scholarly journals De novo lupus-like glomerulonephritis after pediatric non-kidney organ transplantation

2021 ◽  
Author(s):  
Cristina M. Farkas-Skiles ◽  
Robert B. Ettenger ◽  
Jonathan E. Zuckerman ◽  
Meghan Pearl ◽  
Robert S. Venick ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
De Novo ◽  
Solid Organ ◽  
Significant Finding ◽  
Clinically Significant ◽  
Pediatric Recipients ◽  
Pediatric Liver Transplant ◽  
Active Lupus

Abstract Background We propose a novel clinically significant finding, de novo lupus-like glomerulonephritis (DNLLGN), in patients with autoantibodies and kidney abnormalities in pediatric liver transplant (LT) and intestinal inclusive transplants (ITx). Methods We describe the clinical, serologic, and histopathologic presentation and kidney outcomes in eight patients from our center found to have DNLLGN on kidney biopsy. Results Pediatric recipients of non-kidney solid organ transplants developed an unusual de novo immune complex glomerulonephritis with morphologic similarity to lupus nephritis. Six had isolated LT (0.9% of all pediatric LT at our center) and two had ITx (2.1% of all ITx). Five (63%) presented with nephrotic syndrome. Five patients had autoantibodies. Patients underwent kidney biopsy at a mean of 11.5 years in LT and 2.8 years in ITx after the index transplant. Biopsies demonstrated changes similar to focal or diffuse active lupus. Follow-up eGFR at a mean of 6 years after biopsy showed a mean decrease of 30 ml/min/1.73 m2 in all patients (p = 0.11). Conclusions DNLLGN has not been previously recognized in this clinical setting, yet 8 kidney biopsies from pediatric recipients of LT and ITx at our center in 25 years demonstrated this finding. DNLLGN appears to be an under-reported phenomenon of clinical significance. Graphical abstract

scholarly journals Arthroscopic Bristow: 12-Year Experience, Assessments of Safety and Effectiveness

2019 ◽  
Author(s):  
Jose Carlos Garcia
Keyword(s):  
Los Angeles ◽  
Shoulder Instability ◽  
De Novo ◽  
Mean Value ◽  
Anterior Shoulder Instability ◽  
Simple Shoulder Test ◽  
The Mean ◽  
Lateral Rotation ◽  
Clinically Significant

Abstract Objective The open Bristow procedure is a long established and effective method for treating anterior shoulder instability. Following the trends of minimally-invasive surgeries, these procedures were performed arthroscopically, and their outcomes were evaluated. Methods A total of 43 shoulders of patients submitted to Bristow procedures by arthroscopy, using a graft positioned horizontally and a screw, with at least two years of postoperative follow-up, were evaluated regarding quality of life, de novo dislocation index, and loss of lateral rotation. Results The mean follow-up time was of 76 months (range: 129 to 24 months). The University of California at Los Angeles (UCLA) score varied from 25.56 ± 0.50 (standard deviation [SD] = 3.25) to 33.23 ± 0.44 (SD = 2.91) (p < 0.0001). Two or more years after surgery, the mean Rowe score was of 94.25 ± 1.52 (SD = 1.34), whereas the good results standard is 75 (p < 0.0001). The mean value for the simple shoulder test was of 11.35 ± 0.21 (SD = 1.34), while the mean value of the lateral rotation loss was of 10.37° ± 1.36° (SD = 8.58°). There were no de novo dislocations.In total, there were 12 complications, 8 of which had no clinical repercussions. The clinically-significant complications included an infection six months after surgery with a potential hematogenous origin, a coracoid fracture that required an intraoperatively procedure change, and two patients with previous impingement who required synthesis material removal more than six months after surgery. Conclusion Although the arthroscopic Bristow procedure was effective in treating anterior shoulder instability, it is not a complication-free surgery.

