Enhancing the Maturation of Human Pluripotent Stem Cell-Derived Cardiomyocytes with an n-Type Organic Semiconductor Coating.

Human pluripotent stem cell-derived cardiomyocytes (hPSC-CMs) are a promising cell source for cardiac regenerative medicine and in vitro modeling. However, hPSC-CMs exhibit immature structural and functional properties compared with adult cardiomyocytes. Various electrical, mechanical, and biochemical cues have been applied to enhance hPSC-CM maturation but with limited success. In this work, we investigated the potential application of the semiconducting polymer poly{[N,N'-bis(2-octyldodecyl)-naphthalene-1,4,5,8-bis(dicarboximide)-2,6-diyl]-alt-5,5'-(2,2'-bithiophene)} (P(NDI2OD-T2)) as a light-sensitive material to stimulate hPSC-CMs optically. Our results indicated that P(NDI2OD-T2)-mediated photostimulation caused cell damage at irradiances applied long-term above 36 µW/mm2 and did not regulate cardiac monolayer beating (after maturation) at higher intensities applied in a transient fashion. However, we discovered that the cells grown on P(NDI2OD-T2)-coated substrates showed significantly enhanced expression of cardiomyocyte maturation markers in the absence of a light exposure stimulus. A combination of techniques, such as atomic force microscopy, scanning electron microscopy, and quartz crystal microbalance with dissipation monitoring, which we applied to investigate the interface of the cell with the n-type coating, revealed that P(NDI2OD-T2) impacted the nanostructure, adsorption, and viscoelasticity of the Matrigel coating used as a cell adhesion promoter matrix. This modified cellular microenvironment promoted the expression of cardiomyocyte maturation markers related to contraction, calcium handling, metabolism, and conduction. Overall, our findings demonstrate that conjugated polymers such as P(NDI2OD-T2) can be used as passive coatings to direct stem cell fate through interfacial engineering of cell growth substrates.

Salts as Additives: A Route to Improve Performance and Stability of n-Type Organic Electrochemical Transistors.

Organic electrochemical transistors (OECTs) are becoming increasingly ubiquitous in various applications at the interface with biological systems. However, their widespread use is hampered by the scarcity of electron-conducting (n-type) backbones and the poor performance and stability of the existing n-OECTs. Here, we introduce organic salts as a solution additive to improve the transduction capability, shelf life, and operational stability of n-OECTs. We demonstrate that the salt-cast devices present a 10-fold increase in transconductance and achieve at least one year-long stability, while the pristine devices degrade within four months of storage. The salt-added films show improved backbone planarity and greater charge delocalization, leading to higher electronic charge carrier mobility. These films show a distinctly porous morphology where the interconnectivity is affected by the salt type, responsible for OECT speed. The salt-based films display limited changes in morphology and show lower water uptake upon electrochemical doping, a possible reason for the improved device cycling stability. Our work provides a new and easy route to improve n-type OECT performance and stability, which can be adapted for other electrochemical devices with n-type films operating at the aqueous electrolyte interface.

SpyDirect: A Novel Biofunctionalization Method for High Stability and Longevity of Electronic Biosensors.

Electronic immunosensors are indispensable tools for diagnostics, particularly in scenarios demanding immediate results. Conventionally, these sensors rely on the chemical immobilization of antibodies onto electrodes. However, globular proteins tend to adsorb and unfold on these surfaces. Therefore, self-assembled monolayers (SAMs) of thiolated alkyl molecules are commonly used for indirect gold-antibody coupling. Here, a limitation associated with SAMs is revealed, wherein they curtail the longevity of protein sensors, particularly when integrated into the state-of-the-art transducer of organic bioelectronics-the organic electrochemical transistor. The SpyDirect method is introduced, generating an ultrahigh-density array of oriented nanobody receptors stably linked to the gold electrode without any SAMs. It is accomplished by directly coupling cysteine-terminated and orientation-optimized spyTag peptides, onto which nanobody-spyCatcher fusion proteins are autocatalytically attached, yielding a dense and uniform biorecognition layer. The structure-guided design optimizes the conformation and packing of flexibly tethered nanobodies. This biolayer enhances shelf-life and reduces background noise in various complex media. SpyDirect functionalization is faster and easier than SAM-based methods and does not necessitate organic solvents, rendering the sensors eco-friendly, accessible, and amenable to scalability. SpyDirect represents a broadly applicable biofunctionalization method for enhancing the cost-effectiveness, sustainability, and longevity of electronic biosensors, all without compromising sensitivity.

