Plasma C24:0- and C26:0-lysophosphatidylcholines are reliable biomarkers for the diagnosis of peroxisomal ß-oxidation disorders.

The gold-standard diagnostic test for peroxisomal disorders (PDs) is plasma concentration analysis of very long-chain fatty acids (VLCFAs). However, this method's time-consuming nature and limitations in cases which present normal VLCFA levels necessitates alternative approaches. The analysis of C26:0-lysophosphatydylcholine (C26:0-LPC) in dried blood spot samples by tandem-mass spectrometry (MS/MS) has successfully been implemented in certain newborn screening programs to diagnose X-linked adrenoleukodystrophy (ALD). However, the diagnostic potential of very long-chain LPCs concentrations in plasma remains poorly understood. This study sought to evaluate the diagnostic performance of C26:0-LPC and other very long-chain LPCs, comparing them to VLCFA analysis in plasma. The study, which included 330 individuals affected by a peroxisomal ß-oxidation deficiency and 407 control individuals, revealed that C26:0- and C24:0-LPC concentrations demonstrated the highest diagnostic accuracy (98.8% and 98.4%, respectively), outperforming VLCFA when C26:0/C22:0 and C24:0/C22:0 ratios were combined (98.1%). Combining C24:0- and C26:0-LPC gave the highest sensitivity (99.7%), with ALD females exhibiting notably higher sensitivity compared with the VLCFA ratio combination (98.7% vs. 93.5%, respectively). In contrast, C22:0-LPC exhibited suboptimal performance, primarily due to its low sensitivity (75%), but we identified a potential use to help distinguish between ALD and Zellweger spectrum disorders. In summary, MS/MS analysis of plasma C24:0- and C26:0-LPC concentrations represents a rapid and straightforward approach to diagnose PDs, demonstrating superior diagnostic accuracy, particularly in ALD females, compared with conventional VLCFA biomarkers. We strongly recommend integrating very-long chain LPC plasma analysis in the diagnostic evaluation of individuals suspected of having a PD.

Pulse Wave Velocity and Blood Pressure Variability as Prognostic Indicators in Very Elderly Patients.

There is scarce evidence for the prognostic importance of hemodynamic measures, such as blood pressure (BP), BP variability, and arterial stiffness, in the very elderly population with advanced chronic conditions. We aimed to evaluate the prognostic importance of 24 h BP, BP variability, and arterial stiffness in a cohort of very elderly patients admitted to the hospital due to a decompensated chronic disease. We studied 249 patients older than 80 (66% women; 60% congestive heart failure). Noninvasive 24 h monitoring was used to determine 24 h brachial and central BP, BP and heart rate variabilities, aortic pulse wave velocity, and BP variability ratios during admission. The primary outcome was 1-year mortality. Aortic pulse wave velocity (3.3 times for each SD increase) and BP variability ratio (31% for each SD increase) were associated with 1-year mortality, after adjustments for clinical confounders. Increased systolic BP variability (38% increase for each SD change) and reduced heart rate variability (32% increase for each SD change) also predicted 1-year mortality. In conclusion, increased aortic stiffness and BP and heart rate variabilities predict 1-year mortality in very elderly patients with decompensated chronic conditions. Measurements of such estimates could be useful in the prognostic evaluation of this specific population.

CSF neopterin and beta-2-microglobulin as inflammation biomarkers in newborns with hypoxic-ischemic encephalopathy.

