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Study of a novel Ca/P/S-based cement implanted in the osteoporotic goat spine and its clinical application in preventing adjacent vertebral fractures.

Osteoporotic vertebral compression fracture (OVCF) has been increasing over the last decades. In addition to primary OVCF, the adjacent vertebral fracture (AVF) is a frequent serious complication in OVCF patients receiving percutaneous vertebroplasty or kyphoplasty, resulting in poor long-term outcome and recurrent symptoms. The aim of the present study is to investigate the bone regeneration behavior of a novel Ca/P/S-based resorbable cement (Ezechbone® Cement CBC-400) implanted into surgically created vertebral defects of ovariectomized (OVX) goats. Furthermore, the present study provides a

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… Relatively mature lamellar bones are usually aligned in layers, and collagen bundles are usually parallel to each other, causing a birefringence effect that allows identification of their existence under polarized light ( Bullough, 2010 ; Warshaw et al., 2017 ). … The transmitted polarized light microscopy used in the present study could easily distinguish lamellar bone from woven bone based on their different birefringent effects ( Gunson et al., 2015 ; Ip et al., 2016 ). …

Brazilian consensus for diagnosis, management and treatment of hereditary transthyretin amyloidosis with peripheral neuropathy: second edition.

Hereditary transthyretin amyloidosis with peripheral neuropathy (ATTRv-PN) is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy with over 130 pathogenic variants identified in the TTR gene. Hereditary transthyretin amyloidosis with peripheral neuropathy is a disabling, progressive and life-threatening genetic condition that leads to death in ∼ 10 years if untreated. The prospects for ATTRv-PN have changed in the last decades, as it has become a treatable neuropathy. In addition to liver transplantation, initiated in 1990, there are now at least 3 drugs approved in many

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… On peripheral nerve biopsy, amyloid deposits are scattered in the endoneurium and around blood vessels and have a round, amorphous, and orange appearance on Congo red staining, with characteristic apple-green birefringence under polarized light 61 71 ( Figure 1 ). … The section A under polarized light shows the amyloid material birefringence appearing here as apple-green and golden-yellow colors. C. Electropherogram of TTR gene shows the c.148G > A(Val30Met) mutation. D. Semithin section stained with Toluidine Blue shows axonal loss. F. …

Patient with AL amyloidosis with recurrent syncope as the first symptom: case report and literature review.

BACKGROUND: Immunoglobulin light-chain (AL) amyloidosis is a rare systemic disease caused by the extracellular deposition of misfolded light chains. Nonspecific manifestations, especially those dominated by severe autonomic neuropathy, frequently delay diagnosis. METHODS AND RESULTS: A 68-year-old woman was admitted after five months of recurrent syncope. The clinical picture was dominated by refractory orthostatic hypotension, nausea and vomiting alternating with diarrhea and constipation, stocking-glove paresthesias, and proteinuria. Bone marrow examination showed the presence of clonal pla

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… Renal biopsy established the diagnosis: Congo red–positive deposits exhibiting apple-green birefringence under polarized light confirmed renal amyloidosis of the λ light-chain (AL-λ) type, as shown in Figure 1 . Figure 1 Renal puncture pathology. (A) PAS stain. (B) light chain λ (×400). …

Lysosomal storage diseases.

Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy. Sometimes, the lysosomal storage ca

Utility of Bruton's Tyrosine Kinase Inhibitors in Light Chain Amyloidosis Caused by Lymphoplasmacytic Lymphoma (Waldenström's Macroglobulinemia).

