Persistent and non-persistent strains of Listeria monocytogenes: A focus on growth kinetics under different temperature, salt, and pH conditions and their sensitivity to sanitizers.

This study aimed to investigate the effect of different conditions, including temperature (37 °C, 22 °C, and 4 °C), NaCl concentrations (2.5%, 4%, and 8%), and acidity (pH = 5), on the growth response of persistent and non-persistent isolates of Listeria monocytogenes. The resistance to two common sanitizers (benzalkonium chloride and hydrogen peroxide) was also investigated. A selected group of 41 persistent and non-persistent L. monocytogenes isolates recovered from three cheese processing plants during a previous longitudinal study was assembled. Average lag time was similar for persistent and non-persistent isolates grown at 37 °C, 22 °C and 4 °C but significantly shorter (p < 0.05) for persistent isolates grown at 2.5%, 4% and 8% NaCl, and at pH 5. Average growth rates were significantly higher (p < 0.05) for persistent than for non-persistent isolates when grown at 22 °C, 2.5%, 4% and 8% NaCl, and at pH 5. These results suggest that persistent strains may be better adapted to grow under stressful conditions frequently encountered in food processing environments than non-persistent strains. No relation between persistence and resistance to the tested sanitizers was found.

Preferences for medication attributes among patients with type 2 diabetes mellitus in the UK.

AIM: To examine preferences for oral medication attributes among participants with early and advanced type 2 diabetes mellitus (T2DM) in the UK using a discrete choice experiment (DCE). METHODS: A web-based DCE was administered where participants indicated which medication they preferred from two different hypothetical oral anti-diabetic (OAD) medication profiles, each composed of differing levels of seven attributes (efficacy, hypoglycaemic events, weight change, gastrointestinal/nausea side effects, urinary tract infection and genital infection, blood pressure and cardiovascular risk) for 20 sets of pair-wise comparisons. A random effects multinomial logit regression model was used to estimate the preference weight (PW) for each of the attribute levels, and the relative importance (RI) of each attribute was calculated. Analyses were conducted for the overall sample and for medication and gender subgroups. RESULTS: The final sample included 100 participants with a mean age of 62.9 (SD 11.1) years and comparable numbers of participants of each gender (51% male, 49% female). The majority of the participants were White-British (92%). The total PW and corresponding RI were highest for four of the seven attributes: hypoglycaemic events (PW = 1.98; RI = 24.7%), weight change (PW = 1.65; RI = 20.6%), gastrointestinal/nausea side effects (PW = 1.49; RI = 18.6%) and efficacy (PW = 1.44; RI = 18.0%). The RI values differed for some attributes across gender and number of current T2DM medication subgroups. CONCLUSION: The results suggest that hypoglycaemia, weight change, gastrointestinal side effects and efficacy are of primary importance to patients in their OAD preferences in T2DM. These four attributes comprised over 80% of the RI.

[Congenital glycosylation disorders: a study of two patients]. / Trastornos congénitos de la glucosilación. Estudio de 2 pacientes.

BACKGROUND: Congenital glycosylation disorders (CGDs) are a group of disorders caused by a defect in glycoprotein synthesis. Clinical manifestations may affect to different organs. AIMS: To describe two new patients cases with a CGD in order to make paediatricians aware of this disorder. CLINICAL CASES: Two new cases of different age and gender are presented, showing clinical manifestations, and radiological and laboratory findings compatible with CGD. One of the cases was followed up for several years. CONCLUSIONS: Glycosylation disorders are a group of conditions to bear in mind when considering the diagnosis of a patient with neurological symptoms of unexplained origin, particularly in those cases that include a delay in psychomotor activity, low muscle tone, epilepsy, and hepatic or coagulation disorders, as well as in patients with cerebellar or olivopontocerebellar atrophy.

[Cost-effectiveness of the combination therapy of dutasteride and tamsulosin in the treatment of benign prostatic hyperlasia in Spain]. / Coste-efectividad de la combinación dutasterida y tamsulosina en el tratamiento de la hiperplasia benigna de próstata en España.

