfaction and leads to poor patient outcomes but remains underinvestigated in nephrology. We explored the prevalence and determinants of burnout among a sample of nephrologists. Study Design: Cross-sectional. Setting & Participants: The nephrologists were approached via the American Medical Association Physicians Masterfile, National Kidney Foundation listserv, email, and social media between April and August 2019. The predictors were demographics and practice characteristics. The outcome was burnout, defined as responding "once a week" or more on either 1 of the 2 validated measures of emotional exhaustion and depersonalization or both. Analytical Approach: Participant characteristics were tabulated. Responses were compared using χ2 tests. Multivariable logistic regression was used to estimate the odds ratios (ORs) of burnout for risk factors. Free text responses were thematically analyzed. Results: About half of 457 respondents were 40-59 years old (n=225; 49.2%), and the respondents were more predominantly men (n=296; 64.8%), US medical graduates (n=285; 62.4%), and in academic practice (n=286; 62.6%). Overall, 106 (23.2%) reported burnout. The most commonly reported primary drivers of burnout were the number of hours worked (n=27; 25.5%) and electronic health record requirements (n=26; 24.5%). Caring for ≤25 versus 26-75 patients per week (OR, 0.34; 95% confidence interval [95% CI], 0.15-0.77), practicing in academic versus nonacademic settings (OR, 0.33; 95% CI, 0.21-0.54), and spending time on other responsibilities versus patient care (OR, 0.32; 95% CI, 0.17-0.61) were each independently associated with nearly 70% lower odds of burnout after adjusting for age, sex, race, and international medical graduate status. The free text responses emphasized disinterested health care systems and dissatisfaction with remuneration as the drivers of burnout. Limitations: Inability to precisely capture response rate. Conclusions: Nearly one-quarter of the nephrologists in our sample reported burnout. Future studies should qualitatively investigate how the care setting, time spent on electronic medical records, and hours of clinical care drive burnout and explore other system-level drivers of burnout in nephrology.

Skin Colonizers and Catheter Associated Blood Stream Infections in Incident Indian Dialysis Patients.

Introduction: Skin colonization is a risk factor for multi-drug resistant (MDR) catheter-associated bloodstream infections (CABSI). This study aimed to determine the prevalence and spectrum of skin colonizing MDR organisms in incident HD patients and their correlation with CABSI. Methods: This single-center prospective cohort study included consecutive adult incident HD patients who underwent tunneled or non-tunneled internal jugular vein HD catheter insertion between June 1, 2017 and October 31, 2017. Nasal, axillary, and exit site swabs were obtained prior to catheter insertion, at 14-21 days, and 28-35 days after catheter insertion. Results: Forty-three patients (69.7% male, 32.5% diabetic) were included and provided baseline swabs, while 29 and 10 patients respectively were available for follow-up swabs. MDR bacterial colonization, MRSA colonization, and MDR gram-negative colonization on the baseline set of swabs were seen in 76.7%, 69.7%, and 9.3% patients respectively. Of the 29 patients with at least two consecutive sets of swabs, 79.3% showed persistent colonization by MDR gram-positive organisms, most commonly by MRSA. Six patients developed a CABSI during the follow-up period (incidence rate 3.7 per 1000 patient days), 83.4% were gram negative, and in only one instance (16.6%) was the bacterial strain identical to that which had previously colonized the skin. Conclusions: Three-fourths of HD patients were colonized by MDR bacteria prior to HD initiation. Despite the majority being persistently colonized by MDR gram-positive organisms, CABSIs were predominantly gram negative.

Identification and management of chronic kidney disease complications by internal medicine residents: a national survey.

