scholarly journals Trevor’s Disease: A Literature Review regarding Classification, Treatment, and Prognosis apropos of a Case

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Georgios Arealis ◽  
Vassilios S. Nikolaou ◽  
Andrew Lacon ◽  
Neil Ashwood ◽  
Keith Hayward ◽  
...  
Keyword(s):  
Upper Limb ◽  
Lower Limb ◽  
Bone Scan ◽  
Whole Body ◽  
New Classification ◽  
Trevor’S Disease ◽  
Treatment Prognosis ◽  
Dysplasia Epiphysealis Hemimelica

Background. Dysplasia epiphysealis hemimelica (DEH) is rare and its main characteristic is osteochondromas of the epiphysis of long bones.Methods. We report a case of DEH of the ankle in an 8-year-old boy that was resected in 2005. Additionally we collect all the reported cases of DEH. The literature is reviewed regarding the treatment, prognosis, long term function, and patterns and areas affected by DEH.Results. In our case no complications were noted and our patient remains asymptomatic. Reviewing the literature we found that 73 authors have reported 144 cases from 1926 to 2013. We propose and describe a new classification that correlates with prognosis. According to our classification DEH is classified as types 1 with single lower limb involvement, 2 with multiple lower limb, 3 with single upper limb, 4 with multiple upper limb, 5 with upper and lower limb, and 6 with spine.Conclusions. All single lesions should be followed up and if indicated a whole body nuclear bone scan can be useful in identifying the existence of multiple affected joints. Type 1 lesions have better prognosis than 2 and have less chances of developing OA even if not resected. Resection, even if partial, can be a successful treatment for DEH.

scholarly journals Technology for monitoring everyday prosthesis use: a systematic review

2020 ◽  
Author(s):  
Alix Chadwell ◽  
Laura Diment ◽  
Encarna Micó Amigo ◽  
Dafne Zuleima Morgado Ramirez ◽  
Alexander Dickinson ◽  
...  
Keyword(s):  
Upper Limb ◽  
Lower Limb ◽  
Battery Life ◽  
Activity Levels ◽  
Everyday Activity ◽  
Prosthetic Devices ◽  
Clinical Scores ◽  
Standard Clinical Practice ◽  
The Cost

BackgroundUnderstanding how prostheses are used in everyday life is central to the design, provision and evaluation of prosthetic devices and associated services. This paper reviews the scientific literature on methodologies and technologies that have been used to assess the daily use of both upper- and lower-limb prostheses. It discusses the types of studies that have been undertaken, the technologies used to monitor physical activity, the benefits of monitoring daily living and the barriers to long-term monitoring.MethodsA systematic literature search was conducted in PubMed, Web of Science, Scopus, CINAHL and EMBASE of studies that monitored the activity of prosthesis-users during daily-living.Results60 lower-limb studies and 9 upper-limb studies were identified for inclusion in the review. The first studies in the lower-limb field date from the 1990s and the number has increased steadily since the early 2000s. In contrast, the studies in the upper-limb field have only begun to emerge over the past few years. The early lower-limb studies focused on the development or validation of actimeters, algorithms and/or scores for activity classification. However, most of the recent lower-limb studies used activity monitoring to compare prosthetic components. The lower-limb studies mainly used step-counts as their only measure of activity, focusing on the amount of activity, not the type and quality of movements. In comparison, the small number of upper-limb studies were fairly evenly spread between development of algorithms, comparison of everyday activity to clinical scores, and comparison of different prosthesis user populations. Most upper-limb papers reported the degree of symmetry in activity levels between the arm with the prosthesis and the intact arm.ConclusionsActivity monitoring technology used in conjunction with clinical scores and user feedback, offers significant insights into how prostheses are used and whether they meet the user’s requirements. However, the cost, limited battery-life and lack of availability in many countries mean that using sensors to understand the daily use of prostheses and the types of activity being performed has not yet become a feasible standard clinical practice. This review provides recommendations for the research and clinical communities to advance this area for the benefit of prosthesis users.

Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1

2018 ◽  
Vol 231 (02) ◽  
pp. 52-59
Author(s):  
André Lollert ◽  
Katharina Laudemann ◽  
Eugen Mengel ◽  
Christian Hoffmann ◽  
Larissa Moos ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Gaucher Disease ◽  
Disease Type ◽  
Whole Body ◽  
Enzyme Replacement ◽  
Gaucher Disease Type 1 ◽  
Body Magnetic Resonance Imaging

Abstract Purpose We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy. Materials and Methods In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: “early” (age ≤12 years, median 5 years) and “late” (during adulthood, median 32 years). We evaluated occurrence of irreversible avascular necroses (AVN) and applied several semi-quantitative scores, including the Bone-Marrow-Burden (BMB) score, the Düsseldorf-Gaucher score (DGS), the Vertebra-Disc-Ratio (VDR), and the Gaucher disease type 1 Severity Scoring System (GD-DS3). Results MRI assessments showed no AVN in the “early” group. AVN were observed in 2 patients of the “late” group; one also had a splenic Gaucheroma. The follow-up examinations showed slight improvements in the BMB-score, DGS, and VDR, with similar tendencies in both treatment groups. The GD-DS3 score only improved in “late” group. Conclusion This retrospective study supported the ongoing clinical value of enzyme replacement therapy with alglucerase/imiglucerase, as WB-MRI-based scores stayed constant or slightly improved even after long-term treatment. Secondary complications were only observed in the late treatment group. Our results suggest that “early initiation” of enzyme replacement therapy may protect the bone.

scholarly journals NIMG-66. LONG-TERM FOLLOW-UP OF NEUROFIBROMATOSIS TYPE 1 PATIENTS USING WHOLE-BODY MRI DEMONSTRATES DYNAMIC CHANGES IN INTERNAL NEUROFIBROMA SIZE

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi176-vi176
Author(s):  
Ina Ly ◽  
Raquel Thalheimer ◽  
Wenli Cai ◽  
Miriam Bredella ◽  
Vanessa Merker ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Whole Body ◽  
Childhood And Adolescence ◽  
Entire Study ◽  
Whole Body Mri

Abstract BACKGROUND Neurofibromas affect 40–50% of neurofibromatosis type 1 (NF1) patients and can cause significant morbidity and mortality. They grow more rapidly during childhood and adolescence but studies in adults are limited by their retrospective nature and follow-up time < 3 years. The long-term natural history of neurofibromas remains unknown. No guidelines exist on the need and frequency of surveillance imaging for patients. Whole-body MRI (WBMRI) can detect whole-body tumor burden, including internal neurofibromas. METHODS 17 adult NF1 patients who underwent WBMRI between 2007–2010 (Scan 1) underwent repeat WBMRI between 2018–2019 (Scan 2). Internal neurofibromas were segmented on short tau inversion recovery (STIR) sequences and tumor volume was calculated using a computerized volumetry and three-dimensional segmentation software. Circumscribed tumors were defined as discrete; invasive tumors or those involving multiple nerves were defined as plexiform. Tumor growth and shrinkage were defined as volume change ≥ 20% over the entire study period. RESULTS Median patient age was 43 years during Scan 1 and 53 years during Scan 2. Median time between Scan 1 and 2 was 9 years. A total of 140 neurofibromas were assessed. 24% of tumors grew by a median 63% (6.8% per year). 54% of tumors spontaneously decreased in volume by a median 60% (7% per year) without treatment. On a per-patient basis, 18% of patients had overall tumor growth and 41% overall tumor shrinkage. 8 new tumors developed in 7 patients. 16 tumors resolved entirely without medical or surgical intervention. Growth behavior did not correlate with discrete or plexiform morphology. CONCLUSION A subset of internal neurofibromas in adult NF1 patients grow significantly over a long-term period, suggesting that continued monitoring of these patients may be warranted. Surprisingly, more than half of neurofibromas shrink spontaneously without intervention. Continued patient enrollment and correlation of imaging findings with functional outcomes are underway.

