RESUMEN
PURPOSE: To assess responsive neurostimulation (RNS) efficacy in pediatric patients with drug-resistant epilepsy, comparing response (≥ 50% reduction in seizure frequency) rates between patients with two or fewer seizure foci and those with multifocal or generalized epilepsy. This study seeks to address the gap in knowledge regarding RNS effectiveness in pediatric populations. METHODS: A systematic review and meta-analysis included data from PubMed, Embase, and Web of Science through November 2023, including 17 retrospective studies and a case series of 24 patients from our practice for a total of 105 aggregated patients. The inclusion criteria of patients were age ≤ 18 and diagnosis of DRE. Exclusion criteria were nonhuman subjects and cases where RNS was not utilized to treat DRE. Study inclusion criteria were detailing the use of RNS and comparing patients with ≤ 2 foci with other focalities. Study exclusion criteria were failure to specify RNS lead placement or type of epilepsy. The risk of bias was assessed using the ROBINS-I tool for all non-randomized studies. Effect sizes and variances were aggregated to provide a comprehensive measure of RNS efficacy, and heterogeneity among the studies was assessed using I2 statistics and Cochran's Q test to evaluate the consistency of the findings. Statistical analyses were conducted using IBM SPSS. We analyzed demographics, epilepsy history, treatment outcomes, and RNS details using descriptive and inferential statistics, including Wilcoxon-Mann-Whitney, Fisher's exact, and chi-squared tests. This systematic review was not registered. RESULTS: Seventeen retrospective studies and a single-institution case series, encompassing 105 pediatric patients, were analyzed. Effect sizes and confidence intervals were calculated to quantify treatment effects. Analyses revealed that RNS reduces seizure frequency across a spectrum of pediatric epilepsy syndromes, irrespective of the seizures' focal, multifocal, or generalized origins. The effectiveness of RNS was not influenced by the patient's sex, age at epilepsy onset, or presence of neurological and psychiatric comorbidities. Prior vagus nerve stimulation surgery and the presence of an epileptic syndrome were factors associated with a lower likelihood of near-complete seizure remission with RNS, underscoring the complexities of treating patients with generalized epilepsies or previous interventional failures. The necessity of further research into individualized surgical strategies for patients was underscored by the mixed results of comparisons of electrode characteristics with responder rates. Limitations of our study include its reliance on retrospective studies, which introduces potential bias and limits the ability to infer causality. DISCUSSION: RNS is a safe and effective treatment in pediatric patients with DRE across demographic, comorbidity, and focality variability. FDA age and focality restrictions, along with patient and physician hesitancy, may be limiting the potential for effective treatment of pediatric DRE with RNS. Prospective randomized trials are recommended to validate these findings.
RESUMEN
Cyclopentadienone-based iron complexes were used for the first time to successfully catalyze the diastereoselective hydrogenation of enantiopure imines. Chiral amines, including valuable biologically active products, were obtained often as enantiomerically pure compounds. Computational studies helped to elucidate the chemical and stereochemical aspects of the iron-catalyzed reaction.
RESUMEN
From 1991 until 2013, 304 patients with intracranial hematomas from aneurysmal rupture were managed surgically in our department, constituting 17 % of all patients with aneurysmal rupture. Of them, 242 patents presented with isolated intracerebral hematomas (in 69 cases associated with significant intraventricular hemorrhage), 50 patients presented with combined intracerebral and subdural hematomas (in 11 cases associated with significant intraventricular hemorrhage), and 12 presented with an isolated subdural hematoma. The surgical procedure consisted of simultaneous clipping of the aneurysm and evacuation of the hematoma in all cases. After surgery, 16 patients (5 %) submitted to an additional decompressive hemicraniectomy, and 66 patients (21 %) submitted to a ventriculo-peritoneal shunt. Clinical outcomes were assessed at discharge and at 6 months, using the modified Rankin Scale (mRS); a favorable outcome (mRS 0-2) was observed in 10 % of the cases at discharge, increasing to 31 % at 6 months; 6-month mortality was 40 %. Applying uni- and multivariate analysis, the following risk factors were associated with a significantly worse outcome: age >60; preoperative Hunt-Hess grades IV-V; pupillary mydriasis (only on univariate); midline shift >10 mm; hematoma volume >30 cc; and the presence of hemocephalus (i.e., packed intraventricular hemorrhage). Based on these results, an aggressive surgical treatment should be adopted for most cases with aneurysmal hematomas, excluding patients with bilateral mydriasis persisting after rescue therapy.