scholarly journals The Transition From Minimal Change Disease to Focal and Segmental Glomerulosclerosis in a Patient With Nephrotic Syndrome: a Case Report

2021 ◽  
Author(s):  
Long Tang ◽  
Zhen Cai ◽  
Yuan Meng ◽  
Wen-jing Zhao ◽  
Su-Xia Wang
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Kidney Biopsy ◽  
Elevated Serum ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage ◽  
The Relationship

Abstract Background:Although minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) have been described as two separate forms of nephrotic syndrome(NS), they are not completely independent. We report a patient presenting a transition from MCD to FSGS, review the literature and explore the relationship between the two diseases.Case presentation:A 42-year-old male welder, Asian, presenting lower extremity edema and elevated serum creatinine, had laboratory exams indicating NS and end-stage renal disease(ESRD). The patient had a kidney biopsy 20 years earlier for NS, which indicated MCD, and this repeated kidney biopsy suggested FSGS. After treatment follow-up, the patient was eventually admitted to renal replacement therapy. Conclusions:MCD and FSGS may be different stages of the same disease. The transition from MCD to FSGS in this case indicates the progression of the disease, which may be related to the excessive metal caused by occupation.

Is rituximab (R) the optimal therapeutic intervention (TI) for post-transplant lymphoproliferative disorder (PTLD) following solid organ transplant (SOT)?

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 8074-8074
Author(s):  
R. E. Tsao ◽  
T. Jin ◽  
B. Richendollar ◽  
E. Hsi ◽  
B. Pohlman
Keyword(s):  
De Novo ◽  
Univariate Analysis ◽  
Cleveland Clinic ◽  
Organ Transplant ◽  
Clinical Information ◽  
Initial Study ◽  
Solid Organ ◽  
Younger Age ◽  
Study Results

8074 Background: PTLD is a rare, often fatal, complication of SOT. Most are derived from CD20+ B-cells. Historically, patients (pts) received a variety of TIs ranging from decreased immunosuppression to chemotherapy (CT). Based on promising initial study results, most pts now receive rituximab (R) without CT as part of 1st TI. Methods: We searched the Cleveland Clinic pathology archives for SOT pts, who were diagnosed with PTLD between 1987 and 2006; reviewed the medical records; extracted clinical information and outcomes; and analyzed the data by Cox proportional univariate and multivariate analyses. Results: We identified 55 SOT pts (heart, 18; lung, 16; kidney 14; liver, 6; pancreas, 1), who were diagnosed with PTLD at median age 47 years (range 7–66). The median time from SOT to PTLD was 1.7 years (range .2–20.9). 1st TI (usually >1) included “complete” resection (4), decreased immunosuppresion (53), acyclovir or ganciclovir (28), interferon (4), radiation therapy (6), CT (12), and/or R (17). Response to 1st TI was CR (30) or PR (10). The median follow-up among surviving pts is 5.0 years (range .1–11.4). 29 (including 5 CT and 5 R) pts have died; only 2 CT but all 5 R pts died from PTLD. On univariate analysis, younger age+, <2 prior rejection episodes+*, PS <2+*, normal LDH+*, <2 extranodal sites+, lower IPI+*, >1 1st TI+, and 1st TI with CT* were associated with an improved overall survival (OS)+ and/or PTLD-specific survival (PSS)*. On multivariate analysis, only PS <2 (HR 0.04 [CI 0.01–0.14], p<0.001) and >1 1st TI (HR 0.43 [CI 0.19–0.97], p=0.041) were associated with improved OS while PS <2 (HR 0.04 [0.01- 0.16], p<0.001) and 1st TI with CT (HR 0.19 [CI 0.04–0.84], p<0.028) were associated with improved PSS. Conclusions: A significant minority of SOT pts that receive R without CT as part of 1st TI still die from PTLD. PS is the most important predictor of outcome. In conjunction with the improved survival observed in de novo B-cell NHL pts treated with R+CT (compared to CT alone), this retrospective analysis suggests that some SOT PTLD pts should receive R+CT as part of 1st TI. No significant financial relationships to disclose.

