We present a case of a young patient with chest pain. Labs and cardiac imaging were suspicious for acute myocarditis. Genetic testing revealed a diagnosis of desmoplakin cardiomyopathy. Desmoplakin ...cardiomyopathy may be considered in patients with recurrent acute myocarditis or a family history of cardiac disease to avoid the potential for misdiagnosis.
To evaluate the racial and ethnic representation of transarterial therapy for hepatocellular carcinoma (HCC) clinical trials in the United States.
The ClinicalTrials.gov database was examined to ...identify all completed studies with transarterial therapies for the management of HCC in the United States and extract information about the observed number of participants for each racial and ethnic group (based on the Office of Management and Budget definitions). The expected number of participants was calculated by multiplying the total number of participants in a trial with the U.S.-population HCC-based proportion for each group. The effects of the study phase, funding source, number of centers involved in the study, and the location of the participating center on racial and ethnic distribution were explored.
Seventy-nine relevant studies were identified, of which 27 (34.2%) and 18 (22.8%) reported ethnic and race characteristics, respectively. Most study participants were White (81%, 1,591/1,964) by ethnicity and not Hispanic or Latino (93%, 937/1,008) by race. In terms of the observed-to-expected ratios by race and ethnicity in all trials, White and not Hispanic or Latino participants were overrepresented with a ratio of 1.22 (1.10-1.37) and 1.33 (1.26-1.41), respectively, and all other racial and ethnic groups were underrepresented. The enrollment of African Americans and Asian Americans varied by the study phase, and a higher enrollment of African Americans was noted in the National Institutes of Health-funded and multicenter studies (P < .05).
This cross-sectional study demonstrates that in HCC transarterial therapy clinical trials, racial and ethnic minorities were underrepresented and the majority of the studies identified failed to report this demographic information.
Chondromyxoid fibroma is a rare, benign tumor of the bone with excellent prognosis but a high rate of recurrence. We report a patient presenting with pain and a history of chondromyxoid fibroma of ...the distal left femur previously treated with multiple prior curettage and bone graft procedures. Magnetic resonance imaging and histopathology indicated a recurrence of tumor. Due to the small size of the tumor recurrence and challenges associated with prior open surgery, the patient underwent cryoablation of the lesion with computed tomography guidance. Follow-up 18 months later indicated a resolution of pain and improvement on magnetic resonance imaging, and no concerns after 20 months. To our knowledge, this is the first reported case of chondromyxoid fibroma treated with cryoablation. This case suggests cryoablation could be considered in the setting of recurrent chondromyxoid fibroma for local tumor control.
Background
Studies evaluating health-related quality of life (HRQOL) in patients with hereditary hemorrhagic telangiectasia (HHT) have expanded rapidly in the past decade. These studies have ...evaluated QOL aspects ranging from the general QOL for patients living with HHT to intervention-specific outcomes. However, few tools have been fully validated across the spectrum of disease manifestations and interventions in HHT.
Objective
In this scoping review, we aim to map the literature on HHT-QOL metrics, identify gaps, inform future QOL research, and facilitate future metric development.
Methods
We analyzed articles in English that assessed at least 1 measure of general HRQOL, including physical health, mental health, social health, or intervention-specific QOL in patients with HHT. Searches across 2 bibliographic databases (PubMed and Scopus) yielded 186 articles after duplicates were removed. Sixty-three studies met eligibility criteria: 22 prospective studies (34.9%), 20 retrospective studies (31.7%), 12 cross-sectional studies (17.5%), 6 randomized controlled trials or secondary analyses of a randomized controlled trials (9.5%), 2 qualitative studies (3.2%), and 1 case-control study (1.6%). Two additional studies—1 prospective and 1 cross-sectional study—were identified at the October 2022 14th International HHT Conference and included, making a total of 65 studies.
Results
The 65 eligible studies used 30 QOL instruments. Twenty studies characterized baseline HRQOL, and 45 studies evaluated QOL before and after treatment. Of those 45 studies, 37 evaluated HRQOL before and after therapies targeting epistaxis and nasal symptoms, 4 targeted therapies for liver arteriovenous malformations and high-output heart failure, 3 evaluated therapies for both epistaxis and gastrointestinal bleeding, and 1 evaluated treatment targeting gastrointestinal bleeding alone.
Conclusions
Comparison of results across studies remains challenging given the heterogeneity in outcomes measures. Further development of HHT-specific patient-reported outcomes instruments that capture the global illness experience of HHT is needed.
To evaluate interventional radiology (IR) research over time based on the study type of published articles and the visibility of articles to non-radiology clinicians.
We performed a search of all ...PubMed-indexed literature from January 1, 1991, through November 11, 2020, for clinical IR articles classified by their study type, categorized as: 1) meta-analyses/systematic reviews/practice guidelines; 2) randomized controlled trials; 3) non-randomized controlled trials; and 4) longitudinal/observational studies. Clinical IR articles were defined as those that met keyword criteria constructed from Society of Interventional Radiology procedure guides. Data were also collected on medical specialty journal categories that published IR-related articles.