Asunto(s)
Aneurisma Roto/cirugía , Procedimientos Endovasculares , Hematoma Intracraneal Subdural/cirugía , Aneurisma Intracraneal/cirugía , Procedimientos Neuroquirúrgicos , Hemorragia Subaracnoidea/cirugía , Adolescente , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Aneurisma Roto/complicaciones , Aneurisma Roto/diagnóstico por imagen , Angiografía Cerebral , Angiografía por Tomografía Computarizada , Craniectomía Descompresiva , Femenino , Hematoma/diagnóstico por imagen , Hematoma/etiología , Hematoma/cirugía , Hematoma Intracraneal Subdural/diagnóstico por imagen , Hematoma Intracraneal Subdural/etiología , Humanos , Aneurisma Intracraneal/complicaciones , Aneurisma Intracraneal/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Análisis Multivariante , Midriasis/etiología , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Rotura Espontánea , Hemorragia Subaracnoidea/diagnóstico por imagen , Hemorragia Subaracnoidea/etiología , Instrumentos Quirúrgicos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Derivación Ventriculoperitoneal , Adulto JovenRESUMEN
The impairment of the right ventricle (RV) in systemic sclerosis (SSc) is usually related to pulmonary arterial hypertension (PAH). New echocardiographic techniques, such as 3-dimensional echocardiography (3DE) and 2-dimensional speckle tracking (2DSTE), allow an accurate evaluation of the RV function. The aim of this study was to evaluate the RV function using 3DE and 2DSTE in SSc patients with no history of heart disease and no PAH. Forty-five SSc patients, 42 females and 3 males, 28 with limited cutaneous SSc (lcSSc) and 17 with diffuse cutaneous SSc (dcSSc), were studied. Forty-three age- and gender-matched healthy subjects were enrolled as controls. All of them underwent a 3DE and 2DSTE ecocardiographic evaluation of the RV function. Systolic pulmonary arterial pressure (sPAP) and total pulmonary vascular resistance (tPVR) were also estimated by power doppler. RV echocardiographic parameters were compared in the different subsets of SSc patients. A statistical analysis was performed by t-test, ANOVA and multiple logistic regression. RV areas in 2DSTE and volumes in 3DE were higher and RV function parameters were reduced in SSc patients compared with controls. Also sPAP and tVPR were higher, but they did not reach pathological values. Echocardiographic alterations were more pronounced in patients with lcSSc. 3DE and 2DSTE echocardiography allowed us to detect morphological and functional alterations of the RV in a group of SSc patients with no clinical signs of heart disease and no PAH. These patients had significantly higher sPAP and tPVR than healthy controls without reporting values compatible with PAH. These data suggest that RV alterations are related to a pressure overload rather than to an intrinsic myocardial involvement in SSc.
Asunto(s)
Ecocardiografía Tridimensional , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/fisiopatología , Disfunción Ventricular Derecha/diagnóstico , Disfunción Ventricular Derecha/fisiopatología , Adulto , Anciano , Estudios de Casos y Controles , Ecocardiografía/métodos , Ecocardiografía Tridimensional/métodos , Femenino , Humanos , Italia/epidemiología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Reproducibilidad de los Resultados , Esclerodermia Sistémica/epidemiología , Sensibilidad y Especificidad , Disfunción Ventricular Derecha/epidemiologíaRESUMEN
A group of 126 surgical patients with 143 unruptured MCA aneurysms was evaluated in order to determine the risks of treatment and possible adjuncts for safer surgery. The precise location and size of the aneurysms were determined in each case; 21 aneurysms were located on the M1 tract, 109 on the main division - which consisted of more than two branches in 10 cases and was proximally located in 12 cases - and 13 were distal; 36 aneurysms were small (<7 mm), 90 standard, and 17 large or giant (>15 mm); 45 patients harbored multiple aneurysms (12 on the ipsilateral MCA). The aneurysms were excluded by clip in most cases, with the assistance of intraoperative flowmetry in 78 patients. Temporary proximal vessel occlusion was used in 57 patients (>10 min in 8 cases), without significant effects on radiological or clinical outcome. After surgery, newly occurring minor deficits were observed in 5 patients and significant deficits in 4; one patient died from an intractable coagulopathy. The low rate (3.9 %) of unfavorable results (modified Rankin Score > 2) linked to surgery justifies serious consideration for treatment in these patients, especially when facing the high disability rate after the rupture of aneurysms in this anatomical location.
