Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España / Epidemiology of ataxia and hereditary spastic paraplegia in Spain: a cross-sectional study

Introducción: Las ataxias (AT) y paraparesias espásticas hereditarias (PEH) son síndromes neu-rodegenerativos raros. Nos proponemos conocer la prevalencia de las AT y PEH en Espa˜na en2019.Pacientes y métodos: Estudio transversal, multicéntrico, descriptivo y retrospectivo de lospacientes con AT y PEH, desde marzo de 2018 a diciembre de 2019 en toda Espa˜na.Resultados: Se obtuvo información de 1933 pacientes procedentes de 11 Comunidades Autóno-mas, de 47 neurólogos o genetistas. Edad media: 53,64 a˜nos ± 20,51 desviación estándar (DE);938 varones (48,5%), 995 mujeres (51,5%). En 920 pacientes (47,6%) no se conoce el defectogenético. Por patologías, 1.371 pacientes (70,9%) diagnosticados de AT, 562 diagnosticados dePEH (29,1%). La prevalencia estimada de AT es 5,48/100.000 habitantes, y la de PEH es 2,24casos/100.000 habitantes. La AT dominante más frecuente es la SCA3. La AT recesiva más fre-cuente es la ataxia de Friedreich (FRDA). La PEH dominante más frecuente es la SPG4, y la PEHrecesiva más frecuente es la SPG7.Conclusiones: La prevalencia estimada de AT y PEH en nuestra serie es de 7,73 casos/100.000habitantes. Estas frecuencias son similares a las del resto del mundo. En el 47,6% no se haconseguido un diagnóstico genético. A pesar de las limitaciones, este estudio puede contribuira estimar los recursos, visibilizar estas enfermedades, detectar las mutaciones más frecuentespara hacer los screenings por comunidades, y favorecer los ensayos clínicos.(AU)

Introduction: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes.We aimed to determine the prevalence of these disorders in Spain in 2019.Patients and methods: We conducted a cross-sectional, multicentre, retrospective, descrip-tive study of patients with ataxia and hereditary spastic paraplegia in Spain between March2018 and December 2019. Results: We gathered data from a total of 1933 patients from 11 autonomous communities,provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51)years; 938 patients were men (48.5%) and 995 were women (51.5%). The genetic defect wasunidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%)had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegiawere estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequenttype of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia wasFriedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in oursample was SPG4, and the most frequent recessive type was SPG7.Conclusions: In our sample, the estimated prevalence of ataxia and hereditary spastic para-plegia was 7.73 cases per 100 000 population. This rate is similar to those reported for othercountries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, ourstudy provides useful data for estimating the necessary healthcare resources for these patients,raising awareness of these diseases, determining the most frequent causal mutations for localscreening programmes, and promoting the development of clinical trials.(AU)

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1933 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 938 patients were men (48.5%) and 995 were women (51.5%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: a cross-sectional study. / Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1.809 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 920 patients were men (50.8%) and 889 were women (49.2%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Characterization of dsRed2-positive cells in the doublecortin-dsRed2 transgenic adult rat retina.

Doublecortin (DCX) is predominantly expressed in neuronal precursor cells and young immature neurons of the developing and adult brain, where it is involved in neuronal differentiation, migration and plasticity. Moreover, its expression pattern reflects neurogenesis, and transgenic DCX promoter-driven reporter models have been previously used to investigate adult neurogenesis. In this study, we characterize dsRed2 reporter protein-expressing cells in the adult retina of the transgenic DCX promoter-dsRed2 rat model, with the aim to identify cells with putative neurogenic activity. Additionally, we confirmed the expression of the dsRed2 protein in DCX-expressing cells in the adult hippocampal dentate gyrus. Adult DCX-dsRed2 rat retinas were analyzed by immunohistochemistry for expression of DCX, NF200, Brn3a, Sox2, NeuN, calbindin, calretinin, PKC-a, Otx2, ChAT, PSA-NCAM and the glial markers GFAP and CRALBP, followed by confocal laser-scanning microscopy. In addition, brain sections of transgenic rats were analyzed for dsRed2 expression and co-localization with DCX, NeuN, GFAP and Sox2 in the cortex and dentate gyrus. Endogenous DCX expression in the adult retina was confined to horizontal cells, and these cells co-expressed the DCX promoter-driven dsRed2 reporter protein. In addition, we encountered dsRed2 expression in various other cell types in the retina: retinal ganglion cells (RGCs), a subpopulation of amacrine cells, a minority of bipolar cells and in perivascular cells. Since also RGCs expressed dsRed2, the DCX-dsRed2 rat model might offer a useful tool to study RGCs in vivo under various conditions. Müller glial cells, which have previously been identified as cells with stem cell features and with neurogenic potential, did express neither endogenous DCX nor the dsRed2 reporter. However, and surprisingly, we identified a perivascular glial cell type expressing the dsRed2 reporter, enmeshed with the glia/stem cell marker GFAP and colocalizing with the neural stem cell marker Sox2. These findings suggest the so far undiscovered existence of perivascular associated cell with neural stem cell-like properties in the adult retina.

