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1.
Alzheimer Dis Assoc Disord ; 38(2): 128-132, 2024.
Article En | MEDLINE | ID: mdl-38755756

BACKGROUND: We examined drivers of self and study partner reports of memory loss in mild cognitive impairment (MCI) from Alzheimer (AD-MCI) and vascular disease (Va-MCI). METHODS: We performed retrospective cross-sectional analyses of participants with AD-MCI (n=2874) and Va-MCI (n=376) from the National Alzheimer Coordinating Center data set. Statistical analysis utilized 2-sided t test or the Fisher exact test. RESULTS: Compared with AD-MCI, Va-MCI subjects (24.5% vs. 19.7%, P =0.031) and study partners (31.4% vs. 21.6%, P <0.0001) were more likely to deny memory loss. Black/African Americans were disproportionately represented in the group denying memory loss in AD-MCI (20.0% vs. 13.2%, P <0.0001) and Va-MCI (33.7% vs. 18.0%, P =0.0022). Study partners of participants with these features also disproportionately denied memory loss: female (AD-MCI: 60.1% vs. 51.7%, P =0.0002; Va-MCI: 70.3% vs. 52.3%, P =0.0011), Black/African American (AD-MCI: 23.5% vs. 11.98%, P <0.0001; Va-MCI: 48.8% vs. 26.5%, P =0.0002), and <16 years of education (AD-MCI only: 33.9% vs. 16.3%, P =0.0262). In AD-MCI and Va-MCI, participants with anxiety were disproportionately represented in the group endorsing memory loss (AD: 28.2% vs. 17.4%, P <0.0001; Va: 31.5% vs. 16.1%, P =0.0071), with analogous results with depression. CONCLUSION: The findings would suggest extra vigilance in interview-based MCI detection of persons at-risk for self-based or informant-based misreport.


Alzheimer Disease , Cognitive Dysfunction , Memory Disorders , Vascular Diseases , Humans , Female , Male , Aged , Cross-Sectional Studies , Memory Disorders/diagnosis , Retrospective Studies , Vascular Diseases/complications , Aged, 80 and over
2.
BMJ Case Rep ; 17(5)2024 May 21.
Article En | MEDLINE | ID: mdl-38772870

A postpartum woman in her early 40s, with a history of pre-eclampsia and von Willebrand disease (VWD), presented to the emergency room with chest pain suggestive of an acute coronary syndrome. Initial workup revealed an evolving anterior wall ST-segment elevation myocardial infarction on ECG and elevated cardiac biomarkers, confirming myocardial damage. Point-of-care ultrasound showed apical hypokinesis and coronary angiography revealed a distal dissection of the left anterior descending coronary artery. There was TIMI 3 flow and no evidence of plaque rupture. No percutaneous coronary intervention was performed and the patient was managed conservatively.Fibromuscular dysplasia was ruled out on screening CT angiography. Dual antiplatelet therapy was initiated for an amended course of 3 months given the history of VWD. Our patient had an uncomplicated course in the hospital with a downward trend in their cardiac biomarkers, resolving anterior ST elevation on serial ECGs, and no bleeding complications.


Coronary Vessel Anomalies , Electrocardiography , ST Elevation Myocardial Infarction , von Willebrand Diseases , Humans , ST Elevation Myocardial Infarction/complications , ST Elevation Myocardial Infarction/diagnosis , Female , Adult , von Willebrand Diseases/complications , von Willebrand Diseases/diagnosis , Coronary Vessel Anomalies/complications , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessel Anomalies/diagnosis , Vascular Diseases/congenital , Vascular Diseases/complications , Vascular Diseases/diagnostic imaging , Vascular Diseases/diagnosis , Coronary Angiography , Platelet Aggregation Inhibitors/therapeutic use
3.
J Int Med Res ; 52(4): 3000605241240579, 2024 Apr.
Article En | MEDLINE | ID: mdl-38603605

The mortality rate of gastric varices bleeding can reach 20% within 6 weeks. Isolated gastric varices (IGVs) refer to gastric varices without esophageal varices and typically arise as a common complication of left portal hypertension. Although IGVs commonly form in the setting of splenic vein occlusion, the combination of antiphospholipid syndrome and protein S deficiency leading to splenic vein occlusion is rare. We herein present a case of a 28-year-old woman with intermittent epigastric pain and melena. She was diagnosed with antiphospholipid syndrome based on the triad of pregnancy morbidity, unexplained venous occlusion, and positive lupus anticoagulant. Laparoscopic splenectomy and pericardial devascularization were performed for the treatment of IGVs. During the 6-month postoperative follow-up, repeated endoscopy and contrast-enhanced computed tomography revealed disappearance of the IGVs. This is the first description of splenic vein occlusion associated with both antiphospholipid syndrome and protein S deficiency. We also provide a review of the etiology, clinical manifestations, diagnosis, and treatment methods of IGVs.


