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Frontiers in Endocrinology 2022It is currently controversial whether subclinical hyperthyroidism is associated with gene variants. We describe a man with subclinical hyperthyroidism and a gene...
BACKGROUND AND OBJECTIVES
It is currently controversial whether subclinical hyperthyroidism is associated with gene variants. We describe a man with subclinical hyperthyroidism and a gene variant who was diagnosed with Carney complex (CNC), and we performed a systematic review of published studies to assess the association between gene variants and the risk of subclinical hyperthyroidism.
DESIGN AND METHODS
The PubMed, EMBASE, OVID, Science Direct, and gray literature electronic databases were searched for articles published from January 2002 to May 2021 using predefined keywords and inclusion and exclusion criteria. Data on thyroid function from selected studies were extracted and analyzed.
RESULTS
We identified a CNC patient with a subclinical hyperthyroidism phenotype combined with multiple components and genetic sequenced data. In a subsequent systematic review, twenty selected studies (14 case studies and 6 series studies) enrolling 23 individuals were included in the final analysis. The patient's thyroid function data were qualitative in 11 cases and quantitative in 12 cases. The prevalence of subclinical hyperthyroidism in the CNC patients with a gene variant, including our patient, was markedly higher than that in the normal population (12.5% vs. 2%).
CONCLUSIONS
The findings of this systematic review provide helpful evidence that gene variants and subclinical hyperthyroidism are related and suggest that subclinical hyperthyroidism may be a neglected phenotype of gene variants and a novel component of CNC patients.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/PROSPERO, identifier CRD42021197655.
Topics: Carney Complex; Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; Humans; Hyperthyroidism; Phenotype
PubMed: 36213268
DOI: 10.3389/fendo.2022.951133 -
The Journal of Small Animal Practice May 2022To determine the efficacy and adverse events of the administration of angiotensin--converting enzyme inhibitors for the management of preclinical myxomatous mitral valve... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
To determine the efficacy and adverse events of the administration of angiotensin--converting enzyme inhibitors for the management of preclinical myxomatous mitral valve disease in dogs.
MATERIALS AND METHODS
A compre- hensive search using Pubmed/MEDLINE, LILACS and CAB abstracts databases was performed. Ran- domised clinical trials that assessed efficacy and adverse events of angiotensin-converting enzyme inhibitors for the management of preclinical myxomatous mitral valve disease in dogs were included. Certainty of evidence was rated using GRADE methods.
RESULTS
Four randomised clinical trials were included. While safe, angiotensin-converting enzyme inhibitors administration to dogs with myxomatous mitral valve disease and cardiomegaly results in little to no difference in the risk of development congestive heart failure (high certainty of evidence; relative risk: 1.03; 95% confidence interval: 0.87 to 1.23) and may result in little to no difference in cardiovascular-related (low certainty of evidence; relative risk: 1.01; 95% confidence interval: 0.54 to 1.89) and all-cause mortality (low certainty of evidence; relative risk: 0.93; 95% confidence interval: 0.63 to 1.36). Administration of angiotensin-converting enzyme inhibitors to dogs with myxomatous mitral valve disease without cardiomegaly may result in a reduced risk of congestive heart failure development. However, the range in which the actual effect for this outcome may be, the "margin of error," indicates it might also increase the risk of congestive heart failure development (low certainty of evidence; relative risk: 0.86; 95% confidence interval: 0.54 to 1.35).
CLINICAL SIGNIFICANCE
Administration of angiotensin-converting enzyme inhibitors to dogs with -preclinical myxoma- tous mitral valve disease and cardiomegaly results in little to no difference in the risk of the develop- ment of congestive heart failure and may result in little to no difference in -cardiovascular-related and all-cause mortality. The certainty of evidence of the efficacy of angiotensin-converting enzyme inhibi- tors administration to dogs without cardiomegaly was low.
