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Journal of Magnetic Resonance Imaging :... Apr 2018Although cardiac MR and T mapping are increasingly used to diagnose diffuse fibrosis based cardiac diseases, studies reporting T values in healthy and diseased... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Although cardiac MR and T mapping are increasingly used to diagnose diffuse fibrosis based cardiac diseases, studies reporting T values in healthy and diseased myocardium, particular in nonischemic cardiomyopathies (NICM) and populations with increased cardiovascular risk, seem contradictory.
PURPOSE
To determine the range of native myocardial T value ranges in patients with NICM and populations with increased cardiovascular risk.
STUDY TYPE
Systemic review and meta-analysis.
POPULATION
Patients with NICM, including hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM), and patients with myocarditis (MC), iron overload, amyloidosis, Fabry disease, and populations with hypertension (HT), diabetes mellitus (DM), and obesity. FIELD STRENGTH/SEQUENCE: (Shortened) modified Look-Locker inversion-recovery MR sequence at 1.5 or 3T.
ASSESSMENT
PubMed and Embase were searched following the PRISMA guidelines.
STATISTICAL TESTS
The summary of standard mean difference (SMD) between the diseased and a healthy control populations was generated using a random-effects model in combination with meta-regression analysis.
RESULTS
The SMD for HCM, DCM, and MC patients were significantly increased (1.41, 1.48, and 1.96, respectively, P < 0.01) compared with healthy controls. The SMD for HT patients with and without left-ventricle hypertrophy (LVH) together was significantly increased (0.19, P = 0.04), while for HT patients without LVH the SMD was zero (0.03, P = 0.52). The number of studies on amyloidosis, iron overload, Fabry disease, and HT patients with LVH did not meet the requirement to perform a meta-analysis. However, most studies reported a significantly increased T for amyloidosis and HT patients with LVH and a significant decreased T for iron overload and Fabry disease patients.
DATA CONCLUSIONS
Native T mapping by using an (Sh)MOLLI sequence can potentially assess myocardial changes in HCM, DCM, MC, iron overload, amyloidosis, and Fabry disease compared to controls. In addition, it can help to diagnose left-ventricular remodeling in HT patients.
LEVEL OF EVIDENCE
2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:891-912.
Topics: Cardiomyopathies; Cardiovascular Diseases; Heart; Humans; Magnetic Resonance Imaging; Myocardium; Reference Values; Risk Factors
PubMed: 29131444
DOI: 10.1002/jmri.25885 -
World Neurosurgery Aug 2020Solitary spinal amyloidoma (SSA) is a rare and poorly characterized disease. There are few cases described, and the knowledge of this neoplasm is limited. A more...
BACKGROUND
Solitary spinal amyloidoma (SSA) is a rare and poorly characterized disease. There are few cases described, and the knowledge of this neoplasm is limited. A more accurate description of demographics, clinical findings, and outcomes may be useful for a better understanding of this pathology, as well as therapeutic intervention, adding value to the research of localized amyloidosis.
METHODS
A systematic search was carried out from when registries began until February 2020. We also include a case diagnosed and treated in our department. Descriptive statistics were used to evaluate data, demographics, clinical findings, diagnostic modalities, therapeutics, and finally neurologic outcomes. The Kaplan-Meier method was used to assess overall survival and progression-free survival.
RESULTS
The final cohort comprises 35 patients. The mean age at diagnosis was 61.97 years, and 68.60% of the patients were male. SSA developed more frequently in the thoracic spine (48.60%), followed by the cervical spine (17.10%). Intradural lesions were rare, and the average neoplastic score for spinal instability was 9.5 points. The most common symptoms were impaired motor function (74.29%) and axial back pain (65.70%). After surgery, neurologic recovery was reported in 82.90% of cases. Mean progression-free survival and mean overall survival were 47.26 and 156.66 months.