Antiphospholipid Syndrome , Esophageal and Gastric Varices , Protein S Deficiency , Vascular Diseases , Female , Humans , Adult , Esophageal and Gastric Varices/complications , Esophageal and Gastric Varices/diagnosis , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Protein S Deficiency/complications , Gastrointestinal Hemorrhage/etiology , Vascular Diseases/complications
4.
Medicine (Baltimore) ; 103(16): e37871, 2024 Apr 19.
Article En | MEDLINE | ID: mdl-38640308

RATIONALE: The bleeding of Dieulafoy lesion predominantly involves the proximal stomach and leads to severe gastrointestinal bleeding. However, these lesions have also been reported in the whole gastrointestinal tract. Bleeding of Dieulafoy lesions at the anastomosis was seldomly reported and was very easy to be ignored clinically. PATIENT CONCERNS: We describe a 72-year-old woman with a past history of surgery for rectal carcinoma hospitalized with chief complaint of massive rectal bleeding. No gross bleeding lesion was found during the first emergency colonoscopy. Despite multiple blood transfusions, her hemoglobin rapidly dropped to 5.8 g/dL. DIAGNOSIS: She was diagnosed with Dieulafoy lesion at the colorectal anastomosis during the second emergency colonoscopy. INTERVENTIONS: Primary hemostasis was achieved by endoscopic hemostatic clipping. However, she experienced another large volume hematochezia 3 days later, and then received another endoscopic hemostatic clipping. She was improved and discharged. However, this patient underwent hematochezia again 1 month later. Bleeding was arrested successfully after the over-the-scope clip (OTSC) was placed during the fourth emergency colonoscopy. OUTCOMES: This patient underwent 4 endoscopic examinations and treatments during 2 hospitalizations. The lesion was overlooked during the first emergency colonoscopy. The second and third endoscopes revealed Dieulafoy lesion at the colorectal anastomosis and performed endoscopic hemostatic clippings, but delayed rebleeding occurred. The bleeding was stopped after the fourth emergency colonoscopy using OTSC. There was no further rebleeding during hospitalization and after 2-year of follow-up. LESSONS: As far as we know, there is no reported case of lower gastrointestinal bleeding caused by Dieulafoy lesion at the colorectal anastomosis, OTSC is a safe and effective rescue treatment for Dieulafoy lesions.


Colorectal Neoplasms , Hemostasis, Endoscopic , Hemostatics , Vascular Diseases , Humans , Female , Aged , Hemostasis, Endoscopic/adverse effects , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/surgery , Vascular Diseases/complications , Anastomosis, Surgical/adverse effects , Colorectal Neoplasms/therapy
6.
Psychopharmacol Bull ; 54(2): 28-33, 2024 Apr 04.
Article En | MEDLINE | ID: mdl-38601836

As global aging becomes more prominent, neurocognitive disorders (NCD) incidence has increased. Patients with NCD usually have an impairment in one or more cognitive domains, such as attention, planning, inhibition, learning, memory, language, visual perception, and spatial or social skills. Studies indicate that 50-80% of these adults will develop neuropsychiatric symptoms (NPS), such as apathy, depression, anxiety, disinhibition, delusions, hallucinations, and aberrant motor behavior. The progression of NCD and subsequent NPS requires tremendous care from trained medical professionals and family members. The behavioral symptoms are often more distressing than cognitive changes, causing caregiver distress/depression, more emergency room visits and hospitalizations, and even earlier institutionalization. This signifies the need for early identification of individuals at higher risk of NPS, understanding the trajectory of their NCD, and exploring treatment modalities. In this case report and review, we present an 82-year-old male admitted to our facility for new-onset symptoms of depression, anxiety, and persecutory delusions. He has no significant past psychiatric history, and his medical history is significant for extensive ischemic vascular disease requiring multiple surgeries and two episodes of cerebrovascular accident (CVA). On further evaluation, the patient was diagnosed with major NCD, vascular subtype. We discuss differential diagnoses and development of NPS from NCD in order to explain the significance of more thorough evaluation by clinicians for early detection and understanding of NCD prognosis.


