Bone remodeling, around an anatomical hip stem: a one year prospective study using DEXA. / Estudio prospectivo del remodelado óseo femoral alrededor de un vástago anatómico de cadera: un año de seguimiento densitométrico.

INTRODUCTION: The loss of bone mass, as a consequence of bone remodelling, in the proximal third of the femur, is a factor that contributes to the failure of hip prostheses in the medium to long term. This periprosthetic remodelling occurs mainly during the first 12 months after the operation. The aim is to evaluate the behaviour at one year of a new anatomical stem, the ANATO® stem (2015-Stryker®), which is a redesign of its predecessor (ABG-ii®-Stryker stem) by means of bone densitometry. METHOD: Prospective, controlled study in which the changes in bone mineral density (BMD) observed around the seven areas of Gruen in a group of 61 patients affected by primary coxarthrosis, in whom an ANATO® stem was implanted, are analysed densitometrically. The healthy hip was taken as the control group. The existence of differences in the remodelling pattern according to sex, age and body mass index (BMI) was compared. The follow-up was during the first year after the intervention. RESULTS: After one year of follow-up, decreases of bone mineral density in zone seven of -5.9% were observed, being this decrease statistically significant. No differences were found in the remodelling pattern according to age, sex and body mass index. CONCLUSION: The ANATO® stem allows an efficient transmission of loads from the stem to the proximal femur. Only in zone seven significant bone atrophy is observed. Differences in age, BMI and sex do not seem to influence the bone remodelling around this new stem.

Comparison of the open versus the arthroscopic approach in the treatment of femoroacetabular shock. / Comparación del abordaje abierto frente al artroscópico en el tratamiento del choque femoroacetabular.

OBJECTIVE: To compare clinical and imaging results and complications between patients treated for femoroacetabular impingement who underwent either open surgery or an arthroscopic approach. METHODS: This retrospective study included patients who underwent femoroacetabular impingement surgical treatment between June 2009 and January 2018. Patients treated with open surgery were compared with those treated with arthroscopy. Patients were radiographically and clinically assessed by alpha angle, degree of arthritis, Harris Hip Score, hospital stay and complications, as well as progression to total hip arthroplasty. RESULTS: 57 patients with FAI were included; 27 (45.6%) underwent open surgery and 31 (54.4%) underwent arthroscopy. Statistically significant differences were observed in hospital stay, where the patients who underwent arthroscopic surgery showed better outcomes. There were no other statistically significant differences, the results were similar in both groups. CONCLUSIONS: Arthroscopy and open surgery treatments for femoroacetabular impingement provided comparable clinical and radiographic results. However, the latter surgery provides better results in surgery time, hospital stay and postoperative recovery.

Detection of particle motion using image processing with particular emphasis on rolling motion.

Image-processing has been used in granular systems for detecting particle positions and motion near optically accessible surfaces like sediment flow and bedload transport. We review the image-processing techniques used for single and multiple particles. To enhance reliability in particle recognition, tools like Canny edge and Hough transform are intensively used. We show exemplarily how they can be applied to detect not only particle positions but also rotatory motion. The different steps are described in detail and the algorithm is applied to different examples, which are discussed in view of the obtained accuracy.

Influencia de la masa ósea preoperatoria en la remodelación femoral periprotésica tras la implantación de una prótesis ABG-II: resultados tras 10 años de seguimiento / Influence of preoperative bone mass density in periprosthetic bone remodeling after implantation of ABG-II prosthesis: a 10-year follow-up