Many patients with chronic kidney disease (CKD) receive care from primary care physicians. Identification and management of CKD complications in primary care is suboptimal. It is not known if current residency curriculum adequately prepares a future internist in this aspect of CKD care. We performed an online questionnaire survey of internal medicine residents in the United States to determine knowledge of CKD complications and their management. Four hundred seventy-nine residents completed the survey with postgraduate year (PGY) distribution 166 PGY1, 187 PGY2, and 126 PGY3. Most of the residents correctly recognized anemia (91%) and bone disease (82%) as complications at estimated glomerular filtration rate less than 60 mL/min/1.73 m; however, only half of the residents identified coronary artery disease (54%) as a CKD complication. For a patient with estimated glomerular filtration rate less than 60 mL/min/1.73 m, two thirds of the residents would workup for anemia (62%), whereas half of them would check for mineral and bone disorder (56%). With regard to anemia of CKD, less than half of the residents knew the CKD goal hemoglobin level of 11 to 12 g/dL (44%); most would supplement iron stores (86%), whereas fewer would consider nephrology referral (28%). For mineral and bone disorders, many residents would recommend dietary phosphorus restriction (68%) and check 25-hydroxyvitamin D (62%); fewer residents would start 1,25-dihydroxyvitamin D (40%) or refer to the nephrologist (45%). Residents chose to discontinue angiotensin-converting enzyme inhibitor for medication-related complication of greater than 50% decline in estimated glomerular filtration rate (68%) and potassium greater than 5.5 mEq/L (93%). Mean performance score improved with increasing PGY (PGY1 59.4% ± 17.6%, PGY2 63.6% ± 15.6%, and PGY3 66.2% ± 16.5%; P = 0.002). Our study identified specific gaps in knowledge of CKD complications and management among internal medicine residents. Educational efforts such as instruction on use of CKD clinical practice guidelines may help raise awareness of CKD complications, benefits of early intervention, and improve CKD management.

Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent.

Thrombotic thrombocytopenic purpura (TTP) rarely occurs with systemic vasculitis. A 17-year-old girl presented with non-bloody diarrhea, menorrhagia, and syncope. She had severe anemia (hemoglobin = 3.8 g/dl), thrombocytopenia (platelet = 7,000/mm(3)), and acute kidney injury (serum creatinine, Cr = 2.3 mg%). Peripheral smear examination confirmed the presence of microangiopathic hemolytic anemia. Additionally, she had a positive anti-nuclear antibody (1:1600) and normal complement levels. We considered the diagnosis of TTP, possibly associated with systemic lupus erythematosus, and promptly initiated pulse methylprednisolone and daily 3-4 l of plasma exchange therapy. Following resolution of her thrombocytopenia in 48 h, we performed a kidney biopsy that revealed diffuse proliferative, focal crescentic, and necrotizing glomerulonephritis with mild IgG immunofluorescence staining. Concomitantly, autoimmune work-up was significant for positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA = 1:640) and decreased von Willebrand factor cleaving protease activity (<5%). A final diagnosis of TTP with microscopic polyangiitis (p-ANCA-mediated) was made and treatment with daily oral cyclophosphamide and prednisone resolved her renal injury over 2 months (follow-up Cr = 1.0 mg%). Our case highlights the importance of identifying systemic disorders such as ANCA-associated vasculitis with TTP.

Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Kidney Involvement in a Patient with AL Amyloidosis.

Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis has occasionally been associated with other systemic glomerulonephritis, such as anti-glomerular basement membrane disease. Here, we report the first clinical case of ANCA-associated crescentic glomerulonephritis with AL amyloidosis. An 81-years-old gentleman presented to the hospital with acute kidney injury (serum creatinine 4.7 mg/dL) on a background of chronic kidney disease and volume overload. Autoimmune serology was remarkable for p-ANCA and myeloperoxidase positivity. A renal biopsy confirmed pauci-immune glomerulonephritis and lambda light-chain amyloid deposition (confirmed on liquid chromatography and tandem mass spectrometry). The patient was initially managed with rituximab and subsequently transitioned to bortezomib-based chemotherapy but died due to decompensated heart failure. This case report promotes greater awareness of the unusual presentation of amyloidosis and guides future research and treatment.