Delusions , Vascular Diseases , Aged, 80 and over , Humans , Male , Delusions/etiology , Depression/etiology , Hallucinations , Neurocognitive Disorders , Vascular Diseases/complications
7.
Clin Endocrinol (Oxf) ; 100(6): 558-564, 2024 Jun.
Article En | MEDLINE | ID: mdl-38652736

CONTEXT: Epidemiological studies involving patients with acromegaly have yielded conflicting results regarding cancer incidence and causes of mortality in relation to control of growth hormone (GH) excess. OBJECTIVE: The objective of this retrospective cohort study is to clarify these questions and identify goals for treatment and monitoring patients. METHODS: We studied 1845 subjects from the UK Acromegaly Register (1970-2016), obtaining cancer standardised incidence rates (SIR) and all causes standardised mortality rates (SMR) from UK Office for National Statistics, to determine the relationship between causes of mortality-age at diagnosis, duration of disease, post-treatment and mean GH levels. RESULTS: We found an increased incidence of all cancers (SIR, 1.38; 95% CI: 1.06-1.33, p < .001), but no increase in incidence of female breast, thyroid, colon cancer or any measure of cancer mortality. All-cause mortality rates were increased (SMR, 1.35; 95% CI: 1.24-1.46, p < .001), as were those due to vascular and respiratory diseases. All-cause, all cancer and cardiovascular deaths were highest in the first 5 years following diagnosis. We found a positive association between post-treatment and mean treatment GH levels and all-cause mortality (p < .001 and p < .001), which normalised with posttreatment GH levels of <1.0 µg/L or meantreatment GH levels of <2.5 µg/L. CONCLUSION: Acromegaly is associated with increased incidence of all cancers but not thyroid or colon cancer and no increase in cancer mortality. Excess mortality is due to vascular and respiratory disease. The risk is highest in the first 5 years following diagnosis and is mitigated by normalising GH levels.


Acromegaly , Human Growth Hormone , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Acromegaly/blood , Acromegaly/complications , Acromegaly/therapy , Cardiovascular Diseases/blood , Cardiovascular Diseases/complications , Human Growth Hormone/blood , Human Growth Hormone/metabolism , Incidence , Neoplasms/complications , Registries , Respiratory Tract Diseases/complications , Retrospective Studies , United Kingdom , Vascular Diseases/complications
8.
Int J Mol Sci ; 25(5)2024 Feb 24.
Article En | MEDLINE | ID: mdl-38473891

Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While generally benign, these dermatoses can be persistent and aesthetically bothersome. Key clinical features include red to brownish patches with a distinctive "cayenne pepper" appearance, predominantly localized on the lower extremities, particularly the shins. Subtypes include Schamberg disease, Majocchi's disease, Gougerot-Blum disease, Ducas and Kapetanakis pigmented purpura, and lichen aureus. Diagnosis relies primarily on clinical evaluation of skin lesions, with biopsy as a confirmatory tool. Although the exact cause of PPD remains unclear, capillary fragility and red blood cell extravasation are implicated. Treatment strategies for PPD aim to alleviate symptoms, considering the generally benign and chronic nature of the condition. As there is no standardized treatment, various methods with varying efficacy are employed. After searching SCOPUS and PubMed databases, we assessed 42 original articles to present current knowledge regarding therapy of PPD. This review will compare treatment approaches specifically in Schamberg disease and other manifestations of pigmented purpuric dermatoses.


Eczema , Pigmentation Disorders , Purpura , Skin Diseases , Vascular Diseases , Humans , Pigmentation Disorders/etiology , Skin Diseases/diagnosis , Purpura/diagnosis , Purpura/etiology , Purpura/pathology , Vascular Diseases/complications
9.
J Alzheimers Dis ; 98(3): 897-906, 2024.
Article En | MEDLINE | ID: mdl-38461505

