Improving rheumatoid arthritis management within the Spanish National Health System: a social return on investment study.

OBJECTIVES: To define a set of proposals that would improve the current management of patients with rheumatoid arthritis (RA) within the Spanish National Health System (SNHS), and to estimate the impact of their implementation from a social perspective. METHODS: A one-year forecast-type Social Return on Investment (SROI) analysis was performed on the basis of information collected from a scientific literature review, official data, and multiple stakeholders regarding RA. A sub-analysis was performed within the areas of diagnosis, early RA (<2 years from diagnosis), and established RA (≥2 years from diagnosis). RESULTS: Stakeholders agreed on a set of 22 proposals, which included incorporating specialised nursing, addressing adherence issues, providing psychological support, or promoting the role of patient associations, among others. Their implementation would require an investment of 289 million euros and yield a social return of 913 million euros, i.e. a social return of 3.16 euros per euro invested (2.92 euros in the worst-case scenario and 3.40 euros in the best-case scenario). The greatest social return relative to investment and the greatest attributed to intangible aspects were observed within the area of early RA. CONCLUSIONS: Evidence-based recommendations for the management of RA are aspirational. Nevertheless, the present study estimated that the implementation of the set of proposals would result in a positive impact relative to the investment needed to implement them. The results may guide management decisions to reduce the burden associated with RA, and help bridge the gap between evidence-based recommendations and routine clinical practice.

Detection of SARS-CoV-2 in a dog with hemorrhagic diarrhea.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of COVID-19, has infected several animal species, including dogs, presumably via human-to-animal transmission. Most infected dogs reported were asymptomatic, with low viral loads. However, in this case we detected SARS-CoV-2 in a dog from the North African coastal Spanish city of Ceuta presenting hemorrhagic diarrhea, a disease also reported earlier on in an infected dog from the USA. CASE PRESENTATION: In early January 2021, a West Highland Terrier pet dog from Ceuta (Spain) presented hemorrhagic diarrhea with negative tests for candidate microbial pathogens. Since the animal was in a household whose members suffered SARS-CoV-2 in December 2020, dog feces were analyzed for SARS-CoV-2, proving positive in a two-tube RT-PCR test, with confirmation by sequencing a 399-nucleotide region of the spike (S) gene. Furthermore, next-generation sequencing (NGS) covered > 90% SARS-CoV-2 genome sequence, allowing to classify it as variant B.1.177. Remarkably, the sequence revealed the Ile402Val substitution in the spike protein (S), of potential concern because it mapped in the receptor binding domain (RBD) that mediates virus interaction with the cell. NGS reads mapping to bacterial genomes showed that the dog fecal microbiome fitted best the characteristic microbiome of dog's acute hemorrhagic diarrhea. CONCLUSION: Our findings exemplify dog infection stemming from the human SARS-CoV-2 pandemic, providing nearly complete-genome sequencing of the virus, which is recognized as belonging to the B.1.177 variant, adding knowledge on variant circulation in a geographic region and period for which there was little viral variant characterization. A single amino acid substitution found in the S protein that could have been of concern is excluded to belong to this category given its rarity and intrinsic nature. The dog's pathology suggests that SARS-CoV-2 could affect the gastrointestinal tract of the dog.

The impact of improving haemophilia A management within the Spanish National Healthcare System: a social return on investment analysis.

BACKGROUND: Haemophilia A (HA) has been associated with poor health-related quality of life and a large economic burden, accentuated by severity, arthropathy, and inhibitors. To meet global standards of care, the management of HA should align with the principles of care outlined by the World Federation of Haemophilia. The aims of the present study were to establish a set of proposals to improve HA management within the Spanish National Health System (SNHS) and to estimate the impact its hypothetical implementation would generate from a clinical, healthcare, economic, and social perspective. METHODS: A multidisciplinary group of experts agreed on a set of 15 proposals to improve HA management within the SNHS. Thereafter, a forecast-type Social Return on Investment analysis was carried out to estimate the impact of implementing this set of proposals within the SNHS over a one-year timeframe, in relation to the required investment. RESULTS: This study estimated that the implementation of the complete set of 15 proposals would require a total investment of 2.34 M€ and have a total impact of 14.60 M€. Accordingly, every euro invested in the complete set of 15 proposals would yield a social return of €6.23 (€3.37 in the worst-case scenario and €9.69 in the best-case scenario) of both tangible and intangible nature in similar proportions (45.71 and 54.29%, respectively). CONCLUSIONS: These results can be used to inform policy and practice such that interventions that may potentially improve current public health challenges associated with the management of HA may be implemented.

