Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY).

BACKGROUND: Our knowledge of hypertrophic cardiomyopathy (HCM) mainly originates from quarternary centres. The objective is to assess the current management of HCM patients in a large multicentre French register according to the level of expertise. METHODS AND RESULTS: A total of 1431 HCM patients were recruited across 26 (11 expert and 15 non-expert) centres in REMY, a prospective hospital-based register of adult HCM patients. A sarcomeric origin was suspected in 1284 (89.7%) patients [261 (20.3%) with a reported gene mutation, 242 (18.8%) genotype-negative], while 107 (7.5%) had a diagnosis of non-sarcomeric HCM. Patients managed in non-expert centres were older (P < 0.01) and presented more often with NYHA III/IV class dyspnoea (P < 0.01), congestive heart failure (P < 0.01), low LEVF (P < 0.01), less often with a syncope history (P < 0.01) and lower LV obstruction (P < 0.01) than patients in expert centres. Genotype positive sarcomeric aetiologies were less frequent in non-expert centres (P < 0.01). The use of diagnostic and prognostic tests as cardiac MRI (P < 0.001), genetic (P < 0.001) and alpha-galactosidase A enzyme level testing (P < 0.001), Holter ECG (P < 0.001), and exercise test (P < 0.001), was lower in non-expert centres. Septal ablation procedures using alcohol (P < 0.001) or myectomy (P < 0.001) were more frequent in expert centres. CONCLUSION: In real life practice, only a minority of HCM patients are identified as sarcomere positive as per genetic testing. The management of HCM patients varies according to the centre's level of expertise, with less access to diagnostic and prognostic tests in non-expert centres. Non-sarcomeric HCM may therefore be overlooked despite specific treatment in some aetiologies.

Differential Effect of Acute Iron Overload on Oxidative Status and Antioxidant Content in Regions of Rat Brain.

The hypothesis of this study is that the cerebral cortex, hippocampus, and striatum of the rat brain are differentially affected in terms of oxidative stress and antioxidant capacity by acute Fe overload because Fe is distributed in a heterogeneous fashion among different regions and cells of the brain. The effects on the lipophilic and hydrophilic cellular environment were compared between regions and with the whole brain. A single dose of Fe-dextran increased Fe deposits, reaching a maximum after 6 hr. Both in whole brain and in cortex region, the ascorbyl/ascorbate content ratio was increased after 6 hr of Fe administration, while in striatum and hippocampus, there was no significant changes after Fe overload. Total thiol content decreased in whole brain and cortex, while there were no significant changes in striatum and hippocampus after Fe overload. The content of α-tocopherol (α-T), whether measured in the whole brain or in the isolated regions, did not change following Fe treatment. Lipid radical (LR•) generation rate after Fe-dextran overload only increased in the cortex region. The LR•/α-T content ratio was increased by Fe treatment in cortex but not in the whole brain, striatum, or hippocampus, in agreement with the study tested hypothesis.

Hipertensión matinal y comportamiento non-dipper en embarazadas con hipertensión de guardapolvo blanco / Morning Hypertension and Non-dipper Behavior in Pregnant Women with White Coat Syndrome