Introducción. El índice de masa ósea preoperatoria ha demostrado ser un factor importante en la remodelación ósea periprotésica en estudios a corto plazo. Material y métodos. Se utilizó DEXA para realizar un estudio de seguimiento de 10 años a 39 pacientes con una artroplastia de cadera no cementada unilateral. Las mediciones de densidad de masa ósea (DMO) se realizaron a los 6 meses, un año, 3 años, 5 años y 10 años después de la cirugía. El coeficiente de correlación de Pearson se utilizó para cuantificar las correlaciones entre DMO preoperatoria y la densidad mineral ósea periprotésica en las 7 zonas de Gruen a los 6 meses, un año, 3 años, 5 años y 10 años. Resultados. La DMO preoperatoria fue un buen predictor de DMO periprotésica un año después de la cirugía en las zonas 1, 2, 4, 5 y 6 (índice de Pearson 0,61-0,75). Tres años después de la cirugía mantiene un buen poder predictivo en las zonas 1, 4 y 5 (0,71-0,61), aunque en las zonas 3 y 7 se observó baja correlación un año después de la cirugía (0,51 y 0,57 respectivamente). Al final del seguimiento se evidenció baja correlación en las 7 zonas de Gruen. El sexo y el IMC no tuvieron una influencia estadísticamente significativa en la remodelación ósea periprotésica. Conclusión. Aunque la DMO preoperatoria parece ser un factor importante en la remodelación periprotésica un año después de la implantación de una artroplastia, este factor va perdiendo progresivamente poder predictivo; no siendo un factor determinante en la remodelación periprotésica 10 años después de la cirugía (AU)

Introduction. Preoperative bone mass index has shown to be an important factor in peri-prosthetic bone remodelling in short follow-up studies. Material and methods. Bone density scans (DXA) were used to perform a 10-year follow-up study of 39 patients with a unilateral, uncemented hip replacement. Bone mass index measurements were made at 6 months, one year, 3 years, 5 years, and 10 years after surgery. Pearson coefficient was used to quantify correlations between preoperative bone mass density (BMD) and peri-prosthetic BMD in the 7 Gruen zones at 6 months, one year, 3 years, 5 years, and 10 years. Results. Pre-operative BMD was a good predictor of peri-prosthetic BMD one year after surgery in zones 1, 2, 4, 5 and 6 (Pearson index from 0.61 to 0.75). Three years after surgery it has good predictive power in zones 1, 4 and 5 (0.71-0.61), although in zones 3 and 7 low correlation was observed one year after surgery (0.51 and 0.57, respectively). At the end of the follow-up low correlation was observed in the 7 Gruen zones. Sex and BMI were found to not have a statistically significant influence on peri-prosthetic bone remodelling. Conclusion. Although preoperative BMD seems to be an important factor in peri-prosthetic remodelling one year after hip replacement, it loses its predictive power progressively, until not being a major factor in peri-prosthetic remodelling ten years after surgery (AU)

[Influence of preoperative bone mass density in periprosthetic bone remodeling after implantation of ABG-II prosthesis: A 10-year follow-up]. / Influencia de la masa ósea preoperatoria en la remodelación femoral periprotésica tras la implantación de una prótesis ABG-II: resultados tras 10 años de seguimiento.

INTRODUCTION: Preoperative bone mass index has shown to be an important factor in peri-prosthetic bone remodelling in short follow-up studies. MATERIAL AND METHODS: Bone density scans (DXA) were used to perform a 10-year follow-up study of 39 patients with a unilateral, uncemented hip replacement. Bone mass index measurements were made at 6 months, one year, 3 years, 5 years, and 10 years after surgery. Pearson coefficient was used to quantify correlations between preoperative bone mass density (BMD) and peri-prosthetic BMD in the 7 Gruen zones at 6 months, one year, 3 years, 5 years, and 10 years. RESULTS: Pre-operative BMD was a good predictor of peri-prosthetic BMD one year after surgery in zones 1, 2, 4, 5 and 6 (Pearson index from 0.61 to 0.75). Three years after surgery it has good predictive power in zones 1, 4 and 5 (0.71-0.61), although in zones 3 and 7 low correlation was observed one year after surgery (0.51 and 0.57, respectively). At the end of the follow-up low correlation was observed in the 7 Gruen zones. Sex and BMI were found to not have a statistically significant influence on peri-prosthetic bone remodelling. CONCLUSION: Although preoperative BMD seems to be an important factor in peri-prosthetic remodelling one year after hip replacement, it loses its predictive power progressively, until not being a major factor in peri-prosthetic remodelling ten years after surgery.

Improving short term instability for quantitative analyses with portable electronic noses.