Yeast extracts from different manufacturers and supplementation of amino acids and micro elements reveal a remarkable impact on alginate production by A. vinelandii ATCC9046.

BACKGROUND: In research and production, reproducibility is a key factor, to meet high quality and safety standards and maintain productivity. For microbial fermentations, complex substrates and media components are often used. The complex media components can vary in composition, depending on the lot and manufacturing process. These variations can have an immense impact on the results of biological cultivations. The aim of this work was to investigate and characterize the influence of the complex media component yeast extract on cultivations of Azotobacter vinelandii under microaerobic conditions. Under these conditions, the organism produces the biopolymer alginate. The focus of the investigation was on the respiration activity, cell growth and alginate production. RESULTS: Yeast extracts from 6 different manufacturers and 2 different lots from one manufacturer were evaluated. Significant differences on respiratory activity, growth and production were observed. Concentration variations of three different yeast extracts showed that the performance of poorly performing yeast extracts can be improved by simply increasing their concentration. On the other hand, the results with well-performing yeast extracts seem to reach a saturation, when their concentration is increased. Cultivations with poorly performing yeast extract were supplemented with grouped amino acids, single amino acids and micro elements. Beneficial results were obtained with the supplementation of copper sulphate, cysteine or a combination of both. Furthermore, a correlation between the accumulated oxygen transfer and the final viscosity (as a key performance indicator), was established. CONCLUSION: The choice of yeast extract is crucial for A. vinelandii cultivations, to maintain reproducibility and comparability between cultivations. The proper use of specific yeast extracts allows the cultivation results to be specifically optimised. In addition, supplements can be applied to modify and improve the properties of the alginate. The results only scratch the surface of the underlying mechanisms, as they are not providing explanations on a molecular level. However, the findings show the potential of optimising media containing yeast extract for alginate production with A. vinelandii, as well as the potential of targeted supplementation of the media.

Type D Adipsia with Severe Hypernatremia: A Unique Presentation of an Extensive Intracranial Germinoma.

Intracranial germ cell tumors are uncommon brain tumors; germinoma is the most common tumor in children and young adults, and the most common regions affected are pineal gland and suprasellar region. Germinomas of the suprasellar region are accompanied by endocrine alterations, with adipsia being a rare presentation. Here, we present the case of a patient with an extensive intracranial germinoma whose initial presentation was adipsia, without any other endocrinological alteration, with development of severe hypernatremia and unusual manifestations derived from it, such as deep vein thrombosis, myopathy with rhabdomyolysis, and neurological axonal damage.

Contact transfer of engineered nanomaterials in the workplace.

This study investigates the potential spread of cadmium selenide quantum dots in laboratory environments through contact of gloves with simulated dry spills on laboratory countertops. Secondary transfer of quantum dots from the contaminated gloves to other substrates was initiated by contact of the gloves with different materials found in the laboratory. Transfer of quantum dots to these substrates was qualitatively evaluated by inspection under ultraviolet illumination. This secondary contact resulted in the delivery of quantum dots to all the evaluated substrates. The amount of quantum dots transferred was quantified by elemental analysis. The residue containing quantum dots picked up by the glove was transferred to at least seven additional sections of the pristine substrate through a series of sequential contacts. These results demonstrate the potential for contact transfer as a pathway for spreading nanomaterials throughout the workplace, and that 7-day-old dried spills are susceptible to the propagation of nanomaterials by contact transfer. As research and commercialization of engineered nanomaterials increase worldwide, it is necessary to establish safe practices to protect workers from the potential for chronic exposure to potentially hazardous materials. Similar experimental procedures to those described herein can be adopted by industries or regulatory agencies to guide the development of their nanomaterial safety programmes.

Respiration in Azotobacter vinelandii and its relationship with the synthesis of biopolymers

Azotobacter vinelandii is a gram-negative soil bacterium that produces two biopolymers of biotechnological interest, alginate and poly(3-hydroxybutyrate), and it has been widely studied because of its capability to fix nitrogen even in the presence of oxygen. This bacterium is characterized by its high respiration rates, which are almost 10-fold higher than those of Escherichia coli and are a disadvantage for fermentation processes. On the other hand, several works have demonstrated that adequate control of the oxygen supply in A. vinelandii cultivations determines the yields and physicochemical characteristics of alginate and poly(3-hydroxybutyrate). Here, we summarize a review of the characteristics of A. vinelandii related to its respiration systems, as well as some of the most important findings on the oxygen consumption rates as a function of the cultivation parameters and biopolymer production.

