scholarly journals Amyloidosis of the Lung

2017 ◽  
Vol 141 (2) ◽  
pp. 247-254 ◽  
Author(s):  
Andras Khoor ◽  
Thomas V. Colby
Keyword(s):  
Amyloid Fibrils ◽  
Clinical Investigation ◽  
Treatment Options ◽  
Al Amyloidosis ◽  
Age Related ◽  
Amyloid Polyneuropathy ◽  
Pulmonary Amyloidosis ◽  
The Relationship ◽  
Hereditary Attr Amyloidosis ◽  
Tracheobronchial Amyloidosis

Context.—Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy). Three different clinicopathologic forms of amyloidosis can be seen in the lungs: diffuse alveolar-septal amyloidosis, nodular pulmonary amyloidosis, and tracheobronchial amyloidosis. Objective.—To clarify the relationship between the fibril protein–based amyloidosis classification system and the clinicopathologic forms of pulmonary amyloidosis and to provide a useful guide for diagnosing these entities for the practicing pathologist. Data Sources.—This is a narrative review based on PubMed searches and the authors' own experiences. Conclusions.—Diffuse alveolar-septal amyloidosis is usually caused by systemic AL amyloidosis, whereas nodular pulmonary amyloidosis and tracheobronchial amyloidosis usually represent localized AL amyloidosis. However, these generalized scenarios cannot always be applied to individual cases. Because the treatment options for amyloidosis are dependent on the fibril protein–based classifications and whether the process is systemic or localized, the workup of new clinically relevant cases should include amyloid subtyping (preferably with mass spectrometry–based proteomic analysis) and further clinical investigation.

scholarly journals The lung in amyloidosis

2017 ◽  
Vol 26 (145) ◽  
pp. 170046 ◽  
Author(s):  
Paolo Milani ◽  
Marco Basset ◽  
Francesca Russo ◽  
Andrea Foli ◽  
Giovanni Palladini ◽  
...  
Keyword(s):  
Treatment Options ◽  
Accurate Determination ◽  
Systemic Amyloidosis ◽  
External Beam Radiation ◽  
Beam Radiation ◽  
Respiratory Impairment ◽  
Clonal Proliferation ◽  
Amyloid Deposits ◽  
Pulmonary Amyloidosis ◽  
Tracheobronchial Amyloidosis

Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas. Finally, tracheobronchial amyloidosis, which is usually not associated with evident clonal proliferation, may result in airway stenosis.Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis. Nodular pulmonary amyloidosis is usually localised, conservative excision is usually curative and the long-term prognosis is excellent. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.

scholarly journals Short tutorial. Treatment options in light chain amyloidosis and monoclonal gammopathy of renal significance

2021 ◽  
Author(s):  
Maria T. Krauth ◽  
Hermine Agis
Keyword(s):  
B Cell ◽  
Cell Population ◽  
Monoclonal Gammopathy ◽  
Amyloid Fibrils ◽  
Treatment Options ◽  
Organ Damage ◽  
Additional Treatment ◽  
Treatment Modalities ◽  
Al Amyloidosis ◽  
Low Grade

SummaryAL amyloidosis (AL) and monoclonal gammopathy of renal significance (MGRS) are both paraprotein-associated diseases. Both entities are based on a monoclonal paraprotein produced by a clonal plasma-cell population like in monoclonal gammopathy of undetermined significance (MGUS) or by a B-cell population like in low grade lymphoma. Per definition MGUS and low-grade lymphoma do not require treatment. But in rare cases the monoclonal M‑gradient acts as a “toxic” protein inducing severe multimodal organ damage as in AL and MGRS. Urgent treatment is indicated in AL and in MGRS to avoid irreparable loss of organ function or death. No treatment is currently approved in Europe for AL or MGRS. On January 15, 2021 the US Food and Drug Administration approved the monoclonal anti-CD38 antibody for treatment of AL. To minimize the serum M‑gradient concentration, a clone directed therapy as in multiple myeloma or B‑cell malignancies treatment regimens can be applied. In AL, an additional treatment option is under investigation. These special drugs are directed against the typical amyloid-fibrils responsible for deposition formation. An additional and important consideration in this special field of rare diseases is the option of organ transplantation in particular kidney transplantation in MGRS. All these treatment modalities are addressed in this article.

scholarly journals Autologous Stem Cell Transplant for AL Amyloidosis

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Vivek Roy
Keyword(s):  
Stem Cell ◽  
Amyloid Fibrils ◽  
Stem Cell Transplant ◽  
Plasma Cells ◽  
Cell Clone ◽  
Treatment Options ◽  
Proteasome Inhibitors ◽  
Autologous Stem Cell Transplant ◽  
Al Amyloidosis ◽  
Cell Transplant

AL amyloidosis is caused by clonal plasma cells that produce immunoglobulin light chains which misfold and get deposited as amyloid fibrils. Therapy directed against the plasma cell clone leads to clinical benefit. Melphalan and corticosteroids have been the mainstay of treatment for a number of years and the recent availability of other effective agents (IMiDs and proteasome inhibitors) has increased treatment options. Autologous stem cell transplant (ASCT) has been used in the treatment of AL amyloidosis for many years. It is associated with high rates of hematologic response and improvement in organ function. However, transplant carries considerable risks. Careful patient selection is important to minimize transplant related morbidity and mortality and ensure optimal patient outcomes. As newer more affective therapies become available the role and timing of ASCT in the overall treatment strategy of AL amyloidosis will need to be continually reassessed.