BACKGROUND: Inflammation plays a crucial role in the pathogenesis of hypoxic-ischemic encephalopathy (HIE). The aim of this study was to measure inflammation in HIE through an analysis of CSF neopterin and ß2-microglobulin and to study the association with brain injury as shown by MRI findings and neurodevelopmental outcomes. METHODS: CSF biomarkers were measured in study patients at 12 and 72 h. Brain injury was evaluated by MRI, and neurodevelopmental outcomes were assessed at 2-3 years of life. An adverse outcome was defined as the presence of motor or cognitive impairment. RESULTS: Sixty-nine HIE infants were included. Median values of neopterin and ß2-microglobulin paralleled the severity of HIE. Adverse outcomes were associated with early neopterin and ß2-microglobulin values, late neopterin values, and the neopterin percentage change between the two samples. A cutoff value of 75% neopterin change predicted adverse outcomes with a specificity of 0.9 and a sensitivity of 0.75. CONCLUSIONS: CSF neopterin and ß2-microglobulin are elevated in HIE, indicating the activation of inflammation processes. Infants with adverse neurodevelopmental outcomes show higher levels of CSF neopterin and ß2-microglobulin. The evolution of neopterin levels provides a better predictive capacity than a single determination. IMPACT: Brain inflammation in newborns with HIE could be measurable through the analysis of CSF neopterin and ß2-microglobulin, both of which are associated with neurodevelopmental outcomes. Our study introduces two inflammatory biomarkers for infants with HIE that seem to show a more stable profile and are easier to interpret than cytokines. CSF neopterin and ß2-m may become clinical tools to monitor inflammation in HIE and might eventually be helpful in measuring the response to emerging therapies.

Efecto de una intervención educativa repetida frente a una intervención inicial sobre el control de la presión arterial en pacientes hipertensos / Effect of a repeated educational intervention versus an initial intervention on blood pressure control in hypertensive patients

Introducción y objetivos:La hipertensión es uno de los principales factores de riesgo cardiovascular. Una intervención educativa, destinada a aumentar o reforzar los conocimientos del paciente, puede contribuir a un mejor control de su presión arterial. Nuestro objetivo fue evaluar la eficacia de una educación terapéutica continuada frente a una intervención inicial en hipertensos.Métodos:Estudio experimental, de intervención comunitaria multicéntrica, mediante la aplicación de un programa de educación terapéutica en hipertensión arterial (HTA).Resultados:Se incluyeron 980 pacientes en tratamiento antihipertensivo, 490 en cada grupo, con edad media de 65 años, y un 50% mujeres siendo la presión arterial sistólica (PAS) media inicial en ambos grupos de 136 mmHg. Respecto a los conocimientos sobre la hipertensión, aunque se observó una mejoría inicial similar en ambos grupos, al finalizar el estudio, en el grupo de intervención continuado (GIC) se observó una media de 23,06 respuestas acertadas, mientras que en el grupo de intervención inicial (GII) la media fue de 20,91, siendo la diferencia estadísticamente significativa (p < 0,001). Al finalizar el estudio la cifra media de PAS fue de 127,5 mmHg en el GIC y de136.57 mmHg en el grupo GII. Asimismo, se observó un menor porcentaje de pacientes con IMC ≥ 25 en el GIC (78,2%) que en le GII 82,6%, siendo esta diferencia estadísticamente significativa (p < 0,001). Este mismo efecto se observó sobre el perímetro abdominal, observándose en el GIC un perímetro abdominal aumentado en un 46,3% frente a un 68,8% en el GII (p < 0,001).Conclusión:Una intervención educativa continuada aumenta el grado de conocimiento sobre la hipertensión respecto a una intervención inicial. Asimismo, esta intervención se asocia a una mejoría en las cifras de presión arterial, peso, índice de masa corporal y perímetro abdominal. (AU)

Introduction and objectives:Hypertension is one of the main cardiovascular risk factors. An educational intervention, aimed at increasing or reinforcing the patient's knowledge, can contribute to better control of their blood pressure. Our goal was to evaluate the efficacy of a continuing therapeutic education versus an initial intervention in hypertensive patients.Methods:Experimental study of multicenter community intervention, through the application of a therapeutic education program in hypertension.Results:980 patients on antihypertensive treatment were included, 490 in each group, with a mean age of 65 years, and 50% women, baseline mean SBP in both groups was136 mmHg. Regarding knowledge about hypertension, although a similar initial improvement was observed in both groups, at the end of the study, in the continuous intervention group (CIG) an average of 23.06 correct answers was observed, while in the Initial intervention group (IIG) the mean was 20.91, being the difference statistically significant (p < 0.001). At the end of the study, the mean SBP was 127.5 mmHg in the CIG and 136.57 mmHg in the IIG group. Likewise, a lower percentage of patients with a BMI ≥25 was observed in the CIG (78.2%) than in the IIG 82.6%, this difference being statistically significant (p < 0.001). This same effect was observed on the abdominal perimeter, observing an increased abdominal perimeter in 46.3% of the CIG compared to 68.8% in the IIG (p < 0.001).Conclusión:A continuous educational intervention increases the degree of knowledge about hypertension compared to an initial intervention. Likewise, this intervention is associated with an improvement in blood pressure, weight, body mass index and abdominal girth figures. (AU)

The physiology of hemostasis.