Background: Financial capacity is vital for the elderly, who possess a substantial share of global wealth but are vulnerable to financial fraud. Objective: We explored the link between small vessel disease (SVD) and financial capacity in cognitively unimpaired (CU) older adults via both cross-sectional and longitudinal analyses. Methods: 414 CU participants underwent MRI and completed the Financial Capacity Instrument-Short Form (FCI-SF). Subsequent longitudinal FCI-SF data were obtained from 104, 240, and 141 participants at one, two, and four years, respectively. SVD imaging markers, encompassing white matter hyperintensities (WMH), cerebral microbleeds (CMB), and lacune were evaluated. We used linear regression analyses to cross-sectionally explore the association between FCI-SF and SVD severity, and linear mixed models to assess how baseline SVD severity impacted longitudinal FCI-SF change. The false discovery rate method was used to adjust multiple comparisons. Results: Cross-sectional analysis revealed a significant association between baseline WMH and Bank Statement (BANK, ß=-0.194), as well as between lacune number and Financial Conceptual Knowledge (FC, ß= -0.171). These associations were stronger in APOE ɛ4 carriers, with ß= -0.282 for WMH and BANK, and ß= -0.366 for lacune number and FC. Longitudinally, higher baseline SVD total score was associated with severe FCI-SF total score decrease (ß= -0.335). Additionally, baseline WMH burden predicted future decreases in Single Checkbook/Register Task (SNG, ß= -0.137) and FC (ß= -0.052). Notably, the association between baseline WMH and SNG changes was amplified in APOE ɛ4 carriers (ß= -0.187). Conclusions: Severe SVD was associated with worse FCI-SF and could predict the decline of financial capacity in CU older adults.


Cerebral Small Vessel Diseases , Vascular Diseases , White Matter , Humans , Aged , Cross-Sectional Studies , Magnetic Resonance Imaging , Vascular Diseases/complications , Cerebral Small Vessel Diseases/diagnostic imaging , Cerebral Small Vessel Diseases/genetics , Cerebral Small Vessel Diseases/complications , Apolipoproteins E
10.
Int J Mol Sci ; 25(5)2024 Mar 06.
Article En | MEDLINE | ID: mdl-38474287

Endocan is an endothelial-cell-specific proteoglycan (ESM-1) and has emerged as an endothelial dysfunction and inflammatory marker in recent years. Endocan can be used as a marker of inflammatory endothelial dysfunction in endothelium-dependent disease: cardiovascular disease, sepsis, lung and kidney disease and malignancies. Recent data suggest that endothelial dysfunction is a key mechanism in COVID-19 pathogenesis. Endotheliitis and thrombo-inflammation are associated with severe forms of SARS-CoV-2 infection, and endocan is currently under investigation as a potential diagnostic and prognostic marker. The aim of this study was to determine serum endocan levels in patients with COVID-19 to evaluate the correlation between endocan levels and clinical disease diagnosis and prognosis. This study enrolled 56 patients, divided into three groups depending on disease severity: mild (15), moderate (25) and severe (16). The biochemical, demographic, clinical and imagistic data were collected and evaluated in correlation with the endocan levels. Serum endocan levels were significantly higher in the COVID-19 patients compared to the control group; also, endocan concentration correlated with vaccination status. The results revealed significantly elevated serum endocan levels in COVID-19 patients compared to the control group, with a correlation observed between endocan concentration and vaccination status. These findings suggest that endocan may serve as a novel biomarker for detecting inflammation and endothelial dysfunction risk in COVID-19 patients. There was no significant relationship between serum endocan levels and disease severity or the presence of cardiovascular diseases. Endocan can be considered a novel biomarker for the detection of inflammation and endothelial dysfunction risk in COVID-19 patients.


COVID-19 , Cardiovascular Diseases , Vascular Diseases , Humans , Biomarkers , Cardiovascular Diseases/etiology , COVID-19/complications , Inflammation/complications , Neoplasm Proteins , SARS-CoV-2 , Vascular Diseases/complications
11.
J Am Soc Echocardiogr ; 37(5): 518-529, 2024 May.
Article En | MEDLINE | ID: mdl-38467311