RESUMEN
A group of 123 patients with large or critically located arterio-venous malformations (AVMs), operated on between 1990 and 2011 and who underwent preoperative embolization, was assessed in order to clarify the risks of this combined treatment. AVM location, volume, and Spetzler's grade were assessed in each case; AVM volume was over 20 cm(3) in 49 % of the cases; Spetzler's grade was 3 or above in 76 % of the cases (with 34 cases in grades 4-5). A mean of 2.3 embolization procedures per patient were carried out, using bucrylate and, more recently, Onyx and/or Glubran; a 4.5 % procedure-related complication rate was observed. Complications occurring after surgery were classified as hemorrhagic (16 cases, 8 requiring surgical evacuation) or ischemic (4 cases); hemorrhagic complications were more common for AVMs with volumes >20 cm(3) and/or deep feeders. Surgery-related unfavorable results (modified Rankin Score > 2) were observed in 6 % of patients in Spetzler's grade 3, and in 20-25 % of patients in grades 4-5. More recently, a triple treatment (radiosurgery + embolization + surgery) allowed for obtaining favorable results (mRS 0-2) in all patients. It has been concluded that a combined treatment with embolization and surgery constitutes a reasonable choice for complex cerebral AVMs; the association of radiosurgery may improve the patients outcome.
RESUMEN
OBJECTIVES: Hypovitaminosis D is very common in the elderly in Italy and generally in the world, contributing to bone fractures and muscle weakness. The aim of the study was to evaluate bone metabolism in an old population of patients hospitalized not for musculo-skeletal complaints. METHODS: The clinical records of 175 patients, 98 female and 77 male, aged >65 years, hospitalized in a Department of Internal Medicine (Sacile, Western Friuli) were retrospectively reviewed. Serum levels of calcium, phosphorous, alkaline phosphatase, parathyroid hormone (PTH) and 25-OH vitamin D were evaluated. Correlations between these parameters were investigate. RESULTS: Abnormalities of bone metabolism parameters were frequently founded, particularly hypocalcemia, increased PTH and reduced 25-OH vitamin D. Hypovitaminosis D were detected in 88% of patients, low levels in 30.28% and very low levels in 57.72%. Hypovitaminosis D was related to female sex, old age of patients and high levels of PTH. CONCLUSIONS: Our data confirm that hypovitaminosis D is very common in elderly population. The study has been performed in an Italian Region where the supplementation of vitamin D in the elderly is not performed, suggesting that a awareness campaign of the doctors could be very useful to prevent bone metabolism abnormalities.
Asunto(s)
Deficiencia de Vitamina D , Vitamina D , Fosfatasa Alcalina , Calcio , Humanos , Hormona Paratiroidea , Deficiencia de Vitamina D/epidemiologíaRESUMEN
OBJECTIVES: A previous single-country pilot study indicated serum anti-GM2 and anti-GA1 anti-glycolipid antibodies as potential biomarkers for acute canine polyradiculoneuritis. This study aims to validate these findings in a large geographically heterogenous cohort. MATERIALS AND METHODS: Sera from 175 dogs clinically diagnosed with acute canine polyradiculoneuritis, 112 dogs with other peripheral nerve, cranial nerve or neuromuscular disorders and 226 neurologically normal dogs were screened for anti-glycolipid antibodies against 11 common glycolipid targets to determine the immunoglobulin G anti-glycolipid antibodies with the highest combined sensitivity and specificity for acute canine polyradiculoneuritis. RESULTS: Anti-GM2 anti-glycolipid antibodies reached the highest combined sensitivity and specificity (sensitivity: 65.1%, 95% confidence interval 57.6 to 72.2%; specificity: 90.2%, 95% confidence interval 83.1 to 95.0%), followed by anti-GalNAc-GD1a anti-glycolipid antibodies (sensitivity: 61.7%, 95% confidence interval 54.1 to 68.9%; specificity: 89.3%, 95% confidence interval 82.0 to 94.3%) and these anti-glycolipid antibodies were frequently present concomitantly. Anti-GA1 anti-glycolipid antibodies were detected in both acute canine polyradiculoneuritis and control animals. Both for anti-GM2 and anti-GalNAc-GD1a anti-glycolipid antibodies, sex was found a significantly associated factor with a female to male odds ratio of 2.55 (1.27 to 5.31) and 3.00 (1.22 to 7.89), respectively. Anti-GalNAc-GD1a anti-glycolipid antibodies were more commonly observed in dogs unable to walk (OR 4.56, 1.56 to 14.87). CLINICAL SIGNIFICANCE: Anti-GM2 and anti-GalNAc-GD1a immunoglobulin G anti-glycolipid antibodies represent serum biomarkers for acute canine polyradiculoneuritis.