When we examined the first vs. the last decade of our study period, the number of IR articles published increased across all study types: randomized controlled trials (374 to 2620; 601% change), longitudinal/observational studies (2324 to 12,447; 436%), meta-analyses/systematic reviews/practice guidelines (1179 to 6135; 420%), non-randomized controlled trials (471 to 2161; 359%). The journal categories with the highest mean percentage increase of IR articles across all study types were obstetrics and gynecology (659%), peripheral vascular disease (342%), and emergency medicine (221%). We found a decrease of IR articles published in surgery (−6.0%), pediatrics (−14%), and pulmonary (−21%) journals.
The number of IR articles grew quickly and at a similar rate compared with all PubMed-indexed articles and increased as a proportion of articles published in non-imaging specialty journals. This indicates greater visibility of IR studies for all clinicians and is encouraging towards the advancement of IR techniques.
•Number of interventional radiology (IR) articles published has increased at a similar rate to all medical literature.•IR articles are increasingly published in non-imaging journals.•An evaluation of publishing trends helps to guide researchers towards future areas of study.
•MRI-based volumetric analysis can assess outcomes of PS in extremity VMs.•Volumetric assessment of volume and SI change on T2-WI after PS is feasible.•VM volume change is a reliable indicator of ...treatment response after PS.•VM volume and SI correlate with clinical outcomes in specific patient groups.
To investigate quantitative changes in MRI signal intensity (SI) and lesion volume that indicate treatment response and correlate these changes with clinical outcomes after percutaneous sclerotherapy (PS) of extremity venous malformations (VMs).
VMs were segmented manually on pre- and post-treatment T2-weighted MRI using 3D Slicer to assess changes in lesion volume and SI. Clinical outcomes were scored on a 7-point Likert scale according to patient perception of symptom improvement; treatment response (success or failure) was determined accordingly.
Eighty-one patients with VMs underwent 125 PS sessions. Treatment success occurred in 77 patients (95 %). Mean (±SD) changes were –7.9 ± 24 cm3 in lesion volume and –123 ± 162 in SI (both, P <.001). Mean reduction in lesion volume was greater in the success group (–9.4 ± 24 cm3) than in the failure group (21 ± 20 cm3) (P =.006). Overall, lesion volume correlated with treatment response (ρ = –0.3, P =.004). On subgroup analysis, volume change correlated with clinical outcomes in children (ρ = –0.3, P =.03), in sodium tetradecyl sulfate–treated lesions (ρ = –0.5, P =.02), and in foot lesions (ρ = –0.6, P =.04). SI change correlated with clinical outcomes in VMs treated in 1 PS session (ρ = –0.3, P =.01) and in bleomycin-treated lesions (ρ = –0.4, P =.04).
Change in lesion volume is a reliable indicator of treatment response. Lesion volume and SI correlate with clinical outcomes in specific subgroups.
To describe residual arterial supply to the stomach after bariatric surgery via a systematic arterial-phase CT assessment approach that can aid in diagnosis and treatment of postoperative ...complications and facilitate planning for future procedures.PURPOSETo describe residual arterial supply to the stomach after bariatric surgery via a systematic arterial-phase CT assessment approach that can aid in diagnosis and treatment of postoperative complications and facilitate planning for future procedures.Arterial-phase CT of 46 patients who underwent Roux-en-Y gastric bypass (RYGB) or sleeve gastrectomy (SG) at 3 academic institutions were retrospectively reviewed to assess patency of left gastric artery (LGA), right gastric artery (RGA), gastroepiploic artery (GEA), and left inferior phrenic artery (LIPA) and presence of gastric perforators.METHODSArterial-phase CT of 46 patients who underwent Roux-en-Y gastric bypass (RYGB) or sleeve gastrectomy (SG) at 3 academic institutions were retrospectively reviewed to assess patency of left gastric artery (LGA), right gastric artery (RGA), gastroepiploic artery (GEA), and left inferior phrenic artery (LIPA) and presence of gastric perforators.In 25 RYGB and 21 SG patients, mean diameters were LGA 2.2 ± 0.4 mm, RGA 1.6 ± 0.5 mm, and GEA 1.7 ± 0.4 mm. On RYGB scans, all LGAs, RGAs, and 24/25 (96%) of GEAs were identified. Excellent to good patency was seen in 20/25 (80%) LGAs, 21/25 (84%) RGAs, and 23/24 (96%) GEAs. On SG scans, all LGAs, 18/21 (86%) of RGAs, and 20/21 (95%) GEAs were identified. Excellent to good patency was seen in 17/21 (81%) LGAs, 15/18 (83%) RGAs, and 20/20 (100%) GEAs. In terms of gastric perforators, LGA supply was seen on 23/25 (92%) of RYGB and 17/17 (100%) of SG scans. RGA supply was seen on 13/21 (62%) RYGB and 9/18 (50%) SG scans. GEA supply was seen on 19/23 (83%) RYGB scans. No gastric supply via GEA was seen on SG scans.RESULTSIn 25 RYGB and 21 SG patients, mean diameters were LGA 2.2 ± 0.4 mm, RGA 1.6 ± 0.5 mm, and GEA 1.7 ± 0.4 mm. On RYGB scans, all LGAs, RGAs, and 