Review
Biochemistry & Molecular Biology
Sergei A. Fedotov, Maria S. Khrabrova, Anastasia O. Anpilova, Vladimir A. Dobronravov, Aleksandr A. Rubel
Summary: Amyloidoses are a group of diseases characterized by the accumulation of abnormal proteins in organs and tissues. Early diagnosis and treatment are crucial for improving patient survival.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Puneet Rawat, R. Prabakaran, Sandeep Kumar, M. Michael Gromiha
Summary: A machine learning model has been developed to predict the amyloidogenic nature of antibody light chains with an accuracy of 79.7%, providing insights for prognosis improvement, understanding aggregation origins in antibody-based biotherapeutics, large-scale antibody sequence analysis, and rational engineering design.
SCIENTIFIC REPORTS
(2021)
Article
Veterinary Sciences
Robert Adam Harris, Matthew Miller, Dillon Donaghy, Laura Ashton, Catherine Langston, Todd Shockey, A. Russell Moore
Summary: The study successfully detected serum and urine free light chains in dogs and cats using a commercially available human immunofixation kit. Free light chains may be nephrotoxic in dogs.
JOURNAL OF VETERINARY INTERNAL MEDICINE
(2021)
Article
Medicine, General & Internal
Emilia Czyzewska, Agnieszka Wisniewska, Anna Waszczuk-Gajda, Olga Ciepiela
Summary: The study indicates that the presence of monoclonal lambda chains in patients with AL amyloidosis may be associated with more severe damage to cardiomyocytes and dysfunction of the myocardium.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Endocrinology & Metabolism
Eli Muchtar, Matthew T. Drake, Nelson Leung, Angela Dispenzieri, Martha Q. Lacy, Francis K. Buadi, David Dingli, Suzanne R. Hayman, Prashant Kapoor, Yi Lisa Hwa, Amie Fonder, Miriam Hobbs, Wilson Gonsalves, Taxiarchis Kourelis, Rahma Warsame, Stephen Russell, Ronald S. Go, Moritz Binder, Robert A. Kyle, S. Vincent Rajkumar, Shaji K. Kumar, Morie A. Gertz
Summary: The study found that vitamin D deficiency is common in AL amyloidosis patients, particularly among those with heavy proteinuria. Severe 25(OH)D deficiency at diagnosis predicts progression to end-stage renal disease.
FRONTIERS IN ENDOCRINOLOGY
(2022)
Article
Neurosciences
Pietro Manuel Ferraro, Viola D'Ambrosio, Andrea Di Paolantonio, Valeria Guglielmino, Paolo Calabresi, Mario Sabatelli, Marco Luigetti
Summary: This study reports the prevalence of kidney involvement and kidney function over time in ATTRv patients with different transthyretin gene mutations. The results show that kidney involvement is common in ATTRv patients, with abnormal UPE appearing to be a common feature of the disease. No single predictor was associated with either eGFR values or its slope over time.
Article
Urology & Nephrology
Csilla Markoth, Laszlo Bidiga, Laszlo Varoczy, Ibolya File, Jozsef Balla, Janos Matyus
Summary: In this study, the deposition of amyloid in the tubular basement membrane (TBM) in patients with light chain amyloidosis (LA) was investigated. It was found that amyloid deposition in the TBM was present in 4 out of 12 LA patients and these patients had worse renal function. These findings suggest the importance of TBM amyloid deposition in the progression of renal disease.
Article
Urology & Nephrology
Naeun Kwak, Min Ji Park, Hea Min Jang, Hee Sun Baek, Sang In Lee, Man Hoon Han, Yong-Jin Kim, Min Hyun Cho
Summary: This study analyzed the incidence and clinical outcomes of children who showed normal histopathological findings in their percutaneous renal biopsies (PRBs). The results showed that most children with normal PRB histopathologic findings had improved urinary abnormalities during the follow-up period, and none of them developed end-stage renal disease.