OBJECTIVES: to evaluate the incremental cost-effectiveness ratio (ICER) of the combination therapy with dutasteride and tamsulosin (DUT+TAM) as initiation treatment versus the most used drug in Spain, tamsulosin (TAM), in the treatment of moderate to severe benign prostatic hyperplasia (BPH) with risk of progression. METHODS: a semi-Markov model was developed using 4-year and 35-year time horizons and from the Spanish National Healthcare Service perspective. Data were obtained from the CombAT trial. Effectiveness was measured in terms of quality adjusted life years (QALYs). Health care resources were defined by an experts' panel, and unitary costs were obtained from published Spanish sources. Pharmacologic cost is expressed in PTP(WAT); in the case of TAM, the generic price is used, in the case of DUT+TAM the price of a fixed dose combination is used. Costs are expressed in 2010 Euros. RESULTS: combination therapy with DUT+TAM produces an incremental effectiveness of 0.06QALY at year 4 and 0.4QALY at year 35. DUT+TAM represents an incremental cost of € 810.53 at 4 years and € 3,443.62 at 35 years. Therefore, the ICER for DUT+TAM versus TAM is € 14,023.32/QALY at year 4 and € 8,750.15/QALY at year 35. CONCLUSIONS: initiation treatment with DUT+TAM represents a cost-effective treatment versus TAM, the most used treatment in Spain, due to the fact the ICER is below the threshold that usually allows a technology to be considered as cost-effective.

[Alterations in bone mineral metabolism in patients with calcium kidney stone disease and polymorphism of vitamin D receptor. Preliminary results]. / Alteraciones en el metabolismo mineral óseo en pacientes con urolitiasis de repetición y polimorfismos del gen del receptor de la vitamina D. Resultados preliminares.

UNLABELLED: Bone health, within calcium kidney stone disease is a matter of controversy. On the other hand, some genetic studies have shown an association between some Vitamin D receptor polymorphisms and calcium kidney stone disease. MAIN OBJECTIVE: To study the possible association between calcium kidney stone disease with bone metabolism and some Vitamin D receptor polymorphisms. PATIENTS AND METHODS: This is a case-control study, with seventy-two subjects of both genders divided into two groups: Group I: cases, composed by 51 patients suffering from calcium kidney stone disease. Twenty-four of them had no hypercalciuria, 16 had absortive hypercalciuria and 11 had renal hypercalciuria. Group II: controls, composed by 21 people, without either urolithiasis or hypercalciuria. We performed a complete study including biochemical markers of bone mineral remodelling, bone mineral density (BMD) was estimated both in the lumbar spine (L2-L4) and femoral neck, and also VDR polymorphism for the loci b, a and t. RESULTS: Patients with urolithiasis had lower values of BMD both in the lumbar spine and femoral neck, compared to controls. Z-score were lower in the lumbar spine and femoral neck (p =0,045 y 0,031, respectively). Those patients with absorptive hypercalciuria had higher BMD in the femoral neck than those with renal hypercalciuria and non-hypercalciuria. Because they had more weight and height all the statistical study was performed alter adjusting by these two variables and statistical significance was then only stated between patients with hypercalciuria and without it. Patients with urolithiasis had higher values of 1,25 (OH)2 vitamin D (p=0,002), and lower of PTH (p=0,049), without any relationship to hypercalciuria and its subtypes. Seventy six percent of the patients had a daily calcium intake lower than 800 mg/day. The distribution of VDR alleles in patients with urolithiasis was similar to controls, although after grouping genotypes, a lower distribution of BB and tt polymorphisms were observed in patients suffering from urolithiasis. CONCLUSIONS: Calcium kidney stone disease by itself produces a decrease in BMD, more intense in femoral neck, independently the presence or absence of hypercalciuria. Patients suffering from urolitihiasis have higher values of 1,25 (OH)2 vitamin D than non-hypercalciuric patients and lower values of PTH probably due to a low dietary calcium intake. In our population studied there is no relationship between VDR polymorphisms and the presence of calcium kidney stone disease. Because the reduced number of patients of our study, more studies are needed to obtain definitely conclusions.

[Unilateral facial and cerebral hyperplasia associated with neurofibromatosis type 1. Report of four patients]. / Hiperplasia facial y cerebral unilateral asociada a neurofibromatosis tipo 1. Presentacion de cuatro pacientes.

