28 results on '"hypertrophic"'
Search Results
2. Activation of Wnt signaling in human fracture callus and nonunion tissues
- Author
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Michael Hadjiargyrou, Maria Kotsiopriftis, Dominique Lauzier, Reggie C. Hamdy, and Peter Kloen
- Subjects
Wnt ,Nonunion ,Fracture ,Callus ,Hypertrophic ,Oligotrophic ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
The Wnt signaling pathway is a key molecular process during fracture repair. Although much of what we now know about the role of this pathway in bone is derived from in vitro and animal studies, the same cannot be said about humans. As such, we hypothesized that Wnt signaling will also be a key process in humans during physiological fracture healing as well as in the development of a nonunion (hypertrophic and oligotrophic). We further hypothesized that the expression of Wnt-signaling pathway genes/proteins would exhibit a differential expression pattern between physiological fracture callus and the pathological nonunion tissues. We tested these two hypotheses by examining the mRNA levels of key Wnt-signaling related genes: ligands (WNT4, WNT10a), receptors (FZD4, LRP5, LRP6), inhibitors (DKK1, SOST) and modulators (CTNNB1 and PORCN). RNA sequencing from calluses as well as from the two nonunion tissue types, revealed that all of these genes were expressed at about the same level in these three tissue types. Further, spatial expression experiments identified the cells responsible of producing these proteins. Robust expression was detected in osteoblasts for the majority of these genes except SOST which displayed low expression, but in contrast, was mostly detected in osteocytes. Many of these genes were also expressed by callus chondrocytes as well. Taken together, these results confirm that Wnt signaling is indeed active during both human physiological fracture healing as well as in pathological nonunions.
- Published
- 2024
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- View/download PDF
3. Tuberculous hypertrophic pachymeningitis masquerading as meningioma: A case report
- Author
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Yu Peng, Min-li Lv, Hao Feng, and Jian-quan Zhong
- Subjects
Tuberculosis ,Hypertrophic ,Pachymeningitis ,Dura ,Surgery ,RD1-811 - Published
- 2024
- Full Text
- View/download PDF
4. Identification of the miRNAome in human fracture callus and nonunion tissues
- Author
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Michael Hadjiargyrou, Leonidas Salichos, and Peter Kloen
- Subjects
microRNA ,miRNA ,Nonunion ,Fracture ,Callus ,Hypertrophic ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Background: Nonunions remain a challenging post-traumatic complication that often leads to a financial and health burden that affects the patient's quality of life. Despite a wealth of knowledge about fracture repair, especially gene and more recently miRNA expression, much remains unknown about the molecular differences between normal physiological repair (callus tissue) and a nonunion. To probe this lack of knowledge, we embarked on a study that sought to identify and compare the human miRNAome of normal bone to that present in a normal fracture callus and those from two different classic nonunion types, hypertrophic and oligotrophic. Methods: Normal bone and callus tissue samples were harvested during revision surgery from patients with physiological fracture repair and nonunions (hypertrophic and oligotrophic) and analyzed using histology. Also, miRNAs were isolated and screened using microarrays followed by bioinformatic analyses, including, differential expression, pathways and biological processes, as well as elucidation of target genes. Results: Out of 30,424 mature miRNAs (from 203 organisms) screened via microarrays, 635 (∼2.1%) miRNAs were found to be upregulated and 855 (∼2.8%) downregulated in the fracture callus and nonunion tissues as compared to intact bone. As our tissue samples were derived from humans, we focused on the human miRNAs and out of the 4223 human miRNAs, 86 miRNAs (∼2.0%) were upregulated and 51 (∼1.2%) were downregulated. Although there were similarities between the three experimental samples, we also found specific miRNAs that were unique to individual samples. We further identified the predicted target genes from these differentially expressed miRNAs as well as the relevant biological processes, including specific signaling pathways that are activated in all three experimental samples. Conclusion: Collectively, this is the first comprehensive study reporting on the miRNAome of intact bone as compared to fracture callus and nonunion tissues. Further, we identify specific miRNAs involved in normal physiological fracture repair as well as those of nonunions. The translational potential of this article: The data generated from this study further increase our molecular understanding of the roles of miRNAs during normal and aberrant fracture repair and this knowledge can be used in the future in the development of miRNA-based therapeutics for skeletal regeneration.
- Published
- 2023
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5. Temporal trends, prevalence, predictors, and outcomes of heart failure in patients with hypertrophic cardiomyopathy in the United States: Insights from the national inpatient sample.
- Author
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Ismail MF, Obeidat O, Abughazaleh S, Daise MA, Alqudah Q, Tarawneh M, Alzghoul H, Al-Ani H, Iqbal J, and Ismail K
- Subjects
- Humans, Male, Female, United States epidemiology, Prevalence, Middle Aged, Retrospective Studies, Aged, Adult, Risk Factors, Time Factors, Databases, Factual, Cardiomyopathy, Hypertrophic epidemiology, Cardiomyopathy, Hypertrophic therapy, Cardiomyopathy, Hypertrophic complications, Heart Failure epidemiology, Heart Failure therapy, Hospital Mortality trends, Inpatients statistics & numerical data
- Abstract
Objectives: This study aims to delineate the temporal trends, prevalence, predictors, and outcomes of HF among HCM patients using the National Inpatient Sample (NIS) database, with a focus on optimizing therapeutic strategies and healthcare resources., Methods: We conducted a retrospective cohort analysis of anonymized data from the NIS spanning 2016 to 2019. The study population consisted of adults diagnosed with HCM based on specific ICD-10 diagnostic codes. Logistic regression was utilized to explore the association between HF and in-hospital mortality, adjusting for demographic and clinical factors., Results: Our analysis included 215,505 individuals, with 97,875 (45.4 %) experiencing HF. Patients with HF exhibited a higher burden of comorbidities such as diabetes and renal failure, and had increased odds of mortality (OR 1.41). The study also highlighted significant demographic disparities, with marked differences in outcomes based on race and gender. The economic analysis revealed higher healthcare costs and longer hospital stays associated with HF., Conclusion: HF significantly impacts mortality, healthcare costs, and hospitalization length in HCM patients, with substantial demographic and clinical disparities. This study underscores the importance of tailored management strategies and the need for continuous surveillance and research to address the challenges posed by HF in HCM., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)
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- 2024
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6. Unveiling the language of scars: A patient-centric themed framework for comprehensive scar morphology.