The breakdown of vascular integrity triggers a series of reactions, initially involving a vasoconstriction phenomenon that reduces blood loss and leads to platelet plug formation under changing flow conditions. Once the platelet plug has formed, the coagulation system is activated in order to allow the formation of fibrin, which anchors the platelet plug to the vessel breach. Finally, once the damage has resolved, the fibrin is eliminated through fibrinolysis. This article explains all these regulatory mechanisms.

The Role of Arterial Stiffness in the Estimation of Cardiovascular Risk in Liver Transplant Recipients.

BACKGROUND: Long-term cardiovascular (CV) events are a frequent cause of death and disability after liver transplant (LT). Although a more in-depth, risk-adapted control of CV risk factors may result in improved post-LT CV outcomes, an accurate stratification of the CV risk of LT recipients to better implement preventive strategies is lacking. Aortic pulse wave velocity (aPWV) is a surrogate of arterial stiffness that has been suggested as a biomarker of CV risk; it has never been evaluated in adult LT recipients. METHODS: In a single-center prospective study, we included 122 LT recipients at 12 (n = 39), 60 (n = 45), or 120 (n = 38) mo after LT. aPWV estimation by oscillometry, clinical assessment of CV risk factors, and CV risk estimation by standard clinical scores (systematic coronary risk evaluation and pooled cohort equation) were performed. The incidence of CV events during prospective follow-up was registered. RESULTS: aPWV was independently associated with age and the grade of control of blood pressure. After a median follow-up of 35 mo, 15 patients (12%) presented a CV event. Higher aPWV, diabetes, past or present smoking habit, previous CV events, lower eGFR, being in systematic coronary risk evaluation or pooled cohort equation high-risk groups, and higher levels of total cholesterol, LDL-cholesterol, creatinine, and triglycerides were associated with the incidence of CV events at univariate analysis; aPWV, past or present smoking habit, and triglycerides were independent predictors of CV events. CONCLUSIONS: According to our results, aPWV mirrors CV risk in LT recipients and thus may be a useful CV risk biomarker in this population. Considering these preliminary results, its accuracy in stratifying risk requires confirmation in further studies.

Toward an improved assessment of quality of life in endometriosis: evaluation of the Spanish version of the Endometriosis Health Profile 30.

PURPOSE: Our main objective is to evaluate the psychometric properties of the Spanish version of the EHP-30 questionnaire. The secondary aim is to evaluate the differences in the scores of the core EHP-30 scales between patients with either surgical treatment or conservative management of endometriosis. METHODS: Cross-sectional study conducted into a tertiary hospital endometriosis reference unit. All patients (n = 223) pre-surgically completed the core EHP-30 questionnaire, the EQ-5D questionnaire (n = 184) and a visual analogue scale (n = 210) for endometriosis-related pain. Demographical and clinical data were recorded. RESULTS: Psychometric characteristics of the Spanish core EHP-30 questionnaire were investigated. Statistical analyses confirmed the five-structure factor, a high degree of internal consistency and of item-total correlation for all the assessed items. Convergent validity between EQ-5D and EHP-30 items and between VAS and EHP-30 subscale pain was observed. Additionally, patients with surgical management rendered significantly higher scores in the core EHP-30 subscales "pain" and "control and powerlessness". CONCLUSIONS: We present the reliability, validity and acceptability of the Spanish core EHP-30 questionnaire, providing clinicians and researchers with an improved tool to assess the endometriosis-related quality of life. Additionally, we show that patients subsidiaries of surgical treatment for endometriosis present with higher pain and powerlessness than those with conservative management.

