Dialysis amyloidosis

Leukocyte chemotactic factor-2 amyloidosis (ALECT2) is a recently described subtype of amyloidosis. IgG4-related disease is a rare fibroinflammatory condition characterized by dense interstitial lymphoplasmacytic infiltrates and fibrosis. Membranous nephropathy and diabetic nephropathy are common causes of nephrotic syndrome. Here we report a 49-year-old Hispanic male patient with diabetes mellitus who presented with jaundice and pruritus. IgG4-related autoimmune pancreatitis was diagnosed through laboratory workup and ampulla biopsy...

OBJECTIVE: We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still's disease (AOSD). METHODS: We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed...

Amyloid fibril formation is a general property of proteins and peptides. It is a physicochemical phenomenon similar to crystallization, in which amyloid precursor proteins exceeding solubility precipitate through the breakdown of supersaturation. Using the ultrasonication-forced amyloid fibril inducer HANABI, we have discovered that serum albumin acts as an inhibitor in dialysis-related amyloidosis. Exploring the factors that induce or inhibit amyloid fibril formation using HANABI can lead to the development of early diagnosis and prevention methods for amyloidosis...

AL amyloidosis is caused by the excessive production of nonfunctional immunoglobulins, leading to the formation of amyloid fibrils that damage vital organs, especially the heart and kidneys. AL amyloidosis presents with non-specific symptoms such as fatigue, weight loss, numbness, pain, and nephrotic syndrome. Consequently, diagnosis is often delayed, and patients typically present with advanced disease at diagnosis. The Pavia renal staging model stratifies patients based on their likelihood of progressing to dialysis...

BACKGROUND: The kidney is involved in almost 100% of cases of AA amyloidosis, a rare disease caused by persistent inflammation with long overall survival but frequent progression to end-stage kidney failure . Identification of patients with advanced disease at diagnosis is difficult, given the absence of validated staging systems. METHODS: Newly diagnosed patients with AA amyloidosis from the Pavia (n=233, testing cohort) and Heidelberg (n=243, validation cohort) centers were included in the study...

Amyloidosis is a heterogeneous disorder characterized by abnormal protein aggregate deposition that often leads to kidney involvement and end-stage kidney disease. With advancements in diagnostic techniques and treatment options, the prevalence of patients with amyloidosis requiring chronic dialysis has increased. Kidney transplantation is a promising avenue for extending survival and enhancing quality of life in these patients. However, the complex and heterogeneous nature of amyloidosis presents challenges in determining optimal referral timing for transplantation and managing post-transplantation course...

OBJECTIVES: To report healthcare resource utilization (HCRU) and safety outcomes in systemic light chain (AL) amyloidosis from the EMN23 study. MATERIALS AND METHODS: The retrospective, observational, multinational EMN23 study included 4,480 patients initiating first-line treatment for AL amyloidosis in 2004-2018 and assessed, among other objectives, HCRU and safety outcomes. HCRU included hospitalizations, examinations, and dialysis; safety included serious adverse events (SAEs) and adverse events of special interest (AESIs)...

Diffuse thyroid lipomatosis (DTL) is a rare entity of unknown etiology that can be associated with amyloidosis and rarely, thyrotoxicosis. Here, we present a case of DTL with amyloid deposits and concurrent thyrotoxicosis. A 64-year-old South-Asian woman with a several-year history of an enlarging goiter, unintentional weight loss, and work-up 10 months prior suggestive of thyroiditis presented with a viral syndrome in setting of several weeks of progressive fatigue. Her examination was notable for resting sinus tachycardia and massive painless goiter...

BACKGROUND: Dialysis-related amyloidosis (DRA) is a severe complication in end-stage kidney disease (ESKD) patients undergoing long-term dialysis treatment, characterized by the deposition of β2 -microglobulin-related amyloids (Aβ2M amyloid). To inhibit DRA progression, hexadecyl-immobilized cellulose bead (HICB) columns are employed to adsorb circulating β2 -microglobulin (β2M). However, it is possible that the HICB also adsorbs other molecules involved in amyloidogenesis...

From a physicochemical viewpoint, amyloid fibril formation is a phase transition from soluble to crystal-like sates limited by supersaturation. It occurs only above solubility (i.e., the solubility limit) coupled with a breakdown of supersaturation. Although many studies have examined the role of molecular chaperones in the context of proteostasis, the role of supersaturation has not been addressed. Moreover, although molecular chaperone-dependent disaggregations have been reported for preformed amyloid fibrils, amyloid fibrils will not dissolve above the solubility of monomers, even if agitations fragment long fibrils to shorter amyloid particles...