BACKGROUND: The etiology and significance of coronary artery tortuosity (TCA) among patients with spontaneous coronary artery dissection (SCAD) are unknown. The aim of this prospective imaging cohort study was to report echocardiographic findings and evaluate whether TCA correlates with cardiac anatomy and function among patients with SCAD. Comorbidities including fibromuscular dysplasia (FMD) and outcomes were also assessed. METHODS: TCA was determined on coronary angiography performed during the diagnosis of SCAD, and cardiac structure and function were evaluated using prospective comprehensive echocardiography. RESULTS: Among 116 patients with SCAD, the mean age at echocardiography was 50.8 ± 8.8 years, a median of 10.9 months after SCAD. Sixty-two patients (53.4%) had FMD, 41 (35.3%) had histories of hypertension, and 17 (14.8%) were hypertensive during echocardiography. Most patients (n = 78 [69%]) had normal left ventricular geometry with normal median ejection fraction (61%; interquartile range, 56% to 64%) and normal global longitudinal strain (-22.2%; interquartile range, -24.0% to -19.9%). Fifteen patients (13.4%) had diastolic dysfunction that was associated with hypertension at the time of echocardiography. Patients with TCA (n = 96 [82.8%]) were older (mean age, 52.1 ± 8.0 vs 44.7 ± 9.9 years; P < .001) with a higher prevalence of FMD (59.4% vs 25%, P = .007) but a similar prevalence of hypertension (35% vs 35%, P > .99) compared with patients without TCA. Across the age range (31.5 to 66.9 years), each decade of age was associated with an approximately 0.89-unit increase in coronary tortuosity score (P < .0001). Echocardiographic parameters were not significantly different between the two groups. Median follow-up duration was 4.4 years (95% CI, 3.8 to 5.2 years). The Kaplan-Meier 3-year SCAD recurrence rate was 9.4% (95% CI, 3.7% to 14.8%). There were no deaths. CONCLUSIONS: The majority of patients with SCAD had normal or near normal echocardiographic results, including global longitudinal strain, with no differences according to TCA. However, patients with SCAD with TCA were older, with a higher prevalence of FMD.


Coronary Vessel Anomalies , Coronary Vessels , Echocardiography , Fibromuscular Dysplasia , Vascular Diseases , Vascular Diseases/congenital , Humans , Female , Male , Fibromuscular Dysplasia/complications , Fibromuscular Dysplasia/diagnosis , Fibromuscular Dysplasia/epidemiology , Fibromuscular Dysplasia/physiopathology , Middle Aged , Coronary Vessel Anomalies/epidemiology , Coronary Vessel Anomalies/complications , Coronary Vessel Anomalies/diagnosis , Coronary Vessel Anomalies/physiopathology , Echocardiography/methods , Prospective Studies , Vascular Diseases/epidemiology , Vascular Diseases/physiopathology , Vascular Diseases/diagnosis , Vascular Diseases/complications , Coronary Vessels/diagnostic imaging , Coronary Angiography/methods , Adult , Global Longitudinal Strain
12.
Am J Cardiol ; 212S: S77-S82, 2024 Feb 01.
Article En | MEDLINE | ID: mdl-38368039

Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited heart disease encumbered throughout much of its almost 60-year history by the expectation of an unfavorable outcome with shortened longevity. However, it is notable that in 2023, most patients affected with HCM can now achieve normal or extended life expectancy without major disability because of a comprehensive constellation of management strategies that have evolved largely over the last 20 years. Distinct adverse disease pathways dictate high-benefit low-risk personalized treatments, without reliance on genomics and sarcomere mutations, including: primary prevention implantable defibrillators for sudden cardiac death prevention, surgical myectomy and percutaneous alcohol septal ablation to reverse heart failure symptoms, anticoagulation to prevent embolic stroke associated with concomitant atrial fibrillation, external defibrillation and hypothermia for out-of-hospital cardiac arrest, and heart transplant in a small patient subgroup with end-stage disease. Large cohort studies using these contemporary management strategies achieved remarkably low HCM-related mortality (0.5%/year) across all age groups, which is lower than in the other cardiac or noncardiac risks of living, and largely confined to nonobstructive patients with progressive heart failure, including those awaiting heart transplant.


Atrial Fibrillation , Cardiomyopathy, Hypertrophic , Heart Failure , Heart Transplantation , Vascular Diseases , Humans , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/diagnosis , Cohort Studies , Heart Failure/complications , Heart Transplantation/adverse effects , Atrial Fibrillation/complications , Death, Sudden, Cardiac/epidemiology , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Vascular Diseases/complications
13.
J Pediatr Surg ; 59(5): 839-846, 2024 May.
Article En | MEDLINE | ID: mdl-38365473

