Characteristics and Outcomes of NELL1 Membranous Nephropathy in Lipoic Acid Users and Nonusers.

Introduction: Neural epidermal growth factor like 1 membranous nephropathy (NELL1 MN) is associated with various secondary etiologies. However, previous studies on the frequency of these associations and their impact on outcomes are limited. We report a large multiinstitutional series of patients with NELL1 MN with a focus on secondary associations, pathology findings, and their impact on outcome. Methods: We retrospectively reviewed clinicopathologic features of NELL1 MN from 3 institutions and analyzed clinical and histologic associations with outcome. Results: Of 70 patients, 53% were male with a median age of 66 years; median proteinuria was 5.9 g/d. NELL1 MN was associated with lipoic acid (36%), heavy nonsteroidal antiinflammatory drug (NSAID) use (27%), autoimmune disease (23%), malignancy (10% recent, 23% any), mercury exposure (1%), and 11% had no known secondary association. At median follow-up of 11 months, 72% achieved complete or partial remission. Remission rate was 91% in patients with lipoic acid-associated NELL1 MN and ≥6 months of follow-up. On multivariable analyses, patients with primary NELL1 MN (adjusted odds ratio [OR]: 19.7, P = 0.01) and increasing degree of tubular atrophy and interstitial fibrosis (IFTA) (adjusted OR 1.1, P = 0.01) were less likely to achieve any remission, whereas complete remission (CR) was associated with lipoic acid use (adjusted OR: 10.9, P = 0.04, 95% confidence interval [CI]: 1.2-100) and lesser degrees of IFTA (adjusted OR: 0.79, P = 0.16, 95% CI: 0.66-0.96). Conclusion: Our findings strengthen the association between lipoic acid and NELL1 MN. Furthermore, our findings suggest that discontinuation of lipoic acid without immunosuppression should be considered as the first-line treatment.

Home-Based Dialysis: A Primer for the Internist.

Home-based dialysis modalities offer both clinical and practical advantages to patients. The use of the home-based modalities, peritoneal dialysis and home hemodialysis, has been increasing over the past decade after a long period of decline. Given the increasing frequency of use of these types of dialysis, it is important for clinicians to be familiar with how these types of dialysis are performed and key clinical aspects of care related to their use in patients with end-stage kidney disease.

Patient-Facing Clinical Decision Support for High Blood Pressure Control: Patient Survey.

BACKGROUND: High blood pressure (HBP) affects nearly half of adults in the United States and is a major factor in heart attacks, strokes, kidney disease, and other morbidities. To reduce risk, guidelines for HBP contain more than 70 recommendations, including many related to patient behaviors, such as home monitoring and lifestyle changes. Thus, the patient's role in controlling HBP is crucial. Patient-facing clinical decision support (CDS) tools may help patients adhere to evidence-based care, but customization is required. OBJECTIVE: Our objective was to understand how to adapt CDS to best engage patients in controlling HBP. METHODS: We conducted a mixed methods study with two phases: (1) survey-guided interviews with a limited cohort and (2) a nationwide web-based survey. Participation in each phase was limited to adults aged between 18 and 85 years who had been diagnosed with hypertension. The survey included general questions that assessed goal setting, treatment priorities, medication load, comorbid conditions, satisfaction with blood pressure (BP) management, and attitudes toward CDS, and also a series of questions regarding A/B preferences using paired information displays to assess perceived trustworthiness of potential CDS user interface options. RESULTS: We conducted 17 survey-guided interviews to gather patient needs from CDS, then analyzed results and created a second survey of 519 adults with clinically diagnosed HBP. A large majority of participants reported that BP control was a high priority (83%), had monitored BP at home (82%), and felt comfortable using technology (88%). Survey respondents found displays with more detailed recommendations more trustworthy (56%-77% of them preferred simpler displays), especially when incorporating social trust and priorities from providers and patients like them, but had no differences in action taken. CONCLUSIONS: Respondents to the survey felt that CDS capabilities could help them with HBP control. The more detailed design options for BP display and recommendations messaging were considered the most trustworthy yet did not differentiate perceived actions.

Provider Perspectives on Patient- and Provider-Facing High Blood Pressure Clinical Decision Support.

