Relevance of subclinical right ventricular dysfunction measured by feature-tracking cardiac magnetic resonance in non-ischemic dilated cardiomyopathy.

BACKGROUND: Right ventricular (RV) dysfunction in patients with non-ischemic dilated cardiomyopathy (NICM) is associated with cardiovascular events. To analyze the feasibility of assessing RV myocardial deformation by feature tracking (FT)-cardiac magnetic resonance (CMR), and its usefulness as a prognostic marker. METHODS: Retrospective study of NICM patients undergoing CMR. Longitudinal FT-RV free wall (LFT-RVFW) and fractional area change (FAC) were obtained. Correlation with standard RV parameters was studied. An association with combined event (heart failure (HF), ICD implantation or cardiovascular death) was assessed using a logistic regression model. RESULTS: 98 patients (64 ± 13 years) were included. Left ventricular (LV) systolic function (LVEF 29.5 ± 9.6%, 47% with LVEF ≥ 30%) and RV (RVEF 52.2 ± 14.6%, 72% with RVEF ≥ 45%). Follow-up of 38 ± 17 months, 26.5% presented at least one admission for HF. An excellent correlation of LFT-RVFW (r = 0.82) and FAC (r = 0.83) with RVEF was evident. No association of RV-FT parameters with prognosis entire study population was found. However, in patients with LVEF ≥ 30%, admissions for HF were associated with lower LFT-RVFW (-21.6 ± 6.6% vs -31.3 ± 10%; p = 0.006) and FAC (36.6 ± 9.6% vs 50.5 ± 13.4%; p < 0.001) values. Similar differences were observed when only patients with RVEF ≥ 45% were considered. An LFT-RVFW cut-off point of -19.5% and FAC of 36.5% showed good prognostic performance. Decreased LFT-RVFW or FAC represented an independent predictor of combined event in patients with LVEF ≥ 30%. CONCLUSIONS: In NICM patients without severe LV dysfunction, decreased values of LFT-RVFW and/or FAC were associated with HF admissions, independently of RVEF.

Vertebral fractures are increased in rheumatoid arthritis despite recent therapeutic advances: a case-control study.

Prevalence and risk factors of vertebral fractures in postmenopausal RA women were assessed in 323 patients and compared with 660 age-matched women. Of patients, 24.15% had at least one vertebral fracture vs.16.06% of controls. Age, glucocorticoids and falls were the main fracture risks. Vertebral fractures were associated with disease severity. INTRODUCTION: There is little quality data on the updated prevalence of fractures in rheumatoid arthritis (RA) that may have changed due to advances in the therapeutic strategy in recent years. This study was aimed at analysing the prevalence and risk factors of vertebral fractures in postmenopausal women with RA and comparing it with that of the general population. METHODS: We included 323 postmenopausal women diagnosed with RA from 19 Spanish Rheumatology Departments, randomly selected and recruited in 2018. Lateral radiographs of the thoracic and lumbar spine were obtained to evaluate morphometric vertebral fractures and the spinal deformity index. We analysed subject characteristics, factors related to RA, and fracture risk factors. The control group consisted of 660 age-matched Spanish postmenopausal women from the population-based Camargo cohort. RESULTS: Seventy-eight (24.15%) RA patients had at least one vertebral fracture. RA patients had increased fracture risk compared with controls (106 of 660, 16.06%) (p = 0.02). Logistic regression analysis showed that age (OR 2.17; 95% CI 1.27-4.00), glucocorticoids (OR 3.83; 95% CI 1.32-14.09) and falls (OR 3.57; 95% CI 1.91-6.86) were the independent predictors of vertebral fractures in RA patients. The subgroup with vertebral fractures had higher disease activity (DAS28: 3.15 vs. 2.78, p = 0.038) and disability (HAQ: 0.96 vs. 0.63, p = 0.049), as compared with those without vertebral fractures. CONCLUSION: The risk of vertebral fracture in RA is still high in recent years, when compared with the general population. The key determinants of fracture risk are age, glucocorticoids and falls. Patients with vertebral fractures have a more severe RA.

Osteoporotic patients treated with bisphosphonates do not show the increased mortality observed in those untreated.

INTRODUCTION: Osteoporosis has been said to be associated with increased mortality. On the other hand, it is debated whether treatment with bisphosphonates may reduce mortality in osteoporotic patients. To contribute to the clarification of these issues, we have studied in a prospective cohort the mortality in people without osteoporosis and in patients with osteoporosis, untreated or treated with bisphosphonates MATERIAL AND METHODS: At their inclusion in the cohort, four groups of participants were identified: (a) people without osteoporosis (group 1); (b) osteoporotic patients treated with bisphosphonates (group 2); (c) osteoporotic patients who refused to be treated (group 3); and (d) patients who met osteoporosis diagnostic criteria but were not treated because their risk of fracture was considered to be low (group 4). To compare all four groups, unadjusted Kaplan-Meier estimates of survivorship were obtained and they were compared using log-rank test. Hazard ratios were then estimated via Cox regression adjusting for the main confounders. A comparison among the osteoporotic groups was made by means of a Cox regression analysis performed using only these three groups, adjusting for propensity scores. RESULTS: Two thousand six hundred and sixty-five people were included. In the unadjusted analysis, mortality in group 3 was higher than in the other groups (p < 0.001). Taking group 1 as a reference, Cox regression analysis showed the following mortality HRs for groups 2, 3, and 4 after adjusting for confounding factors: 0.82 (0.41-1.63), 1.37 (0.90-2.10), and 0.69 (0.46-1.02). In the analysis of the osteoporotic groups with the PS generated for them, and taking group 2 as a reference, the HRs were as follows: group 3, 2.38 (1.34-4.22); group 4, 1.45 (0.61-3.43). CONCLUSION: Mortality in osteoporotic patients who refused treatment is higher than in osteoporotic patients treated with bisphosphonates. In unadjusted analysis, it was also higher than in non-osteoporotic people; however, this difference disappeared after adjustment for confounding factors.

