Anakinra-Associated Systemic Amyloidosis.

OBJECTIVE: To describe a 41-year-old woman with a history of neonatal onset multisystem inflammatory disease, on treatment with daily subcutaneous injections of 600 mg of recombinant interleukin-1 receptor antagonist (IL-1Ra) protein, anakinra, since the age of 28, who presented with golf-ball size nodules at the anakinra injection sites, early satiety, new onset nephrotic syndrome in the context of normal markers of systemic inflammation. METHODS: Clinical history and histologic evaluation of biopsies of skin, gastric mucosa, and kidney with Congo-red staining and proteomic evaluation of microdissected Congo red-positive amyloid deposits by liquid chromatography-tandem mass spectrometry. RESULTS: The skin, stomach, and kidney biopsies all showed the presence of Congo red-positive amyloid deposits. Mass spectrometry-based proteomics demonstrated that the amyloid deposits in all sites were of AIL1RAP (IL-1Ra protein)-type. These were characterized by high spectral counts of the amyloid signature proteins (apolipoprotein AIV, apolipoprotein E, and serum amyloid P-component) and the amyloidogenic IL-1Ra protein, which were present in Congo red-positive areas and absent in Congo red-negative areas. The amino acid sequence identified by mass spectrometry confirmed that the amyloid precursor protein was recombinant IL-1Ra (anakinra) and not endogenous wild-type IL-1Ra. CONCLUSION: This is the first report of iatrogenic systemic amyloidosis due to an injectable protein drug, which was caused by recombinant IL1Ra (anakinra).

Successful Treatment of Paecilomyces variotii Pneumonia and Lupus Nephritis With Posaconazole-Cyclophosphamide Co-administration Without Drug Interaction-Induced Toxicity.

Paecilomyces variotii is an opportunistic mold that causes pulmonary infections in immunosuppressed humans that are often treated with triazole therapy. Lupus nephritis is a major cause of progressive kidney disease in patients with systemic lupus erythematosus, often requiring cyclophosphamide-based therapies. Triazole-cyclophosphamide co-administration is challenging as triazoles increase cyclophosphamide concentrations, which can worsen cyclophosphamide toxicity. We describe herein a patient with Paecilomyces variotii pneumonia and concomitant lupus nephritis who was successfully treated with posaconazole and echinocandin-bridged interruptions to allow for cyclophosphamide therapy. This regimen was well-tolerated without cyclophosphamide toxicity and achieved improvements in both fungal pneumonia and renal function.

Adapting an Objective Structured Clinical Examination (OSCE) on Conducting Difficult Conversations Between Nephrology Fellows and Patients to a Virtual Platform: A Research Letter.

Background: We previously described a nephrology-specific "Breaking Bad News" Objective Structured Clinical Examination (OSCE) assessing nephrology fellow communication and counseling skills in 3 scenarios: kidney replacement therapy (KRT) in kidney failure, urgent KRT in acute kidney injury (AKI), and kidney biopsy (KBx). Objective: The main objectives of this study is to adapt the OSCE to a virtual platform, simulating nephrology patient telemedicine encounters involving difficult conversations, and to assess fellow and faculty satisfaction with the virtual format. Design: Description of a formative telemedicine simulation for nephrology fellows. Setting: Fully virtual simulation conducted by 2 academic medical simulation centers. Participants: Nephrology faculty and fellows at 3 urban/suburban training programs in the eastern United States. Measurements: Description of the virtual OSCE process. Fellow and faculty satisfaction overall and for each scenario. Faculty and fellow estimates of frequency of virtual patient encounters in the past year. Methods: The OSCE consisted of 3 scenarios: KRT in kidney failure, urgent KRT in AKI, and KBx. Objective Structured Clinical Examinations were administered in May 2021. Each scenario lasted 20 minutes. The AKI scenario was audio only. Fellows telephoned a simulated patient surrogate for urgent KRT consent. Kidney failure and KBx scenarios were video encounters. Faculty observed while muted/video off. Immediately after the OSCE, fellows and faculty were anonymously surveyed regarding their satisfaction with each scenario, the OSCE overall, and their estimate of outpatient encounters and inpatient KRT counseling done virtually in the preceding year. Results: Seventeen fellows completed the OSCE at 2 centers (3 programs). Sixteen (94%) completed the survey. Almost 94% rated the OSCE as a good/very good approximation of telemedicine encounters. Those satisfied/very satisfied with each scenario are as follow: 100% for AKI, 75% for kidney failure, and 75% for KBx. Two commented that they often did urgent KRT counseling by telephone. Fellows estimated a median 20% (interquartile range: 175, 50%) of counseling for acute inpatient KRT and a median 50% (IQR: 33.75, 70%) of outpatient encounters were virtual in the prior year. Two (regarding the kidney failure and KBx scenarios) indicated they would not have counseled similar outpatients virtually. Limitations: The 15-minute interactions may be too short to allow the encounter to be completed comfortably. A small number of programs and fellows participated, and programs were located in urban/suburban areas on the east coast of the United States. Conclusions: Overall, fellows felt that the OSCE was a good approximation of virtual encounters. The OSCE is an opportunity for fellows to practice telemedicine communication skills.