BACKGROUND: Hypertension, persistent high blood pressures (HBP) leading to chronic physiologic changes, is a common condition that is a major predictor of heart attacks, strokes, and other conditions. Despite strong evidence, care teams and patients are inconsistently adherent to HBP guideline recommendations. Patient-facing clinical decision support (CDS) could help improve recommendation adherence but must also be acceptable to clinicians and patients. OBJECTIVE: This study aimed to partly address the challenge of developing a patient-facing CDS application, we sought to understand provider variations and rationales related to HBP guideline recommendations and perceptions regarding patient role and use of digital tools. METHODS: We engaged hypertension experts and primary care respondents to iteratively develop and implement a pilot survey and a final survey which presented five clinical cases that queried clinicians' attitudes related to actions; variations; prioritization; patient input; importance; and barriers for HBP diagnosis, monitoring, and treatment. Analysis of Likert's scale scores was descriptive with content analysis for free-text answers. RESULTS: Fifteen hypertension experts and 14 providers took the pilot and final version of the surveys, respectively. The majority (>80%) of providers felt the recommendations were important, yet found them difficult to follow-up to 90% of the time. Perceptions of relative amounts of patient input and patient work for effective HBP management ranged from 22 to 100%. Stated reasons for variation included adverse effects of treatment, patient comorbidities, shared decision-making, and health care cost and access issues. Providers were generally positive toward patient use of electronic CDS applications but worried about access to health care, nuance of recommendations, and patient understanding of the tools. CONCLUSION: At baseline, provider management of HBP is heterogeneous. Providers were accepting of patient-facing CDS but reported preferences for that CDS to capture the complexity and nuance of guideline recommendations.

Assessing Data Adequacy for High Blood Pressure Clinical Decision Support: A Quantitative Analysis.

OBJECTIVE: This study examines guideline-based high blood pressure (HBP) and hypertension recommendations and evaluates the suitability and adequacy of the data and logic required for a Fast Healthcare Interoperable Resources (FHIR)-based, patient-facing clinical decision support (CDS) HBP application. HBP is a major predictor of adverse health events, including stroke, myocardial infarction, and kidney disease. Multiple guidelines recommend interventions to lower blood pressure, but implementation requires patient-centered approaches, including patient-facing CDS tools. METHODS: We defined concept sets needed to measure adherence to 71 recommendations drawn from eight HBP guidelines. We measured data quality for these concepts for two cohorts (HBP screening and HBP diagnosed) from electronic health record (EHR) data, including four use cases (screening, nonpharmacologic interventions, pharmacologic interventions, and adverse events) for CDS. RESULTS: We identified 102,443 people with diagnosed and 58,990 with undiagnosed HBP. We found that 21/35 (60%) of required concept sets were unused or inaccurate, with only 259 (25.3%) of 1,101 codes used. Use cases showed high inclusion (0.9-11.2%), low exclusion (0-0.1%), and missing patient-specific context (up to 65.6%), leading to data in 2/4 use cases being insufficient for accurate alerting. DISCUSSION: Data quality from the EHR required to implement recommendations for HBP is highly inconsistent, reflecting a fragmented health care system and incomplete implementation of standard terminologies and workflows. Although imperfect, data were deemed adequate for two test use cases. CONCLUSION: Current data quality allows for further development of patient-facing FHIR HBP tools, but extensive validation and testing is required to assure precision and avoid unintended consequences.

Expanding CAPD in Low-Resource Settings: A Distance Learning Approach.

BACKGROUND: Despite growing need, treatment for end-stage renal disease is limited in low- and middle-income countries due to resource restraints. We describe the development of an educational curriculum and quality improvement program to support continuous ambulatory peritoneal dialysis (CAPD) performed primarily by non-nephrology providers in Sri Lanka. METHODS: We developed a program of education, outcome tracking, and expert consultation to support providers in Kandy, Sri Lanka. Education included videos and in-person didactics covering core topics in CAPD. Event-tracking sheets recorded root causes and management of infections and hospitalizations. Conferences reviewed clinical cases and overall clinic management. We evaluated the patient census, peritonitis rates, and root causes and management of infections over 1 year. RESULTS: The curriculum was published through the International Society of Nephrology online academy. High provider turnover limited curriculum assessments. The CAPD patient census rose from 63 to 116 during the year. The peritonitis rate declined significantly, from 0.8 episodes per patient-year in the first 6 months to 0.4 in the latter 6 months, though the most common root causes of peritonitis, related to contamination events and hygiene, persisted. The appropriate ascertainment of culture data and prescription of antibiotics also increased. CONCLUSIONS: Our project supported the expansion of a CAPD program in a resource-limited setting, while also improving peritonitis outcomes. Ongoing challenges include ensuring a durable educational system for rotating providers, tracking outcomes beyond peritonitis, and formalizing management protocols. Our program can serve as an example of how established dialysis programs can support the burgeoning work of providers in resource-limited setting.

A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?

BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases. CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma. Serum anti-neutrophil cytoplasmic antibodies were not detected. Kidney biopsy, including with Pronase digestion, did not reveal pathologic paraprotein deposition. Two previously published similar case reports are also discussed. CONCLUSION: We propose a novel pathologic mechanism involving monoclonal proteins as a trigger for pauci-immune glomerulonephritis, potentially via complement dysregulation and/or neutrophil activation. This requires further epidemiologic and mechanistic study.