[Effects of the COVID-19 pandemic on a cohort of patients with vein occlusion]. / Efectos de la pandemia por SARS-CoV-2 en una cohorte de pacientes con obstrucción venosa retiniana.

BACKGROUND AND OBJECTIVES: A new coronavirus disease in humans, COVID-19, caused by SARS-CoV-2, emerged in December 2019. It has been associated with the development of thrombotic phenomena. Retinal vein occlusion (RVO) is mainly a consequence of vascular risk factors (VRF). This study aimed to analyze cases of COVID-19 in a cohort of patients with RVO (Valdecilla cohort). PATIENTS AND METHODS: Between December 2008 and December 2020, 429 patients with RVO were attended to in our clinic. Ten patients had COVID-19, one of which did not have VRF or thrombophilia. The remaining nine patients had RVO prior to the infection and VRF, six had carotid atherosclerosis, and four had antiphospholipid syndrome. The infection did not cause thrombotic phenomena in any of them. CONCLUSIONS: RVO is a rare manifestation of COVID-19. In our cohort of patients with RVO, COVID-19 disease did not lead to thrombotic events.

The Arf6 activator Efa6/PSD3 confers regional specificity and modulates ethanol consumption in Drosophila and humans.

Ubiquitously expressed genes have been implicated in a variety of specific behaviors, including responses to ethanol. However, the mechanisms that confer this behavioral specificity have remained elusive. Previously, we showed that the ubiquitously expressed small GTPase Arf6 is required for normal ethanol-induced sedation in adult Drosophila. Here, we show that this behavioral response also requires Efa6, one of (at least) three Drosophila Arf6 guanine exchange factors. Ethanol-naive Arf6 and Efa6 mutants were sensitive to ethanol-induced sedation and lacked rapid tolerance upon re-exposure to ethanol, when compared with wild-type flies. In contrast to wild-type flies, both Arf6 and Efa6 mutants preferred alcohol-containing food without prior ethanol experience. An analysis of the human ortholog of Arf6 and orthologs of Efa6 (PSD1-4) revealed that the minor G allele of single nucleotide polymorphism (SNP) rs13265422 in PSD3, as well as a haplotype containing rs13265422, was associated with an increased frequency of drinking and binge drinking episodes in adolescents. The same haplotype was also associated with increased alcohol dependence in an independent European cohort. Unlike the ubiquitously expressed human Arf6 GTPase, PSD3 localization is restricted to the brain, particularly the prefrontal cortex (PFC). Functional magnetic resonance imaging revealed that the same PSD3 haplotype was also associated with a differential functional magnetic resonance imaging signal in the PFC during a Go/No-Go task, which engages PFC-mediated executive control. Our translational analysis, therefore, suggests that PSD3 confers regional specificity to ubiquitous Arf6 in the PFC to modulate human alcohol-drinking behaviors.

High prevalence of non-alcoholic fatty liver disease among hidradenitis suppurativa patients independent of classic metabolic risk factors.

BACKGROUND: Some chronic inflammatory skin diseases, such as psoriasis, have been associated with an increased prevalence of non-alcoholic fatty liver disease (NAFLD). Nevertheless, this prevalence in hidradenitis suppurativa (HS) has not been assessed to date. OBJECTIVES: To determine the prevalence of NAFLD in patients with HS and the risk factors associated with this disorder. METHODS: This case-control study enrolled 70 HS patients and 150 age- and gender-matched controls who were evaluated by hepatic ultrasonography (US) and transient elastography (TE) after excluding other secondary causes of chronic liver disease. The diagnosis of NAFLD was established if US and/or TE were altered. RESULTS: The prevalence of NAFLD was significantly increased in patients with HS compared to controls (72.9% vs. 24.7%: P < 0.001). In the multivariable regression model adjusted for age, sex and classic metabolic risk factors for NAFLD, HS was significantly and independently associated with the presence of NAFLD [OR 7.75 confidence interval (CI) 2.54-23.64; P < 0.001]. CONCLUSIONS: Our results show a high prevalence of NAFLD in HS patients independent of classic metabolic risk factors. Therefore, we suggest HS patients to be evaluated for NAFLD and managed accordingly.

Evidence of the high prevalence of neurological disorders in nonsyndromic X-linked recessive ichthyosis: a retrospective case series.