A qualitative assessment of diabetes care access and disease management in Central America.

BACKGROUND: Diabetes is a growing concern in low-and middle-income countries. Medical missions play a role in increasing access to care and medicines, but often ignore non-communicable disease prevention and advanced management. Increased knowledge of local community needs and resources can lead to the development and implementation of pharmacist-supported interventions to improve diabetes management in rural areas. OBJECTIVES: The purpose of this study was to 1) understand the availability of monitoring for diabetes locally; and 2) describe knowledge and health beliefs regarding diabetes management for those with diabetes, and prevention among those at high risk of developing diabetes. METHODS: This qualitative evaluation used semi-structured interviews with key informants in a community in rural Honduras. Participants included those with diabetes, those at-risk for developing diabetes, and community leaders. Data was analyzed using thematic content analysis through an iterative process of coding and theme development. RESULTS: A total of 35 interviews were conducted with five resulting themes: 1) participants identified multiple barriers to diabetes management including access to monitoring, access to certain medications, and access to advanced levels of care; 2) participants acknowledge the relationship between lifestyle choices and diabetes control, but struggled with adherence to a healthy lifestyle; 3) participants identify that they have limited knowledge of diabetes pathophysiology, diabetes management, and strategies to prevent diabetes; 4) participants felt that opportunities existed within the community to support diabetes education and prevention, and 5) providers should integrate culture, societal norms, and religion in diabetes management. CONCLUSION: This research identifies challenges and resulting opportunities for managing diabetes in rural Honduras. Health care providers including pharmacy personnel should consider strategies to engage communities around self-care and diabetes education. Further, strategies are needed to enhance access to resources and essential medicines for diabetes management. These themes can guide clinicians in supporting communities to enhance diabetes care.

Molecular weight and viscosifying power of alginates produced by mutant strains of Azotobacter vinelandii under microaerophilic conditions.

Alginates are polysaccharides that are of interest in various industrial applications. This is due to the viscosifying properties of alginates, which depends on the weight-average molecular weight. The aim of the present study was to evaluate the changes in alginate quality, in terms of the viscosifying power and weight-average molecular weight of the polymer produced by Azotobacter vinelandii mutant strains in shake flasks under microaerophilic conditions. In cultures developed at oxygen transfer rate (OTR) values close to 5 mmol L-1 h-1, the highest viscosifying power (1.75 L g-1) and weight-average molecular weight (3112 ±â€¯150 kDa) were achieved in cultures performed with the AT9 strain. These values were higher than those obtained for the alginates produced by the parental strain ATCC 9046 grown under similar OTR conditions. In contrast, the alginate produced by the GG9 and OPAlgU + exhibited a very low weight-average molecular weight and therefore a poor viscosifying power. Our results have shown that by the cultivation of AT9 strain under microaerophilic conditions it is possible to obtain a polymer having a high weight-average molecular weight and excellent viscosifying capacity. Therefore, it could be a viable strategy for producing alginates for industrial applications.

Improving glucose and xylose assimilation in Azotobacter vinelandii by adaptive laboratory evolution.

Azotobacter vinelandii is a microorganism with biotechnological potential because its ability to produce alginate and polyhydroxybutyrate. Large-scale biotechnological processes are oriented to sustainable production by using biomass hydrolysates that are mainly composed by glucose and xylose. In the present study, it was observed that A. vinelandii was unable to consume xylose as the sole carbon source and that glucose assimilation in the presence of xylose was negatively affected. Adaptive Laboratory Evolution (ALE) was used as a metabolic engineering tool in A. vinelandii, to improve both carbohydrate assimilation. As a result of ALE process, the CT387 strain was obtained. The evolved strain (CT387) grown in shaken flask cultivations with xylose (8 g L-1) and glucose (2 g L-1), showed an increase of its specific growth rate (µ), as well as of its glucose and xylose uptake rates of 2, 6.45 and 3.57-fold, respectively, as compared with the parental strain. At bioreactor level, the µ, the glucose consumption rate and the relative expression of gluP that codes for the glucose permease in the evolved strain were also higher than in the native strain (1.53, 1.29 and 18-fold, respectively). Therefore, in the present study, we demonstrated the potential of ALE as a metabolic engineering tool for improving glucose and xylose consumption in A. vinelandii.