Asunto(s)
Enfermedades de los Perros , Polirradiculoneuropatía , Animales , Biomarcadores , Enfermedades de los Perros/diagnóstico , Perros , Femenino , Gangliósido G(M2) , Humanos , Inmunoglobulina G , Masculino , Proyectos Piloto , Polirradiculoneuropatía/diagnóstico , Polirradiculoneuropatía/veterinariaRESUMEN
OBJECTIVES: To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc). METHODS: The EUSTAR database was first searched. A case-control study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression. RESULTS: Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction. CONCLUSION: The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective.
Asunto(s)
Esclerodermia Sistémica/complicaciones , Disfunción Ventricular Izquierda/etiología , Adulto , Factores de Edad , Anciano , Bloqueadores de los Canales de Calcio/uso terapéutico , Métodos Epidemiológicos , Europa (Continente)/epidemiología , Femenino , Dedos , Humanos , Masculino , Persona de Mediana Edad , Miositis/complicaciones , Miositis/epidemiología , Esclerodermia Sistémica/epidemiología , Factores Sexuales , Úlcera Cutánea/complicaciones , Úlcera Cutánea/epidemiología , Volumen Sistólico , Disfunción Ventricular Izquierda/epidemiología , Disfunción Ventricular Izquierda/prevención & controlRESUMEN
OBJECTIVES: Antibodies binding to the surface of fibroblasts (anti-fibroblast antibodies: AFA) have been described in systemic sclerosis (SSc). We aimed to assess the effect of AFA on extracellular matrix (ECM) turnover and whether AFA were associated with anti-topoisomerase-I antibody. METHODS: IgG were purified from AFA-positive and AFA-negative sera selected within 20 SSc and 20 healthy individuals, and tested on normal dermal fibroblasts, at protein and mRNA level, for their capacity to induce collagen deposition or degradation. RESULTS: Fibroblasts stimulated with AFA-positive but not with AFA-negative and control IgG showed an increased capacity to digest collagen matrix and produce metalloproteinase-1 (MMP-1) while their production of total collagen, type I collagen and tissue inhibitor of metalloproteinase-1 (TIMP-1) was unaffected. The steady-state mRNA levels of MMP-1, COL1A1 and TIMP-1 paralleled the protein levels. AFA-positive IgG did not induce Smad 2/3 phosphorylation, indicating that this transforming growth factor-beta signalling pathway was not involved. IL-1 and tumour necrosis factor (TNF) neutralization did not reverse the enhanced production of MMP-1, suggesting a direct effect of AFA on fibroblasts. Finally, anti-topoisomerase-I antibodies were present in 11 of 12 AFA-negative IgG, and an anti-topoisomerase-I monoclonal antibody failed to enhance MMP-1 production, thus indicating a lack of correlation between AFA and anti-topoisomerase-I antibody. CONCLUSIONS: These results indicate that SSc antibodies binding to fibroblasts enhance matrix degradation and MMP production events that may favour inflammation but do not directly impact on fibrosis development.
