Análisis de la cultura de seguridad en una Unidad de Cardiología gestionada por procesos / Analysis of the safety culture in a Cardiology Unit managed by processes

Resumen La cultura sobre seguridad se perfila como uno de los requisitos para evitar la aparición de efectos adversos, sin embargo, no se ha estudiado en el ámbito de la cardiología. Objetivo: es evaluar la cultura de seguridad en una unidad de cardiología que tiene implantado y certificado un sistema integrado de gestión de calidad y riesgos para la seguridad del paciente. Método: Se realizó un estudio observacional trasversal en 2 años consecutivos utilizando la encuesta Hospital Survey on Patient Safety Culture de la «Agency for Healthcare Research and Quality¼ en su versión española (42 ítems agrupados en 12 dimensiones) sobre todo el personal. Se comparó el porcentaje de respuestas positivas de cada dimensión en 2014 y 2015, así como con los datos a nivel nacional y en EE. UU., siguiendo las recomendaciones establecidas. Resultados: La valoración global, sobre un máximo de 5, fue de 4.5 en 2014 y de 4.7 en 2015. Identificamos 7 dimensiones como fortaleza. Las peor valoradas fueron: Dotación de personal, Apoyo de la gerencia y Trabajo en equipo entre unidades. La comparación mostró superioridad en todas las dimensiones a nivel nacional, y en 8 respecto a los datos del registro americano. Conclusiones: La cultura de seguridad en una unidad de cardiología con un sistema integrado de gestión de calidad y riesgos y seguridad del paciente es elevada, superior a la nacional en todas sus dimensiones y en la mayoría de ellas respecto al registro de EE. UU.

Abstract Safety culture is one of the requirements for preventing the occurrence of adverse effects. However, this has not been studied in the field of cardiology. The aim of this study is to evaluate the safety culture in a cardiology unit that has implemented and certified an integrated quality and risk management system for patient safety. Methods: A cross-sectional observational study was conducted in 2 consecutive years, with all staff completing the Spanish version of the questionnaire, ''Hospital Survey on Patient Safety Culture'' of the ''Agency for Healthcare Research and Quality'', with 42 items grouped into 12 dimensions. The percentage of positive responses in each dimension in 2014 and 2015 were compared, as well as national data and United States data, following the established rules. Results: The overall assessment out of a possible 5, was 4.5 in 2014 and 4.7 in 2015. Seven dimensions were identified as strengths. The worst rated were: staffing, management support and teamwork between units. The comparison showed superiority in all dimensions compared to national data, and in 8 of them compared to American data. Conclusions: The safety culture in a Cardiology Unit with an integrated quality and risk management patient safety system is high, and higher than nationally in all its dimensions and in most of them compared to the United States.

[Analysis of the safety culture in a Cardiology Unit managed by processes]. / Análisis de la cultura de seguridad en una Unidad de Cardiología gestionada por procesos.

Safety culture is one of the requirements for preventing the occurrence of adverse effects. However, this has not been studied in the field of cardiology. The aim of this study is to evaluate the safety culture in a cardiology unit that has implemented and certified an integrated quality and risk management system for patient safety. METHODS: A cross-sectional observational study was conducted in 2 consecutive years, with all staff completing the Spanish version of the questionnaire, "Hospital Survey on Patient Safety Culture" of the "Agency for Healthcare Research and Quality", with 42 items grouped into 12 dimensions. The percentage of positive responses in each dimension in 2014 and 2015 were compared, as well as national data and United States data, following the established rules. RESULTS: The overall assessment out of a possible 5, was 4.5 in 2014 and 4.7 in 2015. Seven dimensions were identified as strengths. The worst rated were: staffing, management support and teamwork between units. The comparison showed superiority in all dimensions compared to national data, and in 8 of them compared to American data. CONCLUSIONS: The safety culture in a Cardiology Unit with an integrated quality and risk management patient safety system is high, and higher than nationally in all its dimensions and in most of them compared to the United States.

