Monitoring multiple parameters in complex water scenarios using a low-cost open-source data acquisition platform.

Water monitoring faces challenges that are driven by the infrastructure, protection, financial resources, science and innovation policies, among others. A modular, low-cost, fully open-source and small-sized Unmanned Surface Vessel (USV) called EMAC-USV (EMAC: Estación de Monitoreo Ambiental Costero), is proposed for monitoring bathymetry and water quality parameters (i.e. temperature, suspended solids concentration and hydrocarbon concentration) in complex water scenarios. A detailed description of each part of the platform as well as all electronic connections and functioning is presented.The field works were carried out in two small waste stabilization ponds and in a portion of the main tidal channel of the Bahía Blanca port. The EMAC-USV is the result of a cautious design, regarding the balancing performance, communications, payload capacity, among others.

A new giant velvet worm from Costa Rica suggests absence of the genus Peripatus (Onychophora: Peripatidae) in Central America

Introduction: Neotropical onychophoran taxonomy and diversity has been poorly investigated. Recent studies have discovered problems in species classification: they have questioned the accepted genera and the actual number of species. This is true in Costa Rica, where several unidentified species have been reported. Objective: The objective of this investigation was to evaluate the occurrence of the accepted genera in this country, and to describe a new genus and species from Central America. Methods: In 2017, we collected one onychophoran in the Keköldi Indigenous Reserve in Talamanca, Limón, Costa Rica. The specimen gave birth to several offspring. Therefore, seven organisms were analyzed. Light microscopy was used to observe the gross morphology in all samples. The detailed morphology was studied in the biggest specimen with scanning electron microscopy; after that, we performed a phylogenetic analysis with the corresponding sequence of COI. Results: According to our results, a new genus and species of giant onychophoran was found. The genus was identified by its giant size, apical piece of seven scale ranks, large conical primary papillae, dorso-median furrow flanked by two-three accessory papillae, the absence of hyaline organs and a marked sexual dimorphism with respect to the number of legs. The new species presents a particular head pattern, as well as novel structures like cephalic papillae, accessory papillae with rudimentary apical pieces, and a lack of antennal chemoreceptors. Phylogenetic analysis rendered our genus as monophyletic and includes Peripatus solorzanoi, which is grouped within the Central American clade.As our species is clustered inside the Costa Rica-Panamanian group, it is not related to the Caribbean Island nor Guyanan Shield samples, home of Epiperipatus and Peripatus respectively. Therefore, we suggest that those genera do not occur in Central America, and a new genus exists: Mongeperipatus, gen. nov. Conclusion: We concluded that Costa Rica is home to a diversity of undescribed onychophorans that requires specific studies to help clarify the taxonomy and evolutionary relationships of the group to justify their protection.

Introducción: Los onicóforos del neotrópico han sido poco estudiados, especialmente en cuanto a taxonomía y diversidad. Sin embargo, estudios recientes sugieren que los géneros actuales son obsoletos y el número de especies existentes es desconocido. Por ejemplo, en Costa Rica, se han reportado diversos onicóforos que no han sido identificados exitosamente. Objetivo: El objetivo de este trabajo fue evaluar la presencia de los géneros aceptados en el país, y describir un nuevo género y especie para Centroamérica. Métodos: En el 2017, recolectamos un espécimen de onicóforo en la reserva indígena de Keköldi en Talamanca, Limón, Costa Rica, el cual dio a luz a varias crías. Incluyendo a este animal, estudiamos un total de siete especímenes. Mediante el uso de microscopía de luz, observamos las características morfológicas macroscópicas en todas las muestras. Asimismo, utilizamos la microscopía electrónica para analizar detalladamente la morfología en una de estas. Finalmente, realizamos un análisis filogenético con la secuencia correspondiente de COI. Resultados: Un nuevo onicóforo gigante fue descrito. Se propuso un nuevo género, el cual se distingue por su tamaño gigante, el número de filas de escamas en la pieza apical de las papilas primarias, sus grandes papilas primarias cónicas, la línea media flanqueada por dos-tres papilas accesorias, la ausencia de órganos hialinos y el dimorfismo sexual marcado respecto al número de patas. Esta especie presenta un patrón particular de papilas en su cabeza, además de estructuras nuevas como papilas modificadas en la cabeza (papilas cefálicas), papilas accesorias con piezas apicales rudimentarias y la ausencia de quimiorreceptores en las antenas. Los análisis filogenéticos lo sitúan como un género monofilético que incluye a Peripatus solorzanoi, tal clado se encuentra dentro del grupo de especies centroamericanas. Por lo tanto, la nueva especie se agrupa dentro de las muestras pertenecientes a Costa Rica-Panamá. Este grupo no se relaciona con los especímenes de las islas caribeñas ni del Escudo Guyanés, hogares de las especies tipo de Epiperipatus y Peripatus respectivamente. De modo que sugerimos que estos géneros no están presentes en Centroamérica. Así entonces, describimos el nuevo género Mongeperipatus para Costa Rica. Conclusión: Este país alberga una diversidad de onicóforos sin describir, que se requieren estudios puntuales que ayuden a aclarar la taxonomía y relaciones evolutivas del grupo para justificar la protección del filo.