BACKGROUND: X-linked recessive ichthyosis (XLI) is a relatively common type of ichthyosis caused by a deficiency in the steroid sulfatase (STS) enzyme. It is the only type of ichthyosis that can be both syndromic and nonsyndromic. Typical clinical features include dark-brown scale of variable size favouring the extensor surfaces of the extremities. OBJECTIVES: To characterize clinically nonsyndromic XLI, with a particular focus on extracutaneous manifestations. METHODS: This was a multicentre retrospective review of clinical findings from a case series of patients with a clinical and genetic diagnosis of XLI. RESULTS: We identified 30 patients with XLI belonging to 25 different families carrying a deletion in the STS locus. All patients had dark scales of variable size on the extensor surfaces of the extremities. Lack of flexural involvement and pruritus were common but inconsistent findings, whereas palmoplantar hyperlinearity was absent in all but one patient. A history of orchiopexy was present in 10% and thus was more common than expected vs. the general population (3%). Neurological disorders including epilepsy (13%) and attention deficit hyperactivity disorder (ADHD; 30%) were over-represented in patients with XLI. CONCLUSIONS: This was a retrospective study with a limited number of patients. In the absence of confirmatory genetic testing and family history of the disease, dark-brown scale of the extensor surfaces and the absence of palmoplantar hyperlinearity appear to be the most reliable clinical findings supporting a diagnosis of XLI. Dermatologists should be aware of the high prevalence of ADHD and epilepsy in patients with nonsyndromic XLI.

Insulin resistance in hidradenitis suppurativa: a case-control study.

BACKGROUND: The association between chronic inflammatory diseases, such as rheumatoid arthritis and psoriasis, and insulin resistance (IR) has been well established. Hidradenitis suppurativa (HS) is a chronic inflammatory cutaneous disease that affects the apocrine gland-bearing areas of the body. OBJECTIVE: We aimed to determine the prevalence of IR in patients with HS. METHODS: This cross-sectional, case-control study enrolled 137 subjects, 76 patients with HS and 61 age- and gender-matched controls. Demographic data, clinical examination of HS patients, anthropometric measures, cardiovascular risk factors and laboratory studies were recorded. The homeostasis model assessment of IR (HOMA-IR) was calculated in all participants by measuring fasting plasma glucose and insulin levels. RESULTS: The median (IQR) HOMA-IR value in HS patients was significantly higher [2.0 (1.0-3.6)] than in controls [1.5 (0.9-2.3)] (P = 0.01). The prevalence of IR was significantly higher in cases (43.4%) compared with controls (16.4%) (P = 0.001). In the linear regression multivariable analysis after adjusting for age, sex and body mass index (BMI), HS remained as a significant factor for a higher HOMA-IR [2.51 (0.18) vs 1.92(0.21); P = 0.04]. The HOMA-IR value and the prevalence of IR did not differ significantly among HS patients grouped by severity of the disease. CONCLUSION: Our results show an increased frequency of IR in HS. Thus, we suggest HS patients to be evaluated for IR and managed accordingly.

[Management of suspected foreign body aspiration in children. 10-year experience in a single center]. / Sospecha de aspiración de cuerpos extraños en pacientes pediátricos. Nuestra experiencia en 10 años.

INTRODUCTION: Foreign body aspiration (FBA) is a potentially life-threatening event, and is the leading cause of death in children after road traffic injuries. If suspected, a prompt exploration of the airway should be performed. We present our experience in FBA treatment in the last 10 years. MATERIAL AND METHODS: A retrospective study of patients admitted in our center with suspected FBA between 2005 and 2015 was performed. Clinical history, physical findings, radiologic imaging, treatment and evolution were assessed. RESULTS: A total of 115 children (70 M/45 F) with a median age of 2 years old (8 months-13 years), presented with a clinical history of FBA. All of them suffered a choking event and the most frequent symptoms were persistent cough (88.3%) and respiratory distress (46.8%). Pathologic physical examination was registered in 75% and an abnormal chest X-ray was seen in 72%. Rigid bronchoscopy (RB) was performed in 100% based on compatible history, regardless of physical and radiologic exams. A foreign body was found during RB in 78 patients (68.1%) and the most frequent were seeds and nuts (63.4%), located mainly in the right bronchus (46.8%). If the criteria for RB had been based on a compatible history along with clinical findings and abnormal chest X-ray, 21 foreign bodies (26.9%) would have been missed, with the subsequent risk of sudden death. All the foreign bodies were removed with success, without any immediate complication during the procedure. CONCLUSION: FBA is a frequent accident among children. The history, clinical findings and imaging cannot always concur. In case of a positive history of FBA an examination of the tracheobronchial tree must be done.