Malformación de Arnold-Chiari en el síndrome de Noonan y otros síndromes de la vía RAS/MAPK / Arnold-Chiari malformation in Noonan syndrome and other syndromes of the RAS/MAPK pathway

Introducción. El síndrome de Noonan (SN) y otros síndromes con fenotipo similar, como LEOPARD, cardiofaciocutáneo, Costello y Legius, están asociados a mutaciones en genes incluidos en la vía RAS/MAPK (rasopatías), una importante vía de señalización relacionada con la proliferación celular. El descenso de las amígdalas cerebelares dentro del canal medular cervical, conocido como malformación de Arnold-Chiari (MAC), se ha descrito en pacientes afectos de SN, lo que ha llevado a sugerir que la MAC podría formar parte del espectro fenotípico del SN. Presentamos dos casos con SN y MAC. Casos clínicos. Caso 1: mujer de 29 años con fenotipo de Noonan. Fue intervenida a los 9 años de estenosis valvular pulmonar. A los 27 años, presentó MAC sintomática que precisó descompresión quirúrgica. Presentaba mutación c.922A>G (N308D) en el gen PTPN perteneciente a la vía RAS/MAPK. Caso 2: niña de 10 años con fenotipo de Noonan y MAC asintomática detectada en resonancia magnética cerebral. Era portadora de la mutación c.923A>G (N308S) en el gen PTPN11. Conclusiones. Hemos encontrado en la bibliografía seis pacientes con esta asociación, cuatro con fenotipo Noonan y dos con LEOPARD. Nuestros dos pacientes aportan evidencia suplementaria a la hipótesis de que la MAC formaría parte del espectro fenotípico del SN. El escaso número de pacientes publicados con esta asociación no permite extraer recomendaciones sobre el momento y la frecuencia de estudio de neuroimagen; no obstante, una exploración neurológica cuidadosa debería incluirse en la guía anticipatoria de salud en los síndromes de la vía RAS/MAPK (AU)

Introduction. Noonan syndrome (NS) and other syndromes with a similar phenotype, such as LEOPARD, cardiofaciocutaneous, Costello and Legius, are associated to mutations in genes included in the RAS/MAPK pathway (RASopathies), which is an important signalling pathway related to cell proliferation. Tonsillar descent into the upper cervical spinal canal, known as Arnold-Chiari malformation (ACM), has been reported in patients with NS and this has led some researchers to suggest that ACM could be part of the phenotypic spectrum of NS. We report two cases of NS and ACM. Case reports. Case 1: 29-year-old female with Noonan phenotype who underwent surgery at the age of nine years due to pulmonary valve stenosis. At the age of 27, she presented symptomatic ACM that required surgical decompression. She presented the c.922A>G (N308D) mutation in the gene PTPN that belongs to the RAS/MAPK pathway. Case 2: a 10-yearold female with Noonan phenotype and asymptomatic ACM detected in magnetic resonance imaging of the brain. She was a carrier of the c.923A>G (N308S) mutation in gene PTPN11. Conclusions. Six patients with this association have been found in the literature, four with the Noonan phenotype and two with LEOPARD. Our two patients provide supplementary evidence that backs up the hypothesis by which ACM would be part of the phenotypic spectrum of NS. The small number of reported cases of patients with this association does not allow us to draw up recommendations about when and how often neuroimaging studies should be performed; a careful neurological examination, however, should be included in the anticipatory health guidelines in syndromes involving the RAS/MAPK pathway (AU)

[Arnold-Chiari malformation in Noonan syndrome and other syndromes of the RAS/MAPK pathway]. / Malformacion de Arnold-Chiari en el sindrome de Noonan y otros sindromes de la via RAS/MAPK.