Evaluation of Training in Pediatric Ultrasound-guided Vascular Cannulation Using a Model.

BACKGROUND: The study objective was to evaluate a training program and a training model for pediatric ultrasound-guided vascular cannulation (USGVC) by inexperienced operators. METHODS: An observational descriptive study was conducted at the pediatric intensive care unit of a level-III hospital. The study protocol comprised the following parts: (1) pretraining test; (2) theory and practice training session consisting of an explanation of basic vascular ultrasound concepts plus performing vascular cannulation in a model; (3) posttraining test; and (4) evaluation of the training model. RESULTS: A total of 25 health-care professionals participated in the study. All of them possessed the skills to locate vessels and ultrasound planes, and they performed USGVC using the training model. On a 1-5 scale, the model was rated to have 87.6% fidelity with real pediatric patients; the best regarded aspect of it was utility (93%). Differences were found between pre- and post-training scores: 2.72 ± 0.84 versus 4.60 ± 0.50; P < 0.001 (95% confidence interval: -2.28, -1.47). Altogether, 300 ultrasound-guided cannulation procedures were carried out (12 per participant) distributed along the longitudinal axis in plane and the transverse axis out of plane, with 150 punctures in each of them. The success rate for USGVC in the training model was 79.7%, the mean time for the procedure was 115.6 ± 114.9 s, and the mean time for achieving successful cannulation was 87.69 ± 82.81 s. The mean number of trials needed for successful USGVC was 1.49 ± 0.86. CONCLUSION: After undergoing the theory-practice training, participants: (a) improved their knowledge of ultrasound-guided vascular access; (b) positively evaluated the USGVC training model, in particular its utility and fidelity as compared with cannulation in pediatric patients; and (c) achieved a high USGVC success rate in a relatively short time.

Impact of non-cardiovascular comorbidities on the quality of life of patients with chronic heart failure: a scoping review.

PURPOSE: To determine the impact of non-cardiovascular comorbidities on the health-related quality of life (HRQoL) of patients with chronic heart failure (CHF). METHODS: A scoping review of the scientific literature published between 2009 and 2019 was carried out. Observational studies which assessed the HRQoL of patients with CHF using validated questionnaires and its association with non-cardiovascular comorbidities were included. RESULTS: The search identified 1904 studies, of which 21 fulfilled the inclusion criteria to be included for analysis. HRQoL was measured through specific, generic, or both types of questionnaires in 72.2%, 16.7%, and 11.1% of the studies, respectively. The most common comorbidities studied were diabetes mellitus (12 studies), mental and behavioral disorders (8 studies), anemia and/or iron deficiency (7 studies), and respiratory diseases (6 studies). Across studies, 93 possible associations between non-cardiovascular comorbidities and HRQoL were tested, of which 21.5% regarded anemia or iron deficiency, 20.4% mental and behavioral disorders, 20.4% diabetes mellitus, and 14.0% respiratory diseases. Despite the large heterogeneity across studies, all 21 showed that the presence of a non-cardiovascular comorbidity had a negative impact on the HRQoL of patients with CHF. A statistically significant impact on worse HRQoL was found in 84.2% of associations between mental and behavioral disorders and HRQoL (patients with depression had up to 200% worse HRQoL than patients without depression); 73.7% of associations between diabetes mellitus and HRQoL (patients with diabetes mellitus had up to 21.8% worse HRQoL than patients without diabetes mellitus); 75% of associations between anemia and/or iron deficiency and HRQoL (patients with anemia and/or iron deficiency had up to 25.6% worse HRQoL than between patients without anemia and/or iron deficiency); and 61.5% of associations between respiratory diseases and HRQoL (patients with a respiratory disease had up to 21.3% worse HRQoL than patients without a respiratory disease). CONCLUSION: The comprehensive management of patients with CHF should include the management of comorbidities which have been associated with a worse HRQoL, with special emphasis on anemia and iron deficiency, mental and behavioral disorders, diabetes mellitus, and respiratory diseases. An adequate control of these comorbidities may have a positive impact on the HRQoL of patients.