OBJETIVOS: La aspiración de cuerpos extraños (ACE) es una urgencia pediátrica frecuente, descrita como segunda causa de muerte infantil tras los accidentes de tráfico. Presentamos nuestra experiencia en el tratamiento de la ACE en los últimos 10 años. MATERIAL Y METODOS: Estudio retrospectivo de pacientes atendidos por sospecha de ACE entre los años 2005-2015, describiendo historia clínica, sintomatología, pruebas de imagen, procedimiento y evolución. RESULTADOS: Un total de 115 pacientes (70 V/45 M), con una mediana de 2 años (8 meses-13 años), presentaron historia sugestiva de ACE. Los síntomas más frecuentes fueron: tos (88,3%) y dificultad respiratoria (46,8%). El 75% presentaban exploración física patológica y un 72% presentaban alteraciones en la radiografía de tórax. Se realizó broncoscopia rígida (BR) en el 100% de los pacientes con historia compatible, sin importar los resultados de la exploración física o las pruebas de imagen. En 78 pacientes (68,1%) se encontró un cuerpo extraño (CE) durante la BR, siendo los más frecuentes semillas y frutos secos (63,4%); localizándose sobre todo en el bronquio derecho (46,8%). Si el criterio para realizar una BR se hubiese basado en la historia compatible, junto con alteraciones en la exploración física y pruebas de imagen, 21 CE (26,9%) no habrían sido diagnosticados con el consiguiente riesgo de muerte. Todos los CE se extrajeron con éxito, sin ninguna complicación inmediata. CONCLUSIONES: La ACE es frecuente en la infancia. La historia y los hallazgos clínico-radiológicos pueden no concordar, por lo que ante historia sugestiva de ACE es recomendable una exploración de la vía aérea, dado el bajo riesgo que asocia.

MicroRNA (miR)-203 and miR-205 expression patterns identify subgroups of prognosis in cutaneous squamous cell carcinoma.

BACKGROUND: Cutaneous squamous cell carcinoma (CSCC) is the second most widespread cancer in humans and its incidence is rising. These tumours can evolve as diseases of poor prognosis, and therefore it is important to identify new markers to better predict its clinical evolution. OBJECTIVES: We aimed to identify the expression pattern of microRNAs (miRNAs or miRs) at different stages of skin cancer progression in a panel of murine skin cancer cell lines. Owing to the increasing importance of miRNAs in the pathogenesis of cancer, we considered the possibility that miRNAs could help to define the prognosis of CSCC and aimed to evaluate the potential use of miR-203 and miR-205 as biomarkers of prognosis in human tumours. METHODS: Seventy-nine human primary CSCCs were collected at the University Hospital of Salamanca in Spain. We identified differential miRNA expression patterns at different stages of CSCC progression in a well-established panel of murine skin cancer cell lines, and then selected miR-205 and miR-203 to evaluate their association with the clinical prognosis and evolution of human CSCC. RESULTS: miR-205 was expressed in tumours with pathological features recognized as indicators of poor prognosis such as desmoplasia, perineural invasion and infiltrative growth pattern. miR-205 was mainly expressed in undifferentiated areas and in the invasion front, and was associated with both local recurrence and the development of general clinical events of poor evolution. miR-205 expression was an independent variable selected to predict events of poor clinical evolution using the multinomial logistic regression model described in this study. In contrast, miR-203 was mainly expressed in tumours exhibiting the characteristics associated with a good prognosis, was mainly present in well-differentiated zones, and rarely expressed in the invasion front. Therefore, the expression and associations of miR-205 and miR-203 were mostly mutually exclusive. Finally, using a logistic biplot we identified three clusters of patients with differential prognosis based on miR-203 and miR-205 expression, and pathological tumour features. CONCLUSIONS: miR-205 and miR-203 tended to exhibit mutually exclusive expression patterns in human CSCC. This work highlights the utility of miR-205 and miR-203 as prognostic markers in CSCC.

Thyroid autoimmunity in patients with hidradenitis suppurativa: a case-control study.

The aetiopathogenesis of hidradenitis suppurativa (HS) is not fully understood; however, increasing evidence suggests that it may be an immune-mediated disorder. Autoimmune thyroid disease (AITD) has classically been considered as the 'paradigm' of autoimmunity, and it has been linked to a variety of skin disorders. To our knowledge, the prevalence of AITD has not been investigated in patients with HS. The aim of the present study was to assess and compare, for the first time, the prevalence of thyroid autoimmunity in 70 patients with HS and in 70 age- and sex-matched controls. In all participants, thyroid autoantibodies and thyroid function tests were analysed. No statistically significant difference was detected between patients with HS and controls, either for the prevalence of thyroid antibodies or for thyroid function parameters. This lack of an association between HS and thyroid autoimmunity suggests that conventional autoimmune mechanisms may not be implicated in the pathogenesis of HS.

Factors associated with poor outcome for aneurysmal subarachnoid haemorrhage in a series of 334 patients. / Factores asociados a una evolución desfavorable en la hemorragia subaracnoidea aneurismática. Serie de 334 pacientes.