Triple therapy with deferiprone, idebenone and riboflavin in Friedreich's ataxia - open-label trial.

OBJECTIVES: The objective of the study was to test the efficacy, safety and tolerability of triple therapy with deferiprone, idebenone and riboflavin in Friedreich's ataxia (FRDA) patients in a clinical pilot study. PATIENTS AND METHODS: Patients included in this study were 10 males and three females, 14-61 years of age (average 30.2 ± 12.1), diagnosed with FRDA with normal ventricular function. Patients were treated with triple therapy with deferiprone at 5-25 mg/kg/day, idebenone at 10-20 mg/kg/day and riboflavin at 10-15 mg/kg/day for 15-45 months. The efficacy of this triple therapy was assessed by change from baseline on the scale for the assessment and rating of ataxia (SARA) and by the change from baseline in echocardiogram parameters. RESULTS: Four patients discontinued due to adverse events (AEs) related with deferiprone. The annual worsening rate (AWR) was estimated in this series as 0.96 (CI 95%: 0.462-1.608) SARA score, whereas AWR for our FRDA cohort was estimated as 2.05 ± 1.23 SARA score. LVMI only decreased by 6.5 g/m(2) (6.2%) at the end of the first year of therapy. LVEF remained stable, except in case of three patients. CONCLUSION: Our results seem to indicate some uncertain benefit on the neurological and heart functions of this triple therapy in FRDA.

Tendons from non-diabetic humans and rats harbor a population of insulin-producing, pancreatic beta cell-like cells.

Diabetes mellitus is a risk factor for various types of tendon disorders. The mechanisms underlying diabetes associated tendinopathies remain unclear, but typically, systemic factors related to high blood glucose levels are thought to be causally involved. We hypothesize that tendon immanent cells might be directly involved in diabetic tendinopathy. We therefore analyzed human and rat tendons by immunohistochemistry, laser capture microdissection, and single cell PCR for pancreatic ß-cell associated markers. Moreover, we examined the short term effects of a single injection of streptozotocin, a toxin for GLUT2 expressing cells, in rats on insulin expression of tendon cells, and on the biomechanical properties of Achilles tendons. Tendon cells, both in the perivascular area and in the dense collagenous tissue express insulin and Glut2 on both protein and mRNA levels. In addition, glucagon and PDX-1 are present in tendon cells. Intraperitoneal injection of streptozotocin caused a loss of insulin and insulin mRNA in rat Achilles tendons after only 5 days, accompanied by a 40% reduction of mechanical strength. In summary, a so far unrecognized, extrapancreatic, insulin-producing cell type, possibly playing a major role in the pathophysiology of diabetic tendinopathy is described. In view of these data, novel strategies in tendon repair may be considered. The potential of the described cells as a tool for treating diabetes needs to be addressed by further studies.

Evolución de pacientes con esclerosis lateral amiotrófica atendidos en una unidad multidisciplinar / Outcome of patients with amyotrophic lateral sclerosis attending in a multidisciplinary care unit