BACKGROUND: Pulmonary vascular disease (PVD) complicated with pulmonary hypertension (PH) is a leading cause of mortality in congenital diaphragmatic hernia (CDH). Unfortunately, CDH patients are often resistant to PH therapy. Using the nitrogen CDH rat model, we previously demonstrated that CDH-associated PVD involves an induction of elastase and matrix metalloproteinase (MMP) activities, increased osteopontin and epidermal growth factor (EGF) levels, and enhanced smooth muscle cell (SMC) proliferation. Here, we aimed to determine whether the levels of the key members of this proteinase-induced pathway are also elevated in the pulmonary arteries (PAs) of CDH patients. METHODS: Neutrophil elastase (NE), matrix metalloproteinase-2 (MMP-2), epidermal growth factor (EGF), tenascin-C, and osteopontin levels were assessed by immunohistochemistry in the PAs from the lungs of 11 CDH patients and 5 normal age-matched controls. Markers of proliferation (proliferating cell nuclear antigen (PCNA)) and apoptosis (cleaved (active) caspase-3) were also used. RESULTS: While expressed by both control and CDH lungs, the levels of NE, MMP-2, EGF, as well as tenascin-C and osteopontin were significantly increased in the PAs from CDH patients. The percentage of PCNA-positive PA SMCs were also enhanced, while those positive for caspase-3 were slightly decreased. CONCLUSIONS: These results suggest that increased elastase and MMPs, together with elevated tenascin-C and osteopontin levels in an EGF-rich environment may contribute to the PVD in CDH infants. The next step of this study is to expand our analysis to a larger cohort, and determine the potential of targeting this pathway for the treatment of CDH-associated PVD and PH. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: LEVEL III.


Hernias, Diaphragmatic, Congenital , Hypertension, Pulmonary , Vascular Diseases , Humans , Rats , Animals , Hernias, Diaphragmatic, Congenital/complications , Matrix Metalloproteinase 2/analysis , Matrix Metalloproteinase 2/metabolism , Pulmonary Artery , Osteopontin/metabolism , Caspase 3/metabolism , Proliferating Cell Nuclear Antigen/metabolism , Pancreatic Elastase/metabolism , Epidermal Growth Factor , Tenascin/metabolism , Lung/metabolism , Hypertension, Pulmonary/complications , Matrix Metalloproteinases , Vascular Diseases/complications , Phenyl Ethers/metabolism
14.
Medicine (Baltimore) ; 103(7): e37225, 2024 Feb 16.
Article En | MEDLINE | ID: mdl-38363925

Fibro-adipose vascular anomaly (FAVA) is a rare and complex vascular malformation associated with persistent pain, limb contracture, and even restriction of activity. However, the pathophysiology of FAVA remains unclear. Although FAVA is a benign vascular malformation, it is highly misdiagnosed and often thus undergoing repeated surgical resection and interventional sclerotherapy, resulting in worsening of symptoms and irreversible dysfunction. Therefore, aggressive diagnosis and treatment are essential. There are several different treatment options for FAVA, including surgical resection, sclerotherapy, cryoablation, drug therapy, and physical therapy. This article reviews the clinical manifestations, pathological features, pathogenesis, and treatment methods of FAVA.


Fibromyalgia , Vascular Diseases , Vascular Malformations , Humans , Treatment Outcome , Vascular Malformations/therapy , Vascular Malformations/surgery , Vascular Diseases/complications , Fibromyalgia/complications , Pain/etiology , Obesity/complications , Sclerotherapy/methods
15.
Medicine (Baltimore) ; 103(6): e37127, 2024 Feb 09.
Article En | MEDLINE | ID: mdl-38335412

Sickle cell anemia (SCA) is a hereditary blood disorder characterized by abnormal hemoglobin, causing red blood cells to assume a sickle shape, leading to various complications. Climate change has emerged as a significant global challenge, influencing environmental conditions worldwide. This paper explores the implications of climatic variations on the prevalence, management, and outcomes of SCA. Climate change affects weather patterns, leading to altered temperatures, increased frequency of extreme weather events, and variations in humidity levels. These changes can have a profound impact on individuals living with SCA. High temperatures exacerbate the symptoms of SCA, potentially triggering painful vaso-occlusive crises due to dehydration and increased blood viscosity. Conversely, cold temperatures may induce vaso-occlusion by causing blood vessels to constrict. Changes in rainfall patterns might also affect water accessibility, which is crucial for maintaining adequate hydration, particularly in regions prone to droughts. The management of SCA is multifaceted, involving regular medical care, hydration, and avoiding triggers that could precipitate a crisis. Adverse weather events and natural disasters can disrupt healthcare infrastructure and access to essential medications and resources for SCA patients, especially in vulnerable communities. To mitigate the implications of climatic change on SCA, interdisciplinary strategies are essential. These strategies may include enhancing healthcare systems' resilience to climate-related disruptions, implementing adaptive measures to address changing environmental conditions, and promoting public awareness and education on managing SCA amidst climate variability. In conclusion, climatic variations pose significant challenges for individuals with SCA, affecting the prevalence, management, and outcomes of the disease.


