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Introduction: Transthyretin-related amyloidosis (ATTRv) is a progressive multisystem disorder, predominantly involving the peripheral nerve system (PNS) and heart. Quantification of small fiber damage may help guide treatment decisions, as amyloid deposits frequently affect those fibers early in disease course. Corneal confocal microscopy (CCM) is a promising method to monitor patients with ATTRv, due to similarities between corneal nerves and PNS, as the cornea is innervated by Aδ and C fibers., Methods: We compared CCM measures from ATTRv patients to a group of healthy individuals, matched by age and gender. We then investigated the correlations between small fiber tests (SFT): CCM, LDI-Flare and CDT, COMPASS-31 and disability scales (RODS and ONLS) in patients., Results: Of 20 patients (6 with V30M), mean age 50.3±15.3Y, 7 female (35%), six (30%) had polyneuropathy and 10 (50%) carpal tunnel syndrome. CDT was abnormal in 9 and LDI-flare in 6 patients. CCM was abnormal in 19 tested patients and significantly reduced when compared to controls (CNFL: 6.31±0.31 vs. 15.21±1.02mm/mm 2 , p<0.001). Mean COMPASS-31-scores were 22.27±22.84; RODS and ONLS were 38.15±12.33 and 2.05±2.3, with no significant differences between sub-group scores. Disease duration was significantly correlated with ONLS (0.43, p=0.05) and RODS (0.46, p=0.03). There were no significant correlations between measures of disability and SFT., Conclusions: In a diverse cohort of ATTRv patients, CCM was the most frequent abnormal measurement. CCM can be a useful test to triage patients in the early disease stages and with few or equivocal symptoms., (Copyright © 2024 The Authors. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Optical coherence tomography (OCT) has become an essential tool in paediatric ophthalmology. However, none of the currently available OCT devices include any kind of normative database for the paediatric population, which can lead to important interpretation errors., Objective: To review the paediatric OCT normative databases., Material and Methods: The applications and implications of the use of paediatric OCT normative databases are reviewed., Results: The paediatric normative databases that have been published so far in scientific literature with different OCT devices for Spanish and European population are presented., Conclusions: The knowledge and interpretation of paediatric OCT normative databases in our daily clinical practice is crucial in order for the correct interpretation of OCT thickness maps., (Copyright © 2019 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background and Objective: Atherogenic dyslipidemia, which is characterized by increased triglyceride levels and reduced HDL cholesterol levels, is underestimated and undertreated in clinical practice. We assessed its prevalence and the achievement of therapeutic objectives for HDL cholesterol and triglyceride levels in patients treated at lipid and vascular risk units in Spain., Patients and Method: This was an observational, longitudinal, retrospective, multicenter study performed in 14 autonomous Spanish communities that consecutively included 1828 patients aged ≥18 years who were referred for dyslipidemia and vascular risk to 43 lipid clinics accredited by the Spanish Society of Arteriosclerosis. We collected information from the medical records corresponding to 2 visits conducted during 2010 and 2011-12, respectively., Results: Of the 1649 patients who had a lipid profile in the first visit (90.2%), 295 (17.9%) had atherogenic dyslipidemia. The factors associated with atherogenic dyslipidemia were excess weight/obesity, not taking hypolipidemic drugs (statins and/or fibrates), diabetes, myocardial infarction and previous heart failure. Of the 273 (92.5%) patients with atherogenic dyslipidemia that had a lipid profile in the last visit, 44 (16.1%) achieved the therapeutic objectives for HDL cholesterol and triglyceride levels. The predictors of therapeutic success were normal weight and normoglycemia., Conclusion: One of every 6 patients treated in lipid and vascular risk units had atherogenic dyslipidemia. The degree to which the therapeutic goals for HDL cholesterol and triglyceride levels were achieved in these patients was very low., (Copyright © 2014 Elsevier España, S.L.U. All rights reserved.)

Objective: To describe the demographic and clinical profiles of a cohort of environmentally representative severe traumatic brain injury (TBI) cases collected for the past 25 years and to analyse the changes that occurred by dividing the analysis period into 3 equal time periods., Material and Methods: This was an observational cohort study of consecutive adult patients (>14 years of age) with severe closed TBI (Glasgow Coma Scale score [GCS]≤8) who were admitted during the first 48h after injury to the 12 de Octubre hospital from 1987 to 2012. The most relevant epidemiological and clinical variables reported in the literature were defined and compared in 3 equal time periods (1987-1995, 1996-2004 and 2005-2014)., Results: There was a 13% reduction in the frequency of severe TBI from the first to the last time period. An increase in the mean age from 35 to 43 years was observed, whereas the frequency of severe TBI according to sex remained approximately the same during the last decades of life. A distinct change was observed in the injury mechanism; traffic accidents decreased from 76% to 55%, particularly those involving 4-wheeled vehicles. However, falls increased significantly, especially in older women, and contusion and subdural haematoma were the most frequent structural injuries. Motor scores could not be reliably assessed for the last time period because of early intubation and sedative drug use., Conclusions: TBI epidemiology in Western countries has changed. This trend was also observed in our environment as an increase in mean age, which reflected the increase in falls among elderly patients., (Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.)

Severe head injury (SHI) is one of the most important health, social, and economic problems in industrialized countries. Most of the recent studies related to this entity still show pessimistic results, with percentages of mortality and unfavourable outcomes very similar than those reported in the last quarter of century. In order to make predictions for patients with SHI, different "prognostic formulas or models" reviewed in this manuscript, have been developed with the main objective of performing reliable predictions for patients with this pathology. These models are constructed by using a group of "prognostic indicators or factors" and different "prognostic scales" useful for measuring the final outcome. The different "statistical techniques or methods" necessary to develop these prognostic models are also analyzed in this paper.