Of the variety of immunoglobulin related amyloidosis (AL), immunoglobulin M (IgM) related AL represents only 6 to 10% of affected patients, and the majority of these cases are associated with underlying non-Hodgkin's Lymphoma including Waldenström's macroglobulinemia (WM). Ibrutinib, acalabrutinib, and zanubrutinib are Bruton tyrosine kinase (BTK) inhibitors approved for certain indolent B cell non-Hodgkin's lymphoma (NHL). BTK is a nonreceptor kinase involved in B-cell survival, proliferation, and interaction with the microenvironment. We retrospectively evaluated the tolerability and effecti

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… A liver biopsy demonstrated extensive amyloid deposits ( Figure 3(a) ) having an apple-green birefringence when stained with Congo red and observed under polarized light ( Figure 3(b) ). …

Case report: Light-chain amyloidosis responsive to selinexor in combination with daratumumab and dexamethasone (SDd) therapy.

The outcome of AL amyloidosis remains poor, particularly in patients with advanced organ involvement which takes long time to recovery. We conducted an observational study of two patients with AL amyloidosis treated with SDd regimen. Both patients successfully achieved significant hematological and organ responses without severe adverse events, and the time to organ response was remarkably shorter than previously reported. Notably, an over 15% reduction in interventricular septal thickness (IVST) was observed in patient#2 within 6 months. Up to now, SDd therapy has not been previously reported

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… renal involvement in October 2020 at a regional hospital based on the following laboratory findings: proteinuria of 4.3 g/24 h; serum and urine immunofixation electrophoresis revealed presence of λ type monoclonal proteins; a renal biopsy was showed substantial amyloid deposition with positive green birefringence …

Cardiac Amyloidosis: A Rare TTR Mutation Found in an Asian Female.

BACKGROUND: Transthyretin cardiac amyloidosis (ATTR) is a life-threatening, debilitating disease caused by abnormal formation and deposit of transthyretin (TTR) protein in multiple tissues, including myocardial extracellular matrix. It can be challenging to diagnose due to the myriad of presenting signs and symptoms. Additionally, numerous TTR mutations exist in certain ethnicities. Interestingly, our patient was discovered to have a very rare Gly67Ala TTR mutation typically not found in individuals of Asian descent. Recent advances in cardiovascular imaging techniques have allowed for earlier

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… They demonstrate apple-green birefringence with Congo red staining and have nonbranching, rigid filaments typically 7.5–10 nanometers in diameter [ 4 ]. Amyloidosis can be hereditary or acquired. …

Amyloidosis: a case series and review of the literature.

BackgroundSystemic amyloidosis is group of disorders characterized by the accumulation of insoluble proteins in tissues. The most common form of systemic amyloidosis is light chain amyloidosis, which results from the accumulation of misfolded immunoglobulins. The disease is progressive, with treatment targeted at the underlying plasma cell dyscrasia. Since essentially any organ system can be affected, the presentation is variable and delays in diagnosis are common. Given this diagnostic difficulty, we discuss four different manifestations of light chain amyloidosis.Case presentationsIn this ca

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… C Polarization shows “apple green” birefringence of amyloid A cardiac workup was pursued. ECG revealed low-voltage QRS complex. Transthoracic echocardiogram demonstrated intraventricular septum thickness of 1.75 cm. …

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis.

Cardiac amyloidosis (CA) is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart, resulting in increased myocardial stiffness, and restrictive heart wall chamber behavior. Its diagnosis among patients hospitalized for cardiovascular diseases is becoming increasingly frequent, suggesting improved disease awareness, and higher diagnostic capacities. One predominant functional manifestation of patients with CA is exercise intolerance, objectified by reduced peak oxygen uptake (VO2 peak), and assessed by metabolic cart during cardiopulmonary exercise testin

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… Confirmation of cardiac amyloid deposition is achieved through tissue biopsy, using thioflavin dyes (most commonly Congo red), displaying birefringence under cross-polarized light and conferring the characteristic apple-green color to amyloid deposits ( 1 , 7 , 8 ). …

Light-Chain Amyloidosis: The Great Impostor.