INTRODUCTION: Plexiform neurofibroma in any location is one of the commonest complications associated with neurofibromatosis type 1 (NF1). Plexiform neurofibroma of the upper eyelid and orbit is usually associated with ipsilateral hemifacial hyperplasia. We present four patients with NF1 and plexiform neurofibroma of the eyelid and orbit associated with hemifacial hyperplasia, who also showed hyperplasia of the unilateral cerebral hemisphere. CASE REPORTS: There are four patients, three females and one male, who consulted because of NF1 with plexiform neurofibroma of upper eyelid and hemifacial hyperplasia. Upper eyelid involvement was observed since birth and progressed during the first years of life. The patients showed normal neurological and mental development without motor or cerebellar disorders. Magnetic resonance studies demonstrated the asymmetric hyperplasia of the ipsilateral hemisphere in all four cases and of the cerebellar hemisphere in one case. The degree of hemispheric hyperplasia was related to the size and extension of the plexiform neurofibroma, as well as to the severity of the hemifacial hyperplasia. In our case which had the plexiform neurofibroma extended to the neck and the upper thorax, the hyperplasia not only affected the cerebral hemisphere but also the ipsilateral cerebellar hemisphere. All parts of the hemisphere showed increased size. The cortex of the entire hemisphere showed normal differentiation of the subcortical white matter. CONCLUSION: NF1 appears to be related with facial and cerebral ipsilateral hemihyperplasia. The relation between the size and extension of the orbital, eyelid and facial plexiform neurofibroma and the degree of asymmetry of the hemispheric hyperplasia suggest that different influences of a still unknown agent, possibly a gene, obviously related to NF1, causes both the intracranial and extracranial abnormalities.

[Autosomal recessive diseases with mental retardation]. / Enfermedades autosómicas recesivas con retraso mental.

INTRODUCTION: Autosomal recessive diseases with mental retardation are disorders that affect autosomes, and their genetic expression occurs in individuals who are homozygotic for a mutation, while heterozygotic subjects are unaffected carriers. If both parents are carriers, the theoretical possibility of their children also being carriers is 50%, the risk of the children being affected by the disease is 25%, and there is a 25% chance of their being healthy. They are an important source of mental deficiencies and inborn errors of metabolism (IEM) are some of their characteristic syndromes. DEVELOPMENT: The genetic disorders known as IEM can be classified according to the metabolism they affect, that is, purines, pyrimidines, amino acids, and so on. One of the lysosomal disorders is Tay-Sachs disease, which is rare among the general population but is very frequent in populations with a high rate of consanguinity, such as the Ashkenazi Jews. One of the most notable disorders affecting the metabolism of amino acids is the case of phenylketonuria due to mutations in the phenylalanine hydroxylase gene (PAH). It accounts for 0.5-1% of mental diseases and appears with a frequency rate of between 1/11,500 and 1/14,000 in newborn infants. Its early diagnosis through neonatal screening programmes makes it possible to start administering a phenylalanine-free diet and thus prevent mental retardation. CONCLUSIONS: Knowledge of this kind of autosomal diseases with neurological involvement, together with their correct and early diagnosis, makes it possible to establish suitable treatment regimens in some cases and to carry out genetic counselling in all of them.

[Cutaneous hemangiomas and vascular malformations and associated pathology (Pascual-Castroviejo type II syndrome). Study of 41 patients]. / Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes.

AIM: To describe the clinical, diagnostic and therapeutic features of this angiomatous neurocutaneous syndrome, which is the most frequent one, and to report a personal series of 41 patients. PATIENTS AND METHODS: Forty one patients--31 females and 10 males--were studied during childhood and then, several patients were followed during many years, which allowed us to learn about the evolution of the abnormalities. The cutaneous lesions were classified as hemangiomas in 30 patients (73%) and as vascular malformations in 11 patients (27%). RESULTS: A cerebellar anomaly (unilateral hemispheric hypoplasia and Dandy-Walker malformation) was seen in 13 patients (31.5%) cerebral cortical dysplasia in 4 patients (10%), aortic arch coarctation in 6 patients (15%), and congenital cardiopathy in 5 patients (12%). The most frequent abnormalities were intracranial and/or extracranial vascular malformations. Persistence of the trigeminal artery was observed in 7 patients (17%), absence or severe hypoplasia of an internal carotid artery in 13 patients (32%), absence of a vertebral artery in 7 patients (17%), hypoplasia of intracranial arteries in 6 patients (15%) and aneurysmal enlargement of carotid or vertebral arteries in 5 patients (12%). Also were observed 4 patients (10%) with intracranial hemangioma, 2 (5%) with hemangioma in mediastinum, and 3 (7.5%) with intestinal hemangioma, all of which disappeared during the first years of life. Aneurysmal enlargement of the carotid and vertebral arteries and intracranial branches also disappeared after a process of progressive narrowing of the arterial lumen that caused complete obstruction of these arteries. At the same time the cutaneous hemangioma regressed. During this process, collateral vascularization through branches of the external carotid artery and of the non-affected branches of the contralateral intracranial arteries developed. CONCLUSION: This neurocutaneous syndrome is the most frequent one and it is associated with several types of vascular and non-vascular abnormalities which can involve any organ of the body. Internal and external hemangiomas and hemangiomatous lesions progress and tend to regress concomitantly.