OBJECTIVE: This study evaluates care-related sociodemographic, clinical, and imaging factors and influences associated with outcome at discharge in patients with aneurismal subarachnoid haemorrhage. PATIENTS AND METHOD: Retrospective cohort study in 334 patients treated at Hospital Hermanos Ameijeiras in Havana, Cuba between October 2005 and June 2014. RESULTS: Logistic regression analysis determined that the following factors were associated with higher risk of poor outcome: age older than 65 years (OR 3.51, 95% CI 1.79-5.7, P=.031), female sex (OR 2.17, 95% CI 1.22-3.84, P=.0067), systolic hypertension (OR 4.82, 95% CI 2.27-9.8, P=.0001), and hyperglycaemia at admission (OR 3.93, 95% CI 2.10-7.53, P=.0003). Certain complications were also associated with poor prognosis, including respiratory infection (OR 2.73, 95% CI 1.27-5.85, P=.0085), electrolyte disturbances (OR 3.33, 95% CI 1.33-8.28, P=.0073), hydrocephalus (OR 2.21, 95% CI 1.05-4.63, P=.0039), rebleeding (OR 16.50, 95% CI 8.24-41.24, P=.0000), symptomatic vasospasm (OR 19.00, 95% CI 8.86-41.24, P=.0000), cerebral ischaemia (OR 3.82, 95% CI 1.87-7.80, P=.000) and multiplex rebleeding (OR 6.69, 95% CI 1.35-36.39, P=.0019). Grades of iii and iv on the World Federation of Neurological Surgeons (OR 2.09, 95% CI 1.12-3.91, P=.0021) and Fisher scales (OR 5.18, 95% CI 2.65-10.29, P=.0008) were also related to poor outcome. CONCLUSIONS: Outcome of aneurysmal subarachnoid haemorrhage was related to age, sex, clinical status at admission to the stroke unit, imaging findings according to the Fisher scale, blood pressure, glycaemia and such complications as electrolyte disturbances, hydrocephalus, rebleeding, and multiplex rebleeding.

[Results of the mesoportal bypass (Rex shunt) in the treatment of idiopathic extrahepatic portal vein obstruction in children]. / Resultados del shunt mesoportal de Rex en el tratamiento de la trombosis portal extrahepática idiopática.

BACKGROUND: Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. METHODS: Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery. RESULTS: Twelve patients underwent surgery, out of which 10 had prior leukopenia, 11 thrombopenia, 9 longer prothrombin times and all had hypersplenism. One patient suffered a postoperative shunt thrombosis, was reoperated and underwent a change in the operative technique. The remaining patients (92%) have functioning shunts 4.3 ± 2.5 years after surgery, and none have suffered any episode of gastrointestinal bleeding. One year after surgery, there were significant changes in the number of platelets, prothrombin time and spleen size, with no significant changes in the number of leukocytes. However, the number of patients who went from a leukopenic to a normal state was significant, as happened with changes in prothrombin time. CONCLUSIONS: Mesoportal Rex shunt improves some of the disorders caused by portal hypertension in children suffering EPVO, with a high rate of surgical success. This technique should be of first choice in these patients.

OBJETIVOS: La trombosis portal extrahepática (TPEH) es la causa más frecuente de hipertensión portal en el niño. El objetivo de este estudio es analizar la utilidad de la técnica quirúrgica que crea un shunt mesoportal para mejorar o revertir las alteraciones causadas por la TPEH. MATERIAL Y METODOS: Revisión retrospectiva de los pacientes con TPEH idiopática sometidos al shunt mesoportal y análisis de los cambios en la cifra de leucocitos, plaquetas, tiempo de protrombina y tamaño del bazo al año de la cirugía. RESULTADOS: De los 12 pacientes intervenidos, 10 tenían leucopenia, 11 plaquetopenia, 9 un tiempo de protrombina alargado y todos presentaban hiperesplenismo. Una paciente sufrió una trombosis postoperatoria del shunt por la que fue reoperada con cambio de la técnica quirúrgica. El resto de pacientes (92%) tienen un shunt funcionante con un seguimiento 4,3 ± 2,5 años y no han sufrido ningún sangrado gastrointestinal. Al año de la cirugía, observamos cambios significativos en el número total de plaquetas, tiempo de protrombina y tamaño del bazo, no siendo estos cambios significativos para la cifra de leucocitos. Sin embargo, el número de pacientes que pasó de estar leucopénico a tener cifras normales de leucocitos, sí fue significativo, hecho que también ocurrió en el caso de las alteraciones en el tiempo de protrombina. CONCLUSIONES: El shunt mesoportal de Rex mejora de manera efectiva las alteraciones asociadas a la hipertensión portal por TPEH, con una alta tasa de éxito, por lo que debería ser la técnica de elección en estos pacientes.

[Benefits of magnetic resonance for the study of pectus excavatum in children: initial experience]. / Beneficios de la resonancia magnética para el estudio del pectus excavatum en niños: experiencia inicial.

INTRODUCTION/AIM OF THE STUDY: In patients with PE, cardiovascular alterations ensue as a result of the mediastinum compression caused by sternum impingement and is responsible of many of the symptoms. Anatomical and functional assessment is of the utmost importance for a comprehensive understanding of the disease and an adequate treatment plan. Our aim was to describe the use of magnetic resonance image (MRI) in the study of PE and whether it is comparable to imaging techniques. PATIENTS AND METHODS: A retrospective study of the first 10 patients in which an MRI was performed as part of PE assessment within august 2015-2016. PE indexes were evaluated: Haller, correction, asymmetry, as well as sternal rotation. An analysis of right ventricular function was carried out comparing echocardiogram and MRI. RESULTS: MRI scan on 10 patients showed the following findings: Haller index: inspiration: 3.75 (3.5-7.3) and expiration 4,9 (3.9-10.8), correction index of 24% (5-37%) and a sternal rotation of 12º (0-31º). The cardiovascular study showed a median ejection fraction of the right ventricle (EFRV) of 50% (38-64%), with 9 of the 10 patients under the normal value of 61% (54-71%). Echocardiographic findings underestimated functional alterations in all of the patients. CONCLUSION: This initial study suggests that the use of MRI as a test of choice in the evaluation of PE subject to surgical correction is feasible. Absence of radiation offers the capacity of a complete and dynamic anatomical as well as cardiovascular study.