INTRODUCTION: Noonan syndrome (NS) and other syndromes with a similar phenotype, such as LEOPARD, cardiofaciocutaneous, Costello and Legius, are associated to mutations in genes included in the RAS/MAPK pathway (RASopathies), which is an important signalling pathway related to cell proliferation. Tonsillar descent into the upper cervical spinal canal, known as Arnold-Chiari malformation (ACM), has been reported in patients with NS and this has led some researchers to suggest that ACM could be part of the phenotypic spectrum of NS. We report two cases of NS and ACM. CASE REPORTS: Case 1: 29-year-old female with Noonan phenotype who underwent surgery at the age of nine years due to pulmonary valve stenosis. At the age of 27, she presented symptomatic ACM that required surgical decompression. She presented the c.922A>G (N308D) mutation in the gene PTPN that belongs to the RAS/MAPK pathway. Case 2: a 10-year-old female with Noonan phenotype and asymptomatic ACM detected in magnetic resonance imaging of the brain. She was a carrier of the c.923A>G (N308S) mutation in gene PTPN11. CONCLUSIONS: Six patients with this association have been found in the literature, four with the Noonan phenotype and two with LEOPARD. Our two patients provide supplementary evidence that backs up the hypothesis by which ACM would be part of the phenotypic spectrum of NS. The small number of reported cases of patients with this association does not allow us to draw up recommendations about when and how often neuroimaging studies should be performed; a careful neurological examination, however, should be included in the anticipatory health guidelines in syndromes involving the RAS/MAPK pathway.

TITLE: Malformacion de Arnold-Chiari en el sindrome de Noonan y otros sindromes de la via RAS/MAPK.Introduccion. El sindrome de Noonan (SN) y otros sindromes con fenotipo similar, como LEOPARD, cardiofaciocutaneo, Costello y Legius, estan asociados a mutaciones en genes incluidos en la via RAS/MAPK (rasopatias), una importante via de señalizacion relacionada con la proliferacion celular. El descenso de las amigdalas cerebelares dentro del canal medular cervical, conocido como malformacion de Arnold-Chiari (MAC), se ha descrito en pacientes afectos de SN, lo que ha llevado a sugerir que la MAC podria formar parte del espectro fenotipico del SN. Presentamos dos casos con SN y MAC. Casos clinicos. Caso 1: mujer de 29 años con fenotipo de Noonan. Fue intervenida a los 9 años de estenosis valvular pulmonar. A los 27 años, presento MAC sintomatica que preciso descompresion quirurgica. Presentaba mutacion c.922A>G (N308D) en el gen PTPN perteneciente a la via RAS/MAPK. Caso 2: niña de 10 años con fenotipo de Noonan y MAC asintomatica detectada en resonancia magnetica cerebral. Era portadora de la mutacion c.923A>G (N308S) en el gen PTPN11. Conclusiones. Hemos encontrado en la bibliografia seis pacientes con esta asociacion, cuatro con fenotipo Noonan y dos con LEOPARD. Nuestros dos pacientes aportan evidencia suplementaria a la hipotesis de que la MAC formaria parte del espectro fenotipico del SN. El escaso numero de pacientes publicados con esta asociacion no permite extraer recomendaciones sobre el momento y la frecuencia de estudio de neuroimagen; no obstante, una exploracion neurologica cuidadosa deberia incluirse en la guia anticipatoria de salud en los sindromes de la via RAS/MAPK.

Validity of the sluggish cognitive tempo, inattention, and hyperactivity symptom dimensions: neuropsychological and psychosocial correlates.