scholarly journals Rapid and flexible whole body postural responses are evoked from perturbations to the upper limb during goal-directed reaching

2017 ◽  
Vol 117 (3) ◽  
pp. 1070-1083 ◽  
Author(s):  
Catherine R. Lowrey ◽  
Joseph Y. Nashed ◽  
Stephen H. Scott
Keyword(s):  
Upper Limb ◽  
Lower Limb ◽  
Muscle Activity ◽  
Whole Body ◽  
Limb Muscle ◽  
Behavioral Context ◽  
Body Balance ◽  
Postural Responses ◽  
Muscle Responses ◽  
Behavioral Goal

An important aspect of motor control is the ability to perform tasks with the upper limbs while maintaining whole body balance. However, little is known about the coordination of upper limb voluntary and whole body postural control after mechanical disturbances that require both upper limb motor corrections to attain a behavioral goal and lower limb motor responses to maintain whole body balance. The present study identified the temporal organization of muscle responses and center of pressure (COP) changes following mechanical perturbations during reaching. Our results demonstrate that muscle responses in the upper limb are evoked first (∼50 ms), with lower limb muscle activity occurring immediately after, in as little as ∼60 ms after perturbation. Hand motion was immediately altered by the load, while COP changes occurred after ∼100 ms, when lower limb muscle activity was already present. Our secondary findings showed that both muscle activity and COP changes were influenced by behavioral context (by altering target shape, circle vs. rectangle). Voluntary and postural actions initially directed the hand toward the center of both target types, but after the perturbation upper limb and postural responses redirected the hand toward different spatial locations along the rectangle. Muscle activity was increased for both upper and lower limbs when correcting to the circle vs. the rectangle, and these differences emerged as early as the long-latency epoch (∼75–120 ms). Our results demonstrate that postural responses are rapidly and flexibly altered to consider the behavioral goal of the upper limb. NEW & NOTEWORTHY The present work establishes that, when reaching to a target while standing, perturbations applied to the upper limb elicit a rapid response in lower limb muscles. Unlike voluntary movements, postural responses do not occur before corrections of the upper limb. We show the first evidence that corrective postural adjustments are modulated by upper limb behavioral context (target shape). Importantly, this indicates that postural responses take into account upper limb feedback for online control.

Long-Term Follow-Up of a Patient With McCune-Albright Syndrome by Whole-Body Bone Scan and SPECT

2004 ◽  
Vol 29 (11) ◽  
pp. 712 ◽  
Author(s):  
Manabu Fujii ◽  
Shigeru Kosuda ◽  
Motoyuki Jitsu ◽  
Daisuke Maeda ◽  
Shoichi Kusano ◽  
...  
Keyword(s):  
Bone Scan ◽  
Whole Body ◽  
Mccune Albright Syndrome ◽  
Long Term Follow Up ◽  
Albright Syndrome ◽  
Body Bone ◽  
Mccune Albright

Improvements in vascular function in response to acute lower limb heating in young healthy males and females

2021 ◽  
Author(s):  
Jem L. Cheng ◽  
Jennifer S. Williams ◽  
Sven Hoekstra ◽  
Maureen J. MacDonald
Keyword(s):  
Arterial Stiffness ◽  
Endothelial Function ◽  
Upper Limb ◽  
Lower Limb ◽  
Vascular Function ◽  
Water Immersion ◽  
Plasma Concentrations ◽  
Hot Water ◽  
Whole Body ◽  
Vascular Conductance