The Latin American experience with a next generation sequencing genetic panel for recessive limb-girdle muscular weakness and Pompe disease.

BACKGROUND: Limb-girdle muscular dystrophy (LGMD) is a group of neuromuscular disorders of heterogeneous genetic etiology with more than 30 directly related genes. LGMD is characterized by progressive muscle weakness involving the shoulder and pelvic girdles. An important differential diagnosis among patients presenting with proximal muscle weakness (PMW) is late-onset Pompe disease (LOPD), a rare neuromuscular glycogen storage disorder, which often presents with early respiratory insufficiency in addition to PMW. Patients with PMW, with or without respiratory symptoms, were included in this study of Latin American patients to evaluate the profile of variants for the included genes related to LGMD recessive (R) and LOPD and the frequency of variants in each gene among this patient population. RESULTS: Over 20 institutions across Latin America (Brazil, Argentina, Peru, Ecuador, Mexico, and Chile) enrolled 2103 individuals during 2016 and 2017. Nine autosomal recessive LGMDs and Pompe disease were investigated in a 10-gene panel (ANO5, CAPN3, DYSF, FKRP, GAA, SGCA, SGCB, SGCD, SGCG, TCAP) based on reported disease frequency in Latin America. Sequencing was performed with Illumina's NextSeq500 and variants were classified according to ACMG guidelines; pathogenic and likely pathogenic were treated as one category (P) and variants of unknown significance (VUS) are described. Genetic variants were identified in 55.8% of patients, with 16% receiving a definitive molecular diagnosis; 39.8% had VUS. Nine patients were identified with Pompe disease. CONCLUSIONS: The results demonstrate the effectiveness of this targeted genetic panel and the importance of including Pompe disease in the differential diagnosis for patients presenting with PMW.

Dysferlin quantification in monocytes for rapid screening for dysferlinopathies.

INTRODUCTION: In this study, we determined normal levels of dysferlin expression in CD14+ monocytes by flow cytometry (FC) as a screening tool for dysferlinopathies. METHODS: Monocytes from 183 healthy individuals and 29 patients were immunolabeled, run on an FACScalibur flow cytometer, and analyzed by FlowJo software. RESULTS: The relative quantity of dysferlin was expressed as mean fluorescence intensity (MFI). Performance of this diagnostic test was assessed by calculating likelihood ratios at different MFI cut-off points, which allowed definition of 4 disease classification groups in a simplified algorithm. CONCLUSION: The MFI value may differentiate patients with dysferlinopathy from healthy individuals; it may be a useful marker for screening purposes. Muscle Nerve 54: 1064-1071, 2016.

Perspectives on bullying among children who present to the emergency department with behavioral misconduct: a qualitative study.

UNLABELLED: The problem of bullying is an increasing public health threat encountered by emergency physicians especially in inner city emergency departments (EDs). Bullying may result in emotional disturbances and psychological trauma in children. Many children sent to the ED because of behavioral misconduct require immediate stabilization and treatment. The emergency physician performs an initial assessment and stabilization. Emergency departments are increasingly on the frontline of the bullying problem. OBJECTIVES: Our objective was to explore children's perspective of bullying and their views of potential solutions. METHODS: A qualitative study was conducted in a cohort of 50 children (age, 8-17 years), who were referred to the ED from school because of their behavioral misconduct. An interview survey tool about bullying was administered. It focused on what bullying meant to them and what advice they have for a child who is bullied. They were also asked what advice they would have for adults who try to help. We used grounded theory to analyze the data. Similar concepts were grouped, and the categories with similar properties and dimensions were defined. Common themes were then identified. RESULTS: We interviewed 50 children, of whom 27 were boys and 23 were girls. Their mean (SD) age was 12.5 (2.12) years (range, 8-17 years). Bullying was identified by children as including physical, verbal, and emotional actions. Several themes emerged. First, a power imbalance between a bully and victim may render an individual vulnerable to bullying. Being different and weak also increases the risk of being bullied. Second, bullying is wrong, and the bully should be punished. Third, children should learn how to handle bullying situations and develop resilience against bullying. Finally, adults need to be more proactive to prevent or stop bullying. CONCLUSIONS: Our results provide insights into the perceptions of children regarding bullying. We have garnered a better understanding of what these children feel adults should do to prevent bullying.