This study examined the latent structure and validity of inattention, hyperactivity-impulsivity, and sluggish cognitive tempo (SCT) symptomatology. We evaluated mother and teacher ratings of ADHD and SCT symptoms in 140 Puerto Rican children (55.7% males), ages 6 to 11 years, via factor and regression analyses. A three-factor model (inattention, hyperactivity-impulsivity, and SCT) provided the best fit for both sets of ratings. Inattention was the strongest correlate of lower scores on neuropsychological, achievement, and psychosocial measures. Externalizing problems were most strongly associated with hyperactivity-impulsivity, and internalizing problems were most strongly associated with parent-rated SCT and teacher-rated Inattention. SCT was not associated with executive function but was negatively associated with math. Inattention accounted for a disproportionate amount of ADHD-related impairment, which may explain the restricted discriminant validity of DSM-IV types. The distinct factors of hyperactivity-impulsivity and SCT had unique associations with impairing comorbidities and are roughly equivalent in predicting external correlates of ADHD-related impairment.

Tumoral presentation of primary central nervous system lymphomatoid granulomatosis.

PURPOSE: Lymphomatoid granulomatosis (LYG) is an angiocentric Epstein-Barr virus (EBV) related B-cell proliferation associated with a reactive T-cell component with an uncertain malignant potential. LYG present at diagnosis as a mass lesion in the central nervous system (CNS) is rare, and only a few cases have been reported. In this article we present four cases of tumoral CNS-LYG and propose some guidelines for its management. METHODS: Clinical, pathological, imaging and laboratory information of four immunocompetent patients, all of them treated surgically, with a final diagnosis of LYG and presenting with an isolated intracranial tumoral mass is reviewed. RESULTS: Two parenchymal lesions were located in the cerebellum and temporal lobe, and the other two involved the cavernous sinus. At surgery they were avascular, hard, lard-like, necrotic and plastic well-defined lesions, with invasion of the leptomeninges and thrombosis of the small leptomeningeal arteries and veins. Intraoperative pathology excluded any tumor. Pathological studies showed a polymorphic and polyclonal infiltration around, in the wall and into the lumen of medium-sized cortical and leptomeningeal vessels causing their obstruction and tissular necrosis. EBV-infected cells were present. CONCLUSIONS: Making a preoperative diagnosis of CNS-LYG appearing initially as a tumoral mass is difficult because of the lack of pathognomonic clinical symptoms or imaging signs. Surgical management with radical resection of the mass is almost always followed by the long-term local control of the lesion, although the disease may have a disseminated, systemic or malignant evolution.

Parent perceived impact of Spaniard boys' and girls' inattention, hyperactivity, and oppositional defiant behaviors on family life.

OBJECTIVE: This study examined the impact of inattention, hyperactivity, and oppositional defiant disorder (ODD) behaviors and gender on family life. METHOD: We created scales for the Family Experiences Inventory (FEI) in a nonclinical sample of Spaniard families with children ages 6 to 12 years (N = 369) and analyzed the perceived impact of these three behavior dimensions on family experiences. RESULTS: Multiple regression analyses indicated that ODD behaviors were uniquely correlated with Total FEI and its dimensions. Inattention was also uniquely related to higher negative Impact on School Relations and lower Positive Impact on Parents scales. Finally, gender-hyperactivity interactions indicated that boys with higher hyperactivity scores were more likely to score higher on the FEI Total, School Relations, and Siblings scales, and more likely to score lower on the Positive Impact on Parents scale than girls. CONCLUSIONS: These findings suggested that parents perceive greater child-related impact and place greater burden from having a male child with hyperactivity. Inattention, hyperactivity, and oppositional defiant behaviors are associated with global parent-child interactive stress but the pattern of associations will vary depending upon the behavior, child gender, and context of family life examined.

Comparison of the DSM-IV combined and inattentive types of ADHD in a school-based sample of Latino/Hispanic children.

