Plasmacytomas are rare immunoproliferative monoclonal plasma cell diseases of lymphoid lineage that may present in an isolated or systemic manner. Systemic involvement is much more common than occurrences isolated to a particular organ, and for this reason, it is imperative to rule out systemic involvement for osseous and nonosseous isolated neoplasms. These neoplasms present unique challenges due to their location, extent of involvement, vague presentation, and dearth of treatment protocol. We report the case of a 69-year-old man who developed chronic kidney disease stage 4 between 2009 and 2012. Precipitous kidney failure, anorexia, fatigue, and flank pain necessitated clinical follow-up that ultimately led to thorough imaging and bilateral kidney biopsy. Protein electrophoresis, immunohistochemistry, and immunofluorescence were all consistent with bilateral renal extramedullary plasmacytomas. Treatment recommendations are often limited to prior case successes; however, chemotherapy, radiation, and surgery are the mainstay of treatment. Although surgery or combined therapy provides the best results for patients, such options are unfeasible with bilateral kidney involvement. Therefore, a chemotherapy regimen, similar to that for multiple myeloma, was determined to be most reasonable. Treatment consisted of 4 cycles of a bortezomib, cyclophosphamide, and dexamethasone regimen. Three months following chemotherapy, kidney function returned to baseline levels.
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http://dx.doi.org/10.1053/j.ajkd.2018.03.029 | DOI Listing |
BMC Pediatr
January 2025
Geriatric Mental Health Research Center, Department of Psychiatry, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Background: This case report describes a unique presentation of sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) caused by a rare SGPL1 variant, highlighting the diagnostic and management challenges associated with this condition.
Case Presentation: A 2-year-old Iranian female presented with steroid-resistant nephrotic syndrome (NS), primary adrenal insufficiency (AI), growth delay, seizures, and hyperpigmentation. Laboratory evaluation revealed hypoalbuminemia, significant proteinuria, hyperkalemia, and elevated adrenocorticotropic hormone (ACTH) levels.
Am J Obstet Gynecol MFM
January 2025
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Cincinnati College of Medicine, 231 Albert Sabin Way, Cincinnati, Ohio 45267, USA. Electronic address:
Background: Chronic kidney disease is a significant cause of adverse obstetric outcomes. However, there are few studies assessing the risk of severe maternal morbidity and mortality among patients with chronic kidney disease and no studies assessing the association between individual indicators of severe maternal morbidity and chronic kidney disease.
Objective: To evaluate the risk of severe maternal morbidity and mortality among pregnant patients with chronic kidney disease.
Pediatr Nephrol
January 2025
Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong SAR.
Background: This study aimed to evaluate the incidence, contributing factors, and clinical outcomes of acquired cystic kidney disease (ACKD) in children undergoing kidney replacement therapy (KRT).
Methods: We conducted a cross-sectional, territory-wide study at the designated pediatric nephrology center in Hong Kong. ACKD was defined as the presence of ≥ 3 cysts in the native kidneys, excluding congenital or hereditary cystic diseases.
Cardiovasc Drugs Ther
January 2025
Department of Cardiovascular Science, Ascension St, Thomas Hospital, University of Tennessee Health Science Center, Nashville, TN, USA.
Purpose: Heart failure (HF) management is well-defined for reduced ejection fraction (HFrEF) but less so for mildly reduced (HFmrEF) or preserved ejection fraction (HFpEF). This meta-analysis evaluates the impact of Finerenone, a nonsteroidal mineralocorticoid receptor antagonist, on cardiovascular and renal outcomes in these patient populations.
Methods: A systematic search in PubMed and Embase identified randomized controlled trials (RCTs) on Finerenone's cardiovascular and renal effects.
J Gen Intern Med
January 2025
Department of General Internal Medicine, University of Colorado, Anschutz Campus, Aurora, CO, USA.
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