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Azzopardi E, Boyce D, Azzopardi E, Sadideen H, and Mosahebi A
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- Humans, Burns pathology, Patient-Centered Care, Cicatrix pathology, Terminology as Topic
- Abstract
Background: Scarring, a pervasive issue spanning across medical disciplines, lacks a comprehensive terminology for effective communication, patient engagement, and outcome assessment. Existing scar classification systems are constrained by specific pathologies, physician-centric features, and inadequately account for emerging technologies. This study refrains from proposing yet another classification system and instead revisits the foundational language of scar morphology through a theme analysis of primary patient complaints., Method: Data encompassing five years of a high-volume scar practice was analysed. Primary complaints were aggregated into collective descriptors and further organized into theme domains. The resulting hierarchical map of presenting complaints revealed five key domains: Loss of Function, Contour, Texture, Vector, and Colour Presenting complaints were codified into 42 items, which were then categorised into 14 collective descriptor terms. The latter were in turn organised into five overarching themes., Result: Loss of Function, accounting for 10% of primary concerns, signifies reduced function attributed solely to the scar. Contour, encompassing 41% of concerns, pertains to scar height, shape, and depth. Texture, representing 12% of concerns, denotes tactile variations such as hardness, roughness, and moisture. Vector, comprising 13% of concerns, refers to scar tissue tension and associated distortions. Colour, the concern in 24% of cases, encompasses variations in pigmentation, vascularity, and exogenous pigments., Discussion: Standardized terminology enhances patient care, communication, and research. This study underscores the fundamental question of "what bothers the patient," reviving a patient-centred approach to scar management. By prioritizing themes based on patient complaints, this study innovatively integrates function, aesthetics, and patient experience. In conclusion, this study pioneers a paradigm shift in scar management by presenting a patient-driven theme framework that offers a common language for healthcare professionals and patients. Embracing this language harmonizes scar treatment, fosters innovation, and transforms scars from silent reminders into stories of resilience and healing., Competing Interests: Declaration of Competing Interest The authors declare they have no conflict of interest whatsoever in the production of this manuscript This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., (Crown Copyright © 2024. Published by Elsevier Ltd. All rights reserved.)
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- 2024
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7. Tuberculous hypertrophic pachymeningitis masquerading as meningioma: A case report.
- Author
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Peng Y, Lv ML, Feng H, and Zhong JQ
- Subjects
- Female, Humans, Diagnosis, Differential, Hypertrophy, Magnetic Resonance Imaging, Meningitis diagnosis, Meningitis microbiology, Middle Aged, Meningeal Neoplasms diagnosis, Meningeal Neoplasms diagnostic imaging, Meningioma diagnosis, Meningioma diagnostic imaging, Tuberculosis, Meningeal diagnosis
- Published
- 2024
- Full Text
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8. Identification of the miRNAome in human fracture callus and nonunion tissues.
- Author
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Hadjiargyrou M, Salichos L, and Kloen P
- Abstract
Background: Nonunions remain a challenging post-traumatic complication that often leads to a financial and health burden that affects the patient's quality of life. Despite a wealth of knowledge about fracture repair, especially gene and more recently miRNA expression, much remains unknown about the molecular differences between normal physiological repair (callus tissue) and a nonunion. To probe this lack of knowledge, we embarked on a study that sought to identify and compare the human miRNAome of normal bone to that present in a normal fracture callus and those from two different classic nonunion types, hypertrophic and oligotrophic., Methods: Normal bone and callus tissue samples were harvested during revision surgery from patients with physiological fracture repair and nonunions (hypertrophic and oligotrophic) and analyzed using histology. Also, miRNAs were isolated and screened using microarrays followed by bioinformatic analyses, including, differential expression, pathways and biological processes, as well as elucidation of target genes., Results: Out of 30,424 mature miRNAs (from 203 organisms) screened via microarrays, 635 (∼2.1%) miRNAs were found to be upregulated and 855 (∼2.8%) downregulated in the fracture callus and nonunion tissues as compared to intact bone. As our tissue samples were derived from humans, we focused on the human miRNAs and out of the 4223 human miRNAs, 86 miRNAs (∼2.0%) were upregulated and 51 (∼1.2%) were downregulated. Although there were similarities between the three experimental samples, we also found specific miRNAs that were unique to individual samples. We further identified the predicted target genes from these differentially expressed miRNAs as well as the relevant biological processes, including specific signaling pathways that are activated in all three experimental samples., Conclusion: Collectively, this is the first comprehensive study reporting on the miRNAome of intact bone as compared to fracture callus and nonunion tissues. Further, we identify specific miRNAs involved in normal physiological fracture repair as well as those of nonunions., The Translational Potential of This Article: The data generated from this study further increase our molecular understanding of the roles of miRNAs during normal and aberrant fracture repair and this knowledge can be used in the future in the development of miRNA-based therapeutics for skeletal regeneration., Competing Interests: The authors declare that they have no conflicts of interest with the contents of this article., (© 2023 Published by Elsevier B.V. on behalf of Chinese Speaking Orthopaedic Society.)
- Published
- 2023
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9. Predictors of Fabry disease in patients with hypertrophic cardiomyopathy: how to guide the diagnostic strategy?