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Systemic amyloid A amyloidosis is a progressive condition in which sustained elevation of serum amyloid A protein concentration leads to widespread amyloid deposition resulting in multiorgan failure without treatment. The kidney is the most commonly affected organ, and renal amyloid A amyloidosis can cause nephrotic syndrome and chronic kidney disease (CKD) leading to end stage kidney disease (ESKD). Serum Amyloid A protein is produced in the liver in response to chronic inflammation, specifically by inflammatory cytokines, especially IL-6...

Immunotactoid deposition is a rare fibrillary deposition disease that is primarily seen in the kidney and is associated with paraproteinemia. Here, we report a case of hepatic immunotactoid deposition in a 67-year-old male with a history of smoldering myeloma and chronic kidney disease who underwent liver transplantation for metabolic dysfunction-related cirrhosis. Immunotactoid deposition was first identified in the explanted liver and recurred in the allograft within only 7 weeks following transplantation, presenting as ascites with normal liver function tests...

INTRODUCTION: Ultrasonographic examination of joints is a non-invasive method to detect and monitor Dialysis-associated amyloidosis (DRA). METHODS: An ultrasonographic examination of the sternoclavicular (SC) and shoulder joints was performed in patients on maintenance hemodialysis (MHD) and those with normal renal function in the control group. The maximum distance (D) of SC, the rotator cuff thickness (RCs), and the echogenic pads (EPs) of the shoulder were evaluated...

INTRODUCTION: Solitary plasmacytoma is a rare, localized malignancy. Bone localizations are the most common. Extramedullary plasmacytomas are much rarer. They are most often in the upper respiratory tract and can be complicated by amyloidosis. Here is an original report of a mediastinal extramedullary plasmacytoma revealed by type AA renal amyloidosis. CASE PRESENTATION: We present the case of a 52-year-old patient with mediastinal extramedullary plasmocytoma diagnosed by renal failure due to type AA renal amyloidosis...

AIMS: Sevelamer is a phosphate-binding resin implicated in gastrointestinal (GI) injury. This study aimed to investigate the role of sevelamer in GI injury among chronic kidney disease (CKD) patients. METHODS AND RESULTS: The study included 17 CKD patients (cases) with and 18 CKD patients (comparisons) without sevelamer crystals in specimens. All cases were on sevelamer. Six comparison patients were also taking sevelamer, but crystals were absent in tissue sections...

BACKGROUND: Autologous hematopoietic stem cell transplant plays an important role in multiple myeloma (MM) treatment. Increasing incidence of MM and growing awareness of acute kidney injury (AKI) as a complication of hematopoietic stem cell transplant results in the need to better understand AKI in these patients. We aimed to evaluate incidence, risk factors and 5-year prognostic impact of AKI in MM patients undergoing autologous hematopoietic stem cell transplant. METHODS: Retrospective cohort study...

β2 -microglobulin (β2 -m) is a plasma protein derived from physiological shedding of the class I major histocompatibility complex (MHCI), causing human systemic amyloidosis either due to persistently high concentrations of the wild-type (WT) protein in hemodialyzed patients, or in presence of mutations, such as D76N β2 -m, which favor protein deposition in the adulthood, despite normal plasma levels. Here we describe a new transgenic Caenorhabditis elegans ( C. elegans ) strain expressing human WT β2 -m at high concentrations, mimicking the condition that underlies dialysis-related amyloidosis (DRA) and we compare it to a previously established strain expressing the highly amyloidogenic D76N β2 -m at lower concentrations...

RATIONALE: De Novo transplant amyloidosis denotes the condition when a patient develops amyloidosis after transplantation but had not been diagnosed with the disease prior to transplantation. The incidence of de novo amyloidosis in kidney transplants is rare, but few published case reports have described the occurrence of de novo Amyloid A protein (AA) and Light Chain (AL) amyloidosis. However, de novo hereditary fibrinogen A alpha chain (AFib) has not been previously reported. PATIENT PRESENTATION: We present a 72-year-old man, a kidney transplant recipient, who developed progressive rise in his creatinine about 3 years after transplantation...

Dialysis treatment has improved the survival of patients with kidney failure. However, the hospitalization and mortality rates remain alarmingly high, primarily due to incomplete uremic toxin elimination. High-volume hemodiafiltration (HDF) has emerged as a promising approach that significantly improves patient outcomes by effectively eliminating medium and large uremic toxins, which explains its increasing adoption, particularly in Europe and Japan. Interest in this therapy has grown following the findings of the recently published CONVINCE study, as well as the need to understand the mechanisms behind the benefits...