24/25 (96%) of GEAs were identified. Excellent to good patency was seen in 20/25 (80%) LGAs, 21/25 (84%) RGAs, and 23/24 (96%) GEAs. On SG scans, all LGAs, 18/21 (86%) of RGAs, and 20/21 (95%) GEAs were identified. Excellent to good patency was seen in 17/21 (81%) LGAs, 15/18 (83%) RGAs, and 20/20 (100%) GEAs. In terms of gastric perforators, LGA supply was seen on 23/25 (92%) of RYGB and 17/17 (100%) of SG scans. RGA supply was seen on 13/21 (62%) RYGB and 9/18 (50%) SG scans. GEA supply was seen on 19/23 (83%) RYGB scans. No gastric supply via GEA was seen on SG scans.In this study, arterial supply to the stomach through the LGA was consistently identified in all RYGB and SG cases, indicating an uncomplicated surgical approach with regard to preserving the LGA. Dedicated CT angiography protocol or catheter-directed angiography is recommended for accurate and comprehensive assessment of the gastric blood supply, particularly before surgical re-intervention.CONCLUSIONIn this study, arterial supply to the stomach through the LGA was consistently identified in all RYGB and SG cases, indicating an uncomplicated surgical approach with regard to preserving the LGA. Dedicated CT angiography protocol or catheter-directed angiography is recommended for accurate and comprehensive assessment of the gastric blood supply, particularly before surgical re-intervention.
T1-hyperintensity of the basal ganglia (BG) due to manganese deposition is a known radiologic finding in patients with hereditary hemorrhagic telangiectasia (HHT), but risk factors and associated ...clinical manifestations are unclear. This study conducted a quantitative analysis of the association of T1-hyperintensity in HHT patients with specific risk factors, signs, and symptoms.
Patients seen at our center between 2005 and 2020 with a definitive diagnosis of HHT who had an available non-contrast T1-weighted brain MRI were included. Hyperintensity was evaluated using oval regions of interest measurements. The BG: thalamus intensity ratio was used to quantitatively evaluate T1-hyperintensity. Patient laboratory values and clinical findings were collected from electronic medical records. Hyperintensity was analyzed for its association with laboratory values, and clinical findings. Variables were analyzed through regression analysis.
A total of 239 patients were included in this study. On 1.5 T scanners, values that were significant on multivariable regression analysis were age (p < .001), hepatic AVMs (p < .001), iron deficiency anemia (p = .0021), and cirrhosis (p = .016). On 3 T scanners, values that were significant on multivariable analysis were hepatic AVMs (p = .0024) and cirrhosis (p = .0056). On 3 T scanners, hyperintensity was significantly associated with tremor (OR = 1.17, p = .033), restless leg syndrome (OR = 1.22, p = .0086), and memory problems (OR = 1.17, p = .046).
BG hyperintensity due to manganese deposition is significantly associated with hepatic risk factors on 1.5 T and 3 T scanners and iron deficiency anemia on 1.5 T scanners. On 3 T scanners, T1-hyperintensity is associated with neuropsychiatric signs and symptoms, such as tremor, restless leg syndrome, and memory problems.
•T1-hyperintensity in the basal ganglia due to manganese deposition is a known finding in patients with hereditary hemorrhagic telangiectasia•However, the risk factors and clinical significance of these findings are unclear•Significant risk factors for deposition include increased age, iron deficiency anemia, hepatic AVMs, and liver cirrhosis•At a 3T field strength, significant associations were found between T1-hyperintensity and tremors, restless leg syndrome, and memory problems
Idiopathic inflammatory myopathies (IIM) are characterized by muscle inflammation and weakness, myositis-specific autoantibodies (MSAs), and extramuscular organ damage. The role of neutrophil ...dysregulation and neutrophil extracellular traps (NETs) in IIM is unclear. We assessed whether pathogenic neutrophil subsets (low-density granulocytes LDGs) and NETs were elevated in IIM, associated with clinical presentation and MSAs, and their effect on skeletal myoblasts and myotubes. Circulating NETs and LDGs were quantified and correlated with clinical measures. Specific MSAs were tested for their ability to induce NETs. NETs and neutrophil gene expression were measured in IIM biopsies. Whether NETs damage skeletal myoblasts and myotubes was tested. Circulating LDGs and NETs were increased in IIM. IIM LDGs had an enhanced ability to form NETs. LDGs and NETs correlated with IIM disease activity and muscle damage. The serum MSA anti-MDA5 correlated with circulating and tissue NETs and directly enhanced NET formation. An enhanced neutrophil gene signature was present in IIM muscle and associated with muscle injury and tissue IFN gene signatures. IIM NETs decreased the viability of myotubes in a citrullinated histone-dependent manner. Dysregulated neutrophil pathways may play pathogenic roles in IIM through their ability to directly injure muscle cells and other affected tissues.
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