- Author
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Rui Azevedo Guerreiro, Paulo Gaspar, Nuno Sousa, Carolina Lourenço, Gabriel Miltenberger-Miltenyi, N Craveiro, Olga Azevedo, Fernando Sá, Ricardo Faria, Rui Lima, Damião Cunha, Rui Faria, H Antunes, Renata Gomes, Liliana Reis, Inês Cruz, and Nuno Marques
- Subjects
Adult ,Male ,medicine.medical_specialty ,Cardiomyopathy ,macromolecular substances ,030204 cardiovascular system & hematology ,Bifascicular block ,03 medical and health sciences ,Basal (phylogenetics) ,0302 clinical medicine ,Discriminant function analysis ,Internal medicine ,Medicine ,Humans ,In patient ,cardiovascular diseases ,030212 general & internal medicine ,Aged ,Fabry disease ,business.industry ,Hypertrophic cardiomyopathy ,Cardiomyopathy, Hypertrophic ,Middle Aged ,medicine.disease ,Diagnostic strategy ,3. Good health ,Pedigree ,Hypertrophic ,cardiovascular system ,Cardiology ,Etiology ,Fabry Disease ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background Fabry disease (FD) is a treatable cause of hypertrophic cardiomyopathy (HCM). We aimed to determine the independent predictors of FD and to define a clinically useful strategy to discriminate FD among HCM. Methods Multicenter study including 780 patients with the ESC definition of HCM. FD screening was performed by enzymatic assay in males and genetic testing in females. Multivariate regression analysis identified independent predictors of FD in HCM. A discriminant function analysis defined a score based on the weighted combination of these predictors. Results FD was found in 37 of 780 patients with HCM (4.7%): 31 with p.F113L mutation due to a founder effect; and 6 with other variants (p.C94S; p.M96V; p.G183V; p.E203X; p.M290I; p.R356Q/p.G360R). FD prevalence in HCM adjusted for the founder effect was 0.9%. Symmetric HCM (OR 3.464, CI95% 1.151-10.430), basal inferolateral late gadolinium enhancement (LGE) (OR 10.677, CI95% 3.633-31.380), bifascicular block (OR 10.909, CI95% 2.377-50.059) and ST-segment depression (OR 4.401, CI95% 1.431-13.533) were independent predictors of FD in HCM. The score ID FABRY-HCM [−0.729 + (2.781xBifascicular block) + (0.590xST depression) + (0.831xSymmetric HCM) + (2.130xbasal inferolateral LGE)] had a negative predictive value of 95.8% for FD, with a cut-off of 1.0, meaning that, in the absence of both bifascicular block and basal inferolateral LGE, FD is a less probable cause of HCM, being more appropriate to perform HCM gene panel than targeted FD screening. Conclusion FD prevalence in HCM was 0.9%. Bifascicular block and basal inferolateral LGE were the most powerful predictors of FD in HCM. In their absence, HCM gene panel is the most appropriate step in etiological study of HCM.
- Published
- 2020
10. Early physiotherapy experience with a biodegradable polyurethane dermal substitute: Therapy guidelines for use.
- Author
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Schmitt B, Heath K, Kurmis R, Klotz T, Wagstaff MJD, and Greenwood J
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- Adult, Cicatrix, Contracture etiology, Contracture surgery, Humans, Learning Curve, Polyurethanes, Retrospective Studies, Treatment Outcome, Burns surgery, Physical Therapy Modalities, Range of Motion, Articular, Skin, Artificial
- Abstract
Objective: The purpose of this study was to investigate and develop range of motion (ROM) and mobilisation guidelines in adult patients where a newly developed synthetic dermal substitute was applied in our adult burn centre., Method: A retrospective case note audit was conducted on the first 20 acute burn injured patients who had a synthetic dermal substitute applied. Data collected included days to commencement of ROM, days to clearance for mobilisation, and joint ROM achieved after dermal substitute application (prior to delamination) and after split skin grafting (SSG) for the elbow, knee and shoulder joints. Scar assessments were completed at 12 months after injury using two scar assessment scales., Results: Clearance to mobilise occurred at mean 10.4 and 4.9 days after dermal substitute and after skin graft application to lower limbs respectively. ROM commenced at a mean of 9.9 (upper limbs) and 12.7 (lower limbs) days after dermal substitute application. Following skin grafting, ROM commenced at a mean of 6.6 and 6.5 days for upper limbs and lower limbs respectively. Prior to dermal substitute delamination mean flexion at the knee (86.3°), elbow (114.0°) and shoulder (143.4°) was achieved. Mean ROM continued to improve after grafting with knee (133.2°), elbow (126.1°) and shoulder (151.0°) flexion approaching normal ROM in most cases. Mean extension of the elbow (-4.6°) was maintained close to normal levels after skin grafting. There were no recorded instances of knee extension contracture. Patient and Observer Scar Assessment Scale and Matching Assessment of Photographs of Scars scores indicated good cosmetic outcomes with relatively low levels of itch and minimal pain reported at 12 months after injury., Conclusion: A steep learning curve was encountered in providing therapy treatment for patients managed with this relatively new synthetic dermal substitute. Trends indicated that as experience with this new dermal substitute grew, patients progressed toward active therapy earlier. A guideline for therapy treatment has been developed but will continue to be evaluated and adjusted when required., (Crown Copyright © 2020. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2021
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11. Diagnosis of apical hypertrophic cardiomyopathy: T-wave inversion and relative but not absolute apical left ventricular hypertrophy☆
- Author
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Flett, Andrew S., Maestrini, Viviana, Milliken, Don, Fontana, Mariana, Treibel, Thomas A., Harb, Rami, Sado, Daniel M., Quarta, Giovanni, Herrey, Anna, Sneddon, James, Elliott, Perry, McKenna, William, and Moon, James C.