Regular exposure to passive heat stress improves vascular function, but the optimal heating prescription remains undefined. Local limb heating is more feasible than whole body heating, but the evidence demonstrating its efficacy is lacking. The purpose of this study was to determine whether acute improvements in vascular function can be achieved with lower limb heating in 16 young healthy individuals (8 female, 8 male). In separate visits, participants underwent 45-min of ankle- and knee-level hot water immersion (45 °C). A subset of 7 participants also participated in a time-control visit. Endothelial function was assessed through simultaneous brachial and superficial femoral artery flow-mediated dilation (FMD) tests. Macrovascular function was quantified by %FMD, while microvascular function was quantified by vascular conductance during reactive hyperemia. Arterial stiffness was assessed through carotid-femoral and femoral-foot pulse wave velocity (PWV). Plasma concentrations of interleukin-6 and extracellular heat shock protein-72 (eHSP72) were used as indicators of inflammation. Our findings showed that 45-min of lower limb heating - regardless of condition - acutely improved upper limb macrovascular endothelial function (i.e., brachial %FMD; Pre: 4.6±1.7 vs. Post: 5.4±2.0%; P=0.004) and lower limb arterial stiffness (i.e., femoral-foot PWV; Pre: 8.4±1.2 vs. Post: 7.7±1.1 m/s; P=0.011). However, only knee-level heating increased upper limb microvascular endothelial function (i.e., brachial peak vascular conductance; Pre: 6.3±2.7 vs. Post: 7.8±3.5 ml/min⋅mmHg; P≤0.050) and plasma eHSP72 concentration (Pre: 12.4±9.4 vs. Post: 14.8±9.8 ng/ml; P≤0.050). These findings show that local lower limb heating acutely improves vascular function in younger individuals, with knee-level heating improving more outcome measures.

Hand Injury in Rock Climbing: Literature Review

2016 ◽  
Vol 21 (01) ◽  
pp. 13-17 ◽  
Author(s):  
A. Pozzi ◽  
G. Pivato ◽  
L. Pegoli
Keyword(s):  
Literature Review ◽  
Upper Limb ◽  
Lower Limb ◽  
Hand Injury ◽  
Rock Climbing ◽  
Whole Body ◽  
Sport Injuries ◽  
Basic Principles ◽  
Hand Surgeon ◽  
Serious Injuries

With the amazing increasing in number of participants, rock climbing has become a popular sport in the last decade. A growing number of participants, with different skill level, inevitably leads to an increased number of injuries related to this practice. The kind of lesions that can be observed in rock-climbers is very specific and often involves the hand. For this reason is very important for any hand surgeon that is exposed to sport injuries to know which and the most common injuries related to this sport and which are the basic principles for the treatment of those. The aim of this article is to review the literature that has been published in the last ten year in this topic. On the NCBI database 22 articles where found that where related to rock climbing lesion affecting the hand or the whole body. Differences where found according to kind of rock climbing activity that was analyzed, alpine climb leads to more serious injuries, often affecting the lower limb, while in sport and recreational rock climbing the upper limb and the hand are definitely the most affected parts. Flexor pulley lesions, followed by fractures and strains are the most common lesions affecting the hand that are related to this practice.

scholarly journals Analysis of Whole-Body Coordination Patterning in Successful and Faulty Spikes Using Self-Organising Map-Based Cluster Analysis: A Secondary Analysis

Sensors ◽  
2021 ◽  
Vol 21 (4) ◽  
pp. 1345
Author(s):  
Javad Sarvestan ◽  
Zdeněk Svoboda ◽  
Fatemeh Alaei ◽  
Franky Mulloy
Keyword(s):  
Cluster Analysis ◽  
Upper Limb ◽  
Lower Limb ◽  
Secondary Analysis ◽  
Whole Body ◽  
Success Rates ◽  
Ball Impact ◽  
Limb Coordination ◽  
Angular Velocities ◽  
Self Organising Map