[Kabuki make-up syndrome. A report of 18 Spanish cases]. / Síndrome del maquillaje Kabuki. A propósito de 18 casos españoles.

AIM: To present 18 cases of Kabuki make-up syndrome with the associated morphological and neurological alterations. CASE REPORTS: The series consists of 18 patients, 14 males and 4 females, who show the peculiar facial morphology of this disease. Ten cases were studied between 1968 and 1978, before the description of the syndrome. Most of these patients are now over 30 years and two perhaps surmounting the 40, if surviving. The other 8 cases were studied during the last 20 years (a few within the last 3 years). The studies of the patients were anamnesis with genetic history, EEG, image, cytogenetic, laboratory studies, and mental evaluation when the patients collaborated. The most important findings are the peculiar facial characteristics as well as the psychomotor and language retardation, the persistent fingertip pads and clinodactyly that were observed in all patients. Other studies, such as EEG, caryotype, biochemical investigation in blood, urine and CSF (in the studied cases), and the cerebral neuroradiological images (pneumoencephalography, CT or MR) disclosed normal results. None of the patients of this series were neurologically normal. Hyperactivity, respiratory infection and/or otitis were frequently observed. CONCLUSION: Kabuki make-up syndrome is a dysmorphologic and neurological disease of unknown origin. Its main manifestations involve facial appearance and brain function without anatomical anomalies. None of the patients was sent to the Pediatric Neurology service with the suspicion of Kabuki make-up syndrome.

[Malformations of cortical development and their clinical repercussions in a series of 144 cases]. / Malformaciones del desarrollo cortical y su repercusión clínica en una serie de 144 casos.

OBJECTIVE: To show the disorders of the brain cortical development and the possible origin in base to a large series studied in a Pediatric Neurology service. PATIENTS AND METHODS: A series of 144 children with ages ranging between newborn and 12 years was studied from the clinic, image (MR, 3DMR) and evolutive point of views. RESULTS: The diagnosis was: polymicrogyria in 61 cases, lissencephaly in 22, eschizencephaly in 16, heterotopia in 16, cortical dysplasia in 9, hemimegalencephaly in 8, cobblestone in 7, sublobar dysplasia in 3, and 'double cortex' in 2. Mental retardation, motor disorders and epilepsy were the most important anomalies. CONCLUSIONS: Actually, the image is the most important study to make the diagnosis of every type of cerebral malformation. However, to know the specific gene that origin every disorder seems to be the most important thing to make the classification of every malformative type and the possible prevention of this pathology.

[Early treatment of secondary hyperparathyroidism in moderate renal insufficiency: low-phosphorus diet versus calcium carbonate]. / Tratamiento precoz del hiperparatiroidismo secundario en insuficiencia renal moderada: dieta baja en fósforo frente a carbonato cálcico.