- Subjects
Male ,Heart Ventricles ,Magnetic Resonance Imaging, Cine ,hypertrophic ,Coronary Angiography ,Article ,Electrocardiography ,magnetic resonance imaging ,Humans ,cardiovascular diseases ,medicine (all) ,Aged ,Retrospective Studies ,cine ,Cardiomyopathy, Hypertrophic ,Middle Aged ,Hypertrophic cardiomyopathy ,cardiology and cardiovascular medicine ,cardiovascular magnetic resonance ,hypertrophic cardiomyopathy ,aged ,cardiomyopathy, hypertrophic ,coronary angiography ,electrocardiography ,female ,heart ventricles ,humans ,hypertrophy, left ventricular ,magnetic resonance imaging, cine ,male ,middle aged ,retrospective studies ,cardiovascular system ,Cardiovascular magnetic resonance ,Female ,Hypertrophy, Left Ventricular ,hypertrophy ,left ventricular ,cardiomyopathy - Abstract
Background Diagnosis of apical HCM utilizes conventional wall thickness criteria. The normal left ventricular wall thins towards the apex such that normal values are lower in the apical versus the basal segments. The impact of this on the diagnosis of apical hypertrophic cardiomyopathy has not been evaluated. Methods We performed a retrospective review of 2662 consecutive CMR referrals, of which 75 patients were identified in whom there was abnormal T-wave inversion on ECG and a clinical suspicion of hypertrophic cardiomyopathy. These were retrospectively analyzed for imaging features consistent with cardiomyopathy, specifically: relative apical hypertrophy, left atrial dilatation, scar, apical cavity obliteration or apical aneurysm. For comparison, the same evaluation was performed in 60 healthy volunteers and 50 hypertensive patients. Results Of the 75 patients, 48 met conventional HCM diagnostic criteria and went on to act as another comparator group. Twenty-seven did not meet criteria for HCM and of these 5 had no relative apical hypertrophy and were not analyzed further. The remaining 22 patients had relative apical thickening with an apical:basal wall thickness ratio > 1 and a higher prevalence of features consistent with a cardiomyopathy than in the control groups with 54% having 2 or more of the 4 features. No individual in the healthy volunteer group had more than one feature and no hypertension patient had more than 2. Conclusion A cohort of individuals exist with T wave inversion, relative apical hypertrophy and additional imaging features of HCM suggesting an apical HCM phenotype not captured by existing diagnostic criteria., Highlights • The normal left ventricular myocardium tapers towards the apex. • There exists an apical HCM variant with relative rather than absolute apical hypertrophy. • T-wave inversion and relative apical hypertrophy are core features. • Additional features include a dilated left atrium, scarring, apical cavity obliteration and apical microaneurysms.
- Published
- 2015
12. A severe fatal case of Darier-White disease—an extreme phenotype or a new entity?
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Sima Halevy, Elena Kahn, and Guy Shalom
- Subjects
Pathology ,medicine.medical_specialty ,comedonal ,retinoids ,business.industry ,Hyperkeratosis ,Case Report ,hypertrophic ,Dermatology ,Disease ,DWD, Darier-White disease ,medicine.disease ,Phenotype ,Dyskeratosis ,DARIER-WHITE DISEASE ,medicine.anatomical_structure ,dyskeratosis follicularis ,Nail (anatomy) ,medicine ,Darier-White disease ,Oral mucosa ,Keratoderma ,business - Abstract
Darier-White disease (DWD) is an autosomal-dominant skin disease characterized by warty keratotic papules and plaques in a primarily seborrheic distribution. Vegetating papules, erosions, or blisters may sometimes be present. Other findings may include varieties of nail abnormalities, “cobblestoning” of the oral mucosa, filiform keratoderma on the palms and soles, and hyperkeratosis verruciformis of Hopf. DWD has an unpredictable clinical course, and patients may present with variable extents of involvement and diverse morphologies at different body areas.1, 2 Neuropsychiatric abnormalities and immunologic aberrations have been reported in association with DWD.3, 4 Clinical subtypes reported to date include hypertrophic,5 vesicobullous, hypopigmented, cornifying, zosteriform or linear, and comedonal variants.6 DWD has recently been linked to mutations in the SERCA2 gene, which encodes the calcium ATPase pump in the sarcoendoplasmic reticulum. Alterations in calcium signaling cause aberrant keratinocyte differentiation. The result is characteristic dyskeratosis follicularis, demonstrated by the presence of typical dyskeratotic cells, such as, corps ronds and grains. Treatments may include emollients, topical retinoids, topical steroids, and intermittent courses of oral antibiotics and systemic retinoids. We present a unique case of DWD showing overlap between several clinical subtypes of the disease with a 20-year course and fatal outcome.
- Published
- 2015
13. Neurosurgical and neuro-immunological management of IgG4-related hypertrophic sclerosing pachymeningitis. A literature survey and discussion of a unique index case.
- Author
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Elmaci I, Altinoz MA, Akdemir G, Sari R, Baskan O, Ozpinar A, Hacker E, and Sav A
- Subjects
- Autoimmune Diseases diagnosis, Autoimmune Diseases immunology, Diagnosis, Differential, Humans, Hypertrophy immunology, Hypertrophy surgery, Meningitis immunology, Meningitis surgery, Neoplasm Recurrence, Local immunology, Neoplasm Recurrence, Local surgery, Hypertrophy diagnosis, Immunoglobulin G immunology, Meningitis diagnosis, Neoplasm Recurrence, Local diagnosis
- Abstract
Background: Dural thickening is observed in lymphoma, dural carcinomatosis, meningioma, tuberculosis, and autoimmune diseases. We encountered a patient with dural thickening and complaints of neck and back pain, numbness and loss of strength in the hands. The patient also suffered from polychondritis and had previously received steroid and methotrexate treatment for this indication. The patients' serum was also positive for ANA, yet she did not have any other findings suggesting lupus. Our radiological and pathological analysis revealed IHSP (IgG4-related hypertrophic sclerosing pachymeningitis). In this review study, we provided a detailed literature survey to increase the awareness about IHSP in the neurosurgical community., Methods: MRI (magnetic resonance imaging)-based radiological analyses revealed a posterior extramedullary spinal mass extending from C2 to T2-T3 level. The dural mass was surgically excised and a broad panel of immunohistochemical markers including S100, EMA, CD246/ALK-1, CD45, CD20, CD79a, CD138, CD68, CD1a and CD34 was studied. Immunoglobulin heavy chain/kappa chain gene rearrangement analysis was performed which ruled out a lymphoproliferative disorder., Results: MRI and pathological findings suggested IHSP. As the disease relapsed with a new anterior extramedullary multilobulated lesion extending from C5 to T1 level, the patient is now closely monitored for further medical and surgical treatment., Conclusions: IHSP is a relatively novel entity of hypertrophic pachymeningitis and should be included in the differential diagnosis of dural thickening. The fibrosis accompanying IHSP may not respond to medical treatment, which includes steroids and immunosuppressive agents. Additionally, neurological deficits, seizures, spinal decompression, hydrocephalus, or brainstem compression necessitate early surgical intervention. A continued vigilance is also necessary as the disease may relapse long-term following surgical treatment., (Copyright © 2020 Elsevier B.V. All rights reserved.)