Metabolic flux analysis and the NAD(P)H/NAD(P)+ ratios in chemostat cultures of Azotobacter vinelandii.

BACKGROUND: Azotobacter vinelandii is a bacterium that produces alginate and polyhydroxybutyrate (P3HB); however, the role of NAD(P)H/NAD(P)+ ratios on the metabolic fluxes through biosynthesis pathways of these biopolymers remains unknown. The aim of this study was to evaluate the NAD(P)H/NAD(P) + ratios and the metabolic fluxes involved in alginate and P3HB biosynthesis, under oxygen-limiting and non-limiting oxygen conditions. RESULTS: The results reveal that changes in the oxygen availability have an important effect on the metabolic fluxes and intracellular NADPH/NADP+ ratio, showing that at the lowest OTR (2.4 mmol L-1 h-1), the flux through the tricarboxylic acid (TCA) cycle decreased 27.6-fold, but the flux through the P3HB biosynthesis increased 6.6-fold in contrast to the cultures without oxygen limitation (OTR = 14.6 mmol L-1 h-1). This was consistent with the increase in the level of transcription of phbB and the P3HB biosynthesis. In addition, under conditions without oxygen limitation, there was an increase in the carbon uptake rate (twofold), as well as in the flux through the pentose phosphate (PP) pathway (4.8-fold), compared to the condition of 2.4 mmol L-1 h-1. At the highest OTR condition, a decrease in the NADPH/NADP+ ratio of threefold was observed, probably as a response to the high respiration rate induced by the respiratory protection of the nitrogenase under diazotrophic conditions, correlating with a high expression of the uncoupled respiratory chain genes (ndhII and cydA) and induction of the expression of the genes encoding the nitrogenase complex (nifH). CONCLUSIONS: We have demonstrated that changes in oxygen availability affect the internal redox state of the cell and carbon metabolic fluxes. This also has a strong impact on the TCA cycle and PP pathway as well as on alginate and P3HB biosynthetic fluxes.

Molecular mass of Poly-3-hydroxybutyrate (P3HB) produced by Azotobacter vinelandii is influenced by the polymer content in the inoculum.

Poly-3-hydroxybutyrate (P3HB) is a biopolymer produced by Azotobacter vinelandii. The physicochemical properties and applications of P3HB are strongly influenced by its weight-average molecular mass (Mw), and in A. vinelandii, it could be influenced by the culture conditions. The aim of this study was to evaluate the effect of the P3HB content of the inoculum on the Mw of the polymer produced by A. vinelandii OP in bioreactor cultures. A. vinelandii cells containing 20, 50 and 70% of P3HB were used as inoculum. The P3HB content in the inoculum affected the volumetric P3HB productivity (qP3HB) and the Mw of P3HB. Those cultures inoculated with cells containing 20% of P3HB, achieved the highest qP3HB (0.17±0.018gP3HBL-1h-1); whereas a P3HB content of 70% was reflected as a low qP3HB (0.021±0.002gP3HBL-1h-1). On the other hand, using an inoculum with 70% of polymer content, the Mw of the biopolymer remained stable at values close to 3200kDa; whereas, when an inoculum with 20% of P3HB was used, the Mw decreased drastically during early stages of cultivation. These results show that manipulating the P3HB content of the inoculum is possible to produce biopolymers with a suitable Mw.

Analysis of respiratory activity and carbon usage of a mutant of Azotobacter vinelandii impaired in poly-ß-hydroxybutyrate synthesis.

In this study, the respiratory activity and carbon usage of the mutant strain of A. vinelandii AT6, impaired in poly-ß-hydroxybutyrate (PHB) production, and their relationship with the synthesis of alginate were evaluated. The alginate yield and the specific oxygen uptake rate were higher (2.5-fold and 62 %, respectively) for the AT6 strain, compared to the control strain (ATCC 9046), both in shake flasks cultures and in bioreactor, under fixed dissolved oxygen tension (1 %). In contrast, the degree of acetylation was similar in both strains. These results, together with the analysis of carbon usage (% C-mol), suggest that in the case of the AT6 strain, the flux of acetyl-CoA (precursor molecule for PHB biosynthesis and alginate acetylation) was diverted to the respiratory chain passing through the tricarboxylic acids cycle, and an important % C-mol was directed through alginate biosynthesis, up to 25.9 % and to a lesser extent, to biomass production (19.7 %).