Calcitriol deficiency and phosphorus retention are mechanisms involved in the pathogenesis of renal hyperparathyroidism. The aim of this study was to evaluate the effect of dietary phosphorus restriction versus calcium carbonate treatment for one month on PTH and calcitriol levels in patients with mild renal failure. We studied two groups of patients: Group I: 21 patients (14M/7F); mean age 61 years old; mean glomerular filtration rate 51 ml/min. Their diet contained phosphorus 700 mg/day. Group II: 30 patients (21M/9F); mean age 58; mean glomerular rate 56 ml/min. They were divided in two subgroups: 18 patients treated with calcium carbonate 2.5 g/day and 12 patients with 5 g/day. Serum PTH, calcitriol, 25(OH)D3, calcium, phosphorus and urinary excretion of calcium and phosphorus were measured before and after a 30 day period. The low phosphorus diet (Group I) resulted in a significant decrease in PTH levels (81.3 +/- 35 vs 71 +/- 39 pg/ml, p < 0.05) and significant increase in calcitriol levels (22.4 +/- 4.4 vs 33.4 +/- 7.5 pg/ml, p < 0.05). In our study calcium carbonate treatment (Group II) had no effect on PTH and calcitriol levels.

[Neurological complications in patients with heart disease]. / Complicaciones neurológicas del paciente con cardiopatía.

INTRODUCTION: The brain needs a constant supply of oxygen and nutrients from blood. This fact explains its tight dependence on the cardiovascular system as well as the after effects of heart disease on the central nervous system. OBJECTIVES: This study intends to describe the main conditions in which a neurological disorder appears secondarily to or coincidentally with heart disease. Methods. A bibliographical review of publications of neurological disorders related to heart disease is carried out. RESULTS: The main cardiac diseases that can induce neurological disorders include: disturbances of the cardiac rhythm, congenital heart diseases, and complications of cardiac catheterisation and reparative surgery. Likewise, other conditions congenital or acquired in which neurological and heart disorders coexist must be considered. CONCLUSIONS: Medical practice confirms that heart diseases account for different neurological conditions that can manifest by a very variable clinical expressivity.

Unilateral polymicrogyria: a common cause of hemiplegia of prenatal origin.

We describe a series of 13 patients, ten males and three females, with ages ranging between three and fourteen years, who show unilateral polymicrogyria (in the left cerebral hemisphere in four cases and in the right in nine). The first and the main clinical alteration in all 13 cases was hemiparesis that did not change along the years. Ten patients had seizures that were completely controlled in seven. Three cases never presented seizures. Spontaneous threat of abortion or arterial hypertension during the first five months of pregnancy occurred in five cases. Weight less than 3 kg at birth was observed in six cases. The only patient who shows severe mental retardation associated with deafness is a girl who had infantile spasms at 20 days of age and presents duplication of a short arm of the chromosome X. Conventional magnetic resonance (MR) studies, performed in four cases did not disclose polymicrogyria but only an enlarged cortex that was diagnosed as cortical dysplasia. Three-dimensional MR (3DMR) images are very important not only to see the polymicrogyria, but also its extension and severity, especially if, moreover the axial, coronal and sagittal views, oblique, frontal, occipital, basal and superior images of the hemispheric cortical surface are performed.

Antihypertensive effects of the flavonoid quercetin in spontaneously hypertensive rats.

1. The effects of an oral daily dose (10 mg kg(-1)) of the flavonoid quercetin for 5 weeks in spontaneously hypertensive (SHR) and normotensive Wistar Kyoto rats (WKY) were analysed. 2. Quercetin induced a significant reduction in systolic (-18%), diastolic (-23%) and mean (-21%) arterial blood pressure and heart rate (-12%) in SHR but not in WKY rats. 3. The left ventricular weight index and the kidney weight index in vehicle-treated SHR were significantly greater than in control WKY and these parameters were significantly reduced in quercetin-treated SHR in parallel with the reduction in systolic blood pressure. 4. Quercetin had no effect on the vasodilator responses to sodium nitroprusside or to the vasoconstrictor responses to noradrenaline or KCl but enhanced the endothelium-dependent relaxation to acetylcholine (E(max)=58+/-5% vs 78+/-5%, P<0.01) in isolated aortae. 5. The 24 h urinary isoprostane F(2 alpha) excretion and the plasma malonyldialdehyde (MDA) levels in SHR rats were increased as compared to WKY rats. However, in quercetin-treated SHR rats both parameters were similar to those of vehicle-treated WKY. 6. These data demonstrate that quercetin reduces the elevated blood pressure, the cardiac and renal hypertrophy and the functional vascular changes in SHR rats without effect on WKY. These effects were associated with a reduced oxidant status due to the antioxidant properties of the drug.