Introducción: La esclerosis lateral amiotrófica (ELA) es una enfermedad con muy mal pronóstico, con una mortalidad del 50% a los 18 meses tras el diagnóstico. Las unidades multidisciplinares pretenden mejorar la calidad de vida y la supervivencia de los enfermos de ELA. El objetivo de nuestro estudio es evaluar cada 3 meses la evolución de pacientes atendidos en la unidad de ELA desde el momento del diagnóstico y durante 24 meses. Material y métodos: Se realizó un estudio observacional prospectivo de pacientes atendidos en la unidad de ELA siguiendo una vía clínica desde el momento del diagnóstico y con revisiones trimestrales desde 2006 a 2010. La edad de inicio, el deterioro de la situación funcional (escala ALSFRS-r), el deterioro de la función respiratoria y la aparición de disfagia y de signos de depresión y/o de deterioro cognitivo fueron evaluados en relación con la localización inicial de los síntomas (bulbar [B], miembros superiores [MMSS], miembros inferiores [MMII]). Resultados: 42 pacientes (30V y 12M) fueron evaluados (edad media de inicio±desviación estándar de 57,97±14,56 años). Se encontró una distribución igual por localización de inicio de los síntomas (B 14 pacientes, MMSS 14, MMII 14). El deterioro funcional (B –26,89 pts.; MMSS –22,48 pts.; MMII –22,66 pts.), la necesidad de uso de BIPAP (B 64,28%; MMSS 35,71%, MMII 50%), la presencia de disfagia (B 85,71; MMSS 42.85; MMII 71.42%), de signos de depresión (B 78,57%, MMSS 35,71%; MMII 64,28%) y de deterioro cognitivo (B 42,85%; MMSS 21,42; MMII 35,71%) fue mayor a los 24 meses de evolución en los pacientes de inicio bulbar. No hubo diferencias en los datos de mortalidad (global 23,80%). Conclusiones: El tratamiento en unidades multidisciplinares no varía la evolución neurológica de la enfermedad pero favorece la aplicación de cuidados multidisciplinares e incrementa la supervivencia de los enfermos de ELA independientemente de su forma de inicio (AU)

Introduction: Amyotrophic lateral sclerosis (ALS) is a disease with very poor prognosis, and a mortality of 50% at 18 months after diagnosis. Multidisciplinary units attempt to improve the quality of life and survival of patients with ALS. The aim of this study is to evaluate every 3 months, over a 24-month period, the outcome of patients treated at the ALS unit since the time of diagnosis. Material and methods: We performed a prospective observational study of patients treated in the ALS unit following a clinical pathway since the time of diagnosis with quarterly reviews from 2006 to 2010. The age of onset, functional impairment (ALSFRS-r), impairment of respiratory function, dysphagia and signs of depression and/or cognitive impairment were evaluated in relation to the initial location symptoms (bulbar [B], upper limbs [UL], lower limbs [LL]). Results: A total of 42 patients (30 males and 12 females) were evaluated (mean age at onset of 57.97years old, SD 14.56). There was an even distribution by location of onset of symptoms (B 14 patients, UL 14, LL 14.) Functional impairment (B –26,89 points, UL –22,48 points, LL –22,66 points), the need for use of BIPAP (B 64.28%; UL 35.71%; LL 50%), the presence of dysphagia (B 85.71; UL 42.85; LL 71.42%), signs of depression (B 78.57%; UL 35.71%; LL 64.28%) and cognitive impairment (B 42.85%; UL 21.42; LL 35.71%) was higher at 24 months of progression in patients with bulbar onset. There was no difference in mortality data (23.80% overall). Conclusions: The treatment in multidisciplinary units does not change the neurological progression of the disease, but increases the survival of ALS patients regardless of their initial onset, emphasising the use of multidisciplinary care (AU)

Outcome of patients with amyotrophic lateral sclerosis attending in a multidisciplinary care unit.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a disease with very poor prognosis, and a mortality of 50% at 18 months after diagnosis. Multidisciplinary units attempt to improve the quality of life and survival of patients with ALS. The aim of this study is to evaluate every 3 months, over a 24-month period, the outcome of patients treated at the ALS unit since the time of diagnosis. MATERIAL AND METHODS: We performed a prospective observational study of patients treated in the ALS unit following a clinical pathway since the time of diagnosis with quarterly reviews from 2006 to 2010. The age of onset, functional impairment (ALSFRS-r), impairment of respiratory function, dysphagia and signs of depression and/or cognitive impairment were evaluated in relation to the initial location symptoms (bulbar [B], upper limbs [UL], lower limbs [LL]). RESULTS: A total of 42 patients (30 males and 12 females) were evaluated (mean age at onset of 57.97 years old, SD 14.56). There was an even distribution by location of onset of symptoms (B 14 patients, UL 14, LL 14.) Functional impairment (B -26,89 points, UL -22,48 points, LL -22,66 points), the need for use of BIPAP (B 64.28%; UL 35.71%; LL 50%), the presence of dysphagia (B 85.71; UL 42.85; LL 71.42%), signs of depression (B 78.57%; UL 35.71%; LL 64.28%) and cognitive impairment (B 42.85%; UL 21.42; LL 35.71%) was higher at 24 months of progression in patients with bulbar onset. There was no difference in mortality data (23.80% overall). CONCLUSIONS: The treatment in multidisciplinary units does not change the neurological progression of the disease, but increases the survival of ALS patients regardless of their initial onset, emphasising the use of multidisciplinary care.