One of the main problems when working with electronic noses is the lack of reproducibility or repeatability of the sensor response, so that, if this problem is not properly considered, electronic noses can be useless, especially for quantitative analyses. On the other hand, irreproducibility is increased with portable and low cost electronic noses where laboratory equipment like gas zero generators cannot be used. In this work, we study the reproducibility of two portable electronic noses, the PEN3 (commercial) and CAPINose (a proprietary design) by using synthetic wine samples. We show that in both cases short term instability associated to the sensors' response to the same sample and under the same conditions represents a major problem and we propose an internal normalization technique that, in both cases, reduces the variability of the sensors' response. Finally, we show that the normalization proposed seems to be more effective in the CAPINose case, reducing, for example, the variability associated to the TGS2602 sensor from 12.19% to 2.2%.

Estudio morfométrico de las organelas del hepatocito tras la administración de drogas de abuso / Morphometric analysis of the liver cell after administration of different abuse drugs

Se han descrito, diferentes alteraciones producidas por el consumo de las drogas de abuso a nivel de distintos órganos siendo uno de los más relevantes, por las funciones por el desarrolladas, el hígado. Cuantificar estas alteraciones es el objetivo principal marcado en el trabajo. MATERIAL Y MÉTODOS: se realiza un trabajo experimental en el ratón albino con el fin de valorar desde el punto de vista morfométrico las posibles alteraciones que tres drogas de abuso como son la cocaína, el extasis y la heroína , producen a nivel del hígado desde el punto de vista ultraestructural. Se realiza la intoxicación de los animales y se realiza un estudio del hígado, después del procesamiento de las piezas bajo microscopía electrónica de transmisión y valoración morfométrica de las organelas intracelulares. RESULTADOS: el estudio mostró una disminución de la densidad y área mitocondrial con abalonamiento de estas organelas. Incremento del número de lisosomas y de su tamaño también con engrosamiento de las mismas. Diminución muy significativa del retículo endoplásmico y número de ribosomas. También se muestran disminuidas las rosetas de glúcógeno, pero incrementado el tamaño y número de vacuolas grasas. Alteraciones que se muestran en todos los animales intoxicados con las drogas siendo mas relevantes las del grupo de heroína. DISCUSIÓN. Los resultados muestran una grave alteración de las organelas intracelulares hepáticas tanto en el número como en las estructura de las mismas, siendo indicativo de sufrimiento celular y mal funcionamiento del complejo ultraestructural del hepatocito. Estas alteraciones son más relevantes en el grupo intoxicado con heroína y menos en el de extasis y cocaína. Se puede considerar que aunque a las dosis tratadas a los animales no exista una gran destrucción intracelular, si que hay una alteración a nivel de organelas y sobre todo las relacionadas con el aparato energético de la célula o de producción proteica como mitocondrias o ribosomas, apareciendo o incrementándose las de lisis celular como son los lisosomas (AU)

Have been described, various changes caused by the consumption of drugs of abuse at the level of different organs is one of the most relevant, by the developed functions, the liver. Quantifying these changes is the main goal scored at work. MATERIAL AND METHODS: we performed an experimental study in the albino mouse in order to evaluate from the standpoint of possible alterations morphometric three drugs of abuse such as cocaine, ecstasy and heroin, produced in the liver from the ultrastructural view. Poisoning takes animal and a study is made of the liver, after processing of the parts under transmission electron microscopy, and morphometric evaluation of the intracellular organelles. RESULTS: the study showed a decrease in mitochondrial density and area of these organelles ballooning. Increase in number of lysosomes and their size also thickening them. Significant diminution of the endoplasmic reticulum and number of ribosomes. Also shown glycogen rosettes decreased but increased the size and number of fat vacuoles. Alterations shown in all animalsintoxicated with drugs being the most relevant group of heroin. DISCUSSION. The results show a severe disruption of the intracellular organelles liver both the number and the structure thereof, being indicative of cell suffering and malfunctioning complex ultrastructural hepatocyte. These changes are most relevant in the group intoxicated with heroin and less on that of ecstasy and cocaine. Can be considered that although the dose treated animals there is a large intracellular destruction if there is a disturbance at the level of organelles and especially those related to energy device or cell protein production as mitochondria or ribosome, appearing or increasing the cell lysis such as lysosomes (AU)

A compact and low cost electronic nose for aroma detection.