Asunto(s)
Anticuerpos Antiidiotipos/análisis , Colágeno/metabolismo , Metaloproteinasa 1 de la Matriz/metabolismo , Esclerodermia Sistémica/inmunología , Inhibidor Tisular de Metaloproteinasa-1/metabolismo , Adulto , Anticuerpos Antiidiotipos/efectos de los fármacos , Western Blotting , Estudios de Casos y Controles , Células Cultivadas , Ensayo de Inmunoadsorción Enzimática , Femenino , Fibroblastos/efectos de los fármacos , Fibroblastos/inmunología , Técnica del Anticuerpo Fluorescente Indirecta , Humanos , Inmunoglobulina G/farmacología , Interleucina-1/farmacología , Masculino , Metaloproteinasa 1 de la Matriz/efectos de los fármacos , Persona de Mediana Edad , ARN Mensajero/análisis , Esclerodermia Sistémica/fisiopatología , Piel/patología , Inhibidor Tisular de Metaloproteinasa-1/efectos de los fármacosRESUMEN
INTRODUCTION: In children with an unilateral renal tumor, nephron-sparing surgery (NSS) results in a more adequate renal function adaptation compared to nephrectomy. In the present study, we investigated whether nephron-sparing surgery is followed by a different renal structure adaptation compared to nephrectomy. METHODS: Sixteen patients with unilateral renal tumor treated by nephrectomy (Group 1) and 10 treated by nephron-sparing surgery (Group 2) were enrolled in the study. Kidney volume was estimated by ultrasonography, using the formula for a prolate ellipsoid. Kidney volume was adjusted to the patient's weight and kidney laterality and expressed as a percentage of the expected volume of two kidneys in a healthy child. Total kidney volume (TKV) corresponded to the volume of the contralateral kidney in Group 1 patients, and to the volume of contralateral kidney + kidney remnant in Group 2 patients. Renal function was evaluated by serum creatinine values adjusted for sex and age and expressed as standard deviation scores (SDS). RESULTS: Group 2 patients presented with a greater indexed TKV compared to Group 1 patients, (97.4 +/- 18.8 % vs. 77.0 +/- 17.7 %; p = 0.005). Indexed TKV below the reference range for healthy controls with two kidneys was found in 4 of 10 Group 2 patients vs. 14 of 16 Group 1 patients (p = 0.017). In both patient groups, correlation analysis of indexed TKV and creatinine SDS showed a negative correlation (r = - 0.47; p = 0.01). CONCLUSION: In children with unilateral renal tumor, NSS is followed by a more adequate compensatory restoration of TKV compared with nephrectomy.
Asunto(s)
Adaptación Fisiológica/fisiología , Neoplasias Renales/cirugía , Riñón/crecimiento & desarrollo , Nefrectomía/métodos , Recuperación de la Función/fisiología , Niño , Estudios Transversales , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Neoplasias Renales/patología , Masculino , Tamaño de los Órganos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Eating Disorders are very widespread within the adolescent population. A possible interpretation and the comprehension of such forms of psychopathology may revolve around the failure to develop a well-defined personal identity, an incapacity to achieve a sense of differentiation with respect to others, an incapacity to measure oneself against others, dependence on others, the fear of rejection and a sense of inadequacy. This study explores the relational styles and behaviour of individuals suffering from eating disorders and their influence on the development of the personality, with reference being made in particular to self-valuation, dependence on others and levels of differentiation. A sample population of 90 women with eating disorders was studied. The subjects were subdivided into 3 groups (30 with restricting anorexia nervosa, 30 with binge-eating/purging anorexia nervosa and 30 with bulimia nervosa), overlapping in terms of age, duration of disorders and interrelation style, using the Relational Competence Test. The most significant results of this study concern the question of the definition of an autonomous personal identity. This process seems to be in progress in young women suffering from bulimia nervosa who appear to be driven towards a "definition of the self in opposition" with the consequent tendency towards relational experiences outside their own family. In women with binge-eating/purging AN moreover an awareness of the difference between the self and others and of their state of dependence would appear to be present, however behaviour aimed at the determination of an autonomous self is not evident. In women with restricting anorexia nervosa a definition of the identity is totally absent; these women develop an omnipotent self in their 'oneness' with others. These relational aspects lead to the identification of a continuum between restricting anorexia nervosa, binge-eating/purging anorexia nervosa and bulimia nervosa in an evolutionary perspective regarding the self-with-others.