INTRODUCCION/OBJETIVOS: Las alteraciones cardiovasculares provocadas por la impronta del esternón en el mediastino causan muchos de los síntomas que presentan los pacientes con pectus excavatum (PE). Su estudio anatómico y funcional es fundamental para el entendimiento de la enfermedad y para la elección del tratamiento adecuado. Nuestro objetivo es describir el uso de la resonancia magnética (RM) en el estudio de los pacientes con PE, y si esta prueba es equiparable o superior a otras técnicas de imagen. PACIENTES Y METODOS: Evaluación retrospectiva de los resultados de las primeras 10 RM indicadas en pacientes con PE en el período de octubre 2015-2016. Se analizaron los índices de PE: Haller, corrección, asimetría, así como el grado de rotación esternal. Además se realizó un análisis de la función ventricular derecha mediante ecocardiografía y RM. RESULTADOS: Los 10 pacientes evaluados con RM presentaron: índice de Haller en inspiración de 3,75 (3,5-7,3) y en espiración de 4,9 (3,9-10,8), índice de corrección 24% (5-37%) y rotación esternal 12º (0º-31º). Del estudio cardiovascular destaca una fracción de eyección del ventrículo derecho (FEVD) del 50% (38-64%), con 9 de los pacientes por debajo del valor normal 61% (54-71%). La ecocardiografía infraestimó la alteración funcional en la mayoría de casos, al calificarla de normal en el 100%. CONCLUSION: Este estudio inicial sugiere que el uso de la RM como prueba de elección en la evaluación del PE susceptible de corrección quirúrgica es factible. La ausencia de radicación ofrece la capacidad de un estudio tanto anatómico como cardiovascular completo y dinámico.

[Mesenteric edema as a prenatal ultrasound sign of poor prognosis in gastroschisis]. / El edema mesentérico como signo ecográfico prenatal de mal pronóstico en gastrosquisis.

INTRODUCTION/AIM OF THE STUDY: Gastroschisis is a congenital malformation with an easy and early prenatal diagnosis, however, it has a variable post-natal outcome. Our aim was to determine if certain ultrasound markers or early delivery were related with a worse postnatal outcome. PATIENTS AND METHODS: Retrospective study of a cohort of patients with gastroschisis diagnosed between 2005-2014, with emphasis on prenatal ultrasounds, gestational age at delivery and post-natal outcome. Oligohydramnios, peel, mesenteric edema, fixed and dilated bowel with loss of peristalsis and small wall defect were considered ultrasonographic markers associated with poor prognosis. Outcome variables included: length-of-stay, complications, nutritional and respiratory factors. Non-parametric statistical analysis were used with p < 0,05 regarded as significant. RESULTS: Clinical charts of 30 patients with gastroschisis were reviewed (17M/13F). Gestational age at diagnosis was 20 (12-31) and at delivery 36 (31-39) weeks (33% of the patients over 36+3 weeks). A 73% of the patients presented at least one ultrasonographic marker factor during follow-up. Univariate analysis showed that mesenteric edema was associated with poor outcome variables: short-bowel syndrome (p= 0,000), PN-dependence (p= 0,007) and intestinal atresia (p= 0,02). The remaining risk factors analysed, including late delivery (> 36+3 weeks) were not associated with length-of-stay, ventilatory support, digestive autonomy, complications or mortality. CONCLUSIONS: Neither the presence of ultrasonographic markers classically associated with unfavorable outcomes, nor early delivery (< 36 weeks) resulted in worse postnatal outcome. Mesenteric edema was the only alarming ultrasound marker and that may suggest the need of closer follow-up.

INTRODUCCION: La gastrosquisis es una anomalía congénita de fácil diagnóstico prenatal y pronóstico postnatal variable. Nuestro objetivo es determinar si los signos ecográficos prenatales o el momento del parto se relacionan con peor pronóstico postnatal. PACIENTES Y METODOS: Se realiza un estudio retrospectivo de la cohorte de pacientes con gastrosquisis diagnosticados entre 2005-2014, registrando las ecografías prenatales, edad gestacional al parto y evolución postnatal. Se valoraron los hallazgos ecográficos prenatales: oligohidramnios, peel, edema mesentérico, asas fijas, aperistálticas y/o dilatadas y defecto pequeño de pared. Se consideraron variables resultado: la estancia hospitalaria, complicaciones, mortalidad y factores nutricionales y respiratorios. Se utilizaron pruebas no paramétricas, considerándose significativo un valor p < 0,05. RESULTADOS: Se analizaron 30 pacientes con gastrosquisis (17V/13M). La edad gestacional al diagnóstico fue de 20 (12-31) y al parto de 36 (31-39) semanas (33% mayores de 36+3 semanas). El 73% de los pacientes presentaron al menos un signo ecográfico de mal pronóstico. El análisis univariante asoció el edema mesentérico al síndrome de intestino corto (p= 0,000), falta de autonomía digestiva (p= 0,007) y mayor incidencia de atresia (p= 0,02). El resto de los factores, incluyendo la edad gestacional > 36+3 semanas, no tuvieron repercusión negativa en términos de estancia, asistencia respiratoria, autonomía digestiva, complicaciones o mortalidad. CONCLUSIONES: Ni la presencia de signos ecográficos considerados generalmente como desfavorables ni la tendencia a acercar el parto a la semana 36ª tienen repercusiones significativas en el curso postnatal. Únicamente el edema mesentérico parece un signo alarmante que sugiere la necesidad de aumentar la frecuencia de intervenciones (ecografías, pruebas de bienestar fetal).