Anemia, Sickle Cell , Vascular Diseases , Humans , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Erythrocytes , Pain/complications , Vascular Diseases/complications , Climate Change
16.
Medicine (Baltimore) ; 103(2): e36898, 2024 Jan 12.
Article En | MEDLINE | ID: mdl-38215146

Sickle cell anemia (SCA) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells. While much research has focused on the molecular and cellular mechanisms underlying the pathophysiology of SCA, recent attention has turned to the role of apoptosis, or programmed cell death, in the disease progression. This review aims to elucidate the intricate mechanisms of apoptosis in SCA patients and explore its implications in disease severity, complications, and potential therapeutic interventions. Different research search engines such as PubMed central, Scopus, Web of Science, Google Scholar, ResearchGate, Academia Edu, etc were utilized in writing this paper. Apoptosis, a highly regulated cellular process, plays a crucial role in maintaining homeostasis by eliminating damaged or dysfunctional cells. In SCA, the imbalance between pro-apoptotic and anti-apoptotic signals contributes to increased erythrocyte apoptosis, exacerbating anemia and vaso-occlusive crises. Various factors, including oxidative stress, inflammation, and altered cell signaling pathways, converge to modulate the apoptotic response in SCA. Furthermore, the interaction between apoptotic cells and the vascular endothelium contributes to endothelial dysfunction, promoting the pathogenesis of vasculopathy and organ damage seen in SCA patients. In conclusion, unraveling the complexities of apoptosis in SCA provides valuable insights into the disease pathophysiology and offers novel avenues for therapeutic interventions.


Anemia, Sickle Cell , Vascular Diseases , Humans , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , Erythrocytes/metabolism , Vascular Diseases/complications , Inflammation/metabolism , Apoptosis
17.
Ann Vasc Surg ; 102: 110-120, 2024 May.
Article En | MEDLINE | ID: mdl-38296038

BACKGROUND: Nutcracker syndrome is a rare condition that occurs as a result of the entrapment of the left renal vein (LRV) between the aorta and the superior mesenteric artery. It is typically associated with symptoms such as left flank pain, hematuria, proteinuria, and pelvic congestion. The current treatment approach may be conservative in the presence of tolerable symptoms, and surgical or hybrid and stenting procedures in the order of priority in the presence of intolerable symptoms. The aim of this study is to review our experiences to evaluate the results of both methods in this series in which we have a greater tendency toward surgery instead of stenting. METHODS: The clinical data of consecutive patients with nutcracker syndrome who underwent LRV transposition and LRV stenting between July 2019 and October 2023 were retrospectively reviewed. The patients were divided into 2 groups based on the methods of treatment: surgical and stenting. For procedure selection, LRV transposition was primarily recommended, with stenting offered to those who declined. Primary end points were morbidity and mortality. Secondary end points included late complications, patency, freedom from reintervention, and resolution of symptoms. Standard basic statistics and survival analysis methods were employed. RESULTS: Nineteen patients with nutcracker syndrome (female: 100%) were treated with LRV stentings (n = 5) and LRV transposition (n = 14). The mean age was 24 (20-27, interquartile range [IQR]) years. The mean follow-up was 23 (9-32, IQR) months. There were no major complications and mortality after both procedures. The most frequent sign and symptom associated with LRV entrapment were left flank pain 100% (n = 19), proteinuria 88% (n = 15), and hematuria 47% (n = 9). The mean peak velocity ratio on Doppler ultrasound was 6.13 (6-6.44, IQR). Aortomesenteric angle, beak angle (beak sign), and mean diameter ratio on computed tomography were 26° (22.6-28.5, IQR), 25° (23.9-28, IQR), and 5.3 (5-6, IQR), respectively. Venous pressure measurements were only used to confirm the diagnosis in 5 patients in the stenting group. The measured renocaval gradient was 4 (3.9-4.4, IQR) mm Hg. After both procedures, the classical symptoms, including left flank pain, proteinuria, and hematuria, resolved in 89.5% (n = 17), 57.8% (n = 11), and 82.3% (n = 15) of the cases, respectively. A total of 4 patients required reintervention, 3 patients after LRV transposition (occlusion, n = 2; stenosis, n = 1), and 1 patient after stenting (occlusion, n = 1). The 1-year and 3-year primary patency for the 19 patients was 87% and 80%, respectively. Three-year primary-assisted patency was 100%. Similarly, the 1-year and 3-year freedom from reintervention rate was 83% and 72%, respectively. Additionally, the 1-year and 3-year primary patency for the surgical group was 91% and 81%, respectively, and the 1-year and 3-year primary patency for the stenting group was 75%. CONCLUSIONS: Nutcracker syndrome should be kept in mind in cases where flank pain and hematuria cannot be associated with kidney diseases. Radiographic evidence must be accompanied by serious symptoms to initiate the treatment of nutcracker syndrome with LRV transposition and endovascular stenting procedures. Both procedures, along with their respective advantages and disadvantages, can be preferred as primary treatments for nutcracker syndrome. Our study demonstrates that both procedures can be safely and effectively performed, yielding good outcomes.