Light-chain amyloidosis (AL) is a disease of protean manifestations due to a wide spectrum of organs that can be affected. The disorder is caused by the deposition of an extracellular amorphous material, the amyloid, which is produced by malignant plasma cells. The latter usually reside in the bone marrow; plasma cell infiltration is often low, in sharp contrast to what we observe in multiple myeloma. The disease may run below the physician's radar for a while before clinical suspicion is raised and targeted tests are performed. In this short review, we try to answer most of the questions that

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… A few years later, in 1927, Divry and Florkin, at the University of Liege, reported on the birefringence of the Congo red-stained amyloid under polarized light; in this setting, red turns into a characteristic apple green that is hard to miss [ 7 ]. …

Systemic AL amyloidosis: current approach and future direction.

Systemic Light chain (AL) amyloidosis is a monoclonal plasma cell proliferative disorder characterized by deposition of amyloidogenic monoclonal light chain fragments causing organ dysfunction. It is a fatal disease and if not diagnosed and treated early can lead to organ failure and potentially death. The renal system along with the cardiovascular system are the most common organs involved but other organs such as gut and liver can be involved as well. The initial evaluation of patients requires confirming the diagnosis with tissue biopsy and staining with Congo red followed by confirmatory t

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… Upon Congo Red staining of the tissue specimen, the sample should demonstrate green birefringence fibrils on a polarized microscope. Additional typing can be done using immunochemistry or mass spectrometry [ 4 ]. …

State-of-the-Art Management of Complications of Myeloma and Its Treatment.

Multiple myeloma is an incurable disease, although patient survival has increased with the availability of novel agents. Both multiple myeloma and its therapies often affect the renal, immune, skeletal, hematologic, and nervous systems. The resulting organ dysfunctions often impair the quality of life of affected patients, complicate and limit subsequent therapies, and may result in significant mortality. Research on the treatment of complications of multiple myeloma has been limited; hence, preventative and management strategies for patients with these complications are heterogeneous and ofte

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… The diagnosis of amyloidosis requires the demonstration of apple green birefringence on Congo red staining of involved tissue. Immunostain for kappa or lambda light chain and electron microscopy are important adjunctive confirmatory tests. …

State of the Art of Cardiac Amyloidosis.

Cardiac amyloidosis is defined by extracellular deposition of misfolded proteins in the heart. The most frequent cases of cardiac amyloidosis are caused by transthyretin and light chain amyloidosis. This condition is underdiagnosed, and its incidence has been continuously rising in recent studies because of the aging of the population and the development of noninvasive multimodal diagnostic tools. Amyloid infiltration affects all cardiac tunics and causes heart failure with preserved ejection fraction, aortic stenosis, arrythmia, and conductive disorder. Innovative, specific therapeutic approa

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… The confirmation test is the appearance of characteristic birefringence under cross polarized light when the amyloid deposits look apple green. The experts highlight that the term “amyloid fibril” should be used for any cross β-sheet fibril [ 38 , 39 ]. …

A bibliometric analysis of light chain amyloidosis from 2005 to 2024: research trends and hot spots.

BackgroundLight chain (AL) amyloidosis stands as the most prevalent subtype of systemic amyloidosis, encompassing a group of rare diseases. Here, we evaluated the scientific landscape of AL amyloidosis to investigate research trends and identify hotspots within the field.MethodsRelevant studies on AL amyloidosis published over the past two decades were retrieved from the Web of Science Core Collection. The publications between 2005 and 2024 were subjected to bibliometric analyses, leveraging tools including CiteSpace, VOSviewer, RStudio and MS Excel to analyse and visualize the annual publicat

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… Cardiac amyloidosis Cardiac amyloidosis is characterized by the extracellular deposition of misfolded proteins known as amyloid fibrils within the myocardium, typically exhibiting Congo red positive staining and green birefringence when viewed under cross polarized light microscopy. …

When and how do patients with cardiac amyloidosis die?