Serum 25-hydroxyvitamin D, parathyroid hormone, calcium intake, and bone mineral density in Spanish adults.

UNLABELLED: Vitamin D insufficiency is very common among Spanish community-dwelling adult subjects. A threshold of serum 25(OH)D around 30 ng/ml would be necessary for the prevention of secondary hyperparathyroidism and hip bone loss in our population, regardless of the dairy calcium ingestion. INTRODUCTION: This study aims to assess 25-hydroxyvitamin D-25(OH)D-status in Spanish adult subjects and to analyze its relationships with serum PTH levels, calcium intake, and bone mineral density (BMD). METHODS: A total of 1811 individuals (1154 postmenopausal women and 657 men) aged 44-93 years participated in the study. Serum 25(OH)D, intact parathyroid hormone (PTH), aminoterminal propeptide of type I collagen (P1NP), and C-terminal telopeptide of type I collagen (ß-CTX) levels were measured by electrochemiluminescence. BMD was determined by dual x-ray absorptiometry (DXA) at lumbar spine, femoral neck, and total hip. RESULTS: Serum 25(OH)D levels were below 10, 20, and 30 ng/ml in 5, 40, and 83 % of participants, respectively. There was a significant seasonal difference in mean serum 25(OH)D, with higher levels in summer-autumn. In multivariate analysis, 25(OH)D levels were negatively correlated with age, serum PTH and creatinine, body mass index, smoking, alcohol intake, and a number of chronic diseases, but positively with dairy calcium intake. The magnitude of the difference in serum PTH according to 25(OH)D quartiles was not influenced by calcium intake. A threshold of serum 25(OH)D around 30 ng/ml was observed for serum PTH and hip BMD. CONCLUSIONS: Vitamin D insufficiency is very common among Spanish community-dwelling adult subjects. A threshold of serum 25(OH)D around 30 ng/ml would be necessary for the prevention of secondary hyperparathyroidism and hip bone loss in our population, regardless of the dairy calcium ingestion. Programs to improve vitamin D status may be required in our country.

[Gynecological abnormalities in patients with rectovestibular fistula]. / Malformaciones ginecológicas en pacientes con fístula recto vestibular.

Patients with recto vestibular fistula may have gynecological malformations that could be unnoticed at the initial examination. The aim of this paper is to demonstrate the incidence of these malformations and propose a study method to help diagnose these malformations, avoiding unnecessary surgeries. We reviewed the records of patients treated with rectovaginal fistula (RVF) in the last 18 years and studied their gynecological malformations, time at diagnosis and treatment received. Of the 39 patients treated, 5 of them (13.1%) demonstrated 9 gynecological malformations: Hemivaginas (2), hemiuteros (2), uterine agenesis (2), vaginal agenesis (2) and vaginal septum (1). The diagnosis was made after the posterior sagittal approach (PSA) in two patients (acute abdomen and hydrometrocolpos), during the PSA in 2 patients and only one of them was diagnosed before the PSA. The 2 patients with hemivaginas and hemiuterus underwent a hemihysterosalpinguectomy and a vaginoplasty later in adolescence. The patient with vaginal and uterine agenesis diagnosed prior to PSA underwent a posterior sagittal anorectoplasty and a vaginoplasty with colon in the same procedure. The patient with vaginal and uterine agenesis (age 13 months) is waiting for vaginal replacement. The patient with vaginal septum (intraoperative finding) underwent a septum resection during the PSA. Gynecological defects are part of RVF spectrum. Girls with RVF require a complete gynecological examination prior to the definitive repair. Preoperative examinations assist in the timing and type of repair, and ultimately avoids complications and unnecessary interventions.

La fístula recto-vestibular se puede asociar a anomalías ginecológicas que, en numerosas ocasiones, pasan inadvertidas en la exploración inicial. Su reconocimiento en el momento adecuado puede cambiar el plan terapéutico y el pronóstico. Se revisan las historias de las pacientes con fístula recto-vestibular de los últimos 18 años y se describen las malformaciones ginecológicas asociadas, el momento del diagnóstico, el tratamiento y resultado postoperatorio. De 39 pacientes, en 5 (12,8%) se observaron 12 malformaciones ginecológicas: hemivaginas (2), hemiúteros (2), agenesia uterina (2), agenesia vaginal (2) y tabique vaginal (3). En dos pacientes el diagnóstico se realizó durante la anorrectoplastia, en otras dos después de la anorrectoplastia sagital posterior a causa de hidrometrocolpos y solo en una de ellas, antes de la intervención. En las pacientes con hemivagina y hemiútero se realizó, en una, la extirpación del hemiútero y la trompa y, en la otra, plastia vaginal transformándola en una única vagina. Cuando el diagnóstico se hizo antes de la intervención se pudo planificar la plastia adecuadamente. En la paciente con tabique vaginal y hallazgo intraoperatorio la anomalía genital fue tratada durante la anorrectoplastia. La paciente con agenesia vaginal y uterina aún no ha sido intervenida. Las malformaciones ginecológicas se asocian con relativa frecuencia a la fístula recto-vestibular. Por ello se requiere una exploración ginecológica adecuada antes de la anorrectoplastia para poder planificar de manera correcta el momento de la reparación, evitando complicaciones e intervenciones innecesarias. intraútero, la invaginación intestinal postnatal en el prematuro y la invaginación intestinal postnatal en el neonato a término.