Topics: Angiotensin-Converting Enzyme Inhibitors; Angiotensins; Animals; Cardiomegaly; Dog Diseases; Dogs; Heart Failure; Mitral Valve
PubMed: 34905219
DOI: 10.1111/jsap.13461 -
Survey of Ophthalmology 2022Conjunctival myxoma is an uncommon benign tumor that may develop as a localized disease or as part of the Carney complex, in which case it can cause significant... (Review)
Review
Conjunctival myxoma is an uncommon benign tumor that may develop as a localized disease or as part of the Carney complex, in which case it can cause significant morbidity and mortality. Conjunctival myxomas can mimic other pure or mixed tumors and are often overlooked in clinical practice. Histopathological studies are essential for diagnosis; however, controversy regarding their pathological and immunohistochemical features makes the diagnostic process more difficult. Because of the importance of correctly classifying these conjunctival tumors, as well as recognizing when a systematic screening is required owing to the possible association with life-threatening diseases, we comprehensively evaluate the literature on this rare entity based on a systematic approach.
Topics: Conjunctival Neoplasms; Humans; Myxoma
PubMed: 34461119
DOI: 10.1016/j.survophthal.2021.08.007 -
International Journal of Clinical... Nov 2021Aortic valve myxoma is the rarest location of the most common primary tumour of cardiac origin. Because of the paucity of data, there is little known about their...
BACKGROUND
Aortic valve myxoma is the rarest location of the most common primary tumour of cardiac origin. Because of the paucity of data, there is little known about their clinical presentation, diagnosis and complications.
METHODS
PUBMED, EMBASE, SCOPUS and WEB OF SCIENCE were systematically searched to identify all published cases of aortic valve myxoma through October 2020. Descriptive statistics were used to report the data.
RESULTS
Aortic valve myxomas were more prevalent in young (mean age 41 years) male (75%) patients. It most commonly involved the right coronary cusp (50%). Cerebrovascular events (25%), dyspnoea (18.8%), and distal embolisation (18.8%) were found to be the most frequent complications. Echocardiography remains the diagnostic modality of choice in all cases, histopathology is used for confirmation. Most cases were treated with surgical excision (94%); concomitant aortic valve repair and mechanical aortic valve replacement were performed in 25% and 37.5% cases respectively. Sudden cardiac death was noted in one patient.
CONCLUSION
Aortic valve myxomas are more often than not discovered in the context of embolic phenomenon or dyspnoea. The most feared complication is stroke, although mortality remains low in surgically managed cases.
Topics: Adult; Aortic Valve; Cardiac Surgical Procedures; Echocardiography; Humans; Male; Myxoma; Stroke
PubMed: 34165869
DOI: 10.1111/ijcp.14566 -
Journal of Clinical Neuroscience :... Dec 2020Neoplastic cerebral aneurysms (NCAs) are highly rare lesions characterized by invasion of cancerous cells within the wall of an artery leading to aneurysm formation....
Neoplastic cerebral aneurysms (NCAs) are highly rare lesions characterized by invasion of cancerous cells within the wall of an artery leading to aneurysm formation. While NCAs caused by myxomas are well characterized in the clinical literature, rarer etiologies have also been reported and are typically associated worse clinical outcomes. We performed the first PRISMA-compliant systematic literature review of true, non-myxoma neoplastic cerebral aneurysms using the PubMed/MEDLINE, Embase, Scopus, and Google Scholar databases. Data of interest included age, sex, aneurysm size, number of aneurysms, aneurysm location, neoplasm type, aneurysm treatments, cancer treatments, risk of rupture, intracerebral hemorrhage prevalence, subarachnoid hemorrhage prevalence, and survival at 90 and 180 days. A total of 50 studies met our inclusion criteria. The mean age of the patient population was 37.4 years (SD: ±16.8) and had an overall female preponderance (39/50, 78%). Of these NCA cases, 29/50 (58.0%) were choriocarcinomas, 10/50 (20.0%) were related to lung cancer, and 11/50 (22.0%) had other origins of variable pathologies. 90-day survival rates were 60.0% (15/25) for choriocarcinomas, 28.6% (2/7) for the lung cancer group, and 14.3% (1/7) for the other origins group. 180-day survival rates were 52.0% (13/25) for the choriocarcinoma group, 14.3% (1/7) for the lung cancer group, and 0% (0/7) for the other origins group. Prognosis of NCA patients ultimately depends on the course of disease progression and cancer management. Further research is needed to better understand optimal treatment modalities for patients with NCAs.