[Effect of a repeated educational intervention versus an initial intervention on blood pressure control in hypertensive patients]. / Efecto de una intervención educativa repetida frente a una intervención inicial sobre el control de la presión arterial en pacientes hipertensos.

INTRODUCTION AND OBJECTIVES: Hypertension is one of the main cardiovascular risk factors. An educational intervention, aimed at increasing or reinforcing the patient's knowledge, can contribute to better control of their blood pressure. Our goal was to evaluate the efficacy of a continuing therapeutic education versus an initial intervention in hypertensive patients. METHODS: Experimental study of multicenter community intervention, through the application of a therapeutic education program in hypertension. RESULTS: 980 patients on antihypertensive treatment were included, 490 in each group, with a mean age of 65 years, and 50% women, baseline mean SBP in both groups was136 mmHg. Regarding knowledge about hypertension, although a similar initial improvement was observed in both groups, at the end of the study, in the continuous intervention group (CIG) an average of 23.06 correct answers was observed, while in the Initial intervention group (IIG) the mean was 20.91, being the difference statistically significant (p < 0.001). At the end of the study, the mean SBP was 127.5 mmHg in the CIG and 136.57 mmHg in the IIG group. Likewise, a lower percentage of patients with a BMI ≥25 was observed in the CIG (78.2%) than in the IIG 82.6%, this difference being statistically significant (p < 0.001). This same effect was observed on the abdominal perimeter, observing an increased abdominal perimeter in 46.3% of the CIG compared to 68.8% in the IIG (p < 0.001). CONCLUSIóN: A continuous educational intervention increases the degree of knowledge about hypertension compared to an initial intervention. Likewise, this intervention is associated with an improvement in blood pressure, weight, body mass index and abdominal girth figures.

Is there sufficient evidence to justify changes in dietary habits in heart failure patients? A systematic review.

The incidence and prevalence of heart failure (HF) is increasing worldwide, leading to high morbidity and mortality. The global management of HF involves lifestyle changes in addition to pharmacological treatments. Changes include exercise and dietary recommendations, mainly salt and fluid restriction, but without any clear evidence. We conducted a systematic review to analyse the degree of evidence for these dietary recommendations in HF. Only randomized controlled trials (RCT), and observational studies in humans were selected. Studies were considered eligible if they included participants with HF and sodium and/or fluid restriction. Publications in languages other than English or Spanish were excluded. We included 15 studies related to sodium or fluid restriction. Nine RCT and six observational studies showed some improvements in symptoms and quality of life and a degree of reduction in new hospitalizations, but the results are based on limited population groups, applying different methodologies, and with different restriction goals. We found a lack of clear evidence of the benefits of sodium/fluid restriction in chronic HF. The evidence is limited to few studies with conflicting results. Randomized clinical trials are needed to fill this gap in our knowledge.

Diagnostic yield of capillary compared to venous glucose in the diagnosis of hypoglycorrhachia in children: A prospective, observational study.

INTRODUCTION: The ratio of cerebrospinal fluid (CSF) glucose and blood glucose is of major relevance, conducting to the diagnosis of hypoglycorrhachia, which is a sign of neuroinfection, as well as a number of neurological diseases of genetic or neoplastic etiology. Glucose in capillary sample (glucometry) is a low cost, readily available technique, as compared to venous glucose. This study aims to compare glucometry to venous glucose in the diagnosis of hypoglycorrhachia in pediatric population. METHODS: Prospective cross-sectional study based on data obtained from lumbar punctures in the period from February 2017 to January 2019 in a specialized pediatric institution in Colombia. RESULTS: 97 patients were analyzed, aged 1 month to 17 years old, mean 7.67 years, 52 (53.61%) were female. 26 (26.8%) were diagnosed with hypoglycorrhachia. Pearson correlation coefficient for absolute venous and capillary glucose was 0.54, and 0.55 for the ratios of CSF glucose/venous glucose and CSF glucose/glucometry, which support a linear correlation between the variables in both, absolute values and ratios. Intraclass correlation coefficient was calculated for both, the venous glucose and glucometry ratios, which was 0.52, revealing a moderate agreement among the tests. Sensitivity and specificity of CSF glucose/glucometry, as compared to gold standard are 73.1% and 60.6% respectively; whereas predictive positive value (PPV) and negative predictive value (NPV), were 40.4% and 86.0%. CONCLUSION: Glucometry cannot replace the glucose in venous sample in the diagnosis of hypoglycorrhachia in children.