Does written informed consent adequately inform surgical patients? A cross sectional study.

BACKGROUND: Informed consent (IC) is an essential step in helping patients be aware of consequences of their treatment decisions. With surgery, it is vitally important for patients to understand the risks and benefits of the procedure and decide accordingly. We explored whether a written IC form was provided to patients; whether they read and signed it; whether they communicated orally with the physician; whether these communications influenced patient decisions. METHODS: Adult postsurgical patients in nine general hospitals of Italy's Campania Region were interviewed via a structured questionnaire between the second and seventh day after the surgery at the end of the first surgical follow up visit. Physicians who were independent from the surgical team administered the questionnaire. RESULTS: The written IC form was given to 84.5% of those interviewed. All recipients of the form signed it, either personally or through a delegate; however, 13.9% did not know/remember having done so; 51.8% said that they read it thoroughly. Of those who reported to have read it, 90.9% judged it to be clear. Of those receiving the written consent form, 52.0% had gotten it the day before the surgery at the earliest 41.1% received it some hours or immediately before the procedure. The written IC form was explained to 65.6% of the patients, and 93.9% of them received further oral information that deemed understandable. Most attention was given to the diagnosis and the type of surgical procedure, which was communicated respectively to 92.8 and 88.2% of the patients. Almost one in two patients believed that the information provided some emotional relief, while 23.2% experienced increased anxiety. Younger patients (age ≤ 60) and patients with higher levels of education were more likely to read the written IC form. CONCLUSIONS: The written IC form is not sufficient in assuring patients and making them fully aware of choices they made for their health; pre-operative information that was delivered orally better served the patients' needs. To improve the quality of communication we suggest enhancing physicians' communication skills and for them to use structured conversation to ensure that individuals are completely informed before undergoing their procedures.

Deciphering function of the pulmonary arterial sphincters in loggerhead sea turtles (Caretta caretta).

To provide new insight into the pathophysiological mechanisms underlying gas emboli (GE) in bycaught loggerhead sea turtles (Caretta caretta), we investigated the vasoactive characteristics of the pulmonary and systemic arteries, and the lung parenchyma (LP). Tissues were opportunistically excised from recently dead animals for in vitro studies of vasoactive responses to four different neurotransmitters: acetylcholine (ACh; parasympathetic), serotonin (5HT), adrenaline (Adr; sympathetic) and histamine. The significant amount of smooth muscle in the LP contracted in response to ACh, Adr and histamine. The intrapulmonary and systemic arteries contracted under both parasympathetic and sympathetic stimulation and when exposed to 5HT. However, proximal extrapulmonary arterial (PEPA) sections contracted in response to ACh and 5HT, whereas Adr caused relaxation. In sea turtles, the relaxation in the pulmonary artery was particularly pronounced at the level of the pulmonary artery sphincter (PASp), where the vessel wall was highly muscular. For comparison, we also studied tissue response in freshwater sliders turtles (Trachemys scripta elegans). Both PEPA and LP from freshwater sliders contracted in response to 5HT, ACh and also Adr. We propose that in sea turtles, the dive response (parasympathetic tone) constricts the PEPA, LP and PASp, causing a pulmonary shunt and limiting gas uptake at depth, which reduces the risk of GE during long and deep dives. Elevated sympathetic tone caused by forced submersion during entanglement with fishing gear increases the pulmonary blood flow causing an increase in N2 uptake, potentially leading to the formation of blood and tissue GE at the surface. These findings provide potential physiological and anatomical explanations on how these animals have evolved a cardiac shunt pattern that regulates gas exchange during deep and prolonged diving.

A robot-based gait training therapy for pediatric population with cerebral palsy: goal setting, proposal and preliminary clinical implementation.