Establishing a Group Educational Session for Hyperacusis Patients

2019 ◽  
Vol 28 (2) ◽  
pp. 245-250
Author(s):  
Ann E. Perreau ◽  
Richard S. Tyler ◽  
Patricia C. Mancini ◽  
Shelley Witt ◽  
Mohamed Salah Elgandy
Keyword(s):  
Therapeutic Process ◽  
Treatment Options ◽  
Clinical Group ◽  
Education Session ◽  
Group Approach ◽  
Treatment Protocols ◽  
Educational Session ◽  
Evidence Based Treatment ◽  
Relationship Of ◽  
The Relationship

Purpose Audiologists should be treating hyperacusis patients. However, it can be difficult to know where to begin because treatment protocols and evidence-based treatment studies are lacking. A good place to start in any tinnitus and hyperacusis clinic is to incorporate a group educational session. Method Here, we outline our approach to establishing a hyperacusis group educational session that includes specific aspects of getting to know each patient to best meet their needs, understanding the problems associated with hyperacusis, explaining the auditory system and the relationship of hyperacusis to hearing loss and tinnitus, describing the influence of hyperacusis on daily life, and introducing treatment options. Subjective responses from 11 adults with hyperacusis, who participated in a recent clinical group education session, were discussed to illustrate examples from actual patients. Conclusions Due to the devastating nature of hyperacusis, patients need to be reassured that they are not alone and that they can rely on audiologists to provide support and guidance. A group approach can facilitate the therapeutic process by connecting patients with others who are also affected by hyperacusis, and by educating patients and significant others on hyperacusis and its treatment options. Supplemental Material https://doi.org/10.23641/asha.8121197

Cognitive Factors Mediate the Relation Between Age and Flight Path Maintenance in General Aviation

2016 ◽  
Vol 6 (2) ◽  
pp. 81-90 ◽  
Author(s):  
Kathleen Van Benthem ◽  
Chris M. Herdman
Keyword(s):  
Cognitive Functioning ◽  
Flight Path ◽  
General Aviation ◽  
Cognitive Factors ◽  
Pilot Performance ◽  
Pilot Error ◽  
Age Related ◽  
And Performance ◽  
Relationship Of ◽  
The Relationship

Abstract. Identifying pilot attributes associated with risk is important, especially in general aviation where pilot error is implicated in most accidents. This research examined the relationship of pilot age, expertise, and cognitive functioning to deviations from an ideal circuit trajectory. In all, 54 pilots, of varying age, flew a Cessna 172 simulator. Cognitive measures were obtained using the CogScreen-AE ( Kay, 1995 ). Older age and lower levels of expertise and cognitive functioning were associated with significantly greater flight path deviations. The relationship between age and performance was fully mediated by a cluster of cognitive factors: speed and working memory, visual attention, and cognitive flexibility. These findings add to the literature showing that age-related changes in cognition may impact pilot performance.

The Association Between Cardio-Ankle Vascular Index and Primary Idiopathic Complete Atrioventricular Block in an Elderly Population

Angiology ◽  
2021 ◽  
pp. 000331972110287
Author(s):  
Turhan Turan ◽  
Faruk Kara ◽  
Selim Kul ◽  
Muhammet Rasit Sayın ◽  
Sinan Sahin ◽  
...  
Keyword(s):  
Arterial Stiffness ◽  
Atrioventricular Block ◽  
Elderly Population ◽  
Complete Atrioventricular Block ◽  
Age Related ◽  
And Gender ◽  
The Relationship ◽  
Block Frequency ◽  
Complete Atrioventricular ◽  
Relevant Factors

The most common cause of complete atrioventricular block (CAVB) is age-related fibrotic degeneration and is referred to as primary idiopathic complete atrioventricular block (iCAVB). This study aims to investigate the relationship between iCAVB and arterial stiffness using the cardio-ankle vascular index (CAVI). In this study, of 205 CAVB patients, 41 patients with iCAVB implanted with a dual-chamber permanent pacemaker and 40 age- and gender-matched controls were studied. Arterial stiffness was assessed by a VaSera VS-1000 CAVI instrument. The CAVI values of patients with iCAVB were significantly higher compared with the controls (9.63 ± 1.42 vs 8.57 ± 1.12, P < .001). Idiopathic complete atrioventricular block frequency was higher among patients with abnormal CAVI values than those with borderline and normal CAVI ( P = .04). In multivariate analysis, only CAVI was an independent predictor of iCAVB after adjusting for other relevant factors (odds ratio, 2.575; 95% CI [1.390-4.770]; P = .003). The present study demonstrated that CAVI, as a marker of arterial stiffness, was increased among elderly patients with iCAVB. Thus, we provide a possible additional mechanism linking easily measured CAVI with iCAVB.