This article explains the development of a prototype of a portable and a very low-cost electronic nose based on an mbed microcontroller. Mbeds are a series of ARM microcontroller development boards designed for fast, flexible and rapid prototyping. The electronic nose is comprised of an mbed, an LCD display, two small pumps, two electro-valves and a sensor chamber with four TGS Figaro gas sensors. The performance of the electronic nose has been tested by measuring the ethanol content of wine synthetic matrices and special attention has been paid to the reproducibility and repeatability of the measurements taken on different days. Results show that the electronic nose with a neural network classifier is able to discriminate wine samples with 10, 12 and 14% V/V alcohol content with a classification error of less than 1%.

Estudio morfométrico del hepatocito tras la administración de drogas de abuso / Morphometric evaluation of liver cell after adminstration of abuse drugs

Las drogas ocasionan diferentes alteraciones a nivel de los diversos órganos y aparatos del cuerpo humano. Por sus mecanismos de acción, la mayoría de las drogas tienen una especial implicación a nivel del hígado, considerando su papel metabólico a nivel del organismos. En el presente estudio de base experimental, realizado en el ratón se ha evaluado las posibles acciones de tres drogas de abuso como son la cocaína, el éxtasis y la heroína a nivel del hepatocito. Se han valorado aspectos celulares y del núcleo. Los resultados muestran escasa alteraciones constatadas mediante morfometría a nivel del hepatocito y se centran fundamentalmente a nivel de elementos vasculares del espacio porta con alteraciones a nivel de la pared de los vasos y elementos estructurales de esta formación estructural. Los resultados cuantificados no se pueden considerar muy relevantes y los que han presentado alteraciones pueden en parte interpretarse de acuerdo a la relación con la droga suministrada y de acuerdo a otras alteraciones descritas en la bibliografía (AU)

Drugs cause different alterations in the various organs and systems of the human body. For their mechanisms of action, most drugs are particularly liver involvement level, considering its metabolic role at the level of organisms. In the present study experimental basis, conducted in mice was evaluated three possible actions of drugs of abuse such as cocaine, ecstasy and heroin hepatocyte level. We evaluated the nucleus and cellular aspects. The results show little alterations which are established by morphometry hepatocyte level and focus mainly vascular level space holder elements with alterations in the vessel wall and structural elements of the structural formation. The quantified results can not be considered very important and those who submitted alterations may in part be interpreted according to the relationship with the drug tested and according to other conditions described in the literatura (AU)

Repercusiones ponderales y en la morbimortalidad tras la intoxicación de drogas de abuso / Effect of weight, morbidity and mortality after poisoning abuse drugs

La adicción a las drogas es una de las mayores lacras sociales en el momento actual y que desarrollan una importante tasa de morbimortalidad al individuo. Se pretende en el estudio evidenciar las repercusiones de las drogas a nivel de la morbimortalidad, efectos orgánicos y repercusiones ponderales a nivel experimental. Se utilizan en el estudio un total de 48 ratones sometidos de forma subaguda a las drogas con la administración durante 21 días de cocaína, extasis y heroína, comparados con un grupo control. Se valora la tasa de mortalidad, los efectos a nivel corporal y las repercusiones sobre el peso del animal. Se discuten los resultados valorando estas repercusiones de forma comparativa en relación a los diferentes tipos de intoxicación y se intenta relacionar los efectos con respectos de las diferentes actuaciones de cada droga a nivel corporal interpretando cada fenómeno fisiopatológico (AU)

Addiction to drugs is one of the biggest social evils at the moment and have a significant morbidity and mortality rate of the individual. The study is intended to demonstrate the impact of drugs morbidity level, organic effects and repercussions weights experimentally. They are used in the study a total of 48 mice submitted to subacute to drugs for 21 days administration of cocaine, ecstasy and heroin, compared with a control group. We assess the mortality rate, level effects and effects on body weight of the animal. Results are discussed assessing these impacts comparatively in relation to different types of poisoning and attempts to relate the effects respects the different actions of each drug at interpreting body each underlying process (AU)

Studio ultraetructural de los efectos de las drogas de adicción a nivel del hígado. Estudio experimental / Ultraestructural study of the effects on the liver of the abuse drugs. Experimental study