Asunto(s)
Anorexia Nerviosa/psicología , Bulimia Nerviosa/psicología , Identificación Psicológica , Autoimagen , Adolescente , Codependencia Psicológica , Relaciones Familiares , Femenino , Humanos , Relaciones Interpersonales , Modelos Lineales , Pruebas Psicológicas , Identificación SocialRESUMEN
Digital ulcers (DU) at the hands are one of the more frequent and severe complications in systemic sclerosis. Data on their prevalence and distribution in the different subsets of disease are variable in the literature. We studied the frequency of DU in a cohort of 333 scleroderma patients followed in the last 10 years in our Unit. DU have been recorded in 133 patients (39,9%), more frequently in males, in patients with cutaneous diffuse form of disease and in patients with anti-Scl70 ANA specificity. Complications of DU have been observed in 12,3% of cases. Surgery of the hands has been required in 8,7% of patients. The more effective treatment of DU are i.v. prostanoids, performed usually in day hospital, with high costs for the National Health Service. Recently the efficacy of bosentan, an oral receptor antagonist of endothelin, has been demonstrated, thus opening new perspectives in the treatment of DU in systemic sclerosis.
Asunto(s)
Dedos/patología , Esclerodermia Difusa/complicaciones , Úlcera Cutánea/etiología , Adulto , Anciano , Autoanticuerpos/inmunología , Bosentán , Centrómero/inmunología , Estudios de Cohortes , ADN-Topoisomerasas de Tipo I , Antagonistas de los Receptores de Endotelina , Femenino , Humanos , Masculino , Persona de Mediana Edad , Proteínas Nucleares/inmunología , Prevalencia , Prostaglandinas/uso terapéutico , Esclerodermia Difusa/inmunología , Úlcera Cutánea/tratamiento farmacológico , Úlcera Cutánea/epidemiología , Úlcera Cutánea/cirugía , Sulfonamidas/uso terapéuticoRESUMEN
OBJECTIVE: Pulmonary arterial hypertension (PAH) is a rare but severe complication of connective tissue diseases (CTD), with a negative impact on patients survival. Bosentan, a receptor antagonist of endothelin, has been proved effective for the treatment of PAH. The aim of this study was to evaluate the effects and the safety of bosentan administered for 2 years in a group of patients with PAH related to CTD. METHODS: Twelve patients with PAH related to systemic sclerosis (8 cases), SLE (2 cases), mixed connective tissue disease (1 case) and polymyositis (1 case) attending the Rheumatology Unit of Padova University were treated with bosentan for two years. Distance walked in 6 minutes, right ventricular systolic pressure and mean pulmonary artery pressure estimated by doppler echocardiography were evaluated at baseline and after 6, 12, 18 and 24 months of treatment. Safety was assessed by laboratory tests performed every two months. RESULTS: During bosentan treatment, a significant decrease of right ventricular systolic pressure was observed after 6, 12, 18 and 24 months in comparison to baseline, whereas pulmonary artery mean pressure remained unchanged. Distance walked in 6 minutes slightly increased after 6 and 12 months, but significantly decreased after 18 and 24 months, mostly because complications of CTD which compromised the ability to walk arose in 4 patients. Adverse events related to bosentan were observed in 2 cases. CONCLUSIONS: Bosentan has been demonstrated effective in reducing pulmonary arterial pressure in a two-year period of treatment. Exercise capacity improved only in the first year of therapy and worsened thereafter, suggesting the opportunity of a combination therapy for a long-term treatment of PAH related to CTD.