Desarrollo de una vía clínica para la atención a pacientes con esclerosis lateral amiotrófica en un ámbito regional. Red de Atención de ELA-Comunidad de Madrid / Development of a clinical pathway for the attention of patients with amyotrophic lateral sclerosis in a regional network. ALS Assistance Network-Comunidad de Madrid

Introducción. La esclerosis lateral amiotrófica (ELA) precisa una atención multidisciplinaria compleja. Las vías clínicas son planes asistenciales para determinadas enfermedades con curso predecible establecidas en centros aislados, no en regiones multicéntricas. El objetivo es desarrollar una vía clínica capaz de organizar y homogeneizar la atención en la Red de Atención de ELA-Comunidad de Madrid constituida por cinco hospitales, desde el inicio hasta el fin de la enfermedad.Métodos. Neurólogos de estos hospitales y miembros del Servicio Madrileño de Salud en sucesivas reuniones revisaron las guías terapéuticas publicadas y otros documentos utilizados en la atención de la ELA y desarrollaron una vía clínica adaptando la información a la realidad sociosanitaria de la Comunidad de Madrid siguiendo el modelo FOCUSPDCA para el desarrollo de la misma.Resultados. Se crea una vía clínica compuesta por una matriz cientificotécnica que ordena la atención a los pacientes en relación al diagnóstico y tratamiento según el grado de afectación y un cronograma. Se acompaña de unos documentos de información a los pacientes sobre la enfermedad y las pruebas a realizar y evaluación de la atención recibida. Se establecen los estándares a alcanzar en la atención para promover la mejora continua asistencial.Conclusiones. La vía clínica para la atención de la ELA en una red regional organiza la atención y cuidados que deben recibir los pacientes desde el inicio de los síntomas hasta el fin de la enfermedad. Esta ordenación y homogenización mejora la calidad asistencial, disminuye la variabilidad y racionaliza el uso de los recursos sanitarios

Introduction. Amyotrophic lateral sclerosis (ALS) requires complex multidisciplinary attention. Clinical pathways are assistance plans for certain diseases with a predictable course. These plans are established in isolated centers, not in multicenter regions. The aim is to develop a clinical pathway capable of organizing and homogenizing assistance given in ALS Assistance Network-Comunidad de Madrid which is made up of five hospitals, from the beginning until the end of the disease.Methods. In successive meetings, neurologists of these hospitals and members of the Madrid Health Service evaluated published therapeutic guidelines and other documents used in ALS assistance. A clinical pathway was developed adapting this information to social-health care conditions in the Comunidad de Madrid following the FOCUS-PDCA model.Results. A clinical pathway was created consisting of a scientist-technical framework which arranges the attention in relationship to the diagnosis and treatment, according to the degree of disease progression and a chronogram. This is accompanied by several patient information documents on the disease and the tests that are required, and a patient assistance evaluation form. The standards are established to reach and to promote 354 constant improvement in patient care.Conclusions. Clinical pathway for the ALS assistance in a regional network organizes the attention and cares that the patients must receive from the beginning to the end of the disease. This arrangement and homogenization of the attention improves the quality of patient care, diminishes variability and rationalizes the use of the health care resources

[Development of a clinical pathway for the attention of patients with amyotrophic lateral sclerosis in a regional network. ALS Assistance Network-Comunidad de Madrid]. / Desarrollo de una vía clínica para la atención a pacientes con esclerosis lateral amiotrófica en un ámbito regional. Red de Atención de ELA-Comunidad de Madrid.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) requires complex multidisciplinary attention. Clinical pathways are assistance plans for certain diseases with a predictable course. These plans are established in isolated centers, not in multicenter regions. The aim is to develop a clinical pathway capable of organizing and homogenizing assistance given in ALS Assistance Network-Comunidad de Madrid which is made up of five hospitals, from the beginning until the end of the disease. METHODS: In successive meetings, neurologists of these hospitals and members of the Madrid Health Service evaluated published therapeutic guidelines and other documents used in ALS assistance. A clinical pathway was developed adapting this information to social-health care conditions in the Comunidad de Madrid following the FOCUS-PDCA model. RESULTS: A clinical pathway was created consisting of a scientist-technical framework which arranges the attention in relationship to the diagnosis and treatment, according to the degree of disease progression and a chronogram. This is accompanied by several patient information documents on the disease and the tests that are required, and a patient assistance evaluation form. The standards are established to reach and to promote 354 constant improvement in patient care. CONCLUSIONS: Clinical pathway for the ALS assistance in a regional network organizes the attention and cares that the patients must receive from the beginning to the end of the disease. This arrangement and homogenization of the attention improves the quality of patient care, diminishes variability and rationalizes the use of the health care resources.