This study investigated the whole-body coordination patterning in successful and faulty spikes using self-organising map-based cluster analysis. Ten young, elite volleyball players (aged 15.5 ± 0.7 years) performed 60 volleyball spikes in a real-game environment. Adopting the cluster analysis, based on a self-organising map, whole-body coordination patterning was explored between successful and faulty spikes of individual players. The self-organising maps (SOMs) portrayed whole body, lower and upper limb coordination dissimilarities during the jump phase and the ball impact phases between the successful and faulty spikes. The cluster analysis illustrated that the whole body, upper limb and lower limb coordination patterning of each individual’s successful spikes were similar to their faulty spikes. Range of motion patterning also demonstrated no differences in kinematics between spike outcomes. Further, the upper limb angular velocity patterning of the players’ successful/faulty spikes were similar. The SPM analysis portrayed significant differences between the normalized upper limb angular velocities from 35% to 45% and from 76% to 100% of the spike movement. Although the lower limb angular velocities are vital for achieving higher jumps in volleyball spikes, the results of this study portrayed that the upper limb angular velocities distinguish the differences between successful and faulty spikes among the attackers. This confirms the fact that volleyball coaches should shift their focus toward the upper limb velocity and coordination training for higher success rates in spiking for volleyball attackers.

Cardiac troponin I in patients with acute upper and lower limb ischemia

VASA ◽  
2008 ◽  
Vol 37 (4) ◽  
pp. 327-332 ◽  
Author(s):  
Koutouzis ◽  
Sfyroeras ◽  
Moulakakis ◽  
Kontaras ◽  
Nikolaou ◽  
...  
Keyword(s):  
Upper Limb ◽  
Lower Limb ◽  
Cardiac Troponin ◽  
Cardiac Involvement ◽  
Troponin I ◽  
Limb Ischemia ◽  
Elevated Troponin ◽  
Troponin Elevation ◽  
Lower Limb Ischemia ◽  
Ischemia Group

Background: The aim of this study was to investigate the presence, etiology and clinical significance of elevated troponin I in patients with acute upper or lower limb ischemia. The high sensitivity and specificity of cardiac troponin for the diagnosis of myocardial cell damage suggested a significant role for troponin in the patients investigated for this condition. The initial enthusiasm for the diagnostic potential of troponin was limited by the discovery that elevated cardiac troponin levels are also observed in conditions other than acute myocardial infarction, even conditions without obvious cardiac involvement. Patients and Methods: 71 consecutive patients participated in this study. 31 (44%) of them were men and mean age was 75.4 ± 10.3 years (range 44–92 years). 60 (85%) patients had acute lower limb ischemia and the remaining (11; 15%) had acute upper limb ischemia. Serial creatine kinase (CK), isoenzyme MB (CK-MB) and troponin I measurements were performed in all patients. Results: 33 (46%) patients had elevated peak troponin I (> 0.2 ng/ml) levels, all from the lower limb ischemia group (33/60 vs. 0/11 from the acute upper limb ischemia group; p = 0.04). Patients with lower limb ischemia had higher peak troponin I values than patients with upper limb ischemia (0.97 ± 2.3 [range 0.01–12.1] ng/ml vs. 0.04 ± 0.04 [0.01–0.14] ng/ml respectively; p = 0.003), higher peak CK values (2504 ± 7409 [range 42–45 940] U/ml vs. 340 ± 775 [range 34–2403] U/ml, p = 0.002, respectively, in the two groups) and peak CK-MB values (59.4 ± 84.5 [range 12–480] U/ml vs. 21.2 ± 9.1 [range 12–39] U/ml, respectively, in the two groups; p = 0.04). Peak cardiac troponin I levels were correlated with peak CK and CK-MB values. Conclusions: Patients with lower limb ischemia often have elevated troponin I without a primary cardiac source; this was not observed in patients presenting with acute upper limb ischemia. It is very important for these critically ill patients to focus on the main problem of acute limb ischemia and to attempt to treat the patient rather than the troponin elevation per se. Cardiac troponin elevation should not prevent physicians from providing immediate treatment for limb ischaemia to these patients, espescially when signs, symptoms and electrocardiographic findings preclude acute cardiac involvement.

Sign in / Sign up

Export Citation Format

Share Document