BACKGROUND: The use of robotic trainers has increased with the aim of improving gait function in patients with limitations. Nevertheless, there is an absence of studies that deeply describe detailed guidelines of how to correctly implement robot-based treatments for gait rehabilitation. This contribution proposes an accurate robot-based training program for gait rehabilitation of pediatric population with Cerebral Palsy (CP). METHODS: The program is focused on the achievement of some specifications defined by the International Classification of Functioning, Disability and Health framework, Children and Youth version (ICF-CY). It is framed on 16 non-consecutive sessions where motor control, strength and power exercises of lower limbs are performed in parallel with a postural control strategy. A clinical evaluation with four pediatric patients with CP using the CPWalker robotic platform is presented. RESULTS: The preliminary evaluation with patients with CP shows improvements in several aspects as strength (74.03 ± 40.20%), mean velocity (21.46 ± 33.79%), step length (17.95 ± 20.45%) or gait performance (e.g. 66 ± 63.54% in Gross Motor Function Measure-88 items, E and D dimensions). CONCLUSIONS: The improvements achieved in the short term show the importance of working strength and power functions meanwhile over-ground training with postural control. This research could serve as preliminary support for future clinical implementations in any robotic device. TRIAL REGISTRATION: The study was carried out with the number R-0032/12 from Local Ethical Committee of the Hospital Infantil Niño Jesús. Public trial registered on March 23, 2017: ISRCTN18254257 .

[Epidemiology of traumatic spinal cord injury in Gran Canaria]. / Epidemiología de la lesión medular de origen traumático en Gran Canaria.

OBJECTIVE: To evaluate the epidemiological and clinical trends in acute traumatic spinal cord injuries. MATERIAL AND METHODS: A retrospective study was conducted of traumatic spinal cord injury patients in Gran Canaria (Canary Islands, Spain) from 2000 to 2014. Demographic and spinal injury severity trends were analysed by year of injury grouped into 3 periods: 2000-2005, 2006-2010, and 2011-2014. RESULTS: The sample included 141 patients. The mean incidence for the entire period was 12 cases/million/year. There was a decrease in cases in the second and third period. While the male/female ratio was 3.8/1 and was maintained in all periods, the mean patient age increased from 38.8 in 2000-5 to 54.5 years in 2011-4 (P<.05). Falls have been the leading cause of spinal cord injury (48.2%), followed by traffic accidents (37.6%). Falls have increased, especially in the older population. Incomplete tetraplegia has been the most prevalent group (30.5%). A vertebral fracture was suffered by 70.3% of all patients, with 93.2% of them requiring surgery. CONCLUSIONS: There has been a decrease in the incidence of traumatic spinal cord injury in recent years. The target population has changed, and the older population is currently the most affected. This reality suggests the need to change the local prevention campaigns for spinal cord injury in the elderly.

Burden of Disease Study of Patients with Neovascular Age-Related Macular Degeneration in Spain.

INTRODUCTION: Neovascular age-related macular degeneration (nAMD) is a progressive retinal disease that causes severe and irreversible vision loss. The disease can therefore have a significant impact on the life of patients' and their families. The aim of this study was to evaluate the socio-economic burden of nAMD in Spain. METHODS: The annual cost per patient with nAMD was estimated for the first, second, and third year (or beyond) of treatment since diagnosis. Several cost categories were considered including direct healthcare costs (DHC), direct non-healthcare costs (DNHC), labor productivity losses (LPL), and intangible costs (IC) related to loss of quality of life. The average annual cost per patient was estimated by assigning a unit price or financial proxy to the resources consumed per patient. Reference year of costs was 2021. RESULTS: The mean annual cost of nAMD was estimated at €17,265, €15,403, and €14,465 per patient in the first, second, and third year of treatment after diagnosis. There was an additional one-off cost of €744 associated with the diagnosis of nAMD. DHC accounted for most of the total annual cost per patient independent of the year of treatment since diagnosis (48% in year 1; 42% in year 2; 39% in year 3). Similarly, DNHC had an important contribution to the total costs (32% in year 1; 35% in year 2; 37% in year 3), followed by IC (20% in year 1; 23% in year 2; 24% in year 3), while the contribution of patients' LPL was minimal. CONCLUSION: This study estimated a high economic burden associated with nAMD for patients and their families, the healthcare system, and society at large. There is a need to improve the management of these patients to reduce the impact of nAMD disease progression.

Burden of Disease Study of Patients with Diabetic Macular Oedema in Spain.