Asunto(s)
Antihipertensivos/uso terapéutico , Enfermedades del Tejido Conjuntivo/complicaciones , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Sulfonamidas/uso terapéutico , Adulto , Anciano , Bosentán , Enfermedades del Tejido Conjuntivo/fisiopatología , Ecocardiografía Doppler , Tolerancia al Ejercicio , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/fisiopatología , Masculino , Persona de Mediana Edad , Polimiositis/complicaciones , Esclerodermia Sistémica/complicaciones , Resultado del Tratamiento , Función Ventricular Derecha/efectos de los fármacosRESUMEN
Many infants with a repaired esophageal atresia (EA) undergo fundoplication, aortopexy, or glossopexy because the mechanisms most responsible for airway obstruction and/or apparent life-threatening event (AO/ALTE) syndrome are considered to be gastroesophageal reflux (GER), tracheal compression (TC), or obstructive apnea, respectively. In the present study, we investigated whether these mechanisms are independent or interrelated. We developed a database of 120 consecutive patients with EA treated by the senior author between 1967-2002. We studied the clinical manifestations of patients with a cervical esophagostomy and/or blind lower esophageal stump, which ruled out TC and/or proximal esophageal GER as a mechanism for AO/ALTE. Of 25 neonates who underwent section/ligation of lower tracheo-esophageal fistula and/or feeding gastrostomy, 10 critically ill neonates died. Of 15 survivors, 9 infants had a feeding gastrostomy without an esophagostomy. Of these, 6 infants presented one or more episodes of AO, and 8 presented ALTE with or without AO. Subsequently, 5 of the 9 infants underwent an esophagostomy. Eventually, 11 infants had a feeding gastrostomy with an esophagostomy. Of the latter, 5 infants presented one or more episodes of AO, and 6 presented ALTE without AO. In conclusion, oral feeding, proximal esophageal GER, and TC are not essential for AO/ALTE syndrome to occur. They are probably factors which offer evidence of an underlying problem with control of upper airway patency.
Asunto(s)
Atresia Esofágica/etiología , Atresia Esofágica/cirugía , Bradicardia/epidemiología , Comorbilidad , Atresia Esofágica/fisiopatología , Esofagostomía , Femenino , Humanos , Hiperhidrosis/epidemiología , Recién Nacido , Polihidramnios/epidemiología , Embarazo , Infecciones del Sistema Respiratorio/epidemiología , Enfermedades de la Tráquea/epidemiologíaRESUMEN
PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Neuroblastoma/cirugía , Neoplasias de las Glándulas Suprarrenales/mortalidad , Neoplasias de las Glándulas Suprarrenales/patología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Neoplasias del Mediastino/mortalidad , Neoplasias del Mediastino/patología , Neoplasias del Mediastino/cirugía , Estadificación de Neoplasias , Neuroblastoma/mortalidad , Neuroblastoma/patología , Evaluación de Resultado en la Atención de Salud , Estudios RetrospectivosRESUMEN
Arthritis and tenosynovitis are frequently reported as complications of inflammatory bowel diseases. About 10% of patients with ulcerative colitis presents articular inflammation, usually in the phases of activity of intestinal disease. Tenosynovitis is also a frequent complication of ulcerative colitis. We describe here a case of tenosynovitis of both ankles occurring in a patient affected by ulcerative colitis not in active phase. Chest X-ray and TC showed hilar lymph node enlargement and transbronchial biopsy confirmed the diagnosis of sarcoidosis. In this disease tenosynovitis is very rare, unlike arthritis that is rather common. In conclusion we observed a case of ankle bilateral tenosynovitis as onset manifestation of sarcoidosis.