Contexte: Nous avions précédemment décrit un examen clinique objectif structuré (ECOS) de type « annonce d'une mauvaise nouvelle ¼ en néphrologie afin d'évaluer les compétences en communication et consultation des résidents en néphrologie. L'ECOS comportait trois scénarios: thérapie de remplacement rénal (TRR) pour l'insuffisance rénale, TRR urgente dans les cas d'insuffisance rénale aiguë (IRA), et biopsie du rein (BxR). Objectifs: Adapter l'ECOS à une plateforme virtuelle, simuler des rencontres de télémédecine impliquant des conversations difficiles avec des patients en néphrologie, et évaluer la satisfaction des résidents en néphrologie et du corps enseignant à l'égard du format virtuel. Conception: Description d'une simulation de télémédecine pour la formation des résidents en néphrologie. Cadre: Des simulations entièrement virtuelles réalisées dans deux centers universitaires de simulation médicale. Participants: Les professeurs et les résidents en néphrologie de trois programs de formation urbains/suburbains de l'est des États-Unis. Mesures: Description du processus de l'ECOS virtuel. Satisfaction générale des résidents en néphrologie et du corps enseignant pour chacun des scénarios. Estimation, par les professeurs et les résidents, de la fréquence des rencontres virtuelles avec des patients au cours de la dernière année. Méthodologie: L'ECOS était composé de trois scénarios: TRR en insuffisance rénale; TRR urgente en contexte d'IRA et biopsie rénale. Les ECOS ont été réalisés en mai 2021. Chaque scénario durait 20 minutes. Le scénario IRA était audio uniquement; les boursiers devaient téléphoner à un patient simulé afin d'obtenir un consentement pour une TRR urgente. Les scénarios pour l'insuffisance rénale terminale et la BxR étaient sous forme de rencontres vidéo. Les professeurs observaient les scénarios en sourdine/hors vidéo. Immédiatement après l'ECOS, les résidents en néphrologie et les professeurs ont été interrogés de façon anonyme sur leur satisfaction à l'égard de chaque scénario et de l'ECOS dans son ensemble. Ils ont également été invités à estimer le nombre de consultations externes et de conseils prodigués sur la TRR à des patients hospitalisés au cours de l'année précédente. Résultats: Dix-sept résidents en néphrologie ont complété l'ECOS dans les deux centers (trois programs) et seize (94 %) ont répondu au sondage. La très grande majorité (94 %) a évalué l'ECOS comme une bonne/très bonne simulation des rencontres de télémédecine. Le taux de personnes satisfaites/très satisfaites s'établissait à 100 % pour le scénario de l'IRA, à 75 % pour celui de l'insuffisance rénale terminale et à 75 % pour celui de la BxR. Deux personnes ont dit faire régulièrement des consultations par téléphone pour la TRR urgente. Les résidents en néphrologie ont estimé que 20 %, (proportion médiane) ÉIQ= 175; 50 %, des consultations avec des patients hospitalisés et 50 %, (proportion médiane) ÉIQ = 33,75; 70 %, des consultations externes avaient été faites virtuellement au cours de l'année précédente. Pour les scénarios d'insuffisance rénale terminale et de BxR, deux personnes ont indiqué qu'elles ne feraient pas de consultations virtuelles pour ces patients. Limites: Les interactions de 15 minutes sont probablement trop courtes pour compléter confortablement la rencontre. Un faible nombre de programs et de résidents en néphrologie ont participé. Les programs évalués se situaient en zones urbaines et suburbaines de la côte est des États-Unis. Conclusion: Dans l'ensemble, les résidents en néphrologie ont estimé que l'ECOS était une bonne représentation des rencontres virtuelles. L'ECOS est une occasion pour les résidents de mettre en pratique leurs compétences en communication dans un contexte de télémédecine.