INTRODUCTION: Diabetic macular oedema (DMO) is a complication of diabetic retinopathy that can result in vision loss. The disease can impact different spheres of a patient's life, including physical and psychological health, work, and activities of daily living, entailing an important use of healthcare and non-healthcare resources. This study aimed to estimate the socio-economic burden of DMO in Spain. METHODS: The burden of DMO was estimated from a societal perspective, per patient, year of treatment since diagnosis, and type of treatment. Four categories were considered: direct healthcare costs (DHC), direct non-healthcare costs (DNHC), labour productivity losses (LPL), and intangible costs (IC) associated with loss of quality of life. Average annual costs were calculated by multiplying the resources used per patient by their corresponding unit price (or financial proxy). For a more accurate estimation, differences in resource use between treatments (intravitreal anti-vascular endothelial growth factor injections of ranibizumab or aflibercept, and intravitreal dexamethasone implants) and year since diagnosis (first, second, and third year or beyond) were considered and presented separately. The reference year for costs was 2021. RESULTS: The average annual costs of DMO in the first year of treatment after diagnosis was estimated at €18,774, €17,512, and €16,188 per patient treated with ranibizumab, aflibercept, and dexamethasone, respectively. This burden would be reduced to €15,783, €15,701, and €12,233 in the second year, and to €15,119, €15,043, and €12,790 in the third year, respectively. Diagnosis of DMO entails an additional one-off cost of €485. DHC accounted for the greatest proportion of total annual costs per patient, independent of the year, with LPL also making an important contribution to total costs. CONCLUSIONS: The socio-economic impact of DMO on patients, the healthcare system, and society at large is substantial. The constant increase in its prevalence accentuates the need for planning and implementation of healthcare strategies to prevent vision loss and reduce the socio-economic burden of the disease.

The socioeconomic burden of adult attention-deficit/hyperactivity disorder in Spain.

Introduction: The symptoms of attention-deficit/hyperactivity disorder (ADHD) in adults highly interfere with function in multiple dimensions, increasing the economic burden associated with ADHD. The aim of this study was to explore the impact of ADHD in Spanish adults and estimate the associated economic burden within the healthcare, social, economic, and legal domains. Methods: An economic model was developed from a social perspective using a bottom-up approach, based on the scientific literature and a multidisciplinary expert group. Results: The cost incurred per diagnosed adult patient with ADHD included an annual cost of €15,652 and a one-time cost of €7,893 (3,035 M€ and 1,531 M€ for Spain, respectively). Regarding the annual cost, 50% was attributed to costs within the economic domain, of which 53% were work-absenteeism-related. Moreover, 28% was attributed to costs within the social domain, of which 74% were substance-abuse-related. Regarding the one-time cost, 52% was attributed to costs within the healthcare domain, of which approximately 50% were hospitalization-related costs. Moreover, 42% was attributed to costs within the legal domain, of which 62% were imprisonment-related costs. Conclusions: This is the first report on the socioeconomic burden of ADHD in Spanish adults, shedding light on the large burden that adult ADHD poses on the healthcare system and society at large, as symptoms have been shown to impact almost every aspect of life. This is particularly important for undiagnosed/untreated patients with ADHD in Spain, as appropriate treatments have shown positive results in these areas and may reduce its associated socioeconomic burden.

A SARS-CoV-2 full genome sequence of the B.1.1 lineage sheds light on viral evolution in Sicily in late 2020.

To investigate the influence of geographic constrains to mobility on SARS-CoV-2 circulation before the advent of vaccination, we recently characterized the occurrence in Sicily of viral lineages in the second pandemic wave (September to December 2020). Our data revealed wide prevalence of the then widespread through Europe B.1.177 variant, although some viral samples could not be classified with the limited Sanger sequencing tools used. A particularly interesting sample could not be fitted to a major variant then circulating in Europe and has been subjected here to full genome sequencing in an attempt to clarify its origin, lineage and relations with the seven full genome sequences deposited for that period in Sicily, hoping to provide clues on viral evolution. The obtained genome is unique (not present in databases). It hosts 20 single-base substitutions relative to the original Wuhan-Hu-1 sequence, 8 of them synonymous and the other 12 encoding 11 amino acid substitutions, all of them already reported one by one. They include four highly prevalent substitutions, NSP12:P323L, S:D614G, and N:R203K/G204R; the much less prevalent S:G181V, ORF3a:G49V and N:R209I changes; and the very rare mutations NSP3:L761I, NSP6:S106F, NSP8:S41F and NSP14:Y447H. GISAID labeled this genome as B.1.1 lineage, a lineage that appeared early on in the pandemic. Phylogenetic analysis also confirmed this lineage diagnosis. Comparison with the seven genome sequences deposited in late 2020 from Sicily revealed branching leading to B.1.177 in one branch and to Alpha in the other branch, and suggested a local origin for the S:G118V mutation.

