Abstract:
BACKGROUND:A relative inability to capture a sufficiently large patient population in any one geographic location has traditionally limited research into rare diseases. METHODS AND RESULTS:Clinicians interested in the rare disease lymphangioleiomyomatosis (LAM) have worked with the LAM Treatment Alliance, the MIT Media Lab, and Clozure Associates to cooperate in the design of a state-of-the-art data coordination platform that can be used for clinical trials and other research focused on the global LAM patient population. This platform is a component of a set of web-based resources, including a patient self-report data portal, aimed at accelerating research in rare diseases in a rigorous fashion. CONCLUSIONS:Collaboration between clinicians, researchers, advocacy groups, and patients can create essential community resource infrastructure to accelerate rare disease research. The International LAM Registry is an example of such an effort. 82.
journal_name
Lymphat Res Bioljournal_title
Lymphatic research and biologyauthors
Nurok M,Eslick I,Carvalho CR,Costabel U,D'Armiento J,Glanville AR,Harari S,Henske EP,Inoue Y,Johnson SR,Lacronique J,Lazor R,Moss J,Ruoss SJ,Ryu JH,Seyama K,Watz H,Xu KF,Hohmann EL,Moss Fdoi
10.1089/lrb.2009.0028subject
Has Abstractpub_date
2010-03-01 00:00:00pages
81-7issue
1eissn
1539-6851issn
1557-8585journal_volume
8pub_type
杂志文章abstract:BACKGROUND:Surgical manipulations of the axilla may cause a condition known as Axillary Web Syndrome (AWS). The systems compromised and the sequence of events leading to this syndrome remains unknown. This study evaluated clinical, surgical, and vascular factors associated with onset and duration of AWS after breast ca...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0037
更新日期:2018-06-01 00:00:00
abstract:BACKGROUND:Lower limb lymphedema (LLL) is a common complication of cancer treatment. The disease is chronic and progressive with no cure. Although a common and significant source of morbidity, the impact of this condition on health-related quality of life (HRQOL) has only recently been addressed. In effort to identify ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/lrb.2012.0015
更新日期:2013-03-01 00:00:00
abstract::The vasculature of the central nervous system (CNS) is highly specialized with a blood-brain-barrier, reciprocal neuroepithelial-endothelial cell interactions and extensive pericyte coverage. Developmentally, numerous important signaling pathways participate in CNS angiogenesis to orchestrate the precise timing and sp...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/lrb.2008.1014
更新日期:2008-01-01 00:00:00
abstract:: Background: Bioimpedance spectroscopy detects unilateral lymphedema if the ratio of extracellular fluid (ECF) between arms or between legs is outside three standard deviations (SDs) of the normative mean. Detection of bilateral lymphedema, common after bilateral breast or ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0082
更新日期:2018-12-01 00:00:00
abstract:BACKGROUND:Lymphatic stomata are small lymphatic openings in the serosal membrane that communicate with the serosal cavity. Although these stomata have primarily been studied in experimental mammals, little is known concerning the presence and properties of lymphatic stomata in the adult human pleura. Thus, adult human...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2014.0009
更新日期:2015-06-01 00:00:00
abstract:BACKGROUND:We performed experiments to test whether isolated lymphatic vessels would remain viable after overnight long-distance shipment. METHODS AND RESULTS:Freshly isolated segments of rat mesenteric lymphatic vessels were placed into tubes filled by chilled D-MEM/F12 supplemented with antibiotics and shipped via o...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2010.0007
更新日期:2010-12-01 00:00:00
abstract:: Background: Mesenteric lymph (ML) has been implicated in the development of multiple organ dysfunction syndrome in critical illness. Extracellular RNAs play a role in cell-to-cell communication during physiological and disease processes but they are rarely studied in ML. W...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0044
更新日期:2019-10-01 00:00:00
abstract:BACKGROUND:Lymphedema is the abnormal accumulation of protein-rich fluid in the interstitial space. Primary lymphedema is a rare genetic condition with both autosomal dominant and autosomal recessive modes of inheritance. Three genes, FLT4 (VEGFR3), FOXC2, and SOX18 cause varying forms of primary lymphedema. In industr...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2008.1524
