Corticosteroid-induced hyperammonaemic encephalopathy in a woman with late-onset ornithine transcarbamylase deficiency.

Ornithine transcarbamylase deficiency (OTCD) is a rare, X linked disorder that can manifest in late adulthood in heterozygous females as severe hyperammonaemia following environmental stressors. We present a case of hyperammonaemic encephalopathy that was triggered by glucocorticoid administration in an adult woman with heterozygous OTCD with clinical response to haemodialysis, ammonia scavengers and a high-calorie, low-protein diet.

Kidney Outcomes Following Utilization of Molecular Adsorbent Recirculating System.

Introduction: Molecular adsorbent recirculating system (MARS) is an extracorporeal system combining conventional veno-venous hemodiafiltration and adsorption to provide rescue support in fulminant hepatic failure. Acute kidney injury (AKI) is common in patients with hepatic failure warranting continuous kidney replacement therapy (CKRT). Our primary aim was to characterize a cohort of patients who received MARS therapy and examine kidney events given the current paucity of available data. Methods: Patients initiating MARS in a tertiary care setting from January 2014 through December 2020 were assessed for treatment indications, transplantation, CKRT, kidney recovery, and death. Data was collected using the REDCAP software. Results: A total of 49 patients (67% female; 75% White) received MARS therapy with 29 patients (59%) requiring concomitant CKRT. Hepatic encephalopathy (HE) was the most common indication for MARS initiation (55%). In-hospital mortality was 41% (12/29) among patients who received CKRT versus 10% (2/20) among those not requiring CKRT (relative risk [RR] 4.15, 95% confidence interval [CI] 1.04 to 16.52, P = 0.044); this persisted following adjustment for prespecified patient characteristics (all RR ≥ 3.76, all P ≤ 0.060). One-year mortality post-MARS initiation was high overall but highest among the CKRT group (59% [17/29] vs. 25% [5/20] unadjusted RR 2.92, 95% CI 1.08 to 7.94, P = 0.035). Liver transplant after MARS occurred in 41% of patients (20/49). After CKRT, 39% of patients (9/29) recovered kidney function prior to hospital discharge. Conclusions: Patients requiring MARS frequently have AKI warranting the use of concomitant CKRT, which is associated with a high rate of in-hospital and 1-year mortality.

Advanced Practice Provider Care Team Models: Best Practices From an Academic Medical Center.

Health care leaders must be innovative to meet the demands of patient access and cost efficiency, all while never compromising patient safety or experience. A clear understanding of the care team model with optimal utilization of all team members is paramount to success. This article discusses 5 nurse practitioner and physician assistant (collectively called advanced practice providers) care team models that regularly occur in health care organizations across the country. Examples of each practice model, a discussion on when each model would be appropriate, as well as data on patient experience and financial return of investment of each model are provided.

Improving Access for Priority Patient Populations Within Outpatient Specialty Practices.

In an outpatient health care practice, it can be challenging to convert patient demand into completed appointments, even for high-priority patients. One of the barriers to higher conversion rates is excessive appointment lag time, which can lead to nonattendance or cancellation for other reasons. In this article, we develop a mechanism for reducing appointment lag time for priority patient populations. We report on a pilot program with 12 practices, split into pilot and control groups, and involving 11001 patients requesting new appointments. The results of the pilot show that statistically significant improvements to conversion rates can be achieved.

Minimal change disease and subacute interstitial nephritis in association with Edwardsiella tarda gastroenteritis following oyster consumption.

Edwardsiella tarda (E. tarda) is a gram-negative, facultatively anaerobic bacillus that is associated with gastroenteritis and a host of other extra-intestinal manifestations in humans. However, its impact on the kidneys is unclear. Most literature that has explored this association involves fish, marine life in which E. tarda inhabits. We report a rare case of a 72-year-old female who presented with an acute kidney injury (AKI) associated with newfound minimal change disease, subacute interstitial nephritis, and a severe E. tarda infection. Her clinical course resolved with antibiotics and glucocorticoids.

Association of Ancillary Pathology Findings in Non-neoplastic Renal Parenchyma and Renal Outcomes of Robotic-Assisted Partial Nephrectomy.