A Social Return on Investment Analysis of Improving the Management of Chronic Obstructive Pulmonary Disease Within the Spanish National Healthcare System.

Purpose: To define a set of proposals that would improve the current management of chronic obstructive pulmonary disease (COPD) within the Spanish National Healthcare System (SNHS) from a comprehensive multidisciplinary perspective and to assess the impact of its implementation from clinical, healthcare, economic, and social perspectives. Patients and Methods: A group of 20 stakeholders related to COPD (healthcare professionals, patients, and informal caregivers, among others) participated in an online Delphi process to agree on a set of 15 proposals that would improve the current management of COPD within the SNHS in four areas: diagnosis, risk stratification, management of exacerbations, and management of stable COPD. A one-year forecast-type social return on investment (SROI) analysis was used to estimate the impact that implementing the set of proposals would have in relation to the investment required. A sensitivity analysis was used to test the strength of the model when varying assumption-based data-points. Results: The hypothetical implementation of the complete set of 15 proposals would require a €668 million investment and would generate a €2079 million social impact concerning savings for the SNHS and quality of life improvements for patients and their informal caregivers, among others. Accordingly, for every euro invested in the set of proposals, a social return of €3.11 would be generated (€2.71 in the worst-case scenario and €3.62 in the best-case scenario) of both tangible (32.56%) and intangible nature (67.44%). Conclusion: Altogether, implementing this set of 15 proposals would generate a positive social impact, threefold the required investment. The results may inform decisions relative to healthcare policy and practice regarding COPD management within the SNHS, further contributing to reduce the large burden of COPD.

Ultrasound-guided pediatric vascular cannulation by inexperienced operators: outcomes in a training model.

OBJECTIVE: To present the results of an ultrasound vascular cannulation (UGVC) training program for inexperienced operators using a training model. METHOD: This was a descriptive observational study developed in the paediatric intensive care unit (PICU) of a third-level hospital. Operators received basic theoretical training in the USVC technique, followed by practical training with a model designed for USVC-inexperienced healthcare professionals. RESULTS: The study included 25 healthcare professionals, who carried out a total of 300 ultrasound-guided cannulation procedures (12 per participant) at equidistant sites on the longitudinal axis/in-plane (LA/IP) and the transverse axis/out-of-plane (TA/OP). The mean depth of cannulated vessels was 0.90 (0.34) cm and their mean diameter was 0.41 (0.1) cm. In 41.7% of cases, complete view of the needle (CVN) was accomplished; in 49% of cases, repositioning of the needle/guidewire (RNG) was necessary for successful UGVC. The rate of successful UGVC in the training model was 79.7%. The mean time required for the procedure was 74.70 (73.72) seconds. The time to successful cannulation was 58.72 (56.87) seconds. The mean number of attempts needed until successful UGVC was 1.31 (0.72). Complications were: (a) 26.3% vessel perforation/wrong guidewire positioning (VP/WGP) and (b) 4.3% successful vessel puncture followed by failure to accomplish subsequent cannulation. CONCLUSIONS: Through the present theoretical-practical training program for inexperienced operators using a training model: (a) high success rates and short procedural times were attained; (b) complete view of needle and need for repositioning the needle/guidewire occurred in half of the procedures; and (c) complications occurred in a third of the procedures.

Validating 3D indexes in the non-surgical pectus excavatum patient.

BACKGROUND/PURPOSE: In recent years there has been an increased interest in three-dimensional (3D) imaging for the assessment of chest wall deformities. Some studies have proven a correlation between 3D and traditional cross-sectional images but only for patients who already had an indication for a computed tomography (CT) scan prior to surgery; mainly due to their severity. Our aim is to determine the accuracy and reliability of the measures obtained by a portable 3D scanner in a cohort of pectus excavatum (PE) patients with different severity grades, as well as in controls. METHODS: We conducted a study comparing radiological and optical indexes on magnetic resonance imaging (MRI) and 3D surface images. We used a hand-held 3D scanner to obtain the optical Haller Index (3DHI) and Correction Index (3DCI) and a limited MRI scan to obtain the traditional indexes. A statistical analysis was carried out to determine the correlation between optical and radiological measures, plus a subjective severity evaluation. RESULTS: Twenty-eight patients and controls were enrolled in the study. In both the control and PE groups, there was a significant positive correlation between the indexes, especially for the CI. There were no differences in correlation regarding gender, age or severity. CI appears to better discriminate amongst the different severity groups and controls. CONCLUSION: 3D surface imaging is feasible and appropriate to use to assess PE, regardless of the severity or characteristics of the individual patient. Even with a small hand-held device, we can obtain accurate images and measures which are especially useful for the assessment of the nonsurgical pectus patient.