scholarly journals Management of Nephrotic Syndrome: A Case Report from Lao PDR

2017 ◽  
Vol 44 (Suppl. 1) ◽  
pp. 31-34
Author(s):  
Phanekham Souvannamethy
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
Lao Pdr ◽  
Deficiency Anemia ◽  
Laboratory Findings ◽  
Poor Diet ◽  
History Of ◽  
Laboratory Investigations ◽  
Pedal Edema

We report the case of a 23-year-old woman with a 2-week history of swelling around the eyes and both legs, and generalized body swelling. She had a history of chronic constipation and poor diet but no fever, recent illnesses, or hematuria. Examination revealed bilateral pedal edema and mild ascites. Laboratory investigations showed low hemoglobin 79 g/L, low mean corpuscular volume 53 fL, thrombocytosis 973 × 109/L, and marked hypochromia and microcytosis, with low iron and ferritin. She had hypoalbuminemia and reduced serum protein (albumin 1.9 g/dL, globulin 2.8 g/dL) with elevated triglycerides (454 mg/dL). Although kidney biopsy could not be performed due to a lack of facilities in the country, we made a diagnosis of idiopathic nephrotic syndrome (NS) with iron deficiency anemia secondary to poor diet based on clinical and laboratory findings. The patient was admitted and treated with intravenous methylprednisolone and iron supplements. Antiplatelet therapy was instituted with dipyridamole to prevent thromboembolism from the combination of nephrotic syndrome and thrombocytosis. She was later treated with albumin and furosemide due to elevated blood pressure and worsened edema. The edema resolved and her general condition improved. She was discharged to follow up and has remained stable, requiring no further treatment as of 18 months after admission. Kidney biopsy is important for diagnosis of NS. Diagnosis may be made from clinical and laboratory findings alone in some cases; however, biopsy is required to determine the type of NS and improve further management and treatment outcomes for patients.

scholarly journals DOP50 Recommencement of Inflammatory Bowel Disease medications following colectomy for Ulcerative Colitis

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S085-S086
Author(s):  
O Ledder ◽  
R Lujan ◽  
E Orlanski-Meyer ◽  
C Friss ◽  
Y Loewenberg Weisband ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
De Novo ◽  
Large Population ◽  
Medication Use ◽  
Health Maintenance ◽  
True Incidence ◽  
Repeated Surgery ◽  
Clinically Significant ◽  
Acute Severe Colitis

Abstract Background Total colectomy (TC) is often considered a curative procedure for patients with chronic refractory ulcerative colitis (UC) or acute severe colitis. Chronic pouchitis and de novo Crohn’s disease (CD) are well recognized sequelae following TC, yet the true incidence of these are poorly characterized in large population models. We assessed the rate of subsequent utilization of IBD medications as a proxy marker of clinically significant pouchitis or de novo CD following TC for UC. Methods This study utilized data from the Epi-IIRN project, a meta-database incorporating patient data from all four health maintenance organisations (HMO) in Israel, representing 98% of the population. We included all patients identified in the prevalence cohort with an initial diagnosis of UC who underwent TC from January 2000, with ≥ 6 month follow-up. Primary outcome was utilization of IBD medications (including thiopurines, methotrexate, infliximab, adalimumab, vedolizumab, ustekinumab and tofacitinib) following TC. Secondary outcomes were time to commencement of medications, hospitalizations and repeated surgery. Potential predictors of IBD medication use were identified using multivariable models. Results Overall 23,506 patients with UC were identified in the prevalence cohort of whom 456 patients underwent TC for UC and were included in our analysis. 51% of our sample were female, with a median follow up of 8.5 years (IQR 3.8–13.2) and 3956 patient-years. Median age at UC diagnosis was 44.1 (26.5–56.2) years and at TC was 50.2 (34.2–61.7). IBD medications were commenced in 88 (19%) of patients, including 54 (12%) biologics, 56 (12%) immunomodulators and 3 (1%) tofacitinib. A diagnosis of CD was formally assigned to 65 (75%) of these patients. The need for IBD medications was gradual (figure 1). Patients recommenced on IBD medications were younger at diagnosis (30.3 years (18.3–49.7) vs 41.4 (24.2–55.6), p&lt;0.001), at colectomy (34.3 (21.5–54.1) vs 47.5 (30.4–60.6), p&lt;0.001) and with shorter interval from UC diagnosis to TC (2.2 years (1.0–3.3) vs 2.8 (1.4–5.1), p=0.03). TC during childhood was performed in 33 (7%) patients and these had higher utilization of IBD medications post TC (16/33 (49%) vs 72/423 (17%), p=0.001). Conclusion In this nationwide analysis we show that almost 20% of patients require ongoing IBD medications following TC for UC. Younger age is associated with higher rate of subsequent medication use. Patient expectations need be adjusted to account for the potential ongoing requirement of long-term medication following TC.