Designing the modern pump: engineering aspects of continuous subcutaneous insulin infusion software.

Insulin delivery systems attracted the efforts of biological, mechanical, electrical, and software engineers well before they were commercially viable. The introduction of the first commercial insulin pump in 1983 represents an enduring milestone in the history of diabetes management. Since then, pumps have become much more than motorized syringes and have assumed a central role in diabetes management by housing data on insulin delivery and glucose readings, assisting in bolus estimation, and interfacing smoothly with humans and compatible devices. Ensuring the integrity of the embedded software that controls these devices is critical to patient safety and regulatory compliance. As pumps and related devices evolve, software engineers will face challenges and opportunities in designing pumps that are safe, reliable, and feature-rich. The pumps and related systems must also satisfy end users, healthcare providers, and regulatory authorities. In particular, pumps that are combined with glucose sensors and appropriate algorithms will provide the basis for increasingly safe and precise automated insulin delivery-essential steps to developing a fully closed-loop system.

Efficacy of intravenous immunoglobulin in multifocal motor neuropathy.

Multifocal motor neuropathy is a distinct entity, whose treatment differs from that of other chronic immune-mediated neuropathies, mainly chronic inflammatory demyelinating polyradiculoneuropathy, and its variant, multifocal acquired demyelinating sensory and motor neuropathy, although they share some electrophysiological characteristics. From the first descriptions, intravenous immunoglobulins (IVIg) have been considered to be the gold standard of treatment for multifocal motor neuropathy. However, if the effectiveness of IVIg has been confirmed by several randomized, double-blind, placebo-controlled trials, only a few patients experience persistent improvement after a single or few courses of therapy, and the long-term efficacy of IVIg in this disease is currently debated. Consequently, there is a need for new therapeutic strategies that focus on the effects and the costs of this therapy over long-term follow-up.

Western and Asian features of multiple sclerosis in Mexican Mestizos.

OBJECTIVES: The objective of this study was to compare the clinical expression of MS in Mexican Mestizos with that of patients of European or Asian descent; as well as to compare the annual frequency of new cases with that observed in the previous decades. PATIENTS AND METHODS: All patients with diagnosis of definite MS seen at the National Institute of Neurology and Neurosurgery of Mexico from January 1993 to December 2003 were studied (n=312). Sociodemographic and clinical characteristics were compared with reports of patients from either Western or Asian origin; the long-term disability score was analyzed according to gender, age of onset of MS and the initial symptom. RESULTS: The clinical expression of MS in Mexican Mestizos shares some characteristics with both, Asian and Western forms of MS indicating that the genetic composition of Mexican Mestizos participates in the clinical expression of the disease. Also, at the prevalence date, the mean age of patients and the duration of the disease were lower in our patients than in MS patients from endemic countries suggesting a true increasing incidence in recent times, rather than only improved case ascertainment. CONCLUSIONS: Clinical expression of MS in Mexican Mestizos shows the coexistence of some features common in European and in Asian cases.

[Multifocal motor neuropathy with persistent conduction blocks. A chronic immune-mediated neuropathy]. / Neuropathie motrice multifocale avec blocs de conduction persistants. Une neuropathie dysimmunitaire chronique.

Multifocal motor neuropathy with persistent conduction blocks was first specifically identified in 1986. Its major criterion is conduction blocks in motor nerves only. Clinically, this is a multifocal, thus asymmetric, neuropathy that begins and predominant touches upper limbs; it especially affects men after the age of 50 years and has a chronic course with relapses. Approximately 40-50% of patients also have IgM serum antibodies directed against GM1 ganglioside. There are no other laboratory criteria, although moderately high protein levels are found in cerebrospinal fluid. Its course is unpredictable, because the neuropathy may remain limited to one or two motor nerves or extend progressively to all the motor nerves of all four limbs. In general, there is no damage to sensory or cranial nerves or to the autonomic or central nervous systems. Intravenous polyvalent immunoglobulins at high doses are remarkably effective in the short term in 70 to 80% of cases. Corticosteroids and plasma exchange are generally ineffective and may aggravate the neuropathy. The long-term efficacy of intravenous immunoglobulins in delaying motor decline and axon loss in the affected motor nerves is controversial. No information is currently available about the long-term efficacy of other immunomodulatory treatment.