CONCLUSIONS
SSA is a rare subgroup of localized amyloidosis, usually being diagnosed in male patients between the sixth and eighth decades. The gold standard treatment seems to be surgical resection. SSA patients have excellent long-term survival and a low rate of local recurrence.
Topics: Amyloidosis; Female; Humans; Male; Spinal Diseases; Spine; Treatment Outcome
PubMed: 32461181
DOI: 10.1016/j.wneu.2020.05.159 -
Journal of Lower Genital Tract Disease Jul 2019The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically.
OBJECTIVES
The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically.
METHODS
A database search of PubMed and Google Scholar were extracted between March 1, 2009, and March 1, 2019, using the search terms "foreskin," "prepuce," "penis," "pathology," "dermatology," and "rare." The search was limited to "humans" and "dermatopathology." Full article texts were reviewed. Reference lists were screened for additional articles. Patient details (diagnosis, dermatopathology, treatment, and follow-up if available) were extracted. We excluded articles written in the non-English language, unusual variants of common conditions, and cases of common dermatologic conditions.
RESULTS
A list of 369 articles was identified and another screening identified 30 articles for rare foreskin pathologies. Those are divided into categories based on the following etiologies: (a) benign, including congenital (e.g., aposthia), infectious (graft versus host disease and histoplasma), autoimmune (Crohn's disease and pyoderma gangrenosum), and benign neoplasms (neurofibroma, apocrine hidrocystoma, verruciform xanthoma, porokeratosis, penile cutaneous horn, localized amyloidosis) and (b) malignancies, including primary (myeloid sarcoma, basal cell carcinoma, Kaposi's sarcoma, mucosal-associated lymphoid tissue lymphoma), and metastasis.
CONCLUSIONS
We reviewed and discussed unusual benign and malignant dermatopathology conditions that can affect the foreskin.
Topics: Adult; Aged; Autoimmune Diseases; Child; Child, Preschool; Dermatitis; Foreskin; Humans; Male; Middle Aged; Neoplasms; Penile Neoplasms
PubMed: 31149956
DOI: 10.1097/LGT.0000000000000478 -
Journal of Alzheimer's Disease : JAD 2023The negative effects of periodontitis on systemic diseases, including diabetes, cardiovascular diseases, and Alzheimer's disease (AD), have been widely described.
BACKGROUND
The negative effects of periodontitis on systemic diseases, including diabetes, cardiovascular diseases, and Alzheimer's disease (AD), have been widely described.
OBJECTIVE
This systematic review aimed to gather the current understanding of the pathophysiological mechanisms linking periodontitis to AD.
METHODS
An electronic systematic search of the PubMed/MEDLINE, Scopus, and Embase databases was performed using the following PECO question: How can periodontitis or periodontal bacteria influence Alzheimer's disease features?". Only preclinical studies exploring the biological links between periodontitis and AD pathology were included. This study was registered at the International Prospective Register of Systematic Reviews (PROSPERO), and the Syrcle and Camarades protocols were used to assess the risk of bias.
RESULTS
After a systematic screening of titles and abstracts (n = 3,307), thirty-six titles were selected for abstract reading, of which 13 were excluded (k = 1), resulting in the inclusion of 23 articles. Oral or systemic exposure to periodontopathogens or their byproducts is responsible for both in situ brain manifestations and systemic effects. Significant elevated rates of cytokines and amyloid peptides (Aβ) and derivate products were found in both serum and brain. Additionally, in infected animals, hyperphosphorylation of tau protein, hippocampal microgliosis, and neuronal death were observed. Exposure to periodontal infection negatively impairs cognitive behavior, leading to memory decline.
CONCLUSIONS
Systemic inflammation and brain metastatic infections induced by periodontal pathogens contribute to neuroinflammation, amyloidosis, and tau phosphorylation, leading to brain damage and subsequent cognitive impairment.