更新日期:2008-01-01 00:00:00
abstract:: Background: The Lymphedema Functioning, Disability, and Health questionnaire for Upper Limb Lymphedema (Lymph-ICF-UL) is a health-related quality-of-life questionnaire for patients with breast cancer-related lymphedema. Previous testing of this questionnaire showed very go...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2019.0073
更新日期:2020-08-01 00:00:00
abstract:BACKGROUND:Analysis of the innervation pattern of the thoracic duct in young and elderly human subjects has been performed. The subdivision of the vessels in cervical and lumbar region were taken in consideration. METHODS AND RESULTS:Immunostaining for general nerve fibers with a PGP 9.5 marker disclosed a diffuse inn...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0014
更新日期:2012-06-01 00:00:00
abstract::Hemangiomas of infancy are very common tumors, but they are heterogeneous in their behavior. A small, but significant, subset causes medical complications or permanent disfigurement, but due to their heterogeneity, there is no appropriate "one size fits all" approach to management. In addition, the rapid evolution of ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/153968503322758120
更新日期:2003-01-01 00:00:00
abstract:: Background: The use of bioimpedance spectroscopy (BIS) for the detection of unilateral upper limb lymphedema is growing. Currently, normatively determined diagnostic thresholds using bioimpedance are available for females only. It is unclear if they are suitable for males ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0051
更新日期:2020-02-01 00:00:00
abstract:: Background: The aims of this study were to explore, using visual art methodology, how children and adolescents perceive their lymphedema and conceptualize the barriers and enablers in self-management and to explore the role of an educational camp in promoting self-efficacy...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,多中心研究
doi:10.1089/lrb.2018.0075
更新日期:2019-04-01 00:00:00
abstract:BACKGROUND:Lymphedema of the arm and/or hand is a well-established complication of breast cancer therapy. The objective of the study was to determine the interest of auto-adjustable MOBIDERM® Autofit night arm sleeve in controlling edema rebound effect in breast cancer-related lymphedema patients just after a successfu...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0026
更新日期:2017-12-01 00:00:00
abstract::The objective of the study was to evaluate the intra- and inter-rater reliability of bioimpedance in determining upper limb volume in women with lymphedema resulting from breast cancer treatment, as well as its correlation with the upper limb volume calculated by circumferential measurement. A blind cross-sectional st...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0036
更新日期:2018-06-01 00:00:00
abstract:BACKGROUND:Lymphedema leads to adipose tissue deposition. Water-fat magnetic resonance imaging (MRI) can quantify and localize fat and water. The presence of excess fat and excess water/muscle in the subfascial compartment of the lymphedematous limb has not been investigated before. The aim of this study was to investi...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0042
更新日期:2018-04-01 00:00:00
abstract:BACKGROUND:Lymph flow depends on both the rate of lymph production by tissues and the extent of passive and active pumping. Here we aim to characterize the passive mechanical properties of a lymphangion in both mid-lymphangion and valve segments to assess regional differences along a lymphangion, as well as evaluating ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0015
更新日期:2012-12-01 00:00:00
abstract:BACKGROUND:The composition of breast cancer-related lymphedema (BCRL) has been shown to evolve from the initial accumulation of fluid to the development of fibrotic lesions and abnormal fat deposition. Therefore, precise and reliable assessments of BCRL are required to develop accurate staging and management. Although ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2012.0020
更新日期:2013-06-01 00:00:00
abstract::Exciting studies involving the molecular regulation of lymphangiogenesis in lymphatic-associated disorders (e.g., wound healing, lymphedema and tumor metastasis) have focused renewed attention on the intrinsic relationship between lymphatic endothelial cells (LECs) and extracellular matrix (ECM) microenvironment. ECM ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/lrb.2006.4.83
更新日期:2006-01-01 00:00:00