Routine assessment of cognitive function in older patients with hypertension seen by primary care physicians: why and how-a decision-making support from the working group on 'hypertension and the brain' of the European Society of Hypertension and from the European Geriatric Medicine Society.

: The guidelines on hypertension recently published by the European Societies of Hypertension and Cardiology, have acknowledged cognitive function (and its decline) as a hypertension-mediated organ damage. In fact, brain damage can be the only hypertension-mediated organ damage in more than 30% of hypertensive patients, evolving undetected for several years if not appropriately screened; as long as undetected it cannot provide either corrective measures, nor adequate risk stratification of the hypertensive patient.The medical community dealing with older hypertensive patients should have a simple and pragmatic approach to early identify and precisely treat these patients. Both hypertension and cognitive decline are undeniably growing pandemics in developed or epidemiologically transitioning societies. Furthermore, there is a clear-cut connection between exposure to the increased blood pressure and development of cognitive decline.Therefore, a group of experts in the field from the European Society of Hypertension and from the European Geriatric Medicine Society gathered together to answer practical clinical questions that often face the physician when dealing with their hypertensive patients in a routine clinical practice. They elaborated a decision-making approach to help standardize such clinical evaluation.

COVID-19 in chronic hemodialysis patients: A report of 9 cases in Madrid, Spain.

INTRODUCTION: In December 2019, a new respiratory disease, named COVID-19, caused by a novel coronavirus, emerged in Wuhan and since then spread rapidly throughout China and worldwide. Hemodialysis patients are an especially vulnerable subgroup of the general population. However, there are only a few reports on the course of COVID-19 in maintenance hemodialysis patients. CASE REPORT: We describe in depth the clinical, analytical, and radiological details of 9 hemodialysis patients from a single center in Madrid (Spain) diagnosed with COVID-19. Furthermore, we describe and discuss the therapeutic aspects and the patients' outcomes. DISCUSSION: We present one of the first case series of chronic hemodialysis patients with COVID-19. Between March 14 and April 8, 2020, out of 76 prevalent patients in our hemodialysis unit, 9 (11.8%) patients were diagnosed with COVID-19. The most common symptoms were fever (77.8%), asthenia (77.8%), cough (55.6%), and dyspnea (33.3%). A total of 3 patients developed severe pneumonia, whereas 4 patients developed mild pneumonia. In 2 patients, no pathologic changes were found on chest radiography. All patients presented lymphopenia. While 6 (66.7%) patients required hospitalization, none of them was admitted to intensive care unit. The most common treatments used were azithromycin (100%), hydroxychloroquine (66.7%), lopinavir/ritonavir (55.6%) and ß-interferon (22.2%). In general, we observed a mild to moderate severity of disease in our case series. One patient died, however due to a cause not related to COVID-19.

A regular wide QRS complex tachycardia with fusion beats?

A case of wide complex tachycardia with isolated QRS complexes of different amplitude suggesting that this was ventricular tachycardia.

Cerebrospinal fluid neopterin as a biomarker of neuroinflammatory diseases.