Wilson disease: revision of diagnostic criteria in a clinical series with great genetic homogeneity.

BACKGROUND: Wilson disease is an autosomal recessive disorder of copper metabolism caused by mutations in the ATP7B gene. An early diagnosis is crucial to prevent evolution of the disease, as implantation of early therapeutic measures fully prevents its symptoms. As population genetics data predict a higher than initially expected prevalence, it was important to define the basic diagnostic tools to approach population screening. METHODS: A highly genetically homogeneous cohort of 70 patients, belonging to 50 unrelated families, has been selected as a framework to analyze all their clinical, biochemical and genetic characteristics, to define the disease in our population, with an estimated prevalence of 1 in 12,369, and determine the most useful features that reach diagnostic value. RESULTS: Serum ceruloplasmin below 11.5 mg/dL and cupremia below 60 µg/mL, were the best analytical predictors of the disease in asymptomatic individuals, while cupruria or hepatic copper determination were less powerful. Genetic analysis reached a conclusive diagnosis in all 65 patients available for complete testing. Of them, 48 were carriers of at least one p.Leu708Pro mutant allele, with 24 homozygotes. Nine patients carried a promoter deletion mutation, revealing that extended sequencing beyond the ATP7B gene-coding region is essential. All mutations caused hepatic damage since early ages, increasing its severity as diagnosis was delayed, and neurological symptoms appear. CONCLUSION: Serum ceruloplasmin determination followed by genetic screening would reduce costs and favor the prioritization of non-invasive procedures to reach a definitive diagnosis, even for asymptomatic cases.

Medial gastrocnemius structure and gait kinetics in spastic cerebral palsy and typically developing children: A cross-sectional study.

To compare medial gastrocnemius muscle-tendon structure, gait propulsive forces, and ankle joint gait kinetics between typically developing children and those with spastic cerebral palsy, and to describe significant associations between structure and function in children with spastic cerebral palsy.A sample of typically developing children (n = 9 /16 limbs) and a sample of children with spastic cerebral palsy (n = 29 /43 limbs) were recruited. Ultrasound and 3-dimensional motion capture were used to assess muscle-tendon structure, and propulsive forces and ankle joint kinetics during gait, respectively.Children with spastic cerebral palsy had shorter fascicles and muscles, and longer Achilles tendons than typically developing children. Furthermore, total negative power and peak negative power at the ankle were greater, while total positive power, peak positive power, net power, total vertical ground reaction force, and peak vertical and anterior ground reaction forces were smaller compared to typically developing children. Correlation analyses revealed that smaller resting ankle joint angles and greater maximum dorsiflexion in children with spastic cerebral palsy accounted for a significant decrease in peak negative power. Furthermore, short fascicles, small fascicle to belly ratios, and large tendon to fascicle ratios accounted for a decrease in propulsive force generation.Alterations observed in the medial gastrocnemius muscle-tendon structure of children with spastic cerebral palsy may impair propulsive mechanisms during gait. Therefore, conventional treatments should be revised on the basis of muscle-tendon adaptations.

Ultrasound-guided vascular cannulation. Experience in critically-ill pediatric patients. / Canalización vascular ecoguiada. Experiencia en el paciente pediátrico crítico.