scholarly journals Association of Kidney Biopsy Findings With Short- and Medium-Term Outcomes in Children With Moderate-to-Severe Iga Vasculitis Nephritis

2021 ◽  
Author(s):  
Stéphanie Clavé ◽  
Maud Sordet ◽  
Michel Tsimaratos ◽  
Stéphane Decramer ◽  
Marc Fila ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Kidney Biopsy ◽  
Immunoglobulin A ◽  
International Study ◽  
Clinical Severity ◽  
Grade Ii ◽  
Kidney Impairment ◽  
Grade Iii

Abstract Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is challenging important due to its determining effect on kidney management and outcomes. This study paper aims to describe describes a multicentre paediatric multicenter pediatric cohort of IgAV-N patients and discusses whilst investigating the relationships among between clinical presentation, histological features, and kidney outcome. A cohort consisting of 170 children requiring early kidney with biopsy because of IgAV-N, which was diagnosed between 2007 and 2017, was assessed including 27% of children with nephrotic syndrome (NS). One-quarter of the cohort (27%) presented with initial nephrotic syndrome (NS). Kidney biopsy revealed International Study of Kidney Disease (ISKDC) grade II or grade III in 83% of cases. An International Study of Kidney Disease (ISKDC) grade II or grade III was revealed through kidney biopsy in 83% of cases. Endocapillary proliferation was were observed in 73% of patients, and chronic lesions were observed in 25%of patients. Data analysis demonstrated showed a significant association between clinical severity (NS at onset and histological findings such asendocapillary proliferation and cellular crescents. After a median follow-up of 21 months (IQR 12-39), 30% of patients had persistent kidney impairment (proteinuria or decreased eGFR. WorseAt the end of follow-up, kidney outcome impairment was significantly associated more often observed in patients with NS at onset and those with cellular crescents and chronic lesions on initial kidney biopsy.Conclusions: This study highlights the relationship between the clinical and histological presentation of IgAV-N and the factors that affect kidney outcome. The ISKDC classification may be improved by including lesions that are more discriminating for disease severity and prognosis.

Role of a Doxorubicin-Containing Regimen in Relapsed and Resistant Lymphomas: An 8-Year Follow-Up Study of EPOCH

2000 ◽  
Vol 18 (21) ◽  
pp. 3633-3642 ◽  
Author(s):  
Martin Gutierrez ◽  
Bruce A. Chabner ◽  
Debra Pearson ◽  
Seth M. Steinberg ◽  
Elaine S. Jaffe ◽  
...  
Keyword(s):  
De Novo ◽  
International Prognostic Index ◽  
Oral Prednisone ◽  
Prognostic Index ◽  
Resistant Disease ◽  
Highly Effective ◽  
Clinically Significant ◽  
Hodgkin's Lymphomas