Topics: Adult; Brain Neoplasms; Cerebral Hemorrhage; Choriocarcinoma; Female; Humans; Intracranial Aneurysm; Lung Neoplasms; Male; Myxoma; Pregnancy; Prognosis; Subarachnoid Hemorrhage
PubMed: 33246908
DOI: 10.1016/j.jocn.2020.11.010 -
BMC Cardiovascular Disorders Jul 2020The risk factors contributing to embolism in cardiac myxoma (CM) are yet controversial. This systematic review and meta-analysis aimed to clarify the risk factors of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The risk factors contributing to embolism in cardiac myxoma (CM) are yet controversial. This systematic review and meta-analysis aimed to clarify the risk factors of embolism for the CM patients.
METHODS
PubMed, Embase, Cochrane library, Web of Science, China National Knowledge Infrastructure, Wan Fang, and Wei Pu databases were searched from inception to June 2019. Statistical analysis was conducted using Stata version 14.0. The pooled odds ratio or mean difference with 95% confidence interval was estimated for each risk factor.
RESULTS
Herein, 12 studies, encompassing 1814 patients, were included. The pooled results suggested that New York Heart Association (NYHA) class I/II (P < 0.01), hypertension (P = 0.03), irregular tumor surface (P < 0.01), tumor in atypical location (P = 0.01), narrow base of tumor (P < 0.01), and increased fibrinogen (FIB) (P < 0.01) are significant risk factors of embolism in CM patients. However, sex, age, body mass index, smoking, left ventricular ejection fraction, diabetes, hyperlipidemia, atrial fibrillation, valvular heart disease, coronary heart disease, tumor size, platelet count, white blood cells, and hemoglobin were not associated with embolism (all P > 0.05).
CONCLUSIONS
NYHA class (I/II), hypertension, irregular tumor surface, atypical tumor location, the narrow base of tumor, and increased FIB were significant risk factors of embolism in CM patients. For CM patients with these factors, early surgery might be beneficial to prevent embolism.
Topics: Adult; Aged; Biomarkers; Embolism; Female; Fibrinogen; Heart Neoplasms; Humans; Hypertension; Male; Middle Aged; Myxoma; Prognosis; Risk Assessment; Risk Factors; Up-Regulation
PubMed: 32711463
DOI: 10.1186/s12872-020-01631-w -
International Journal of Hematology Jul 2020The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic...
The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspects must be discussed further in light of recent advances in lymphoma research. We collected cardiac lymphoma case reports published from January 2009 to January 2019, collected statistics from each patient, and performed a systematic analysis. The epidemiological characteristics, clinical manifestations, treatments, responses, and survival of primary cardiac lymphoma (PCL) patients are described. We obtained 158 cases of heart lymphoma, of which 101 were defined as PCL. There were more male than female cases. Most cases were diffuse large B-cell lymphoma. Six cases of PCL in cardiac myxomas were described. Patients with arrhythmia had shorter progression-free survival compared those without (HR 0.334, 95% CI 0.112-0.999, log-rank P = 0.042). Surgery did not improve patients' long-term prognosis or reduce the risk of death within 1 month. These data suggest that central nervous system prophylaxis is necessary. The overall survival was longer than that in data from 1949 to 2009.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Arrhythmias, Cardiac; Female; Heart Neoplasms; Humans; Lymphoma; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prognosis; Survival; Survival Analysis; Time Factors; Young Adult
PubMed: 32285360
DOI: 10.1007/s12185-020-02881-2 -
International Journal of Surgery... Apr 2020Cardiac tumors and their associated outcomes are poorly characterized. This study sought to comprehensively assess the epidemiology and natural history of primary and... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Cardiac tumors and their associated outcomes are poorly characterized. This study sought to comprehensively assess the epidemiology and natural history of primary and secondary malignant cardiac tumors (PMCT and SMCT), a well as establish predictors of mortality.