[Benign intracranial hypertension. History, clinical features and treatment in a series of 41 patients]. / Hipertensión intracraneal benigna. Antecedentes, clínica y tratamiento en una serie de 41 pacientes.

INTRODUCTION: Benign intracranial hypertension (BIH) is a syndrome characterized by the abnormal elevation of the intracranial pressure with a normal composition of the cerebrospinal fluid (CSF) and in absence of ventriculomegaly or some intracranial expansive lesion. AIM: The present work seeks, by means of the analysis of diagnosed BIH patients to evaluate its epidemic, clinical and therapeutic features. PATIENTS AND METHODS: 87 histories from intracranial hypertension diagnosed patients with normal cerebral CT were reviewed, between 1999 and 2002. 41 BIH patients were selected. RESULTS: The reached results allow us to draw the following profile, a woman (> 70%) of between 21 and 30 years (29%), smoker, obese (59%) with an recent increase of weight (37%) that consults after spending more than three months with headache (89%), alterations of the visual acuity (> 50%) and nauseas with some vomiting (> 40%). In the exploration, it presents with bilateral papilledema (100%), a CSF pressure bigger than 20 cmH2O (40,78 15,55 cmH2O) with normal composition, without alterations in the neuroradiological study results. CONCLUSION: The treatment with acetazolamide was favourable (51,2%), being definitive (70%) the lumbar peritoneal shunt when it is specified (30,7%), being improved these figures in those patients with a smaller pressure of the CSF in the moment of the diagnosis (p<0,035).

Mortality due to liver failure and impact on survival of hepatitis virus infections in HIV-infected patients receiving potent antiretroviral therapy.

The aim of the present study was to examine the causes of death, the mortality attributable to liver failure, and the impact of hepatitis virus infections on the survival of a cohort of HIV-infected patients before and after the extensive use of highly active antiretroviral therapy (HAART). Liver disease associated with hepatitis C virus (HCV) seems to be accelerated in patients infected with the human immunodeficiency virus (HIV). On the other hand, the effect of HCV on HIV progression was controversial before the introduction of HAART. However, the last study to report changes in mortality due to liver failure was published in 1997, and the impact of HCV carriage on the survival of HIV-infected patients receiving HAART needs to be clarified. In this investigation, 492 patients who were prescribed antiretroviral drugs between April 1989 and September 2000 were included in the study cohort. The median duration of follow-up of the cohort was 1,392 days. HCV infection was present in 323 (68%). Mortality attributable to AIDS decreased from 4.5 to 1.8 per 100 persons per year. Mortality due to liver failure increased from 0.3 to 0.5 per 100 persons per year ( P<0.01). The survival of patients with and without HCV infection was similar ( P=0.8). Although liver failure is an increasing cause of death among HIV-infected patients receiving HAART, HCV infection has still no impact on the survival of HIV-infected patients.

Mesenteric angioplasty in the treatment of chronic intestinal ischemia.

PURPOSE: This study was undertaken to determine the safety and efficacy of percutaneous transluminal angioplasty (PTA) in the treatment of chronic mesenteric ischemia (CMI) in very high-risk surgical patients. METHODS: Twenty-four focal mesenteric stenoses treated from 1984 to 1994 by PTA in 19 patients with CMI were reviewed. All 19 patients were considered poor surgical candidates. Seventeen patients had classic symptoms of CMI, and two patients had atypical abdominal complaints. Vessels dilated included the superior mesenteric artery (18), celiac artery (3), inferior mesenteric artery (1), aorta-superior mesenteric artery vein graft (1), and aorta-splenic artery vein graft (1). Complete follow-up was possible in all patients, with the exception of one patient who had no symptoms when last seen 17 months after the procedure. RESULTS: PTA was technically successful in 18 of 19 patients (95%) and 23 of 24 stenoses (96%). The lone technical failure resulted in superior mesenteric artery dissection with thrombosis and bowel infarction; the patient died despite emergent laparotomy and revascularization (mortality rate, 5%). Complete symptomatic relief was attained in 15 patients (79%), with follow-up showing continued relief of symptoms for a mean of 39 months (range, 4 to 101 months). Partial symptomatic relief was attained in three patients. Recurrent symptoms developed in three patients (20%) at a mean interval of 28 months (range, 9 to 43 months). Repeat PTA performed in two patients provided good technical results and relief from clinical symptoms. One patient had a symptomatic axillary sheath hematoma that required surgical decompression. CONCLUSIONS: Mesenteric PTA is a valuable treatment option in patients who have CMI and are considered very high operative risks. The initial technical success rate is excellent, with the majority of patients having complete symptomatic improvement and continued relief of symptoms at short-term follow-up.