Education in Nephrology Fellowship: A Survey-Based Needs Assessment.

Educational needs assessments for nephrology fellowship training are limited. This study assessed fellows' perceptions of current educational needs and interest in novel modalities that may improve their educational experience and quantified educational resources used by programs and fellows. We distributed a seven-question electronic survey to all United States-based fellows receiving complimentary American Society of Nephrology (ASN) membership at the end of the 2015-2016 academic year in conjunction with the ASN Nephrology Fellows Survey. One third (320 of 863; 37%) of fellows in Accreditation Council for Graduate Medical Education-accredited positions responded. Most respondents rated overall quality of teaching in fellowship as either "good" (37%) or "excellent" (44%), and most (55%) second-year fellows felt "fully prepared" for independent practice. Common educational resources used by fellows included UpToDate, Journal of the American Society of Nephrology/Clinical Journal of the American Society of Nephrology, and Nephrology Self-Assessment Program; others-including ASN's online curricula-were used less often. Fellows indicated interest in additional instruction in several core topics, including home dialysis modalities, ultrasonography, and pathology. Respondents strongly supported interventions to improve pathology instruction and increase time for physiology and clinical review. In conclusion, current nephrology fellows perceive several gaps in training. Innovation in education and training is needed to better prepare future nephrologists for the growing challenges of kidney care.

Education in consultation: A comparative survey-based assessment of perceived educational effectiveness within a university and community-based internal medicine residency.

This article was migrated. The article was marked as recommended. Background: Education in consultation is a potentially valuable, but understudied, element of medical education. Inpatient consultation is an opportunity for significant subspecialist contact for resident trainees and an avenue for improving their knowledge and patient care across content areas. We evaluated the perceived educational effectiveness of education in consultation among internal medicine residents, within a university and a community-based program, as well as the role of barriers in medical training that may limit education. Methods: We used a web-based survey expanded from a previously published survey consisting of 12 questions, including one free-response, on education in consultation. Data were analyzed descriptively and qualitatively. We surveyed residents from two internal medicine programs in 2016. One within a large university-based hospital and the second within a smaller community-based safety-net hospital. Results: 91/198 (46%) of residents responded. Overall results from both programs were similar despite their structural differences. Residents viewed education in consultation as a priority and the majority felt it was at least moderately effective but underutilized. Importantly, educational interactions are largely dependent on outreach from residents. While in-person teaching interactions were the most effective, key barriers to these interactions include a lack of time, difficulty locating residents, and the perception of residents as being too busy. Conclusions: Inpatient consultation offers a unique opportunity for specialist-led education for internal medicine residents. It is potentially effective but constrained extensively in modern training environments. Interventions aimed at emphasizing education in consultation within fellowships and residencies, increasing in-person resident-specialist interactions, and addressing structural barriers, may improve resident knowledge across specialties and strengthen patient care.

Do children with mental disorders have higher prevalence of hypovitaminosis D?

Inadequate vitamin D level is associated with various adverse medical outcomes. There is a growing concern that insufficient vitamin D may play a role in the development of psychiatric symptoms. This study aims to answer the question: do children with mental disorders have a higher prevalence of hypovitaminosis D? A retrospective chart review examined 25 hydroxyvitamin D (25(OH)D) levels in youth ages 7 to 17 (n=67) at two Oregon psychiatric residential facilities. Vitamin D deficiency is defined as <20 ng/ml and insufficiency as <30 ng/ml. Diagnoses were organized into six categories. 25(OH)D levels were compared across genders and diagnostic groups using a two-sample t-test and ANOVA, respectively. Statistical differences in prevalence across diagnostic categories were calculated using a Pearson chi-square test. Using the data from Saintonge's NHANES III study on healthy US children for comparison, 21% of our cohorts were found to be vitamin D deficient and 64% insufficient, in contrast to 14% and 48%, respectively. While our results are not statistically significant, mainly because of small sample size, the overall mean 25(OH)D level in our cohort was insufficient (27.59 ± 9.35 ng/ml), compared to a sufficient mean value of 32.1 ng/ml in the general population. No statistical significant difference was found in the prevalence across diagnostic categories. This study found that children with psychiatric disorders might have a higher prevalence of hypovitaminosis D than the general pediatric population. Although a causal relationship between hypovitaminosis D and psychiatric disorders cannot be derived based on the study design, our study provides initial descriptive data on the prevalence of hypovitaminosis D in children with psychiatric disorders, which has not been previously reported to our knowledge. Prospective studies with a larger sample size and controlled variables would allow more precise analysis of the relationship between hypovitaminosis D and childhood mental disorders.