Background: To evaluate robotic-assisted partial nephrectomy (RAPN) renal outcomes associated with ancillary pathology findings in non-neoplastic renal parenchymal tissue. Methods: Tissue samples from 378 RAPNs were analyzed for glomerular disease (GD), vascular disease (VD), and tubulointerstitial disease (TD). One hundred and fifty-two patients were excluded due to insufficient non-neoplastic tissue for analysis and 4 patients were excluded due to calyceal diverticulum. Non-neoplastic tissue was evaluated for GD (negative, moderate, or global), VD (absent, mild, moderate, or severe), and TD (present or absent). Associations of ancillary pathology factors with patient characteristics were explored using the non-parametric Kendall tau-test and propensity score adjusted longitudinal mixed effects regression models were used to evaluate associations of these pathology factors with changes in estimated glomerular filtration rate (eGFR) following RAPN. Results: One hundred and fifty-three (68.9%) patients had hypertension and 50 (22.5%) patients had diabetes. The majority of patients did not have any GD (N = 158, 71.2%) or TD (N = 186, 83.8%) while 129 (58.1%) had VD. VD was categorized as absent (N = 93, 41.9%), mild (N = 45, 20.3%), moderate (N = 76, 34.2%), and severe (N = 8, 6.8%). Older age (P = 0.018), hypertension (P < 0.001), and high grade MAP score (P = 0.047) were associated with a higher number of ancillary pathology factors. High grade MAP score (P = 0.03, P = 0.002) and hypertension (P = 0.02, P < 0.001) were individually associated with GD severity and VD severity, respectively. Older age was also individually associated with VD severity (P = 0.002) and hypertension was associated with TD (P = 0.04). Moderate-to-severe VD was associated with a worse change in eGFR from pre-RAPN to 1-month post-RAPN compared to those with mild or no VD (difference in mean change, -3.4 ml/kg/1.73m2; 95% CI, -6.6 to -0.2 ml/kg/1.73m2; P = 0.036). Conclusions: Moderate-to-severe VD in non-neoplastic renal parenchyma is associated with post-operative changes in eGFR. Older age, hypertension, and high grade MAP scores are associated with the number of ancillary pathologies observed in RAPN specimens.

Impact on urinary oxalate levels with use of ezetimibe.

Background: Calcium oxalate stones are the most common cause of nephrolithiasis in the United States. Smaller studies of <15 patients investigating ezetimibe, a selective cholesterol absorption inhibitor, have suggested increased urine oxalate levels with use of the drug. We attempt to better define this relationship of ezetimibe on urinary oxalate using a larger patient sample analysing multiple urine collections on and off treatment. Materials and Methods: We retrospectively reviewed all consecutive patients from 01/2018 through 04/2019 evaluated for nephrolithiasis with use of ezetimibe documented in their medical record at Mayo Clinic Florida. Primary outcomes included increase in urinary oxalate with use of ezetimibe and reduction in urinary oxalate with discontinuation of medication. Results: Of 57 reviewed patients, 30 (53%) met inclusion criteria yielding 117 24-h urine measurements either on ezetimibe (72 measurements) or off ezetimibe (41 measurements). The mean urinary oxalate level off ezetimibe was 39.86 mg versus 40.45 mg with ezetimibe. After adjusting for age and sex, the estimated difference was 1.239 mg (95% CI, -4.856 to 7.335 mg; p = 0.93). A subset of six patients with urinary oxalate values both on and off ezetimibe showed a difference in 24-h urinary oxalate levels ranged from -16.40 to 14.95 mg (mean difference = 0.93 mg; median difference = 3.84 mg). Conclusion: Use of ezetimibe does not provide clear evidence of a difference in urinary oxalate levels.

Sugammadex use in patients with end-stage renal disease: a historical cohort study.

PURPOSE: While sugammadex (SGX) is not approved for use in patients with end-stage renal disease (ESRD), its administration in this patient population has been reported. We designed the current study to review all instances of patients with ESRD receiving SGX and to describe their clinical outcomes. METHODS: This is a historical cohort study of 219 patients with chronic kidney disease stage 5 who received SGX in one of three hospital locations within the same academic health system. Data were collected between 7 March 2016 and 1 August 2019 and included demographics, notable events from the anesthesia records, and postoperative complications. The primary outcome included any complication possibly related to SGX such as hypersensitivity reactions, need for reintubation, hypoxemia, pneumonia, and residual neuromuscular blockade. Secondary outcomes included any other complication not included in the primary outcome and/or patient mortality within 30 days after the procedure. RESULTS: No patient experienced a hypersensitivity reaction. Three patients required reintubation while two patients developed hypoxemia that did not require reintubation. One patient developed hospital-acquired pneumonia. Fifty (23%) patients developed other postoperative complications (different from our primary outcome) and nine patients (4%) died during the subsequent 30 postoperative days. None of the primary or secondary outcomes appeared to be related to SGX use. CONCLUSIONS: We provide incremental evidence that SGX could be considered as an alternative neuromuscular blockade reversal agent in patients with ESRD.

