Assessment, pharmacological therapy and rehabilitation management of musculoskeletal pain in children with mucopolysaccharidoses: a scoping review.

BACKGROUND: Pain of musculoskeletal origin is very common in young patients affected by Mucopolysaccharidoses. This scoping review evaluates the evidence for assessment, pharmacological treatment and rehabilitation management for musculoskeletal pain of the latter. METHODS: A Medline search through PubMed has been performed for studies published in English at least for the past twenty years. Two investigators independently reviewed all search results and extracted those that met the inclusion criteria. RESULTS: 29 studies have been selected and analysed in depth, of which 10 related to pain assessment, 11 concerned pharmacological approach, and 8 reported rehabilitation approaches. CONCLUSION: Few data are available in literature concerning the classification and management of pain in children with Mucopolysaccharidoses. Notwithstanding, pain evaluation methods are effectively used to classify pain intensity, according to the age group and communication abilities of young Mucopolysaccharidoses patients. The review emphasizes that drug therapies have a palliative purpose, while rehabilitation reduces musculoskeletal pain and can provide a therapeutic effect on disabilities.

Ultrasound-guided collagen injections in the treatment of supraspinatus tendinopathy: a case series pilot study.

Aim of the present pilot study was to verify, for the first time ever, the effects of collagen injections in patients with chronic supraspinatus tendinopathy. Eighteen patients with chronic supraspinatus tendinopathy were treated with a series of 4 type I porcine collagen ultrasound-guided injections, at weekly intervals. The effects were verified at 2-week, 1-month and 3-month follow-up by means of shoulder scoring systems and sonography. A very strong evidence (p<0.001) of a statistically significant main effect amongst the multiple clinical observation was found. Ultrasound imaging highlighted improvement in the structural integrity of the tendon. Compared to other injection therapies, collagen injections proved to be at least equally effective, faster acting and safer.

Use of ultrasound shear wave to measure muscle stiffness in children with cerebral palsy.

PURPOSE: Cerebral palsy (CP) is a disorder characterized by an increased muscle stiffness that can be contingent on both neurological and biomechanical factors. The neurological aspects are related to hyper-excitability of the stretch reflex, while the biomechanical factors are related to modifications in muscle structure. We used smart-shear wave elastography (S-SWE) to analyze muscle properties and to compare shear wave speed in soleus muscles of patients affected by CP and typically developing children. METHODS: We enrolled 21 children (15 males and 6 females; age range 3-16) with spastic hemiplegia CP and 21 healthy children (11 males and 10 females; age range 3-14). Measurements of soleus S-SWE were performed using a Samsung RS80A ultrasound scanner with Prestige equipment (Samsung Medison Co. Ltd., Seoul, Korea), with a convex array transducer (CA1-7; Samsung Medison Co. Ltd., Seoul, Korea). For each CP child clinical assessment included Modified Ashworth Scale (MAS) score. RESULTS: Children with CP showed greater S-SWE values than the healthy ones (p < 0.001). Our data suggest a significant correlation between the S-SWE values and the MAS scores (Spearman correlation coefficient 0.74; p < 0.001 at Kruskal-Wallis test) in children with CP. CONCLUSIONS: Measuring muscle properties with SWE, a non-invasive and real-time technique, may integrate the physical exam. SWE may be a reliable clinical tool for diagnosis and longitudinal monitoring of muscle stiffness, as well as particularly suitable for grading and for assessing the response to treatments.

A micro-perfusion bioreactor for on line investigation of ECM remodeling under hydrodynamic and biochemical stimulation.