Los consumidores de drogas de adicción suelen desarrollar alteraciones hepáticas con lesiones a nivel histopatológico. El propósito del presente trabajo es evaluar a nivel ultraestructural, la acción de diferentes drogas de abuso en el hígado del ratón. La lesión hepática, es inducida en el estudio por dosis repetitivas durante 21 días de diferentes drogas como la cocaína, el éxtasis y la heroína en cada grupo de estudio. Se ha realizado un estudio ultraestructural, tras el procesado de las muestras hepáticas tratadas por las diferentes drogas administradas a los animales integrados en los diferentes grupos de estudio. En los resultados se muestran a nivel de las diferentes estructuras y organelas intracelulares los cambios inducidos por la administración de las diferentes drogas. Se intenta correlacionar las alteraciones ultraestructurales con las repercusiones desde el punto de vista fisiopatológico y sus consecuencias clínicas en el humano (AU)

Drugs abusers are frequently found to have abnormal liver function tests and hepatic histology. The aim of the present work was to analyse, at the ultrastructural level, the action of drugs abuse in the liver mice. Liver injury was induced by a repetitive dose of cocaine, ecstasy and heroin in male albino Swiss mice during 21 days. There has been an ultrastructural study, after processing of the liver samples treated by different drugs given to animals integrated into different groups. The results are shown different level of intracellular organelle structures and changes induced by the administration of different drugs. It attempts to correlate ultrastructural changes with repercussions from the physiological point of view and its clinical consequences in humans (AU)

Estudio histológico de los efectos de diferentes drogas de adicción a nivel del hígado / Histologic study of the effects on the liver of the abuse drugs. Experimental study

INTRODUCCIÓN. Los consumidores de drogas de abuso presentan graves alteraciones psicológicas, orgánicas y con frecuenciapresentan pruebas anormales de la función hepática y de la histología hepática. Los autores realizan un estudio experimental en ratones, con el objeto de hacer la valorización de los efectos de las drogas de adicción a nivel del hígado y en especial sus repercusiones, en el hepatocito, el núcleo, la vena y arteria del sistema portal, la vena hepática centrolobulillar y también en otras estructuras como los conductos canaliculares biliares. El objetivo del presente trabajo fue analizar, a nivel histopatológico, el efecto de las drogas de abuso en el hígado de ratones. MATERIAL Y MÉTODOS. La lesión hepática fue inducida por una dosis de droga de cocaína, heroína y MDMA durante un periodode exposición de 21 días, en ratones macho albinos. Se realizó tras el tiempo de exposición, la extracción de un segmento de hígado para su procesamiento y posterior evaluación bajo microscopio óptico. RESULTADOS. Las estructuras evaluadas en las preparaciones histológicas, mostraron los efectos de la exposición de las drogas a nivel del hepatocito y los diferentes cambios inducidos a nivel celular y en el parénquima hepático. DISCUSIÓN. La presente investigación pone de relieve que, la posible inducción de lesiones hepáticas que se añadirían a las distintas alteraciones provocadas por las drogas a otros niveles, considerando la alta incidencia de enfermedades agudas y crónicas que afectan de forma asociada a los drogadictos, y que podrían dañar gravemente órganos vitales con la consiguientealteración del estado de salud de los consumidores de drogas de abuso (AU)

INTRODUCTION. Drugs abusers are frequently found to have abnormal liver function tests and hepatic histology. The authors have a experimental study in the mice with the object of make un valorization of effects of addiction drugs on hepatic cells and others structures as arterial, portal vein and intralobulillar hepatic vein and canallicular biliar ducts. The aim of the present work was to analyse, at the histopathological level, the action of drugs abuse in the liver mice. MATERIAL AND METHODS. Liver injury was induced by a daily dose of cocaine, MDMA and heroin in male albino Swiss mice during 21 days. After the exposure time, the animals were sacrificed, and liver samples, processed for examination under optical microscopy.RESULTS. The results evaluated with observations preparations and morphologic methods show the effects of the different factorsrisks and the proportional changes with respect groups of study. DISCUSSION. The present investigation emphasizes that, in addition to the risk of death by overdose, the high incidence ofacute and chronic diseases could seriously undermine the health status of different abuse drug consumers (AU)

Induction of thrombosis of the abdominal aneurysm sac through the application of radiofrequency. An experimental study.