Renal Nutcracker Syndrome , Vascular Diseases , Humans , Female , Renal Veins/diagnostic imaging , Renal Veins/surgery , Flank Pain/etiology , Hematuria/etiology , Retrospective Studies , Treatment Outcome , Renal Nutcracker Syndrome/complications , Renal Nutcracker Syndrome/diagnostic imaging , Renal Nutcracker Syndrome/surgery , Vascular Diseases/complications , Proteinuria/complications
18.
J Cardiovasc Electrophysiol ; 35(4): 654-663, 2024 Apr.
Article En | MEDLINE | ID: mdl-38288888

INTRODUCTION: In hypertrophic cardiomyopathy (HCM), atrial fibrillation (AF) has historically been regarded to have a deleterious impact on clinical course, strongly associated with progressive heart failure (HF) symptoms. However, there is a paucity of information regarding the impact of AF on HCM employing validated quality of life (QoL) surveys. Therefore, we evaluated the impact of AF on QoL utilizing patient reported outcome measures (PROMs). METHODS: 218 consecutive HCM patients with or without AF at the Lahey HCM center in 2022 completed PROMs at their most recent visit evaluating HF (Kansas City Cardiomyopathy Questionnaire [KCCQ]) and AF symptoms (AF Effect on QoL [AFEQT]). RESULTS: Among the 218 patients, 50 (23%) had a history of AF and comprise the primary study cohort. AF was diagnosed at 55 ± 10 years of age, median of 5.5 years before PROM, with 66% of patients treated with a rhythm control strategy with antiarrhythmic drug and/or AF ablation. AFEQT indicated that 52% of patients experienced no or minimal AF-related disability, mild to moderate in 22%, and severe in 26%. There was no substantial difference in HCM phenotype in patients with no or minimal AF disability compared to those with severe disability. HF symptoms for most HCM patients with prior AF history was consistent with no or minimal (59%) or only mild (27%) disability as measured by KCCQ overall summary scores. In addition, with multivariate analysis, AF history was associated with less HF symptoms and improved QoL (OR 0.4, p = 0.02). CONCLUSION: In contrast to prior perceptions, HCM patients with prior AF history were less likely to incur HF symptoms impairing QoL compared to HCM patients without AF. After treatment, prior history of AF did not substantially impact current QoL. These data provide a realistic appraisal for the impact that AF has on HCM patients and also offers a measure of reassurance for this patient subgroup.


Atrial Fibrillation , Cardiomyopathy, Hypertrophic , Vascular Diseases , Humans , Quality of Life , Anti-Arrhythmia Agents/therapeutic use , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/therapy , Vascular Diseases/complications
20.
Asia Pac J Ophthalmol (Phila) ; 13(1): 100036, 2024.
Article En | MEDLINE | ID: mdl-38244930

Decades of studies on age-related macular degeneration (AMD), cardiovascular disease and stroke have not found consistent associations between AMD and systemic vascular disease. This study suggests that there is in fact no general relationship, but instead a strong, specific association between only the subretinal drusenoid deposit (SDD) phenotype of AMD on retinal imaging and certain co-existent vascular diseases that are high risk for compromised cardiac output or internal carotid artery stenosis. Future screening initiatives for these high -risk vascular diseases (HRVDs) with fast, inexpensive retinal imaging could make a significant contribution to public health and save lives. Likewise, screening patients with known HRVDs for unrecognized AMD of the SDD form could enable needed treatment and save vision.


Cardiovascular Diseases , Macular Degeneration , Retinal Drusen , Vascular Diseases , Humans , Retinal Drusen/diagnosis , Retinal Drusen/complications , Cardiovascular Diseases/complications , Cardiovascular Diseases/diagnosis , Tomography, Optical Coherence/methods , Macular Degeneration/complications , Macular Degeneration/diagnosis , Vascular Diseases/complications , Fluorescein Angiography
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