The elevation of neopterin in cerebrospinal fluid (CSF) has been reported in several neuroinflammatory disorders. However, it is not expected that neopterin alone can discriminate among different neuroinflammatory etiologies. We conducted an observational retrospective and case-control study to analyze the CSF biomarkers neopterin, total proteins, and leukocytes in a large cohort of pediatric patients with neuroinflammatory disorders. CSF samples from 277 subjects were included and classified into four groups: Viral meningoencephalitis, bacterial meningitis, acquired immune-mediated disorders, and patients with no-immune diseases (control group). CSF neopterin was analyzed with high-performance liquid chromatography. Microbiological diagnosis included bacterial CSF cultures and several specific real-time polymerase chain reactions. Molecular testing for multiple respiratory pathogens was also included. Antibodies against neuronal and glial proteins were tested. Canonical discriminant analysis of the three biomarkers was conducted to establish the best discriminant functions for the classification of the different clinical groups. Model validation was done by biomarker analyses in a new cohort of 95 pediatric patients. CSF neopterin displayed the highest values in the viral and bacterial infection groups. By applying canonical discriminant analysis, it was possible to classify the patients into the different groups. Validation analyses displayed good results for neuropediatric patients with no-immune diseases and for viral meningitis patients, followed by the other groups. This study provides initial evidence of a more efficient approach to promote the timely classification of patients with viral and bacterial infections and acquired autoimmune disorders. Through canonical equations, we have validated a new tool that aids in the early and differential diagnosis of these neuroinflammatory conditions.

Isolated palpitations and ventricular pre-excitation.

A 27-year-old male was referred for further assessment after being evaluated by his general practitioner for isolated palpitations. A twelve-lead electrocardiogram was performed in which sinus rhythm with ventricular pre-excitation were observed. Electrophysiologic study demonstrated the presence of a fasciculoventricular accessory pathway.

Grado de conocimiento de la hipertensión en pacientes hipertensos / Grade of knowledge of hypertension in hypertensive patients

Objetivo: Evaluar el grado de conocimientos de la hipertensión en pacientes hipertensos. Método: Estudio multicéntrico descriptivo transversal, realizado en centros de atención primaria y especializada de España. Participaron 980 pacientes hipertensos, mayores de 18 años, con tratamiento farmacológico. Variables principales: años de diagnóstico, factores de riesgo, presión arterial, perímetro abdominal, índice de masa corporal, adherencia a la medicación (test de Morisky-Green), y los ítems de un cuestionario validado sobre conocimientos. Resultados: El 50% mujeres, edad media 65 años. El 46% con más de 10 años del diagnóstico de hipertensión. En relación a la adherencia a la medicación, el 56,6% no lo era, siendo el olvido la primera causa. Al analizar los conocimientos sobre la hipertensión el 41,7% refería que nadie les había informado, el 35,8% no consideraba que la hipertensión fuera para toda la vida y solo el 42,3% conocía los objetivos de presión arterial. El mayor desconocimiento sobre los riesgos que provoca la hipertensión en el organismo fue el relacionado con el riñón, donde solo el 48,6% afirmaba conocer ese dato. En relación a la medicación, solo el 41,4% conocía que era para toda la vida. También se observó que hay diferencias estadísticamente significativas entre nivel de conocimientos y nivel de estudios (p < 0,001). Conclusión: Los conocimientos básicos sobre la hipertensión siguen siendo bajos en estos pacientes. Estos resultados permiten orientar futuras intervenciones. orientando la educación terapéutica al paciente y mejorar su participación en la enfermedad para controlar mejor su hipertensión

Aims: To evaluate the grade of knowledge of hypertension in hypertensive patients. Method: Cross-sectional descriptive multicentre study. Primary and specialized care centres in Spain. Participants: 980 hypertensive patients, older than 18 years, with pharmacological treatment. Main variables: Years of diagnosis, risk factors, blood pressure, abdominal circumference, body mass index, adherence to medication (Morisky-Green test) and the items of a validated questionnaire on knowledge. Results: 50% were women, mean age 65 years. Seventy-eight percent lived with family, 46% over 10 years old diagnosed with hypertension. In relation to adherence to medication, 56.6% were not compliant, forgetting being the first cause. Knowledge about hypertension 41.7% said that no one had explained it, 35.8% did not consider hypertension a lifelong process, and only 42.3% knew the objectives of blood pressure. The area of major lack of knowledge of the risk of hypertension was the problems caused by hypertension in the kidney, only 48.6% answered affirmatively. In relation to medication, only 41.4% knew it is for life. Statistically significant differences were also observed between the level of knowledge and the level of studies (p < .001). Conclusions: Basic knowledge about hypertension remains low in these patients. These results enable future interventions to be oriented by promoting therapeutic education to the patient and improving their involvement in the disease in order to better control their hypertension