- Published
- 2021
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14. Pediatric burn contractures in low- and lower middle-income countries: A systematic review of causes and factors affecting outcome.
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Meng F, Zuo KJ, Amar-Zifkin A, Baird R, Cugno S, and Poenaru D
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- Body Surface Area, Burns complications, Burns pathology, Child, Cicatrix, Hypertrophic epidemiology, Cicatrix, Hypertrophic etiology, Contracture etiology, Developing Countries, Health Care Costs statistics & numerical data, Health Knowledge, Attitudes, Practice, Humans, Risk Factors, Trauma Severity Indices, Burns surgery, Contracture epidemiology, Educational Status, Health Services Accessibility, Plastic Surgery Procedures statistics & numerical data, Social Class, Time-to-Treatment statistics & numerical data
- Abstract
In low- and lower middle-income countries (LMICs), timely access to primary care following thermal injury is challenging. Children with deep burns often fail to receive specialized burn care until months or years post-injury, thus suffering impairments from hypertrophic scarring or joint and soft tissue contractures. We aimed to examine the correlation between limited access to care following burn injury and long-term disability in children in LMICs and to identify specific factors affecting the occurrence of late burn complications. A systematic literature search was conducted to retrieve articles on pediatric burns in LMICs using Medline, Embase, the Cochrane Library, LILACS, Global Health, African Index Medicus, and others. Articles were assessed by two reviewers and reported in accordance with PRISMA guidelines. Of 2896 articles initially identified, 103 underwent full-text review and 14 met inclusion criteria. A total of 991 children who developed long-term burn sequelae were included. Time from injury to consultation ranged from a few months to 17 years. Factors associated with late complications included total body surface area burned, burn depth, low socio-economic status, limited infrastructure, perceived inability to pay, lack of awareness of surgical treatment, low level of maternal education, and time elapsed between burn injury and reconstructive surgery., (Copyright © 2019. Published by Elsevier Ltd.)
- Published
- 2020
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15. Lifestyle Modification and Medical Management of Hypertrophic Cardiomyopathy.
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Heitner SB and Fischer KL
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- Adrenergic beta-Antagonists therapeutic use, Calcium Channel Blockers therapeutic use, Cardiotonic Agents therapeutic use, Diet, Healthy, Disopyramide therapeutic use, Exercise Therapy methods, Health Status, Humans, Obesity prevention & control, Ranolazine therapeutic use, Sleep Apnea Syndromes prevention & control, Cardiomyopathy, Hypertrophic therapy, Healthy Lifestyle, Risk Reduction Behavior
- Abstract
Hypertrophic cardiomyopathy is a heterogenous condition associated with a myriad of symptoms. Just as in other disease states, the aim of medical therapy is the alleviation of suffering, improvement of longevity, and the prevention of complications. This article focuses on the associated comorbidities seen in patients with hypertrophic cardiomyopathy, potential lifestyle interventions, and conventional medical treatments for symptomatic hypertrophic cardiomyopathy., (Copyright © 2018 Elsevier Inc. All rights reserved.)
- Published
- 2019
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16. Cost Analyses of Genomic Sequencing: Lessons Learned from the MedSeq Project.
- Author
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Christensen KD, Phillips KA, Green RC, and Dukhovny D
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- Cardiology Service, Hospital standards, Cost-Benefit Analysis, Genomics instrumentation, Genomics trends, Humans, Primary Health Care standards, Whole Genome Sequencing standards, Genomics methods, Whole Genome Sequencing economics
- Abstract
Objective: To summarize lessons learned while analyzing the costs of integrating whole genome sequencing into the care of cardiology and primary care patients in the MedSeq Project by conducting the first randomized controlled trial of whole genome sequencing in general and specialty medicine., Methods: Case study that describes key methodological and data challenges that were encountered or are likely to emerge in future work, describes the pros and cons of approaches considered by the study team, and summarizes the solutions that were implemented., Results: Major methodological challenges included defining whole genome sequencing, structuring an appropriate comparator, measuring downstream costs, and examining clinical outcomes. Discussions about solutions addressed conceptual and practical issues that arose because of definitions and analyses around the cost of genomic sequencing in trial-based studies., Conclusions: The MedSeq Project provides an instructive example of how to conduct a cost analysis of whole genome sequencing that feasibly incorporates best practices while being sensitive to the varied applications and diversity of results it may produce. Findings provide guidance for researchers to consider when conducting or analyzing economic analyses of whole genome sequencing and other next-generation sequencing tests, particularly regarding costs., (Copyright © 2018 ISPOR–The Professional Society for Health Economics and Outcomes Research. Published by Elsevier Inc. All rights reserved.)
- Published
- 2018
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17. Triamcinolone injection for treatment of Mitrofanoff stomal stenosis: Optimizing results and reducing cost of care.