RéSUMé: OBJECTIF : Bien que le sugammadex (SGX) ne compte pas parmi ses indications une utilisation chez les patients atteints d'insuffisance rénale terminale (IRT), son administration à cette population de patients est rapportée. Nous avons conçu cette étude afin de passer en revue tous les cas de patients atteints d'IRT ayant reçu du SGX et décrire leurs devenirs cliniques. MéTHODE : Il s'agit d'une étude de cohorte historique portant sur 219 patients atteints d'insuffisance rénale chronique de stade 5 ayant reçu du SGX dans l'un de trois hôpitaux appartenant au même réseau de santé universitaire. Les données démographiques, les événements significatifs notés dans les dossiers anesthésiques et les complications postopératoires ont été colligés entre le 7 mars 2016 et le 1er août 2019. Le critère d'évaluation principal incluait toute complication possiblement liée à l'utilisation de SGX telle qu'une réaction d'hypersensibilité, une réintubation, une hypoxémie, une pneumonie, et un bloc neuromusculaire résiduel. Les critères d'évaluation secondaires comprenaient toute autre complication non incluse dans notre critère d'évaluation principal et/ou la mortalité des patients dans les 30 jours suivant l'intervention. RéSULTATS : Aucun patient n'a eu de réaction d'hypersensibilité. Trois patients ont dû être réintubés, et deux patients ont développé une hypoxémie qui n'a pas nécessité de réintubation. Un patient a contracté une pneumonie nosocomiale. Cinquante (23 %) patients ont souffert d'autres complications postopératoires (différentes de notre critère d'évaluation principal) et neuf patients (4 %) sont décédés au cours des 30 jours postopératoires subséquents. Aucun de nos critères d'évaluation primaire ou secondaires ne semblaient liés à l'utilisation de SGX. CONCLUSION : Nous proposons des données probantes supplémentaires selon lesquelles le SGX pourrait être envisagé comme agent décurarisant alternatif chez les patients atteints d'IRT.

Acid-base disturbances: an emergency department approach.

Acid-base disturbances are physiological responses to a wide variety of underlying conditions and critical illnesses. Homeostasis of acid-base physiology is complex and interdependent with the function of the lungs, kidneys, and endogenous buffer systems. Traditionally, these disturbances have been classified in terms of being caused by either a primary respiratory or a metabolic insult and by chronicity and compensation. While existing literature consists largely of physiology reviews, several well-designed studies and clinical practice guidelines provide relevant new perspectives on interpreting and managing acid-base disturbances. This review outlines several approaches to characterizing disturbances, with a case-based format and algorithms to aid in diagnostic testing and interpretation of arterial blood gases.

Prevalence of Nephrolithiasis in Patients with Chronic Liver Disease: A Case-Control Study.

BACKGROUND AND AIMS: Nephrolithiasis is known to be associated with several systemic diseases including chronic kidney disease and renal failure, which can also occur as a complication of chronic liver disease (CLD). This study aimed to assess the prevalence of nephrolithiasis in patients with CLD. METHODS: A short survey was completed by 198 patients with CLD and 322 controls matched by age, sex, and state of residence. A primary diagnosis of liver disease was confirmed with health record review. RESULTS: The median age of the liver disease group was 63 years and 128 (65%) were male; the median age of the control group was 63 and 199 (63%) were male. Body mass index was higher in the liver disease group (27.8 vs 26.7, P < .01). The most common liver disease diagnosis was hepatitis C (60 [30%]) followed by alcoholic cirrhosis (42 [21.2%]). The self-reported prevalence of nephrolithiasis in the liver disease group was 26%, compared to 14% in the control group (P < .01). This association remained significant after adjusting for age, sex, body mass index, and family history of kidney stones or liver disease. CONCLUSIONS: In this case-control, survey-based study, the prevalence of nephrolithiasis was 2 times higher in patients with CLD.

Predictors of early mortality and readmissions among dialysis patients undergoing lower extremity amputation.

OBJECTIVE: Patients receiving dialysis are at increased risk for lower extremity amputations (LEAs) and postoperative morbidity. Limited studies have examined differences in 30-day outcomes of mortality and health care use after amputation or the preoperative factors that relate to worsened outcomes in dialysis patients. Our objective was to examine dialysis dependency and other preoperative factors associated with readmission or death after LEA. METHODS: A retrospective cohort study was conducted of dialysis-dependent and nondialysis patients undergoing major LEA in the 2012 to 2013 American College of Surgeons National Surgical Quality Improvement Program. Primary outcomes included death and hospital readmission within 30 days of amputation. RESULTS: Of 6468 patients, 1166 (18%) were dialysis dependent. The dialysis cohort had more blacks (39% vs 23%), diabetes (76% vs 58%), below-knee amputations (62% vs 55%), and in-hospital deaths (8% vs 3%; all P < .001). The 30-day postoperative death rates (15% vs 7%) and readmission rates (35% vs 20% per 30 person-days; both P < .001) were higher in dialysis patients. Among the live discharges, the rate of any readmission or death within 30 days from amputation was highest in those aged ≥50 years (40% per 30 person-days). Multivariable analyses in the dialysis cohort revealed increased age, above-knee amputation, decreased physical status, heart failure, high preoperative white blood cell count, and low platelet count to be associated with death (P < .05; C statistic, 0.75). The only preoperative factor associated with readmission in dialysis patients was race (P = .04; C statistic, 0.58). CONCLUSIONS: Readmission or death after amputation is increased among dialysis patients. Predicting which dialysis patients are at highest risk for death is feasible, whereas predicting which will require readmission is less so. Risk factor identification may improve risk stratification, inform reimbursement policies, and allow targeted interventions to improve outcomes.