Tissue-on-chip (TOC) systems aim at replicating complex biological dynamics in vitro with the potential either to improve the understanding of human biology or to develop more accurate therapeutic strategies. To replicate faithfully the intricate interrelationships between cells and their surrounding microenvironment, the three-dimensional (3D) tissue model must possess a responsive extracellular matrix (ECM). ECM remodeling plays a pivotal role in guiding cells and tissues functions and such aspect is somewhat denied during in vitro studies. For this purpose, we fabricated a micro-perfusion bioreactor capable to sustain the viability of 3D engineered tissue models recapitulating the process of the native ECM deposition and assembly. Engineered human dermis micro-tissue precursors (HD-µTP) were used as building blocks to generate a final tissue. HD-µTP were loaded in the perfusion space of the micro-perfusion bioreactor and, under the superimposition of different fluid dynamic regimes and biochemical stimulation, they synthesized new collagen proteins that were, then, assembled in the perfusion space forming a continuum of cells embedded in their own ECM. The micro-perfusion bioreactor was fabricated to allow the on-line monitoring of the oxygen consumption and the assembly of the newly formed collagen network via real time acquisition of the second harmonic generation (SHG) signal. The possibility to detect the collagen reorganization due to both fluid dynamic and biochemical stimulation, let us to define the optimal perfusion configuration in order to obtain a TOC system based on an endogenous and responsive ECM.

Clinical risk in rehabilitation: an exploratory investigation in Campania region.

BACKGROUND: Clinical risk management is a comprehensive programme that encompasses all the measures implemented to improve the quality of the healthcare service and ensure patient safety, which is based on learning through error. This process is intended to bring about ongoing improvements in clinical practice, starting with risk identification, before moving on to risk assessment and analysis, in order to reduce risks where possible. When clinical risk management is applied in rehabilitation, the first step involves identifying errors by assessing adverse events, which are considered to indicate the existing risk. Our work aims to explore the characteristics of the clinical risk in rehabilitation so as to learn more about its extent, its components, and its implications for the user. METHODS: Our study involved numerous workers operating in four different branches of rehabilitation - speech therapy, physiotherapy, psychomotor education and occupational therapy - at forty-nine private rehabilitation centres in the province of Naples, an area that has not been studied before. A questionnaire was drafted regarding the main errors committed in the rehabilitation sector. It was then distributed and collected in again, after which the results were analysed and outcomes measured. Out of a total of 556 questionnaires distributed, 493 were returned (88.6% response rate.). RESULTS: The study revealed that for all the rehabilitation branches considered, the macro-category of errors linked to technical and professional aspects accounted for the highest percentage of the total errors (39%). In this study, the most frequent errors linked to technical and professional aspects were: wrong dose errors, treatment planning errors and functional assessment errors. CONCLUSIONS: There is an evident need to take action in order to manage the clinical risk in rehabilitation: to promote a concept of errors as opportunities for learning and improvement; to maintain the focus on both individual responsibility and on any systemic failings; to share fundamental values such as transparency, collaboration between workers, communication with patients, and a commitment to ongoing improvements in healthcare quality.

The disease profile of poverty: morbidity and mortality in northern Uganda in the context of war, population displacement and HIV/AIDS.

The population of Gulu District (northern Uganda) has been severely incapacitated by war, epidemics and social disruption. This study is aimed at describing disease patterns and trends in this area through a retrospective analysis of discharge records for 155205 in-patients of Lacor Hospital in the period 1992-2002. The burden of infectious diseases in childhood is overwhelming, with malaria accounting for the steepest increase in admissions. Admissions for war-related injuries and malnutrition fluctuated with the intensity of the war and the severity of famine. Emerging and re-emerging infections, such as HIV/AIDS, tuberculosis and Ebola, accounted for a heavy disease burden; however, there has been a trend for admissions related to HIV/AIDS and tuberculosis to decrease since the implementation of community-based services. Vulnerable groups (infants, children and women) accounted for 79.8% of admissions. Long-term war, population displacement, the collapse of social structures and the breakdown of the health system place people at a much greater risk of persistent, emerging and re-emerging infectious diseases, malnutrition and war-related injuries, shaping the 'disease profile of poverty'. Most of the disease burden results from infectious diseases of childhood, whose occurrence could be dramatically reduced by low-cost and effective preventive and curative interventions.

Selective launching of higher-order modes into an optical fiber with an optical phase shifter.

We demonstrate a simple, effective technique for launching higher-order modes into a double-mode optical fiber by using an optical phase shifter at the input end. The suppression of the lowest-order mode was measured (but not limited) to be 27 dB.