Modifying Yeast Tolerance to Inhibitory Conditions of Ethanol Production Processes.

Saccharomyces cerevisiae strains having a broad range of substrate utilization, rapid substrate consumption, and conversion to ethanol, as well as good tolerance to inhibitory conditions are ideal for cost-competitive ethanol production from lignocellulose. A major drawback to directly design S. cerevisiae tolerance to inhibitory conditions of lignocellulosic ethanol production processes is the lack of knowledge about basic aspects of its cellular signaling network in response to stress. Here, we highlight the inhibitory conditions found in ethanol production processes, the targeted cellular functions, the key contributions of integrated -omics analysis to reveal cellular stress responses according to these inhibitors, and current status on design-based engineering of tolerant and efficient S. cerevisiae strains for ethanol production from lignocellulose.

The effect of copper on the color of shrimps: redder is not always healthier.

The objective of this research is to test the effects of copper on the color of pacific white shrimp (Litopenaeus vannamei) in vivo. Forty-eight shrimps (L. vannamei) were exposed to a low concentration of copper (1 mg/L; experimental treatment) and forty-eight shrimps were used as controls (no copper added to the water). As a result of this experiment, it was found that shrimps with more copper are significantly redder than those designated as controls (hue (500-700 nm): P=0.0015; red chroma (625-700 nm): P<0.0001). These results indicate that redder color may result from exposure to copper and challenge the commonly held view that highly pigmented shrimps are healthier than pale shrimps.

Riesgo toxicológico ambiental y manipulación del mercurio en el consultorio odontológico de dos unidades comunitarias de salud familiar de San Salvador / Environmental toxicological risk and manipulation of mercury in the dental office of two community family health units in San Salvador

Objetivo: Determinar el riesgo toxicológico derivado de la manipulación de mercurio, por el personal de las clínicas odontológicas de las Unidades Comunitarias de Salud Familiar de Mejicanos y Monserrat de San Salvador. Materiales y Métodos: El diseño corresponde a un estudio descriptivo transversal, utilizando una guía de observación y empleando el Método Colorimétrico en vapor frío; para determinar la presencia de vapores mercuriales, producidos durante el proceso de manipulación de la amalgama de plata como material restaurativo dental. Las observaciones y mediciones fueron efectuadas en diferentes horarios de la jornada laboral, en las Unidades Comunitarias de Salud familiar de Mejicanos y Monserrat; durante los meses de Septiembre y Octubre de 2013. Los resultados obtenidos, fueron ingresados a una base de datos y analizados por SPSS 1.8. Los resultados son presentados en tablas de distribución de frecuencias y contingencia Resultados: Al evaluar el manejo de los excedentes de mercurio, se realizó un total de doce guías de observación por cada Unidad Comunitaria de Salud Familiar. En la Unidad Comunitaria de Salud Familiar de Mejicanos se encontró en tres ocasiones, el recipiente con la tapadera enroscada y humedad en su interior. En cinco ocasiones se observó tapado inadecuadamente y siempre con humedad interior. En cuatro se encontró que utilizaban otro tipo de recipientes para el manejo de los excedentes. Con respecto a la cantidad de mercurio empleada durante cuatro semanas, para el consultorio de Mejicanos fue de 2.8 onzas y de 3.5 onzas en el de Monserrat. De un total de dieciséis mediciones, en ambas se encontraron tres valores por arriba del valor límite de 0.05 mg Hg/m³. Uno fue de 0.12 mg Hg /m³ y dos de 0.10 mg Hg/m³. Estos valores exceden al promedio de exposición al cual una persona puede estar expuesta (0.05 mg Hg/m³), sin que presente efectos adversos repetitivos, durante una jornada laboral de 40 horas semanales. Conclusiones: En tres de las dieciséis mediciones, se sobrepasó el límite de saturación de vapores de mercurio, permitido por la NIOSH (0.05 mg Hg /m³). En cuanto al manejo de los excedentes de mercurio, pudo comprobarse que en los consultorios, no se cumple con la Norma Técnica del Ministerio de Salud.