Topics: Animals; Alzheimer Disease; Systematic Reviews as Topic; Periodontitis; Inflammation; Cognitive Dysfunction
PubMed: 37927257
DOI: 10.3233/JAD-230478 -
Journal of Neurology, Neurosurgery, and... Mar 2012The aim of this study was to determine the strength of the association between intracerebral haemorrhage (ICH) and cerebral amyloid angiopathy (CAA) in a systematic... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The aim of this study was to determine the strength of the association between intracerebral haemorrhage (ICH) and cerebral amyloid angiopathy (CAA) in a systematic review of published neuropathological studies.
METHODS
In April 2011, Ovid Medline (from 1950) and Embase (from 1980) were searched for neuropathological studies that quantified the prevalence of CAA in patients with ICH and in a control group without ICH. Two authors extracted data from each study and meta-analysed their results using a random effects model.
RESULTS
10 neuropathological cross sectional or case control studies were identified, involving 481 cases with ICH and 3219 controls. There was no association between CAA and ICH in any location (OR 1.21, 95% CI 0.87 to 1.68; 10 studies, I(2) 29%), deep ICH (OR 0.81, 95% CI 0.30 to 2.19; five studies, I(2) 58%) or cerebellar ICH (OR 2.05, 95% CI 0.55 to 7.63; four studies, I(2) 0%). CAA was significantly associated with lobar ICH, both overall (OR 2.21, 95% CI 1.09 to 4.45; six studies, I(2) 40%) and in the three studies where average ages for cases and controls were comparable (OR 3.24, 95% CI 1.02 to 10.26).
CONCLUSIONS
There is an association between CAA and lobar ICH, although the association might be stronger if potential confounding factors, distinctive clinical and imaging features of ICH due to CAA and CAA neuropathological severity are taken into account.
Topics: Brain; Cerebral Amyloid Angiopathy; Cerebral Hemorrhage; Humans
PubMed: 22056966
DOI: 10.1136/jnnp-2011-300371 -
BioMed Research International 2015This paper aims to review the morphological and functional characteristics of patients affected by familial amyloid polyneuropathy (FAP), with greater focus on type I... (Review)
Review
PURPOSE
This paper aims to review the morphological and functional characteristics of patients affected by familial amyloid polyneuropathy (FAP), with greater focus on type I and its progression after liver transplantation. We also analyse therapeutic options for the ophthalmic manifestations.
METHODS
The literature from 2002 through 2015 was reviewed, with a total of 45 articles studied, using the key terms related to amyloidosis and its therapeutic approaches. Information was collated, evaluated, critically assessed, and then summarised in its present form. PATHOPHYSIOLOGY AND TREATMENT: FAP results from mutation of the transthyretin gene, with Val30Met being the most frequent substitution. The symptoms are those typical of a sensorimotor autonomic neuropathy and can be halted with liver transplantation. Nowadays there are new medical therapies that delay the progression of the systemic neuropathy. However, there are still no options to avoid ocular disease.
CONCLUSION
The main ocular manifestations in patients with FAP type I are amyloid deposition in the vitreous, dry eye, and secondary glaucoma. Despite liver transplantation, eye synthesis of amyloid persists and is associated with progressive ocular manifestations, which require continued ophthalmologic follow-up. New therapeutic strategies are therefore needed, particularly to target the ocular synthesis of the abnormal protein.
Topics: Adult; Amyloid Neuropathies, Familial; Female; Humans; Iris; Male; Middle Aged
PubMed: 26558262
DOI: 10.1155/2015/282405 -
Journal of Cutaneous Pathology May 2024Macular amyloidosis (MA) is a primary localized cutaneous amyloidosis, characterized by amyloid deposition in the papillary dermis. The clinical presentation includes... (Review)
Review
Macular amyloidosis (MA) is a primary localized cutaneous amyloidosis, characterized by amyloid deposition in the papillary dermis. The clinical presentation includes pruritic hyperpigmented macules and patches with a reticulated or rippled pattern, primarily found on the upper back and extremities. Biopsy is an essential diagnostic tool for confirming MA. This systematic review focused on the biopsy outcomes in patients diagnosed with MA.