METHODS
A comprehensive literature review was performed to identify articles reporting on PMCTs and SMCTs. The prevalence of important cardiac tumor (CT) subtypes was evaluated and further stratified based on the continental region. Outcomes of interest included short- and long-term mortality and utilization of heart transplantation (HTX). A random effect model was adopted, and a meta-regression was performed to determine predictors of the prevalence of CTs as well as predictors of operative mortality.
RESULTS
Of the 1,226 retrieved articles, 74 were included in our study (n = 8,849 patients). The mean follow-up was 2.27 years, mean age was 42.9 years, and 55% of the patients were females. There was a total number of 7,484 benign primary cardiac tumors (PCTs) (5,140 were myxoma), 862 (9.7%) malignant PCTs, and 355 secondary cardiac tumors. The prevalence of PMCTs among PCTs was 10.83% [95%CI = 09.11; 12.83%] with a trend towards being lower in South America compared to other continents (Prevalence = 5.80%). The prevalence of HTX among all patients was 2.45% [1.36; 4.38%]. The pooled short-term mortality was 5.90% [4.70; 7.39%] and the incidence of late mortality in all CTs, benign CT and PMCTs was 2.55% [1.76; 3.72%], 0.79% [0.46; 1.37%] and 14.77% [9.32; 23.40%], respectively. On meta-regression, the annual volume of cardiac tumor cases per center was the only predictor of lower early mortality (Beta = -0.14 ± 0.03, P < 0.0001).
CONCLUSIONS
PMCTs represent the minority of PCT (~10%) and have a higher prevalence in Europe and North America. Survival is higher in benign pathology and is significantly improved by treatment in specialized high-volume centers. Approximately 2% of patients with CTs undergo heart transplantation.
Topics: Adult; Europe; Female; Heart Neoplasms; Humans; Incidence; Male; North America; Prevalence; Time Factors
PubMed: 32169566
DOI: 10.1016/j.ijsu.2020.02.039 -
The British Journal of Oral &... Apr 2020
PubMed: 32061381
DOI: 10.1016/j.bjoms.2020.02.001 -
European Journal of Clinical... Apr 2020Odontogenic myxoma (OM) is a rare neoplasm, which originates from odontogenic ectomesenchyme. There is no study in the literature that analyses the best standards for OM...
BACKGROUND
Odontogenic myxoma (OM) is a rare neoplasm, which originates from odontogenic ectomesenchyme. There is no study in the literature that analyses the best standards for OM diagnosis and how the treatment modalities may influence the recurrence rates.
OBJECTIVE
To evaluate the best standards for odontogenic myxoma (OM) diagnosis and treatment, and how these may influence the recurrence rates.
STUDY DESIGN
Two independent researchers performed a systematic review in many databases. Fifty-two eligible studies were included for qualitative analysis. Bias analysis was conducted according to Oxford Centre for Evidence-Based Medicine.
RESULTS
A total of 1363 OM cases were reported on, and female gender with average age of 27 years is the most common patient profile. Conventional microscopic findings were observed in 93.43% of the reported cases. In 57.49% of the cases, multilocular radiographic appearance was present, followed by unilocular appearance (32.87%). Posterior mandible was the site with the major prevalence, while surgical resection was the most common treatment modality, followed by enucleation. Recurrence rates for both treatment modalities were approximately close (13.04% and 25.0%, respectively).
CONCLUSION
The correct diagnosis of OM relies on the association of clinical, radiographic and microscopic findings. About imaging examinations, panoramic radiography and computed tomography are sufficient for the evaluation of OM. Recurrence rates were closely among the two most used surgery treatments. So according to some clinical-radiological aspects, conservative surgery may be preferred than aggressive surgery modalities.
Topics: Bias; Humans; Jaw Neoplasms; Magnetic Resonance Imaging; Myxoma; Neoplasm Recurrence, Local; Odontogenic Tumors; Radiography, Panoramic; Tomography, X-Ray Computed
PubMed: 32048275
DOI: 10.1111/eci.13214