Objective: To determine the toxicological risks associated with handling mercury, by the staff of the dental clinics of the Community Family Health Units of (Unidades Comunitarias de Salud Familiar) Mejicanos and Monserrat of San Salvador. Materials and methods: The design was a descriptive study, using an observation guide and employing the colorimetric method using cold steam to determine the presence of mercury vapor produced during the handling process of silver amalgam as a dental restorative material. The observations and measurements were made at different times of the workday, in the Community Family Health Units of Mejicanos and Montserrat; during September and October 2013. The results obtained, were entered into a database and analyzed by SPSS 1.8. The results are presented in tables of frequency distribution and contingency. Results: In assessing the management of excess of mercury, a total of twelve observation guides were made for each Community Family Health Units (UCSF). In the UCSF of Mejicanos in three separate occasions the container was found with the lid screwed on and humidity in its interior. In five separate occasions the lid was found fastened inadequately and humidity was always found in the inside. Four were found that they utilized a different type of container for the handling of excess. In respect to the amount of mercury used during the four weeks period by the health clinic of Mejicanos the amount was 2.8 ounces, and 3.5 ounces for the health clinic of Montserrat A total of sixteen measurements were taken in total, in both clinics, three positive values were found (method colorimetric cold steam is limited to 0.05 mg Hg /m3 ) the first value of 0.12 mg Hg /m3 and two values of 0.10 mg Hg /m3 each. These values exceed 0.05 mg Hg /m3 which are the average exposure that a worker may be repeatedly exposed to, without adverse effect, over an 8- hour shift and a 40-hour workweek. Conclusions: In three of the sixteen measurements taken, the limit of saturation of mercury vapors allowed by NIOSH (0.05 mg Hg /m³) was exceeded. Regarding the handling of the excess of mercury, it was found that both clinics are not complying with the Technical Regulations of the Ministry of Health.

The acetylation degree of alginates in Azotobacter vinelandii ATCC9046 is determined by dissolved oxygen and specific growth rate: studies in glucose-limited chemostat cultivations.

Alginates are polysaccharides that may be used as viscosifiers and gel or film-forming agents with a great diversity of applications. The alginates produced by bacteria such as Azotobacter vinelandii are acetylated. The presence of acetyl groups in this type of alginate increases its solubility, viscosity, and swelling capability. The aim of this study was to evaluate, in glucose-limited chemostat cultivations of A. vinelandii ATCC9046, the influence of dissolved oxygen tension (DO) and specific growth rate (µ) on the degree of acetylation of alginates produced by this bacterium. In glucose-limited chemostat cultivations, the degree of alginate acetylation was evaluated under two conditions of DO (1 and 9 %) and for a range of specific growth rates (0.02-0.15 h⁻¹). In addition, the alginate yields and PHB production were evaluated. High DO in the culture resulted in a high degree of alginate acetylation, reaching a maximum acetylation degree of 6.88 % at 9 % DO. In contrast, the increment of µ had a negative effect on the production and acetylation of the polymer. It was found that at high DO (9 %) and low µ, there was a reduction of the respiration rate, and the PHB accumulation was negligible, suggesting that the flux of acetyl-CoA (the acetyl donor) was diverted to alginate acetylation.

Síndrome de Ehlers Danlos, ¿subregistro clínico en ortopedia pediátrica? / Ehlers Danlos syndrome, a clinical subregister in pediatric orthopedics?

Introducción. El comportamiento del síndrome de Ehlers Danlos hizo que se interpretara como un subregistro clínico, poco conocido y con escasas referencias. Esta genodermatosis generalmente está condenada a no tener un tratamiento específico. El objetivo del presente estudio fue, principalmente, describir la morbilidad de este síndrome, desde el punto de vista ortopédico. Métodos. Se realizó un estudio descriptivo prospectivo de 5 años, que incluyó a todos los pacientes con síndrome de Ehlers Danlos, atendidos en la consulta de ortopedia y traumatología entre julio de 2001 y julio del 2006, en el Hospital Pediátrico Docente José Martí (Sancti Spiritus, Cuba). Se consideró un tiempo mínimo de seguimiento de 6 meses para la validación de los resultados. Resultados. Fueron estudiados 41 pacientes afectos de 72 enfermedades de origen ortopédico. La frecuencia estuvo próxima a 1,7 enfermedades por paciente, con predominio no significativo del sexo femenino (n = 24). Uno de los antecedentes perinatales más importantes fue la presencia de grados diversos de displasia de la cadera. La presencia de otras afecciones no ortopédicas no fue significativa. Los principales hallazgos ortopédicos fueron el pie plano flexible (37), el genus recurvatum (11) y la cifoescoliosis (9). La cirugía estética y la cirugía correctora ortopédica fueron las más utilizadas. Conclusiones. La dispensarización de esta enfermedad y su tratamiento oportuno es un método de control eficaz que ayudaría a evitar la degeneración articular, generalmente antesala de la osteoartrosis(AU)