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Reddy PP, Strine AC, Reddy T, Noh PH, DeFoor WR Jr, Minevich E, Sheldon CA, and VanderBrink BA
- Subjects
- Child, Constriction, Pathologic, Female, Humans, Injections, Male, Retrospective Studies, Urinary Catheterization instrumentation, Anti-Inflammatory Agents administration & dosage, Cystostomy adverse effects, Surgical Stomas pathology, Triamcinolone administration & dosage, Urinary Catheterization adverse effects
- Abstract
Introduction: Stomal stenosis is the most common complication after the creation of a continent catheterizable channel (CCC), but is challenging to treat., Objective: To describe early experience with triamcinolone injection for the treatment of stomal stenosis., Materials and Methods: A retrospective review was performed of patients who had undergone a triamcinolone injection (40 mg/ml) for the treatment of stomal stenosis at the present institution. The primary outcome of success was defined as a patient-reported or caregiver-reported return to ease with catheterization, and avoidance of stomal revision or indwelling catheter. The cost of care with various techniques for the treatment of stomal stenosis was also assessed by representing the cost as a percentage of charges for a re-operative Monti ileovesicostomy., Results: A total of 22 injections were performed in 18 CCCs of 17 patients. Demographic and clinical data are provided in the Summary Table. Thirteen CCCs (72%) were successfully treated with a single injection. Three patients (10%) required a total of five repeat injections at a median of 5.1 months between injections (IQR, 4.6-12.4). One patient required a stomal revision at 34.9 months after the initial injection, while one patient also required a Chait cecostomy catheter. The median length of time for ease with catheterization was 11.6 months (IQR, 3.5-18.0) after the initial injection, and 10.4 months (IQR, 4.5-16.0) after any injection. No adverse effects or complications occurred from the injection. There were no variables associated with failure after the initial or any injection on univariate analysis. Represented as a percentage of charges for a reoperative Monti ileovesicostomy, the cost of care was 11.3% for a stomal revision, 5.8% for triamcinolone injection in the operating room, and 0.3% as an office-based procedure., Discussion: The success rate for triamcinolone injection favorably compared with other options for the treatment of stomal stenosis. It surpassed the reported success rate of stomal dilation and approached that of stomal revision. Other studies have similarly observed a minimal risk of adverse effects and complications from both topical and intralesional corticosteroids. The limitations of the present study included its retrospective design at a single institution. A larger cohort of patients with a longer follow-up is necessary to determine its long-term durability., Conclusions: Triamcinolone injection was an effective and well-tolerated option for the treatment of stomal stenosis in any cutaneous stoma, thereby avoiding the need for a more invasive and costly stomal revision., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2017
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18. Hypertrophic olivary degeneration: Case series and review of literature.
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Sabat S, Mannering N, and Agarwal A
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- Adult, Aged, Diagnosis, Differential, Female, Humans, Hypertrophy, Male, Middle Aged, Neurodegenerative Diseases therapy, Olivary Nucleus diagnostic imaging, Neurodegenerative Diseases diagnostic imaging, Neurodegenerative Diseases physiopathology
- Abstract
Hypertrophic Olivary Degeneration (HOD) is a rare neurological condition caused by trans-synaptic degeneration in the brainstem and cerebellum, resulting in varied clinical symptoms, classical being palatal tremors, along with characteristic imaging presentation. Large number of pathologic lesions can cause this condition, ranging from ischemic stroke to neoplasm. The most common conditions include hemorrhage, vascular malformations and infarct. Magnetic resonance scan (MRI) is the imaging modality of choice which shows signal changes with hypertrophy of the inferior olivary nucleus, as well as the primary underlying pathology. Through this pictorial essay, we present the imaging and clinical findings in a number of patients with HOD secondary to varied causes and explain the mechanism behind the changes., (Copyright © 2016 Elsevier B.V. All rights reserved.)
- Published
- 2016
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19. The role of intraoperative transesophageal echocardiographic monitoring in a patient with hypertrophic cardiomyopathy undergoing laparoscopic surgery.
- Author
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Gregory SH and Fierro MA
- Subjects
- Aged, Humans, Insufflation adverse effects, Male, Prostatectomy adverse effects, Robotic Surgical Procedures adverse effects, Ventricular Outflow Obstruction physiopathology, Cardiomyopathy, Hypertrophic diagnosis, Echocardiography, Transesophageal, Laparoscopy adverse effects, Monitoring, Intraoperative methods, Perioperative Care methods, Prostatic Neoplasms surgery
- Abstract
Hypertrophic cardiomyopathy (HCM) presents a significant perioperative challenge. Anesthetic drugs, patient positioning, and surgical technique can provoke worsening left ventricular outflow tract obstruction and hemodynamic deterioration. In this case report, we present the perioperative management of a 70-year-old male with a history of HCM who underwent a robotic laparoscopic prostatectomy. Discussion focuses on the utilization of echocardiographic guidance in the care of patients with HCM undergoing noncardiac surgery, as well as the pathophysiology of laparoscopic insufflation and its effects on left ventricular outflow tract obstruction in HCM., (Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.)
- Published
- 2016
- Full Text
- View/download PDF
20. Noncalcified Hypertrophic Ligamentum Flavum Causing Severe Cervical Stenosis and Myelopathy: Case Report and Review of the Literature.
- Author
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Coughlin DJ, Rymarczuk GN, and Dirks MS
- Subjects
- Cervical Vertebrae surgery, Decompression, Surgical, Female, Humans, Hypertrophy, Ligamentum Flavum surgery, Middle Aged, Spinal Cord Compression etiology, Spinal Cord Compression surgery, Spinal Fusion, Spinal Stenosis complications, Spinal Stenosis surgery, Cervical Vertebrae diagnostic imaging, Ligamentum Flavum pathology, Spinal Cord Compression diagnostic imaging, Spinal Stenosis diagnostic imaging
- Abstract
Background: Calcified hypertrophic ligamentum flavum is a known entity that causes myeloradiculopathy of the cervical, thoracic, and lumbar spine and is seen more commonly in Asian populations. Noncalcified hypertrophic changes are less common and may mimic other epidural space-occupying lesions., Case Description: A 59-year-old woman presented with progressive myelopathy, and imaging studies were consistent with an epidural space-occupying lesion from C4-T3. The patient underwent posterior cervical decompression and fusion with instrumentation. Pathology specimens revealed noncalcified hypertrophic ligamentum flavum., Conclusions: To our knowledge, noncalcified hypertrophic ligamentum flavum causing progressive cervical myelopathy has never been reported in the English literature. This entity should be considered in cases with epidural masses causing progressive myelopathy., (Published by Elsevier Inc.)
- Published
- 2016
- Full Text
- View/download PDF
21. Left ventricular noncompaction mimicking hypertrophic obstructive cardiomyopathy.
- Author
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Hamada M, Takamura Y, Otani T, Ohshima K, Ogimoto A, Ikeda S, and Horii T
- Subjects
- Adult, Diagnosis, Differential, Female, Humans, Cardiomyopathy, Hypertrophic diagnostic imaging, Cardiomyopathy, Hypertrophic physiopathology, Isolated Noncompaction of the Ventricular Myocardium diagnostic imaging, Isolated Noncompaction of the Ventricular Myocardium physiopathology
- Published
- 2016
- Full Text
- View/download PDF
22. Exercise restrictions for patients with inherited cardiac conditions: Current guidelines, challenges and limitations.