Topics: Humans; Amyloidosis; Skin; Amyloidosis, Familial; Biopsy; Skin Diseases; Skin Diseases, Genetic
PubMed: 38328942
DOI: 10.1111/cup.14592 -
Journal de Mycologie Medicale Dec 2019Secondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly...
Secondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly affected organ and manifest as nephrotic syndrome with or without renal failure. Chronic pulmonary aspergillosis (CPA) is a chronic infection of lung parenchyma affecting those with an underlying structural lung disease. Herein, we present a case of CPA where the initial manifestation was that of nephrotic syndrome due to renal amyloidosis. We also perform a systematic review for studies describing secondary amyloidosis due to CPA.
Topics: Adult; Amyloidosis; Chronic Disease; Female; Humans; Kidney; Nephrotic Syndrome; Pulmonary Aspergillosis
PubMed: 31570305
DOI: 10.1016/j.mycmed.2019.100898 -
Hematology/oncology and Stem Cell... Jun 2019Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The...
OBJECTIVE/BACKGROUND
Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.
METHODS
An electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.
RESULTS
We identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53-66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.
CONCLUSIONS
We reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.
Topics: Adult; Female; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Multiple Myeloma; Neoplasm Proteins; Sex Factors; Skin Neoplasms
PubMed: 30261180
DOI: 10.1016/j.hemonc.2018.09.003 -
JACC. Cardiovascular Imaging May 2024Computed tomography (CT)-derived extracellular volume fraction (ECV) is a noninvasive method to quantify myocardial fibrosis. Although studies suggest CT is a suitable... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Computed tomography (CT)-derived extracellular volume fraction (ECV) is a noninvasive method to quantify myocardial fibrosis. Although studies suggest CT is a suitable measure of ECV, clinical use remains limited.
OBJECTIVES
A meta-analysis was performed to determine the clinical value of CT-derived ECV in cardiovascular diseases.
METHODS
Electronic database searches of PubMed, Web of Science Core Collection, Cochrane advanced search, and EMBASE were performed. The most pivotal analysis entailed the comparison of ECV ascertained through CT-ECV among the control, aortic stenosis, and cardiac amyloidosis cohorts. The diagnostic test accuracy for detecting cardiac amyloidosis was assessed using summary receiver-operating characteristics curve.
RESULTS
Pooled CT-derived ECV values were 28.5% (95% CI: 27.3%-29.7%) in the control, 31.9% (95% CI: 30.2%-33.8%) in the aortic stenosis, and 48.9% (95% CI: 44.5%-53.3%) in the cardiac amyloidosis group. ECV was significantly elevated in aortic stenosis (P = 0.002) (vs controls) but further elevated in cardiac amyloidosis (P < 0.001) (vs aortic stenosis). CT-derived ECV had a high diagnostic accuracy for cardiac amyloidosis, with sensitivity of 92.8% (95% CI: 86.7%-96.2%), specificity of 84.8% (95% CI: 68.6%-93.4%), and area under the summary receiver-operating characteristic curve of 0.94 (95% CI: 0.88-1.00).
CONCLUSIONS
This study is the first comprehensive systematic review and meta-analysis of CT-derived ECV evaluation in cardiac disease. The high diagnostic accuracy of CT-ECV suggests the usefulness of CT-ECV in the diagnosis of cardiac amyloidosis in preoperative CT planning for transcatheter aortic valve replacement.
Topics: Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Amyloidosis; Aortic Valve Stenosis; Cardiomyopathies; Fibrosis; Myocardium; Predictive Value of Tests; Prognosis; Reproducibility of Results; Tomography, X-Ray Computed
PubMed: 37999657
DOI: 10.1016/j.jcmg.2023.10.008