Introducción: La hipertensión de guardapolvo blanco (HGB) es común en el embarazo, aunque su evolución clínica durante la gestación se conoce poco. La hipertensión matinal y el comportamiento non-dipper, medidos por monitoreo ambulatorio de la presión arterial (MAPA), se asocian con mayor riesgo de eventos cardiovasculares; no obstante ello, son escasos los estudios en la gestación. Objetivos: El objetivo primario del estudio fue evaluar la hipertensión matinal y el comportamiento non-dipper en embarazadas con HGB versus un grupo control de normotensas. Se planteó como objetivo secundario evaluar si la HGB, la hipertensión matinal y el comportamiento non-dipper en el segundo trimestre del embarazo se relacionan con mayor hipertensión sostenida en el tercer trimestre. Material y métodos: Estudio prospectivo en el que se incluyeron 95 primigestas en la semana 20 de gestación, 50 con HGB y 45 normotensas como grupo control. Se registraron laboratorio de rutina, presión de consultorio y MAPA en la inclusión y a las 32 semanas de gestación. La hipertensión matinal y el comportamiento non-dipper se evaluaron por MAPA. Resultados: La edad, la glucemia y la presión diurna y nocturna por MAPA fueron similares en el examen basal en ambos grupos. Por el contrario, las pacientes con HGB presentaron valores significativamente superiores de hipertensión matinal y comportamiento non-dipper, lo cual se asoció en forma independiente con hipertensión sostenida en el tercer trimestre del embarazo. Conclusiones: Las gestantes con HGB en la semana 20 de embarazo presentaron mayor hipertensión matinal y comportamiento non-dipper y evolucionaron con más frecuencia a hipertensión sostenida que el grupo control de normotensas.

Background: White coat syndrome (WCS) is common during pregnancy, although little is known about its clinical outcome during gestation. Morning hypertension and the non-dipper behavior, measured by ambulatory blood pressure monitoring (ABPM), are associated with greater risk of cardiovascular events. However, there are few studies during pregnancy. Objectives: The primary aim of the study was to evaluate morning hypertension and the non-dipper behavior in pregnant women with WCS versus a control normotensive group. A secondary objective was to evaluate whether WCS, morning hypertension and the non-dipper behavior in the second trimester of pregnancy were associated with greater hypertension in the third trimester. Methods: This prospective study included 95 primiparae in the 20th week of gestation, 50 with WCS and 45 as normotensive control group. Routine lab tests, office blood pressure and ABPM at inclusion and in the 32th week of gestation were recorded. Morning hypertension and the non-dipper behavior were evaluated by ABPM. Results: Age, and baseline blood glucose level and daytime and nighttime blood pressure by ABPM were similar in both groups. Conversely, patients presenting WCS had significantly higher values of morning hypertension and non-dipper behavior, which were independently associated with sustained hypertension in the third trimester of pregnancy. Conclusions: Pregnant women with WCS in the 20th week of gestation presented greater morning hypertension and non-dipper behavior and progressed more frequently to sustained hypertension than the control normotensive group.

Evolution and challenges in the design of computational systems for triage assistance.

Compared with expert systems for specific disease diagnosis, knowledge-based systems to assist decision making in triage usually try to cover a much wider domain but can use a smaller set of variables due to time restrictions, many of them subjective so that accurate models are difficult to build. In this paper, we first study criteria that most affect the performance of systems for triage assistance. Such criteria include whether principled approaches from machine learning can be used to increase accuracy and robustness and to represent uncertainty, whether data and model integration can be performed or whether temporal evolution can be modeled to implement retriage or represent medication responses. Following the most important criteria, we explore current systems and identify some missing features that, if added, may yield to more accurate triage systems.

Novel left ventricular assist systems I and II for cardiac recovery: the driver.

We have recently described the Novel Left Ventricular Assist Systems (Novel LVAS) I and II, which avoid cannulation of cardiac chambers and synchronize pumping with the patient's electrocardiogram. We now describe the drive system in more detail. The drive unit is an air-driven pulsatile system. The driver's parameters can be programmed. This electro-pneumatic unit contains 3 modules. A remarkable feature of the driver system is that it contains 2 pneumatic units that alternate in their function every 15 minutes. This prevents overheating and component fatigue or failure, and it enables the use of smaller units. If one of the units fails, an alarm will warn of the problem, and the other will continue indefinitely. This LVAS is synchronized with the patient's ECG, which enables it to eject the stroke volume during diastole and in this way to act as a chronic counterpulsator. We have designed the Novel LVAS to operate at a low-frequency rate. This fact, together with the electrocardiographic synchronization, offers the best prospect for myocardial recovery in patients who are also receiving beta-adrenergic blocking agents. This dual therapy will help adjust heart rate to pump frequency.