UNLABELLED: This is an experimental study of endovascular aortic surgery for evaluation of the effect of thrombosis induced in the aneurysmal sac using a radiofrequency method. PURPOSE: To determine the efficacy of the induction of thrombosis of the abdominal aneurysm sac as a prevention of endoleaks after endovascular treatment. MATERIALS AND METHODS: Six dogs underwent radiofrequency blood clotting of the aneurysm sac and where afterwards treated with stent-grafting. A comparison with a historical group of 4 dogs with an abdominal aneurysm treated with stent-grafting was used. Radiofrequency was applied to each sac for 1 minute at 127 mA ± 33 (mean ± SD) (tip temperature, 92°C ± 2). IVUS ultrasonography and histology studies were used for the evaluation of the method. RESULTS: The results show the efficacy of the radiofrequency method and its possible future inclusion as a standard clinical application. DISCUSSION AND CONCLUSIONS: Endovascular repair of abdominal aortic aneurysms is dependent on the complete exclusion of the aneurysm from the arterial circulation. Later growth of the aneurysm is originated from retrograde flow into the sac. This study demonstrates how the use of radiofrequency induced thrombosis in a canine model of aneurysm sac. Ultrasound IVUS enabled further control for assessment of blood coagulation after radiofrequency treatment. This approach may be considered a tool for future prevention of endoleaks, in clinical practice.

In vivo genetic engineering of murine pancreatic beta cells mediated by single-stranded adeno-associated viral vectors of serotypes 6, 8 and 9.

AIMS/HYPOTHESIS: The genetic engineering of pancreatic beta cells could be a powerful tool for examining the role of key genes in the cause and treatment of diabetes. Here we performed a comparative study of the ability of single-stranded (ss) adeno-associated viral vectors (AAV) of serotypes 6, 8 and 9 to transduce the pancreas in vivo. METHODS: AAV6, AAV8 and AAV9 vectors encoding marker genes were delivered to the pancreas via intraductal or systemic administration. Transduced cells were analysed by immunostaining. AAV9 vectors encoding hepatocyte growth factor (HGF) were delivered intraductally to a transgenic mouse model of type 1 diabetes and glycaemia was monitored. RESULTS: AAV6, AAV8 and AAV9 mediated efficient and long-term transduction of beta cells, with AAV6 and AAV8 showing the highest efficiency. However, alpha cells were poorly transduced. Acinar cells were transduced by the three serotypes tested and ductal cells only by AAV6. In addition, intraductal delivery resulted in higher AAV-mediated transduction of the pancreas than did systemic administration. As proof of concept, intraductal delivery of AAV9 vectors encoding for the beta cell anti-apoptotic and mitogenic HGF preserved beta cell mass, diminished lymphocytic infiltration of the islets and protected mice from autoimmune diabetes. CONCLUSIONS/INTERPRETATION: Intraductal administration of AAV6, AAV8 and AAV9 is an efficient way to genetically manipulate the pancreas in vivo. This technology may prove useful in the study of islet physiopathology and in assessment of new gene therapy approaches designed to regenerate beta cell mass during diabetes.

Deficiency of CB2 cannabinoid receptor in mice improves insulin sensitivity but increases food intake and obesity with age.

AIMS/HYPOTHESIS: The endocannabinoid system has a key role in energy storage and metabolic disorders. The endocannabinoid receptor 2 (CB2R), which was first detected in immune cells, is present in the main peripheral organs responsible for metabolic control. During obesity, CB2R is involved in the development of adipose tissue inflammation and fatty liver. We examined the long-term effects of CB2R deficiency in glucose metabolism. METHODS: Mice deficient in CB2R (Cb2 ( -/- ) [also known as Cnr2]) were studied at different ages (2-12 months). Two-month-old Cb2 (-/-) and wild-type mice were treated with a selective CB2R antagonist or fed a high-fat diet. RESULTS: The lack of CB2R in Cb2 (-/-) mice led to greater increases in food intake and body weight with age than in Cb2 (+/+) mice. However, 12-month-old obese Cb2 (-/-) mice did not develop insulin resistance and showed enhanced insulin-stimulated glucose uptake in skeletal muscle. In agreement, adipose tissue hypertrophy was not associated with inflammation. Similarly, treatment of wild-type mice with CB2R antagonist resulted in improved insulin sensitivity. Moreover, when 2-month-old Cb2 (-/-) mice were fed a high-fat diet, reduced body weight gain and normal insulin sensitivity were observed. CONCLUSIONS/INTERPRETATION: These results indicate that the lack of CB2R-mediated responses protected mice from both age-related and diet-induced insulin resistance, suggesting that these receptors may be a potential therapeutic target in obesity and insulin resistance.