During the past "Decade of the Brain" several neuroprotective agents have been tested in phase III clinical trials for severe head injury (SHI) but unfortunately none of them significantly improved the outcome of these patients. In contrast to the success achieved by these drugs in animal laboratory studies, the results in terms of neuroprotection in the clinical setting have been disappointing. This paper has been divided in three parts: in the first one, we summarize the pathophysiological mechanisms related to SHI, targeted by the neuroprotective agents. In the second part we review the main clinical trials carried out for SHI to date, and in the third one, we analyze the possible reasons that explain why these agents have failed to show efficacy.

Introduction: Gallbladder adenocarcinoma is an aggressive tumor and is one of the digestive tract malignancies with the poorest prognosis. Because of loco-regional extension and delayed diagnosis, curative resection is often impossible. To determine histological prognostic factors and survival in relation to tumoral stage at diagnosis, we performed a retrospective study of our patients with gallbladder carcinoma., Patients and Method: Sixty-two patients with gallbladder adenocarcinoma diagnosed over a 15-year period were retrospectively included in this study. The surgical procedures performed in this group of patients were laparoscopic cholecystectomy, open cholecystectomy and palliative surgery in patients with unresectable tumors. For each tumoral stage, age, sex, cellular differentiation, tumor size, the presence of metastatic nodes, histological variables linked to poor prognosis, and survival were compared., Results: Of the 62 patients included, 45 were women and 17 were men. The mean age was 75 years. No significant differences were found in relation to age or sex among the different tumoral stages. Cellular differentiation and survival were poorer with advanced tumoral stage. A significant predominance of histological factors of poor prognosis was found in T2 and T3 tumors., Conclusions: Preoperative diagnosis of gallbladder adenocarcinoma is difficult except in advanced cases. It is often incidentally diagnosed at histological examination of gallbladders, and shows little local advancement and a good degree of cellular differentiation. The etiology of this tumor is unknown but its prevalence is greater among women. Clinical symptoms are similar to those caused by gallstones. In this study no relationship was found between age and sex and tumoral stage. In advanced tumoral stages poor cellular differentiation is predominant as well as other histological markers of poor prognosis. Good survival was found in T3 tumors, possibly linked to good cellular differentiation. Due to high associated comorbidity, none of the patients underwent reintervention.

The knowledge of the so called prognostic factors or indicators involved in severe head injury (SHI) is an issue of great interest to make predictions about the future of patients with this pathology. Those indicators constitute the basic elements of the different prognostic formulas or models carried out in order to make predictions in SHI. The mentioned models, therefore, will be constructed by a group of variables (prognostic indicators or factors) and several scales (prognostic scales) that are useful for measuring the final outcome of these patients. In this paper we resume, after an exhaustive review of the literature, the knowledge about the prognostic factors related to SHI. These indicators have been classified as follows: clinical, radiological, physiological, and biochemical. Moreover, we have briefly described the prognostic scales more commonly used in SHI.

P53 protein expression, synonymous to p53 gene mutation, is a common event in head and neck squamous cell carcinoma (HNSCC). However, the correlation between p53 expression and clinico-pathologic parameters remains unclear. Primary tumors for analysis were obtained from 99 patients with HNSCC. The expression of p53 was analyzed with monoclonal D07 antibody (Dako Corp., Glostrup, Denmark). Seventy five percent of 79 valid cases expressed the protein. A correlation was found between alcohol intake and p53 expression. None of the other clinico-pathologic factors were associated to p53 expression. Our results suggest that p53 may be involved in the development of HNSCC. However its clinical significance is still not completely understood.

Background and Objectives: Little is known about the differences between children and adults in the results of head-up tilt test. This study sought to investigate the potential differences concerning: a) the clinical profile and circumstances of spontaneous syncope; b) the overall diagnostic performance of the test, and c) the type of positive response obtained., Material and Methods: We studied 31 children and 123 adults with unexplained syncope. If baseline test (tilting at 70 degrees for 30 min) resulted negative, it was repeated under isoprenaline low-dose infusion., Results: There were no differences in either clinical profile, except for severe traumatism more frequent in adults (25% vs. 3% in children; p < 0.05), or overall diagnostic performance (39% in children vs. 33% in adults; p = NS). However, the way the test rendered positive (via basal tilting in 92% of children vs. 50% in adults; p < 0.05) and the rate of cardioinhibitory response (42% in children vs 8% in adults; p < 0.01) were significantly different., Conclusions: In this study children, in contrast to adults, rarely have a positive response in tilting under isoprenaline infusion. They also present a much higher rate of cardio-inhibitory response than adults.

We report the case of an infant operated on at 11 months, having been clinically, echocardiographically and hemodynamically diagnosed when 2 days at the age. In the Echo-Doppler study the visualisation of the tunnel and the presence of aortic regurgitation within it and in the left ventricular outflow tract confirmed the diagnosis. Surgical access to both orifices, aortic and left ventricular, through the tunnel (after incision in the same) allowed it to be closed with two Goretex patches from its external face. This technique has a double advantage: a) it avoids annular distortions or aortic valvular lesions, b) excluding the tunnel (partial resection of edges and subsequent continuous suture) any possibility of subpulmonary stenosis through compression is eradicated.