BACKGROUND: The aim of this investigation was to examine the construct validity and distinctiveness of the inattentive type (IT) and combined type (CT) of Attention-Deficit/Hyperactivity Disorder (ADHD) in a Latino/Hispanic sample. METHOD: A comprehensive assessment was conducted with a clinically diagnosed school-based sample of 98 children aged 6 to 11 (CT=44; IT=25; control group=29). RESULTS: Both ADHD groups were impaired on academic achievement measures, presented more ADHD-type behaviors during math and vigilance tasks, and exhibited greater internalizing symptoms. The IT group had a later onset of inattention symptoms, presented more sluggish cognitive tempo symptoms, was less prone to initiate social interactions or to be assertive and more self-controlled in social interchanges, was less likely to have externalizing behaviors, had mothers who reported less child-related family stress, and was less impaired in their adaptive functioning. CONCLUSIONS: Findings supported the construct validity of ADHD in this culturally different sample and suggested that the CT and IT represent distinct disorders.

Time estimation and performance on reproduction tasks in subtypes of children with attention deficit hyperactivity disorder.

This study compared Hispanic children (ages 7 to 11) with combined type (CT, n=33) and inattentive type (IT, n=21) attention deficit hyperactivity disorder (ADHD) and a control group (n=25) on time-estimation and time-reproduction tasks. The ADHD groups showed larger errors in time reproduction but not in time estimation than the control group, and the groups did not differ from each other on their performance on this task. Individual differences could not be accounted for by oppositional-defiance ratings and low math or reading scores. Although various measures of executive functioning did not make significant unique contributions to time estimation performance, those of interference control and nonverbal working memory did so to the time-reproduction task. Findings suggest that ADHD is associated with a specific impairment in the capacity to reproduce rather than estimate time durations and that this may be related to the children's deficits in inhibition and working memory.

Survival analysis of the surgical outcome of temporal lobe epilepsy due to hippocampal sclerosis.

PURPOSE: Surgical results in patients with mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE/HS) are often reported in conjunction with other etiologies of TLE. METHODS: We prospectively collected surgical outcome data for 2 to 11 years for 134 consecutive patients who specifically had MTLE and unilateral HS, according to magnetic resonance imaging (MRI) and confirmed by histopathology. Sixty-five had postoperative neuropsychological testing. Outcome was analyzed by using Engel's classification (a) through Kaplan-Meier estimated survival curves (as a function of the time to seizure recurrence), (b) as percentage of patients in each outcome class on a yearly basis, and (c) at the last updated follow-up. RESULTS: Kaplan-Meier estimates of complete seizure freedom (Engel's class IA) for years 1, 2, 5, and 10 were 85%, 77%, 74%, and 66%, and of Engel's class I were 89%, 86%, 83%, and 81%. Only nine (6.7%) patients had outcome classes III or IV at any point during follow-up. Of the patients tested, 26% of those operated on the left side and 22% of those operated on the right had postoperative decline of >1 SD in verbal or visual memory, respectively. CONCLUSIONS: High rates of seizure freedom can be obtained and remain stable over the years in patients operated on for unilateral MTLE/HS, even in countries with limited resources.

[Prognostic value of persistent ST-segment elevation after successful primary angioplasty]. / Valor pronóstico de la persistencia del segmento ST elevado después de una angioplastia primaria realizada con éxito.

INTRODUCTION AND OBJECTIVES: A variable percentage of patients with myocardial infarction treated with successful primary angioplasty and restoration of coronary flow show persistent ST-segment elevation, probably due to inadequate cellular reperfusion. We studied if persistent ST-segment elevation was a predictor of worse prognosis. PATIENTS AND METHODS: We comparatively studied the clinical and angiographic results of 116 acute myocardial infarction patients after successful primary angioplasty, which were classified into two groups depending on the persistence (> 50%) or reduction (

Klippel-Feil syndrome associated with posterior fossa dermoid cyst. Case report.

A patient with association of Klippel-Feil syndrome and posterior fossa dermoid cyst is presented. The patient, a 36-year-old man, presented with an acute obstructive hydrocephalus due to the cyst and exhibited the typical triad of the Klippel-Feil abnormality with short neck, low hairline implantation and limited neck motion along with a complex cervical vertebrae fusion. The anatomical and clinical features as well as the pathophysiology of this rare association are discussed after a review of the literature.