[Clinical presentation, diagnosis, and long-term outcome of 29 patients with Wilson's disease]. / Presentación clínica, diagnóstico y evolución a largo plazo en 29 pacientes con enfermedad de Wilson.

OBJECTIVE: To analyze the clinical characteristics, treatment, and follow-up of a cohort of 29 patients with Wilson's disease (WD) within the region of Murcia. PATIENTS AND METHOD: We reviewed the medical records of 29 cases of WD (mean age, 20.3 +/- 13.4 years) diagnosed during the last 16 years. RESULTS: The most frequent reason for consultation was upon discovering a high transaminase level in almost half the patients, followed by tremors or dystonia in 17% of patients, respectively. A Kayser-Fleischer ring was observed in 17/29 (58.6%) of patients (100% of patients with pure neurological involvement and 35% of patients with pure clinical hepatic disease; p < 0.001). Blood copper levels not associated with ceruloplasmin as well as cupruria were notably superior in patients with neurological symptoms and in those with liver cirrhosis at the time of diagnosis. Patient clinical symptoms remained stable with D-penicillamine or trientine, or improved during the observation period, for 18 out of 29 patients (62%), while 11 out of 29 patients (38%) got worse. CONCLUSIONS: In our region patients with WD are diagnosed at a younger age, and in most cases for hepatic disease. Patients with neurological disease or liver cirrhosis had a high level of free copper not associated to ceruloplasmin and cupruria. The disease had a favorable evolution in all patients but those diagnosed with hepatic disease or advanced neurological disease.

[Giant hepatic hemangioma associated with Kasabach-Merrit syndrome]. / Hemangioma hepático gigante asociado a síndrome de Kasabach-Merrit.

Hepatic hemangioma is the most frequent liver's tumor. The majority are small, asymptomatic and have an excellent prognosis. Those larger than 5 cm can be associated to a consumptive coagulopathy called Kasabach-Merrit syndrome. We present a patient with a giant hepatic hemangioma with multiple hemangimatosis associated to Kasabach-Merrit syndrome.

IGF-I mediates regeneration of endocrine pancreas by increasing beta cell replication through cell cycle protein modulation in mice.

AIMS/HYPOTHESIS: Recovery from diabetes requires restoration of beta cell mass. Igf1 expression in beta cells of transgenic mice regenerates the endocrine pancreas during type 1 diabetes. However, the IGF-I-mediated mechanism(s) restoring beta cell mass are not fully understood. Here, we examined the contribution of pre-existing beta cell proliferation and transdifferentiation of progenitor cells from bone marrow in IGF-I-induced islet regeneration. METHODS: Streptozotocin (STZ)-treated Igf1-expressing transgenic mice transplanted with green fluorescent protein (GFP)-expressing bone marrow cells were used. Bone marrow cell transdifferentiation and beta cell replication were measured by GFP/insulin and by the antigen identified by monoclonal antibody Ki67/insulin immunostaining of pancreatic sections respectively. Key cell cycle proteins were measured by western blot, quantitative RT-PCR and immunohistochemistry. RESULTS: Despite elevated IGF-I production, recruitment and differentiation of bone marrow cells to beta cells was not increased either in healthy or STZ-treated transgenic mice. In contrast, after STZ treatment, IGF-I overproduction decreased beta cell apoptosis and increased beta cell replication by modulating key cell cycle proteins. Decreased nuclear levels of cyclin-dependent kinase inhibitor 1B (p27) and increased nuclear localisation of cyclin-dependent kinase (CDK)-4 were consistent with increased beta cell proliferation. However, islet expression of cyclin D1 increased only after STZ treatment. In contrast, higher levels of cyclin-dependent kinase inhibitor 1A (p21) were detected in islets from non-STZ-treated transgenic mice. CONCLUSIONS/INTERPRETATION: These findings indicate that IGF-I modulates cell cycle proteins and increases replication of pre-existing beta cells after damage. Therefore, our study suggests that local production of IGF-I may be a safe approach to regenerate endocrine pancreas to reverse diabetes.