Use of stents covered with polytetrafluoroethylene in experimental abdominal aortic aneurysm.

PURPOSE: To establish the effectiveness of covered stents in the treatment of aortic aneurysms, to investigate the histopathologic healing patterns of the device, and to determine the long-term endurance and integrity of modified polytetrafluoroethylene (PTFE). MATERIALS AND METHODS: Experimental aneurysms were created in dogs by enlarging the aortic lumen with a patch of abdominal fascia. After 5 months, eight animals underwent an endoluminal bypass. The bypass device consisted of a 6-cm-long stent covered with thin PTFE. After surgery, the animals were killed at 3, 6, and 12 months in groups of three, three, and two, respectively. Specimens were processed for luminal surface studies and cross-sectional histologic study. Explanted PTFE material was analyzed for its physical characteristics and performance and was compared with retained control samples. RESULTS: Before the animals were killed, aortography showed patent bypass conduits in all animals, although two of eight had leaks into the aneurysmal sac. Endothelialized neointima largely covered the luminal surface of the PTFE stent. The percentage of prosthetic surface covered by tissue did not change from 3 months to 1 year. Physical testing of the explanted PTFE material showed no structural deterioration and no change in the internodal distance. Thickness and axial tensile strength varied 12% and 17% from controls, respectively. CONCLUSION: Thin-walled PTFE seems to have physicochemical characteristics that make this material adequate for endovascular use. Though limited, this study supports the establishment of preliminary clinical evaluation of metallic stents combined with PTFE for the treatment of abdominal aortic aneurysm.

Transjugular intrahepatic portosystemic shunt placement for variceal bleeding: predictors of mortality.

PURPOSE: To identify factors that predict survival in patients with variceal bleeding who have undergone transjugular intrahepatic portosystemic shunt (TIPS) placement. PATIENTS AND METHODS: TIPS was performed in 64 of 65 patients. Indications were bleeding esophagogastric varices in 64 patients and hemorrhoidal bleeding in one. Child-Pugh classifications were A in two patients, B in 32, and C in 31. Acute bleeding was controlled before TIPS in 26 patients in stable condition but not in 39 patients whose condition was unstable. RESULTS: Twelve patients died within 30 days of TIPS, and another 14 died thereafter. The cumulative survival rate was 67% at 6 months and 56% at 1 year. Cumulative 30-day survival was 96% for stable and 69% for unstable patients, a significant difference (P = .0135). Thirty-day survival was 91% for patients in Child-Pugh classes A and B combined and 71% for patients in class C (P = .042). CONCLUSION: Efforts to control acute bleeding and improve a patient's metabolic status before TIPS are likely to improve 30-day survival.

Dissection of the external iliac artery in highly trained athletes.

We report a series of four acute external iliac artery dissections occurring in three patients within days of completion of ultraendurance athletic events. Acute dissection of the external iliac artery in highly trained athletes after competition has not been previously documented. A retrospective review of three cases was performed with subsequent follow-up, including imaging and hemodynamic measurements. Dissection was suspected on the basis of duplex imaging results in one case, and arteriography confirmed dissection in all cases. All patients were endurance athletes over the age of 40 years. One patient was found to have bilateral lesions. Treatment in two cases was initiated with percutaneous transluminal angioplasty, one with a successful result and subsequent Plamaz stent placement. In the other case percutaneous transluminal angioplasty was unsuccessful, and operative repair was required with the placement of a graft. The final patient who had bilateral involvement was treated conservatively. At a mean follow-up of 32 months, there have been no complications, and all patients have normal resting hemodynamics. Follow-up duplex imaging shows healing of the dissections in the untreated patient. Histopathologic study in the patient treated with operation disclosed dissection in an otherwise normal arterial wall. Highly trained athletes over the age of 40 are susceptible to external iliac artery dissection, and successful treatment has been achieved by surgical, endovascular, and conservative therapies.