PURPOSE: Curative up-front regimens for non-Hodgkin’s lymphomas contain doxorubicin, vincristine, and cyclophosphamide, whereas salvage regimens generally contain non–cross-resistant agents. We hypothesized that up-front agents may be highly effective for salvage and developed an infusional regimen based on in vitro evidence of increased efficacy. PATIENTS AND METHODS: A prospective phase II study of etoposide, vincristine, and doxorubicin over 96 hours with bolus cyclophosphamide and oral prednisone (EPOCH) was performed in 131 patients with relapsed or resistant lymphoma. RESULTS: Seventy-nine percent of patients had aggressive histologies, 46% were considered high risk by the International Prognostic Index, and 34% had resistant disease. Eighty-eight percent of patients had received at least four of the agents in EPOCH, and 94% had received doxorubicin. In 125 assessable patients, 29 (24%) achieved complete responses and 60 (50%) achieved partial responses. Among 42 patients with resistant disease, 57% responded, and in 28 patients with relapsed aggressive de novo lymphomas, 89% responded with 54% complete responses. With a median follow-up of 76 months, the overall and event-free survivals (EFS) were 17.5 and 7 months, respectively. In 33 patients with sensitive aggressive disease who did not receive stem-cell transplantation, EFS was 19% at 36 months. Toxicity was primarily hematologic, with an 18% incidence of febrile neutropenia. No clinically significant cardiac toxicity was observed, despite no maximum cumulative doxorubicin dose. CONCLUSION: EPOCH is highly effective in patients who had previously received most/all of the same drugs and produces durable remissions in curable subtypes. Salvage regimens need not contain non–cross-resistant agents, and infusional schedules may partially reverse drug resistance and reduce toxicity.

scholarly journals Minimal Change Disease After First Dose of Pfizer-BioNTech COVID-19 Vaccine: A Case Report and Review of Minimal Change Disease Related to COVID-19 Vaccine

2021 ◽  
Vol 8 ◽  
pp. 205435812110582
Author(s):  
Jessica Hanna ◽  
Alistair Ingram ◽  
Tiffany Shao
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Kidney Biopsy ◽  
De Novo ◽  
Rare Complication ◽  
Minimal Change ◽  
Steady Increase ◽  
Rapid Weight Loss ◽  
Tubular Injury ◽  
Number Of Patients

Rationale: While severe complications are generally uncommon with novel coronavirus disease 2019 (COVID-19) vaccine, there has been a steady increase in the number of patients presenting with nephrotic syndrome and acute kidney injury after the administration of COVID-19 vaccine. Physicians should be made aware of minimal change disease as a potential complication associated with COVID-19 vaccine. Presenting concerns: A 60-year-old male without significant past medical history presented with new onset of nephrotic syndrome approximately 10 days after his first dose of Pfizer-BioNTech COVID-19 vaccine. Laboratory findings showed hypoalbuminemia (20 g/L), elevated urine albumin/creatinine ratio (668 mg/mmol), and elevated creatinine of 116 µmol/L from a baseline of 79 µmol/L. Diagnosis: A diagnostic kidney biopsy was performed 6 weeks after the onset of the edema and approximately 8 weeks after his first dose of Pfizer-BioNTech COVID-19 vaccine. The kidney biopsy findings were consistent with minimal change disease with focal acute tubular injury. Interventions: The patient was treated conservatively with ramipril 10 mg and furosemide 80 mg daily 5 weeks after the onset of swelling. Prednisone 1 mg/kg was initiated immediately when the kidney biopsy result became available (approximately 6 weeks after the onset of edema). Outcomes: The patient remitted with rapid weight loss starting 2 weeks post prednisone initiation. Novel findings: De novo minimal change disease with acute tubular injury is a kidney manifestation following the administration of Pfizer-BioNTech COVID-19 vaccine. Minimal change disease is potentially a rare complication of Pfizer-BioNTech COVID-19 vaccine.

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