BackgroundCardiac amyloidosis (CA) is an underappreciated cause of morbidity and mortality. Light-chain (AL) and transthyretin (ATTR) amyloidosis have different disease trajectories. No data are available on subtype-specific modes of death (MOD) in patients with CA.Methods and resultsWe retrospectively investigated 66 with AL and 48 with wild-type ATTR amyloidosis (ATTRwt) from 2000 to 2018. ATTRwt differed from AL by age (74.6 ± 5.4 years vs. 63 ± 10.8 years), posterior wall thickness (16.8 ± 3.3 mm vs. 14.3 ± 2.2 mm), left ventricular mass index (180.7 ± 63.2 g/m2 vs. 133.5 ± 42.2 g/m2), and

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… Diagnostic definition Diagnosis of systemic amyloidosis was defined by histological documentation of Congo Red staining and apple-green birefringence under cross-polarized light in at least one involved organ. …

Multidisciplinary supportive care in systemic light chain amyloidosis.

The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidat

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… Fig. 1 Perivascular amyloid deposits demonstrated as apple green birefringence under polarized light in this Congo Red stain specimen from a patient with systemic AL amyloidosis due to lymphoplasmacytic lymphoma (Bone marrow Congo Red, ×20). …

Tubulin binding potentially clears up Bortezomib and Carfilzomib differential neurotoxic effect.

Proteasome inhibitors (PIs) represent the gold standard in the treatment of multiple myeloma. Among PIs, Bortezomib (BTZ) is frequently used as first line therapy, but peripheral neuropathy (PN), occurring approximately in 50% of patients, impairs their life, representing a dose-limiting toxicity. Carfilzomib (CFZ), a second-generation PI, induces a significantly less severe PN. We investigated possible BTZ and CFZ off-targets able to explain their different neurotoxicity profiles. In order to identify the possible PIs off-targets we used the SPILLO-PBSS software that performs a structure-base

Systemic AL Amyloidosis: Current Approaches to Diagnosis and Management.

AL amyloidosis is characterized by a low-level expansion of an indolent, small plasma cell clone that produces amyloidogenic light chains. Amyloid aggregates or preceding intermediaries cause direct cell damage through their proteotoxicity, and amyloid deposits distort tissue architecture, and, eventually, lead to organ impairment. It is a rare, underdiagnosed disease with a diverse clinical presentation depending on the organ tropism of the amyloid fibrils; cardiac and renal involvement is most common, but any organ can be affected, excluding the central nervous system. A high level of awaren

Inflammation in Cardiomyopathies: Cellular Mechanisms Across Cardiac Phenotype.

Cardiomyopathies are traditionally classified by structural and genetic phenotypes, but emerging evidence highlights chronic myocardial inflammation as a pivotal driver of disease progression across different etiologies. This review synthesizes the current literature on the cellular and molecular inflammatory mechanisms underlying hypertrophic cardiomyopathy, Anderson-Fabry disease, cardiac amyloidosis, arrhythmogenic cardiomyopathy, and dilated cardiomyopathy. Across these distinct conditions, endogenous triggers such as metabolic substrates, misfolded amyloid fibrils, mechanical stress, or v

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… Histologically, amyloidosis is defined by extracellular Congo red-positive deposits showing apple-green birefringence under polarized light, but the identification of the amyloid precursor is essential for therapeutic decision-making. …

Cardiac Amyloidosis: A Narrative Review of Diagnostic Advances and Emerging Therapies.

Background/Objectives: Cardiac amyloidosis (CA) is an underdiagnosed and potentially life-threatening infiltrative cardiomyopathy characterized by the extracellular deposition of misfolded amyloid fibrils in cardiac tissue. It is most commonly associated with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, either hereditary or wild-type. The disease often presents with non-specific symptoms, leading to delayed diagnosis and treatment. This study aims to provide a comprehensive overview of the pathophysiology, diagnostic strategies, and current therapeutic approaches for card

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… CA is characterized by the extracellular buildup of misfolded proteins within the heart, marked by a unique histological feature: green birefringence under cross-polarized light following Congo red staining. …