- Author
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Hammond-Haley M, Patel RS, Providência R, and Lambiase PD
- Subjects
- Brugada Syndrome diagnosis, Brugada Syndrome epidemiology, Brugada Syndrome physiopathology, Cardiac Conduction System Disease, Death, Sudden, Cardiac epidemiology, Heart Diseases diagnosis, Heart Diseases epidemiology, Humans, Death, Sudden, Cardiac prevention & control, Exercise physiology, Heart Diseases physiopathology, Practice Guidelines as Topic standards
- Abstract
Inherited primary arrhythmia syndromes are a clinically heterogeneous group of relatively uncommon but important inherited cardiac conditions that are associated with an increased risk of sudden cardiac death (SCD) in the setting of a structurally normal heart. These include long-QT syndrome (LQTS), Short-QT syndrome (SQTS), Brugada syndrome (BrS) and Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT). The cardiomyopathies represent the other major group of inherited cardiac conditions associated with SCD, of which hypertrophic cardiomyopathy (HCM) is the most common. Exercise is a known trigger of ventricular arrhythmias in many of these conditions, however marked genetic and clinical heterogeneity within individual diseases means that certain patients are at a much greater risk of lethal ventricular arrhythmias during exercise than others. For instance, LQTS type 1 (LQT1) and CPVT patients are at particular risk during exertion, whilst in patients with other genetic variants of LQTS, BrS and SQTS, alternative triggers are more significant precipitants. Many channelopathy (principally Brugada, CPVT) & cardiomyopathy (mainly HCM) patients receive primary or secondary prevention therapy with an implantable cardiac defibrillator (ICD). Exercising with an ICD in situ carries a range of additional risks including inappropriate shocks and lead complications. This review will focus on the risk of exercise-induced SCD in patients with inherited cardiac conditions, the current clinical guidelines in this area and the special consideration of patients with an ICD., (Copyright © 2016. Published by Elsevier Ireland Ltd.)
- Published
- 2016
- Full Text
- View/download PDF
23. Measuring the impact of burn scarring on health-related quality of life: Development and preliminary content validation of the Brisbane Burn Scar Impact Profile (BBSIP) for children and adults.
- Author
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Tyack Z, Ziviani J, Kimble R, Plaza A, Jones A, Cuttle L, and Simons M
- Subjects
- Adolescent, Adult, Aged, Burns psychology, Caregivers psychology, Child, Cross-Sectional Studies, Female, Health Status, Humans, Male, Middle Aged, Prospective Studies, Surveys and Questionnaires, Young Adult, Burns complications, Cicatrix psychology, Quality of Life, Sickness Impact Profile
- Abstract
Introduction: No burn-scar specific, health-related quality of life (HRQOL) measure exists. This study aimed to develop a patient-reported, evaluative HRQOL measure to assess the impact of burn scarring in children and adults., Method: Semi-structured interviews, content validation surveys, and cognitive interviews were used to develop and test content validity of a new measure - the Brisbane Burn Scar Impact Profile (BBSIP)., Results: Participants comprised Australian adults (n=23) and children (n=19) with burn scarring; caregivers of children with burn scarring (n=28); and international scar management experts (n=14). Items distinct from other burn scar measures emerged. Four versions of the BBSIP were developed; one for children aged 8-18 years, one for adults, one for caregivers (as proxies for children aged less than 8-years), and one for caregivers of children aged 8-18 years. Preliminary content validity of the BBSIP was supported. Final items covered physical and sensory symptoms; emotional reactions; impact on social functioning and daily activities; impact of treatment; and environmental factors., Conclusion: The BBSIP was developed to assess burn-scar specific HRQOL and will be available at http://www.coolburns.com.au under a creative commons license. Further testing is underway., (Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.)
- Published
- 2015
- Full Text
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24. Role of Imaging in the Evaluation of Patients at Risk for Sudden Cardiac Death: Genotype-Phenotype Intersection.
- Author
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Nagueh SF and Zoghbi WA
- Subjects
- Animals, Arrhythmogenic Right Ventricular Dysplasia diagnosis, Arrhythmogenic Right Ventricular Dysplasia genetics, Cardiomyopathy, Dilated diagnosis, Cardiomyopathy, Dilated genetics, Cardiomyopathy, Hypertrophic genetics, Genetic Testing, Heart Defects, Congenital diagnosis, Heart Defects, Congenital genetics, Humans, Death, Sudden, Cardiac, Diagnostic Imaging, Genetic Association Studies
- Abstract
Several monogenic cardiac disorders are associated with sudden cardiac death. These include primary electrophysiological disorders (long QT, short QT, Brugada, and catecholaminergic ventricular tachycardia syndromes) and cardiomyopathies, including hypertrophic and dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. This review presents the genotype-phenotype relations in these diseases. In addition to establishing a diagnosis, imaging can play an important role in identifying the pathophysiological abnormalities and hence the potential to guide therapy in some patients. Furthermore, imaging findings in some patients add incremental prognostic information for risk stratification of sudden cardiac death. Finally, along with genetic testing, imaging is important for screening and, in some cases, can identify subjects with positive mutations who have not yet developed the classical phenotype., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)
- Published
- 2015
- Full Text
- View/download PDF
25. Prognostic difference between paroxysmal and non-paroxysmal atrial fibrillation in patients with hypertrophic cardiomyopathy.