scholarly journals Plasma angiopoietin-2 is associated with age-related deficits in cognitive sub-scales in Ugandan children following severe malaria

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Benson J. Ouma ◽  
Paul Bangirana ◽  
John M. Ssenkusu ◽  
Dibyadyuti Datta ◽  
Robert O. Opoka ◽  
...  
Keyword(s):  
Severe Malaria ◽  
Parental Education ◽  
Preschool Education ◽  
Kidney Injury ◽  
Malaria Episode ◽  
Enzyme Linked Immunosorbent Assay ◽  
Testing Time ◽  
Age Related ◽  
Angiopoietin 2 ◽  
The Relationship

Abstract Background Elevated angiopoietin-2 (Angpt-2) concentrations are associated with worse overall neurocognitive function in severe malaria survivors, but the specific domains affected have not been elucidated. Methods Ugandan children with severe malaria underwent neurocognitive evaluation a week after hospital discharge and at 6, 12 and 24 months follow-up. The relationship between Angpt-2 concentrations and age-adjusted, cognitive sub-scale z-scores over time were evaluated using linear mixed effects models, adjusting for disease severity (coma, acute kidney injury, number of seizures in hospital) and sociodemographic factors (age, gender, height-for-age z-score, socio-economic status, enrichment in the home environment, parental education, and any preschool education of the child). The Mullen Scales of Early Learning was used in children < 5 years and the Kaufman Assessment Battery for Children 2nd edition was used in children ≥ 5 years of age. Angpt-2 levels were measured on admission plasma samples by enzyme-linked immunosorbent assay. Adjustment for multiple comparisons was conducted using the Benjamini–Hochberg Procedure of False Discovery Rate. Results Increased admission Angpt-2 concentration was associated with worse outcomes in all domains (fine and gross motor, visual reception, receptive and expressive language) in children < 5 years of age at the time of severe malaria episode, and worse simultaneous processing and learning in children < 5 years of age at the time of severe malaria who were tested when ≥ 5 years of age. No association was seen between Angpt-2 levels and cognitive outcomes in children ≥ 5 years at the time of severe malaria episode, but numbers of children and testing time points were lower for children ≥ 5 years at the time of severe malaria episode. Conclusion Elevated Angpt-2 concentration in children with severe malaria is associated with worse outcomes in multiple neurocognitive domains. The relationship between Angpt-2 and worse cognition is evident in children < 5 years of age at the time of severe malaria presentation and in selected domains in older years.

scholarly journals A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis

2021 ◽  
Vol 2021 (2) ◽  
Author(s):  
Feihong Ding ◽  
Yun Li ◽  
Shailesh Balasubramanian ◽  
Subha Ghosh ◽  
Jason N Valent ◽  
...  
Keyword(s):  
Light Chain ◽  
Systemic Disease ◽  
Amyloid Deposition ◽  
Misfolded Proteins ◽  
Immunoglobulin Light Chain ◽  
Anatomic Site ◽  
Unique Case ◽  
Pulmonary Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Tracheobronchial Amyloidosis

ABSTRACT Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.

scholarly journals Advances in Targeting Cutaneous Melanoma

Cancers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 2090
Author(s):  
Dimitri Kasakovski ◽  
Marina Skrygan ◽  
Thilo Gambichler ◽  
Laura Susok
Keyword(s):  
Cutaneous Melanoma ◽  
Clinical Investigation ◽  
Early Stage ◽  
Treatment Options ◽  
Treatment Resistance ◽  
Immune Checkpoint Blockade ◽  
Oncolytic Viruses ◽  
Cancer Site ◽  
Western World ◽  
Pharmacological Agents

To date, the skin remains the most common cancer site among Caucasians in the western world. The complex, layered structure of human skin harbors a heterogenous population of specialized cells. Each cell type residing in the skin potentially gives rise to a variety of cancers, including non-melanoma skin cancer, sarcoma, and cutaneous melanoma. Cutaneous melanoma is known to exacerbate and metastasize if not detected at an early stage, with mutant melanomas tending to acquire treatment resistance over time. The intricacy of melanoma thus necessitates diverse and patient-centered targeted treatment options. In addition to classical treatment through surgical intervention and radio- or chemotherapy, several systemic and intratumoral immunomodulators, pharmacological agents (e.g., targeted therapies), and oncolytic viruses are trialed or have been recently approved. Moreover, utilizing combinations of immune checkpoint blockade with targeted, oncolytic, or anti-angiogenic approaches for patients with advanced disease progression are promising approaches currently under pre-clinical and clinical investigation. In this review, we summarize the current ‘state-of-the-art’ as well as discuss emerging agents and regimens in cutaneous melanoma treatment.

Patient-Physician Concordance for Quantitative Formats and Treatment Options and the Relationship with State Anxiety

2021 ◽  
Author(s):  
Alicia J. Smallwood ◽  
Joan M. Neuner ◽  
Kathlyn E. Fletcher ◽  
Cindy M. Walker ◽  
Marilyn M. Schapira
Keyword(s):  
State Anxiety ◽  
Treatment Options ◽  
The Relationship
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