Reliability of radiologic evaluation of abdominal aortic calcification using the 24-point scale.

OBJECTIVE: Calcification of the abdominal aorta is associated with increased cardiovascular morbidity, so a reliable method to quantify it is clinically transcendent. The 24-point scale (AAC-24) is the standard method for assessing abdominal aortic calcification on lateral plain films of the lumbar spine. The aim of this study was to determine the intraobserver and interobserver agreements for the AAC-24, taking into account the heterogeneity of the distribution of the calcifications in the design of the statistical analysis. MATERIAL AND METHODS: We analyzed the intraobserver agreement (in plain films from 81 patients, with a four-year separation between observations) and the interobserver agreement (in plain films from 100 patients, with three observers), using both intraclass correlation and Bland-Altman plots. RESULTS: The intraobserver intraclass correlation coefficient was 0.93 (95% confidence interval [CI95%]: 0.6-0.9), and the interobserver intraclass correlation coefficient was 0.91 (CI95%: 0.8-0.9) with an increase in the coefficient in the tercile with the greatest discrepancy. The difference in means ranged from 0.3 to 1.2 points, and the distance between the limits of agreement ranged from 4.7 to 9.4 points. These differences increased significantly as the calcification progressed. CONCLUSIONS: Using the AAC-24 on lateral plain films of the lumbar spine is a reliable and reproducible method of assessing calcification of the abdominal aorta; both intraobserver and interobserver agreement are higher during the initial phases of calcification.

Serum uric acid is associated with quantitative ultrasound parameters in men: data from the Camargo cohort.

UNLABELLED: This study analyzes the association between serum uric acid levels and heel quantitative ultrasound (QUS) parameters in men aged 50 or more from the Camargo cohort. We found that higher serum uric acid levels are positively associated with all QUS measurements, suggesting a better bone quality in men with elevated serum uric acid values. INTRODUCTION: Higher serum uric acid concentrations have been associated with higher bone mineral density and lower prevalence of fractures. However, there are no studies that have assessed the bone quality properties in Caucasians. Therefore, we have analyzed the association between quantitative ultrasound (QUS) and serum uric acid levels in adult men from a population-based cohort. METHODS: A total of 868 men older than 50 were recruited from a larger cohort (Camargo Cohort) after excluding those with any known condition or drug treatment with a possible influence on bone metabolism, or those with a previous diagnosis of gout or taking hipouricemic agents. Bone turnover markers (PINP and CTX), 25OH-vitamin D and PTH levels were measured by electrochemiluminiscence. BMD was determined by DXA, and heel QUS with a gel-coupled device. RESULTS: Lumbar, femoral neck and total hip BMD was significantly higher in men with higher serum uric acid levels. QUS parameters were also significantly higher in men with high uric acid levels than those with lower values, and increased continuously across quartiles after adjustment for confounding variables. In multiple regression analysis, serum uric acid was significantly associated with all QUS parameters. Finally, men with serum acid levels above median showed higher values in all the QUS parameters than men with lower values. CONCLUSIONS: Higher serum uric acid levels in men older than 50 years are positively associated with QUS parameters. These data might suggest a better bone quality in men with elevated serum uric acid values.

Cutaneous vasculitis associated with severe bacterial infections. A study of 27 patients from a series of 766 cutaneous vasculitis.

OBJECTIVES: To assess the clinical spectrum of severe bacterial infections presenting as cutaneous vasculitis (CV) in a defined population. METHODS: Unselected series of 766 patients with CV diagnosed at a single university referral center. RESULTS: An underlying severe bacterial infection was diagnosed in 27 (22 men/5 women; mean age ± standard deviation [SD]: 53 ± 18 years) of 766 cases presenting with CV (3.5%). These infections were: pneumonia (n=8), endocarditis (n=6), meningitis (n=4), intra-abdominal infections (n=3), septic arthritis (n=2), septicaemia (n=2), septic bursitis (n=1), and urinary tract infection (n=1). All the patients were admitted for suspected CV. The median delay from admission to the diagnosis of infection was 4 days. A typical palpable purpura without relevant visceral vasculitic involvement was the main clinical manifestation. Patients with severe bacterial infections were older, with male predominance, had more frequently fever, constitutional symptoms, focal infectious features, and leukocytosis with left shift and anaemia than the remaining patients with CV. Although antibiotics were prescribed in all the patients, seven also required the use of low-dose corticosteroids to achieve complete resolution of the cutaneous lesions. Most patients experienced full recovery but two of them underwent prosthetic cardiac valve replacement, and another two died due to infection-related complications. CONCLUSIONS: CV may be the presenting manifestation of a severe underlying bacterial infection. Physicians should keep in mind this fact to make an early diagnosis of infection and, consequently, prevent life-threatening complications.