- Author
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Yashiro B, Minami Y, Terajima Y, and Hagiwara N
- Subjects
- Adult, Aged, Atrial Fibrillation classification, Atrial Fibrillation diagnosis, Cohort Studies, Electrocardiography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Multivariate Analysis, Prognosis, Risk, Syncope etiology, Tachycardia, Ventricular etiology, Time Factors, Atrial Fibrillation complications, Cardiomyopathy, Hypertrophic complications, Death, Sudden, Cardiac etiology
- Abstract
Background: The association of atrial fibrillation (AF) with sudden death and the difference in prognostic significance between paroxysmal and non-paroxysmal AF remains unclear in patients with hypertrophic cardiomyopathy (HCM). Our aim was to investigate the clinical significance of AF, and to assess the prognostic difference between paroxysmal and non-paroxysmal AF in HCM patients., Methods: The study included 430 HCM patients. Documentation of AF was based on electrocardiograms obtained either after the acute onset of symptoms or fortuitously during routine examination of asymptomatic patients., Results: AF was detected in 120 patients (27.9%). In the patients with AF, syncope and non-sustained ventricular tachycardia were more frequent and the left atrial dimension was larger. Multivariate analysis showed that AF was an independent determinant of the outcome, including the risk of HCM-related death (adjusted hazard ratio 3.57, p<0.001) and sudden death (adjusted hazard ratio 2.61, p=0.038). When patients with AF were divided into subgroups with paroxysmal AF (n=75) or non-paroxysmal AF (n=45), only paroxysmal AF was identified as an independent determinant of the outcome, including the risk of HCM-related death (adjusted hazard ratio 5.24, p<0.001) and sudden death (adjusted hazard ratio 4.67, p=0.002)., Conclusions: AF is a common supraventricular arrhythmia in HCM and has an adverse influence on the prognosis. In addition, each type of AF had a different clinical impact, with paroxysmal AF being a significant independent determinant of an adverse outcome, including sudden death., (Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2014
- Full Text
- View/download PDF
26. Pregnancy in inherited and acquired cardiomyopathies.
- Author
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Herrey AS
- Subjects
- Arrhythmogenic Right Ventricular Dysplasia diagnosis, Arrhythmogenic Right Ventricular Dysplasia physiopathology, Arrhythmogenic Right Ventricular Dysplasia therapy, Cardiomyopathies genetics, Cardiomyopathies physiopathology, Cardiomyopathy, Dilated diagnosis, Cardiomyopathy, Dilated physiopathology, Cardiomyopathy, Dilated therapy, Cardiomyopathy, Hypertrophic diagnosis, Cardiomyopathy, Hypertrophic physiopathology, Cardiomyopathy, Hypertrophic therapy, Cardiomyopathy, Restrictive diagnosis, Cardiomyopathy, Restrictive physiopathology, Cardiomyopathy, Restrictive therapy, Directive Counseling, Female, Humans, Peripartum Period, Preconception Care, Pregnancy, Pregnancy Complications, Cardiovascular genetics, Pregnancy Complications, Cardiovascular physiopathology, Cardiomyopathies diagnosis, Cardiomyopathies therapy, Pregnancy Complications, Cardiovascular diagnosis, Pregnancy Complications, Cardiovascular therapy
- Abstract
Cardiomyopathy encompasses a wide spectrum of heart muscle disease, which can have an impact on the patient's ability to sustain the increased cardiac workload of pregnancy. Pregnancy can also unmask previously unknown cardiomyopathy. The outcome for both mother and baby is often related to the patient's functional class prior to pregnancy, and a multidisciplinary approach to managing this challenging group of patients is pivotal., (Copyright © 2014 Elsevier Ltd. All rights reserved.)
- Published
- 2014
- Full Text
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27. Subendocardial ischemia in hypertrophic cardiomyopathy.
- Author
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Kawasaki T and Sugihara H
- Subjects
- Bridged Bicyclo Compounds, Heterocyclic, Cardiomyopathy, Hypertrophic drug therapy, Diagnostic Imaging, Diltiazem administration & dosage, Echocardiography, Follow-Up Studies, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Myocardial Ischemia drug therapy, Positron-Emission Tomography, Prognosis, Prospective Studies, Pyrimidinones, Tomography, Emission-Computed, Single-Photon, Verapamil administration & dosage, Cardiomyopathy, Hypertrophic complications, Myocardial Ischemia diagnosis, Myocardial Ischemia etiology
- Abstract
Hypertrophic cardiomyopathy (HCM) patients often develop subendocardial ischemia in the left ventricle without atherosclerotic coronary stenosis. Myocardial ischemia plays an important role in the pathophysiology of HCM, but diagnostic techniques for the detection of subendocardial ischemia have not been widely available. We developed specific techniques to quantify subendocardial ischemia on stress scintigraphy, and have compared the results with various clinical features in patients with HCM. This article reviews our understanding of subendocardial ischemia in HCM based on more than 20 years of experience., (Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2014
- Full Text
- View/download PDF
28. Anti-hypertensive drugs have different effects on ventricular hypertrophy regression.
- Author
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Ferreira Filho C, Abreu LC, Valenti VE, Ferreira M, Meneghini A, Silveira JA, Riera AR, Colombari E, Murad N, Santos-Silva PR, Silva LJ, Vanderlei LC, Carvalho TD, and Ferreira C
- Subjects
- Animals, Humans, Hypertension prevention & control, Hypertrophy, Left Ventricular mortality, Rats, Remission Induction methods, Risk Factors, Treatment Outcome, Antihypertensive Agents therapeutic use, Hypertrophy, Left Ventricular drug therapy
- Abstract
Objectives: There is a direct relationship between the regression of left ventricular hypertrophy (LVH) and a decreased risk of mortality. This investigation aimed to describe the effects of anti-hypertensive drugs on cardiac hypertrophy through a meta-analysis of the literature., Methods: The Medline (via PubMed), Lilacs and Scielo databases were searched using the subject keywords cardiac hypertrophy, antihypertensive and mortality. We aimed to analyze the effect of anti-hypertensive drugs on ventricle hypertrophy., Results: The main drugs we described were enalapril, verapamil, nifedipine, indapamina, losartan, angiotensin-converting enzyme inhibitors and atenolol. These drugs are usually used in follow up programs, however, the studies we investigated used different protocols. Enalapril (angiotensin-converting enzyme inhibitor) and verapamil (Ca(++) channel blocker) caused hypertrophy to regress in LVH rats. The effects of enalapril and nifedipine (Ca(++) channel blocker) were similar. Indapamina (diuretic) had a stronger effect than enalapril, and losartan (angiotensin II receptor type 1 (AT1) receptor antagonist) produced better results than atenolol (selective beta1 receptor antagonist) with respect to LVH regression., Conclusion: The anti-hypertensive drugs induced various degrees of hypertrophic regression.
- Published
- 2010
- Full Text
- View/download PDF
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