Asunto(s)
Tobillo , Colitis Ulcerosa/complicaciones , Sarcoidosis/complicaciones , Tenosinovitis/etiología , Adulto , Humanos , MasculinoRESUMEN
OBJECTIVE: To characterize serum autoantibody profiles of patients with idiopathic inflammatory myopathies (IIM) by searching for myositis-specific (MSA) and myositis-associated (MAA) antibodies with sensitive and specific laboratory tests. METHODS: We tested the sera from 46 Caucasian patients diagnosed as affected with IIM at the Division of Rheumatology of Padova University (21 polimyositis, PM; 22 dermatomyositis, DM; 3 myositis overlap syndrome). All patients had definite IIM according to the criteria of Bohan-Peter. MSA including anti-tRNA synthetase (anti-Jo-1 and others) and anti-Mi-2 were determined by RNA immunoprecipitation and a modified immunoblot test, respectively. MAA (-U1RNP, -U2RNP, RoRNP, PM/Scl, Ku) were detected by counterimmunoelectrophoresis and immunoblot. RESULTS: Serum MSA and/or MAA were found in 30/46 (65%) patients with IIM. Twenty-three patients (50%) were positive for at least one MSA: anti-Jo-1 in 15 (33%), anti-Mi-2 in 6 (13%), and other anti-tRNA synthetase in 3 (6%). One patient was anti-Jo-1/Mi-2 positive. Moreover, 18 patients (39%) were positive for at least one MAA: anti-Ro/SSA in 13 (28%), anti-U1RNP in 4 (9%), anti-PM/Scl in 1 (2%) and anti-Ku in 1 (2%). Coexisting MSA and MAA were observed in 8 patients (17%), anti-Jo-1/SSA positive in most cases. Anti-Jo-1 was predominantly associated with PM (57% in PM vs 14% in DM), whereas anti-Mi-2 was exclusively found in DM patients (27%). Anti-synthetase antibodies were closely associated with interstitial lung disease and polyarthritis; anti-Mi-2 positive DM patients did not have lung involvement. Notably, anti-Ro/SSA antibody was frequently observed and almost equally detected in either PM or DM (about 30%): in more than 50% of cases the antibody was associated with one MSA. CONCLUSIONS: By means of analytically reliable methods, MSA was detected in 50% of our IIM patients. Searching for MSA in patients with IIM is recommended because of its diagnostic and prognostic value.
Asunto(s)
Autoanticuerpos/sangre , Miositis/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miositis/sangre , Pruebas SerológicasRESUMEN
The clinical and physiologic features of 28 infants with Pierre Robin syndrome and those of 20 infants with various types of nasal obstruction were reviewed to determine whether different causes of upper airway obstructure may lead to a common syndrome. The patients had no significant differences in distribution of main clinical manifestations. Their features included cyanosis with respiratory distress, apneic spells, oropharyngeal dysphagia, vomiting, failure to thrive, cor pulmonale, brain damage, and sudden death during sleep. The common physiologic manifestation appeared to be an oropharyngeal obstruction caused by glossoptosis, which occurred mainly during wakefulness. Upper airway obstruction led to hypoxemia, which, in many instances, was not associated with hypercapnia and was not relieved by oxygen administration. It is concluded that regardless of a specific cause, any airway obstruction that results in a decreased inspiratory pressure overcoming the airway maintaining genioglossus action causes a glossoptosis-apnea syndrome.
Asunto(s)
Obstrucción de las Vías Aéreas/fisiopatología , Apnea/etiología , Síndrome de Pierre Robin/fisiopatología , Lengua/fisiopatología , Factores de Edad , Obstrucción de las Vías Aéreas/complicaciones , Obstrucción de las Vías Aéreas/diagnóstico por imagen , Apnea/fisiopatología , Femenino , Humanos , Lactante , Recién Nacido , Pulmón/diagnóstico por imagen , Masculino , Micrognatismo/complicaciones , Micrognatismo/diagnóstico por imagen , Micrognatismo/fisiopatología , Síndrome de Pierre Robin/complicaciones , Síndrome de Pierre Robin/diagnóstico por imagen , Radiografía , Insuficiencia Respiratoria/diagnóstico por imagen , Insuficiencia Respiratoria/etiología , Insuficiencia Respiratoria/fisiopatología , Síndromes de la Apnea del Sueño/etiología , Síndromes de la Apnea del Sueño/fisiopatologíaRESUMEN
A modified poly(ethylene glycol) (PEG) has been developed for the soluble-polymer-supported synthesis of beta-lactams. The monomethylether of PEG (MeOPEG) with an average M(W) of 5000 was used as the support, a 4-(3-propyl)phenyl residue as the spacer, and a 4-oxyphenylamino group as the moiety with the reactive functionality. From this modified PEG representative aromatic, heteroaromatic, unsaturated, and aliphatic imines were obtained in high yields by different procedures. The polymer-supported imines were then employed to prepare several beta-lactams by enolate/imine condensation and ketene/imine cycloaddition. Examples of the control of the absolute stereochemistry during the azetidinone ring formation are also reported. The reactions carried out on the polymer-bound imines showed a remarkable similarity to those performed on nonimmobilized imines, both in terms of yields and stereoselectivities. Removal of the beta-lactams from the polymer has also been accomplished to directly deliver the N-unsubstituted azetidinones.