Introduction. The behavior of Ehlers Danlos syndrome caused it to be interpreted as a clinical subregister, little known, and with a few references. This genodermatosis is generally condemned not to have a specific treatment. The objective of this study was mainly to describe the morbidity of this syndrome from the orthopedic point of view. Methods. A 5-year prospective and descriptive study was conducted among all the patients with Ehlers Danlos syndrome that were seen at the Orthopedics and Traumatology department of José Martí Teaching Pediatric Hospital (Sancti Spiritus, Cuba) from July 2001 to July 2006. A minimum follow-up time of 6 months was considered for the validation of the results. Results. 41 patients affected with 72 diseases of orthopedic origin were studied. The frequency was approximately 1.7 diseases per patient, with a non significant predominance of females (n = 24). One of the most important perinatal antecedents was the presence of diverse hip dysplasia degrees. The presence of other non-orthopedic affections was not remarkable. The main orthopedic findings were the flexible flat foot (37), the genus recurvatum (11) and kyphoscoliosis (9). Aesthetic surgery and the corrective orthopedic surgery were the most used. Conclusions. The categorization of this disease and its timely treatment is an efficient method of control that would help to prevent the articular degeneration that generally precedes osteoarthritis(AU)

Síndrome de Ehlers Danlos, ¿subregistro clínico en ortopedia pediátrica? / Ehlers Danlos syndrome, a clinical subregister in pediatric orthopedics?

Introducción. El comportamiento del síndrome de Ehlers Danlos hizo que se interpretara como un subregistro clínico, poco conocido y con escasas referencias. Esta genodermatosis generalmente está condenada a no tener un tratamiento específico. El objetivo del presente estudio fue, principalmente, describir la morbilidad de este síndrome, desde el punto de vista ortopédico. Métodos. Se realizó un estudio descriptivo prospectivo de 5 años, que incluyó a todos los pacientes con síndrome de Ehlers Danlos, atendidos en la consulta de ortopedia y traumatología entre julio de 2001 y julio del 2006, en el Hospital Pediátrico Docente José Martí (Sancti Spiritus, Cuba). Se consideró un tiempo mínimo de seguimiento de 6 meses para la validación de los resultados. Resultados. Fueron estudiados 41 pacientes afectos de 72 enfermedades de origen ortopédico. La frecuencia estuvo próxima a 1,7 enfermedades por paciente, con predominio no significativo del sexo femenino (n = 24). Uno de los antecedentes perinatales más importantes fue la presencia de grados diversos de displasia de la cadera. La presencia de otras afecciones no ortopédicas no fue significativa. Los principales hallazgos ortopédicos fueron el pie plano flexible (37), el genus recurvatum (11) y la cifoescoliosis (9). La cirugía estética y la cirugía correctora ortopédica fueron las más utilizadas. Conclusiones. La dispensarización de esta enfermedad y su tratamiento oportuno es un método de control eficaz que ayudaría a evitar la degeneración articular, generalmente antesala de la osteoartrosis.

Introduction. The behavior of Ehlers Danlos syndrome caused it to be interpreted as a clinical subregister, little known, and with a few references. This genodermatosis is generally condemned not to have a specific treatment. The objective of this study was mainly to describe the morbidity of this syndrome from the orthopedic point of view. Methods. A 5-year prospective and descriptive study was conducted among all the patients with Ehlers Danlos syndrome that were seen at the Orthopedics and Traumatology department of José Martí Teaching Pediatric Hospital (Sancti Spiritus, Cuba) from July 2001 to July 2006. A minimum follow-up time of 6 months was considered for the validation of the results. Results. 41 patients affected with 72 diseases of orthopedic origin were studied. The frequency was approximately 1.7 diseases per patient, with a non significant predominance of females (n = 24). One of the most important perinatal antecedents was the presence of diverse hip dysplasia degrees. The presence of other non-orthopedic affections was not remarkable. The main orthopedic findings were the flexible flat foot (37), the genus recurvatum (11) and kyphoscoliosis (9). Aesthetic surgery and the corrective orthopedic surgery were the most used. Conclusions. The categorization of this disease and its timely treatment is an efficient method of control that would help to prevent the articular degeneration that generally precedes osteoarthritis.