Collapsing glomerulopathy in systemic lupus erythematosus.

Collapsing glomerulopathy (CG) is a rare disease that can be associated with multiple other disorders. It usually leads to poor prognosis with a high percentage of patients progressing to end-stage renal disease. In this article, we illustrate a clinical case of CG associated with systemic lupus erythematosus that had a prompt response to mycophenolate and prednisone. The condition started after sudden cessation of the already established mycophenolate treatment regimen. The patient then presented with acute kidney injury due to kidney biopsy-proven CG. In that circumstance, we hypothesised that mycophenolate may play a role in prevention and development of CG.

Career interest and perceptions of nephrology: A repeated cross-sectional survey of internal medicine residents.

BACKGROUND: Interest in nephrology careers among internal medicine residents in the United States is declining. Our objective was to assess the impact of the presence of a nephrology fellowship training program on perceptions and career interest in nephrology among internal medicine residents. A secondary objective was to identify commonly endorsed negative perceptions of nephrology among internal medicine residents. METHODS: This was a repeated cross-sectional survey of internal medicine residents before (Group 1) and 3 years after (Group 2) the establishment of nephrology fellowship programs at two institutions. The primary outcome was the percentage of residents indicating nephrology as a career interest in Group 1 vs. Group 2. Secondary outcomes included the frequency that residents agreed with negative statements about nephrology. RESULTS: 131 (80.9%) of 162 residents completed the survey. 19 (14.8%) residents indicated interest in a nephrology career, with 8 (6.3%) indicating nephrology as their first choice. There was no difference in career interest in nephrology between residents who were exposed to nephrology fellows during residency training (Group 2) and residents who were not (Group 1). The most commonly endorsed negative perceptions of nephrology were: nephrology fellows have long hours/burdensome call (36 [28.1%] of residents agreed or strongly agreed), practicing nephrologists must take frequent/difficult call (35 [27.6%] agreed or strongly agreed), and nephrology has few opportunities for procedures (35 [27.3%] agreed or strongly agreed). More residents in Group 2 agreed that nephrology is poorly paid (8.9% in Group 1 vs. 20.8% in Group 2, P = 0.04), whereas more residents in Group 1 agreed that nephrologists must take frequent/difficult call (40.0% in Group 1 vs. 18.1% in Group 2, P = 0.02). CONCLUSIONS: The initiation of a nephrology fellowship program was not associated with an increase in internal medicine residents' interest in nephrology careers. Residents endorsed several negative perceptions of nephrology, which may affect career choice.

Rare allergic reaction of the kidney: sitagliptin-induced acute tubulointerstitial nephritis.

A 56-year-old man with a history of diabetes mellitus type-2 and stage-2 chronic kidney disease secondary to diabetic nephropathy presented with an acute deterioration of kidney function. Non-invasive work-up failed to reveal the underlying aetiology for the acute kidney failure. Kidney biopsy revealed acute tubulointerstitial nephritis (ATIN) which was attributed to sitagliptin use. Only few case reports have shown this correlation. Our aim is to alert physicians and other providers of the potential effect of sitagliptin to cause ATIN with this biopsy-proven case.

Inpatient warfarin management: pharmacist management using a detailed dosing protocol.

Hospitalized patients receiving anticoagulants such as warfarin are at increased risk for adverse events because of difficulties maintaining a therapeutic international normalized ratio (INR). We sought to determine whether a detailed warfarin dosing protocol administered by pharmacists with minimal physician oversight significantly reduced the proportion of hospitalized patients with a supratherapeutic INR. We conducted a prospective, nonrandomized trial with patients on cardiology, internal medicine, and family medicine inpatient services who received at least 1 dose of warfarin while hospitalized. The baseline group included 293 patients, and the intervention group comprised 217 patients. Baseline characteristics were similar in each group, except that more patients received antibiotics in the intervention group. The defect rate (INR > 5 after receiving warfarin) in the baseline group was significantly higher than in the intervention group (7.85 vs. 1.85%). Conversely, the percentage of patients with an INR less than 1.7 after 4 warfarin doses was lower in the intervention patients, indicating overall improvement in therapeutic levels. Dosing discussions were required between the pharmacist and a physician for only 6% of intervention patients. The protocol effectively reduced overanticoagulation without increasing under anticoagulation during hospitalization and reduced the need for close physician oversight.