KIDNEY RESEARCH AND CLINICAL PRACTICE
(2022)
Article
Immunology
Jipeng Yan, Jin Zhao, Xiaoxuan Ning, Yunlong Qin, Yan Xing, Yuwei Wang, Qing Jia, Boyong Huang, Rui Ma, Changhui Lei, Meilan Zhou, Zixian Yu, Yumeng Zhang, Wei-Feng Guo, Shiren Sun
Summary: This study demonstrates for the first time the alterations of gut microbiota in patients with AL amyloidosis and successfully establishes and validates a microbial-based diagnostic model. This provides a foundation for future research on microbe-based strategies for diagnosis and treatment in patients with AL amyloidosis.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Environmental Sciences
Tomasz Golebiowski, Jakub Kuzniar, Tomasz Porazko, Renata Wojtala, Andrzej Konieczny, Magdalena Krajewska, Marian Klinger
Summary: This study reports on a coal miner with silicosis who developed rapidly progressing primary amyloidosis, primarily affecting the heart, kidneys, and lungs. The patient died due to severe heart failure caused by myocardial hypertrophy. The research suggests that long-term exposure to environmental silica and silica deposition may contribute to the development of monoclonal gammopathy and amyloidosis through chronic stimulation and immune system dysregulation.
INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH
(2022)
Article
Medicine, General & Internal
Fatma Yazilitas, Evrim Kargin cakici, Eda Didem Kurt Sukur, Semanur Ozdel, Tulin Gungor, Esra Baglan, Evra Celikkaya, Deniz Karakaya, Diclehan Orhan, Mehmet Bulbul
Summary: This study identified risk factors associated with renal involvement in children with IgA vasculitis, including older age, presence of hematuria and/or proteinuria in urine examination, and disease onset in the spring season. The optimal timing for predicting renal involvement in the sixth month was found to be the urine examination conducted in the first month after diagnosis. Patients with IgA vasculitis who show abnormal urine findings in the first month should receive closer follow-up.
POSTGRADUATE MEDICINE
(2022)
Article
Biology
Rebecca Sternke-Hoffmann, Thomas Pauly, Rasmus K. Norrild, Jan Hansen, Florian Tucholski, Magnus Haraldson Hoie, Paolo Marcatili, Mathieu Dupre, Magalie Duchateau, Martial Rey, Christian Malosse, Sabine Metzger, Amelie Boquoi, Florian Platten, Stefan U. Egelhaaf, Julia Chamot-Rooke, Roland Fenk, Luitgard Nagel-Steger, Rainer Haas, Alexander K. Buell
Summary: Researchers introduce a new systematic workflow for the biophysical characterization of multiple myeloma patient-derived immunoglobulin light chains (IgLCs) and find that in vivo aggregation behavior is unlikely to be linked to any single biophysical or biochemical parameter. They also discover that the potential to form amyloid fibrils is widespread in IgLC sequences and not limited to those sequences that form fibrils in patients.
Review
Oncology
Linchun Xu, Yongzhong Su
Summary: Immunoglobulin light chain amyloidosis (AL) is a chronic plasma cell disorder characterized by FLC misfolding and amyloid deposition, classified into non-hyperdiploidy and hyperdiploidy subtypes with distinct treatment responses and prognostic outcomes based on cytogenetic patterns. Other genetic factors, such as specific immunoglobulin light chain germline genes and somatic mutations, also contribute to amyloid formation in AL.
EXPERIMENTAL HEMATOLOGY & ONCOLOGY
(2021)
Article
Medicine, General & Internal
Yue Wang, Xueyao Wang, Jinyu Yu, Shan Wu, Zhonggao Xu, Weixia Sun
Summary: This study reports a rare case of a 39-year-old man with idiopathic membranous nephropathy (IMN) combined with immunoglobulin light chain amyloidosis nephropathy. Attention should be paid to the subtype of the disease and the exclusion of secondary factors. Comprehensive clinical and pathological examinations are crucial for accurate diagnosis and staging.
FRONTIERS IN MEDICINE
(2022)
Article
Clinical Neurology
Min Qian, Lan Qin, Kaini Shen, Hongzhi Guan, Haitao Ren, Yanhuan Zhao, Yuzhou Guan, Daobin Zhou, Bin Peng, Jian Li, Lin Chen
Summary: This study retrospectively reviewed the clinical data of 8 AL amyloidosis patients with peripheral neuropathy as the initial presentation, and found that early recognition and chemotherapy are crucial for better outcomes. Nerve biopsy can help to make the diagnosis.
FRONTIERS IN NEUROLOGY
(2021)