INTRODUCTION: Central vascular cannulation in children is a highly complex technique and poses many difficulties. Vascular ultrasound can make this procedure easier. OBJECTIVE: To describe the characteristics of ultrasound-guided vascular cannulation in critically-ill pediatric patients. POPULATION AND METHODS: Outcome measures prospectively recorded were vessels most frequently cannulated, their localization, the measurement of their diameter/depth, the success rate and complications developed, among others. RESULTS: One hundred and twenty four vascular punctures were performed in 86 pediatric patients. Vascular accesses were the femoral vein (39.7%), followed by the femoral artery (27.2%) and the internal jugular vein (14.7%). Femoral vessels were localized at a depth of 0.75 ± 0.25 mm, with a mean diameter of 0.31 ± 0.16 mm. The depth of jugular vein vessels was smaller (0.64 ± 0.24 mm) and their overall diameter, larger (0.44 ± 0.19 mm). The mean number of attempts in ultrasound-guided cannulations was 2.2 ± 1.3. The success rate was 79% and was associated to a larger vessel diameter (0.39 ± 0.20 mm vs. 0.28 ± 0.13 mm, p= 0.01) and a lower number of attempts (1.90 ± 1.16 vs. 3.45 ± 1.77, p= 0.001). Complications were accidental puncture of another vessel (5.3%) and hematoma formation during puncture (2.3%). CONCLUSIONS: In the pediatric patients studied, ultrasound-guided vascular cannulation allowed vessel visualization and measurement of their depth and diameter; the success rate was high and it was associated to a low complication rate.

Introducción. La canalización vascular central en niños presenta alta complejidad técnica y dificultades. La ecografía vascular puede facilitar este procedimiento. Objetivo. Describir las características de las canalizaciones vasculares ecoguiadas en el paciente pediátrico crítico. Población y métodos. Las variables de interés registradas prospectivamente fueron los vasos más comúnmente canalizados, su localización, la medición del diámetro/profundidad, la tasa de éxito y las complicaciones presentadas, entre otras. Resultados. En 86 pacientes pediátricos, se realizaron 124 punciones vasculares. Los accesos vasculares fueron la vena femoral (39,7%), seguida de la arteria femoral (27,2%) y la vena yugular interna (14,7%). Los vasos femorales se localizaron a una profundidad de 0,75 ± 0,25 mm con un diámetro medio de 0,31 ± 0,16 mm. La profundidad de los vasos venosos yugulares fue menor (0,64 ± 0,24 mm) y su diámetro global, mayor (0,44 ± 0,19 mm). El número medio de intentos en las canalizaciones vasculares ecoguiadas fue de 2,2 ± 1,3. La tasa de éxito fue del 79% asociada a un mayor diámetro del vaso (0,39 ± 0,20 mm vs. 0,28 ± 0,13 mm, p 0,01) y un menor número de intentos (1,90 ± 1,16 vs. 3,45 ± 1,77, p= 0,001). Las complicaciones, fueron la punción accidental de otro vaso (5,3%) y el desarrollo de un hematoma durante la punción (2,3%). Conclusiones. La canalización vascular ecoguiada en los pacientes pediátricos estudiados permite visualizar los vasos y medir su profundidad y diámetro; presenta una alta tasa de éxito y se asocia a una baja tasa de complicaciones.

Impact of gas emboli and hyperbaric treatment on respiratory function of loggerhead sea turtles (Caretta caretta).

Fisheries interactions are the most serious threats for sea turtle populations. Despite the existence of some rescue centres providing post-traumatic care and rehabilitation, adequate treatment is hampered by the lack of understanding of the problems incurred while turtles remain entrapped in fishing gears. Recently it was shown that bycaught loggerhead sea turtles (Caretta caretta) could experience formation of gas emboli (GE) and develop decompression sickness (DCS) after trawl and gillnet interaction. This condition could be reversed by hyperbaric O2 treatment (HBOT). The goal of this study was to assess how GE alters respiratory function in bycaught turtles before recompression therapy and measure the improvement after this treatment. Specifically, we assessed the effect of DCS on breath duration, expiratory and inspiratory flow and tidal volume (VT), and the effectiveness of HBOT to improve these parameters. HBOT significantly increased respiratory flows by 32-45% while VT increased by 33-35% immediately after HBOT. Repeated lung function testing indicated a temporal increase in both respiratory flow and VT for all bycaught turtles, but the changes were smaller than those seen immediately following HBOT. The current study suggests that respiratory function is significantly compromised in bycaught turtles with GE and that HBOT effectively restores lung function. Lung function testing may provide a novel means to help diagnose the presence of GE, be used to assess treatment efficacy, and contribute to sea turtle conservation efforts.