abstract:: Introduction: Mass drug administration and morbidity management and disability prevention (MMDP) though considered as two pillars of global program for elimination of filariasis, implementation of MMDP was disregarded in several endemic countries. Limb hygiene is the main ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2019.0058
更新日期:2020-12-01 00:00:00
abstract:: Background: Conservative nonsurgical treatment has traditionally been applied to lymphedema. Early surgical procedures were invasive and disfiguring and their long-term success was often limited. In contrast, contemporary surgical techniques are much less invasive and have...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0038
更新日期:2019-08-01 00:00:00
abstract:BACKGROUND:Lymphangiomatosis is a rare disorder of the lymphatic system that can impact the dermis, soft tissue, bone, and viscera and can be characterized by lymphangiomas, swelling, and chylous discharge. Whether disordered lymphangiogenesis in lymphangiomatosis affects the function and anatomy of the entire systemic...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2015.0013
更新日期:2015-09-01 00:00:00
abstract:BACKGROUND:Bioimpedance spectroscopy (BIS) is commonly used in the assessment and monitoring of lymphedema. This study investigated electrodes as a source of variability that could impact the accuracy of BIS in the clinic and determined if Ag/AgCl electrocardiograph (ECG) electrodes could be used as an alternative to i...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0002
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Compared to volume/circumference measurements to diagnose lymphedema, thickening and disturbance of echogenicity of cutis and subcutis associated with the development of lymphedema is measured directly by ultrasonography. The aim of the present study was to investigate the evolution of thickness and echogeni...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2013.0028
更新日期:2014-03-01 00:00:00
abstract:OBJECTIVE:This study sought to evaluate the effect of elastic compression, functional compressive bandaging, and kinesiotherapy on blood flow of the upper limb with lymphedema secondary to the treatment of breast cancer. METHODS:This was a randomized blind crossover clinical trial with a washout period of 7 days betwe...
journal_title:Lymphatic research and biology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1089/lrb.2016.0022
更新日期:2017-03-01 00:00:00
abstract:BACKGROUND:The purpose of this study is to investigate the effects of the aqua-lymphatic therapy (ALT) on unilateral lower extremity lymphedema in the maintenance phase. MATERIALS AND METHODS:This is a randomized controlled trial with a blinded assessor. The study was completed with 30 ALT and 27 control group partici...
journal_title:Lymphatic research and biology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1089/lrb.2017.0017
更新日期:2017-09-01 00:00:00
abstract:: Background: Lymphedema may impact patients' health-related quality of life (HRQoL). The purpose of this study was to evaluate HRQoL after two different treatments to the 12-month follow-up point. Methods and Results: Study part...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0065
更新日期:2020-08-01 00:00:00
abstract:BACKGROUND:Tissue dielectric constant (TDC) and spot bioimpedance measurement (BIA) have a role in the assessment of tissue composition changes in breast cancer-related lymphedema (BCRL). Our aims were to determine whether TDC and spot BIA measures could detect inter-limb differences in BCRL, explore the relationship b...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2014.0032
更新日期:2015-03-01 00:00:00
abstract::The present report describes a case with the triad of yellow nail syndrome (YNS) and the use of low-pressure compression pump as treatment of lymphedema in YNS. A 71-year-old woman presented with bilateral lower extremity lymphedema, yellow nails, and recurrent bilateral pleural effusion. In this case, we specifically...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0012
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND:In arm lymphedema secondary to axillary surgery and radiotherapy (breast cancer-related lymphedema), the swelling is largely epifascial and lymph flow per unit epifascial volume is impaired. The subfascial muscle compartment is not measurably swollen despite the iatrogenic damage to its axillary drainage pat...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/153968503321642615
更新日期:2003-01-01 00:00:00