A modification of the staining technique of reticular fibres for image analysis of the cardiac collagen network.

INTRODUCTION: Silver stain of reticular fibres demonstrates the fine structure of the cardiac collagen network. However, nuclei are also stained with current techniques, a drawback that makes computer image analysis difficult. To solve this problem our study was designed to modify Gomori's method. Reactive concentrations and action times represent the core of that modification. Only stromal tissue is stained. The technique was tested for repeatability and reproducibility to assess the precision of the measurement procedure in the assessment of myocardial collagen in a consecutive series of myocardial samples from patients with and without heart muscle disease. In addition, we checked the reliability of the method by comparing our results with the point-counting method (PCM). METHODS: The right ventricular myocardium was fixed in 10% buffered formaldehyde and routinely processed. Paraffin sections (4 microm) were stained with several modifications of Gomori's method. Variable concentrations of potassium permanganate, silver solutions and different oxidation times were tried. A field from each sample was digitized. Additionally, the technique was tested for repeatability and reproducibility. RESULTS: We obtained absence of background and nuclear staining together with a highly contrasted image of the collagen network with 3 min oxidation with 2% potassium permanganate and a silver concentration of 1% during 5 min. In this way, it was very easy to perform acquisition, thresholding and area measurement without any further manual processing of the image. CONCLUSIONS: This technique appears very helpful for the quantitative study of the cardiac collagen network by means of computerized image analysis systems.

Histology, histochemistry and fine structure of the lacrimal and nictitans gland in the South American armadillo Chaetophractus villosus (Xenarthra, Mammalia).

The anatomical, histological, histochemical and ultrastructural characteristics of the lacrimal gland (LG) and nictitans gland (NG) of the armadillo Chaetophractus villosus were described. The histochemical and histological features of both glands in male and female adult animals were compared. The tissues were processed with conventional techniques for light and transmission electron microscopy. Fixed specimens were submitted to a battery of tests for glycans, glycosaminglycans, glycoconjugates, proteins, and lipids. The LG of the armadillo may be considered within the set of glandulae lacrimales superior in which primates, carnivores, perisodactyls and artiodactyls are included. The localization of the NG was similar to that of other mammals. Lacrimal and NG were histologically and histochemically identical. The secretory endpieces consisted of three cell types: (1) Mucous cells (MC) with different types of mucous secretory granules with neutral and sialic acid-containing glycoconjugates (GCs). (2)Seromucous cells (SMC) showing a variety of moderately electron dense secretory granules with flocculent material with carboxylated acidic, neutral, and sialic acid-containing GCs. Intercellular canaliculi with junctional complexes and basolateral intercellular spaces were frequent. (3) Serous cells (SC) with electron dense secretory granules. Histochemically, they showed the strongest reaction for proteins and neutral, weakly acid and carboxylated acidic GCs. The epithelium of the intra- and inter-lobular excretory ducts showed secretory activity, junctional complexes, and wide basolateral intercellular spaces with lateral folds. The endpieces and ducts were surrounded by myoepithelial cells. The stroma was characterized by fenestrated endothelium, unmyelinated axons, and abundant plasma cells. MC, SMC, and the duct system were richly innervated by hypolemmal nerve terminals.

A technique for chronically recording the EKG of the lymph hearts in the toad Bufo arenarum (Hensel)

A simple technique for recording the EKG of the posterior lymphatic hearts of the toad Bufo arenarum (Hensel) in free moving unanesthetized specimens is described. This technique permits long term chronic recordings in varied physiological and behavioral conditions whereas it overcomes some of the technical difficulties encountered in obtaining reliable recordings.

A technique for chronically recording the EKG of the lymph hearts in the toad Bufo arenarum (Hensel)

A simple technique for recording the EKG of the posterior lymphatic hearts of the toad Bufo arenarum (Hensel) in free moving unanesthetized specimens is described. This technique permits long term chronic recordings in varied physiological and behavioral conditions whereas it overcomes some of the technical difficulties encountered in obtaining reliable recordings.(AU)