Acute glomerulonephritis in a hematopoietic blood stem cell donor.

Use of granulocyte colony-stimulating factor (G-CSF) has been associated with side effects including reports of acute glomerulonephritis (GN), almost all of which have been immune complex associated. There is one prior report of pauci-immune GN in a child, but was negative for ANCA (anti-neutrophilic cytoplasmic antibodies). We describe the first case of ANCA-positive pauci-immune GN exacerbated by the use of G-CSF for peripheral blood stem cell (PBSC) donation in a patient with no prior history of vasculitis. Given the use of G-CSF in PBSC donation and neutropenias associated with various conditions, it is important that both the nephrologist and the hematologist are aware of the renal risks associated with its use.

Growth and Immune Response of Pangasius hypophthalmus Fed Diets Containing Seaweed Extracts as Immunostimulant

Abstract Growth and immune response of Pangasius hypophthalmus were evaluated after feeding the fish with diets containing hot-water extracts (HWE) of Sargassum oligocystum as immunostimulant at 100, 300, and 500 mg kg-1 diet. Basal diet for P. hypophthalmus served as the control. The experimental diets were administered for 12 weeks. At the end of the feeding experiment, growth and haematological profile of fish were evaluated. Result showed that final weight, weight gain, daily growth rate and feed conversion ratio were significantly increased in the fish that received 300 and 500 mg kg-1 HWE of S. oligocystum. Evaluation of the haematological profile showed that white blood cells red blood cells, hemoglobin, hematocrit and platelet of P. hypophthalmus that received the HWE of S. oligocystum were significantly higher than the control group. Overall, our results indicate that the use of S. oligocystum HWE improves growth and haematological profile in P. hypophthalmus.

Hyperkalemia and Dialysis in the Deployed Setting.

Acute kidney injury is a recognized complication of combat trauma. The complications associated with acute kidney injury, such as life-threatening hyperkalemia, are usually delayed in onset. In the recent conflicts, rapid evacuation of U.S. and coalition personnel generally resulted in these complications occurring at higher echelons of care where renal replacement therapies were available. In the future however, deployed providers may not have this luxury and should be prepared to temporize patients while they await transport. In this clinical practice guideline, recommendations are made for the management of patients with, or at risk for, acute kidney injury and hyperkalemia in the austere, deployed environment.

Outcomes After Post-Traumatic AKI Requiring RRT in United States Military Service Members.

BACKGROUND AND OBJECTIVES: Mortality and CKD risk have not been described in military casualties with post-traumatic AKI requiring RRT suffered in the Iraq and Afghanistan wars. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This is a retrospective case series of post-traumatic AKI requiring RRT in 51 military health care beneficiaries (October 7, 2001-December 1, 2013), evacuated to the National Capital Region, documenting in-hospital mortality and subsequent CKD. Participants were identified using electronic medical and procedure records. RESULTS: Age at injury was 26±6 years; of the participants, 50 were men, 16% were black, 67% were white, and 88% of injuries were caused by blast or projectiles. Presumed AKI cause was acute tubular necrosis in 98%, with rhabdomyolysis in 72%. Sixty-day all-cause mortality was 22% (95% confidence interval [95% CI], 12% to 35%), significantly less than the 50% predicted historical mortality (P<0.001). The VA/NIH Acute Renal Failure Trial Network AKI integer score predicted 60-day mortality risk was 33% (range, 6%-96%) (n=49). Of these, nine died (mortality, 18%; 95% CI, 10% to 32%), with predicted risks significantly miscalibrated (P<0.001). The area under the receiver operator characteristic curve for the AKI integer score was 0.72 (95% CI, 0.56 to 0.88), not significantly different than the AKI integer score model cohort (P=0.27). Of the 40 survivors, one had ESRD caused by cortical necrosis. Of the remaining 39, median time to last follow-up serum creatinine was 1158 days (range, 99-3316 days), serum creatinine was 0.85±0.24 mg/dl, and eGFR was 118±23 ml/min per 1.73 m(2). No eGFR was <60 ml/min per 1.73 m(2), but it may be overestimated because of large/medium amputations in 54%. Twenty-five percent (n=36) had proteinuria; one was diagnosed with CKD stage 2. CONCLUSIONS: Despite severe injuries, participants had better in-hospital survival than predicted historically and by AKI integer score. No patient who recovered renal function had an eGFR<60 ml/min per 1.73 m(2) at last follow-up, but 23% had proteinuria, suggesting CKD burden.