Increased myo-inositol in parietal white and gray matter as a biomarker of poor prognosis in neuropsychiatric lupus: a case report.

Neuropsychiatric manifestations can be a serious complication of systemic lupus erythematosus, affecting nearly 56% of these patients. Frequently, acceptable clinical outcome is observed in neurolupus with immunosuppressive therapy. Different metabolites identified with MR spectroscopy may be associated with modifications in the natural history of this disease, specifically in the central nervous system. We report a case of neurolupus with progressive neurologic impairment despite aggressive immunosuppressive treatment. We describe clinical features, laboratory and MRI results, as well as characteristic findings on MR spectroscopy. Serial MRI identified atrophy of the left temporal lobe. MR spectroscopy showed an increase of myo-inositol/creatine ratio intensity, accompanied by a decrease of N-acetylaspartate/creatine ratio in both parietal white and gray matter. During follow-up, the patient developed progressive cognitive deficiency despite the intensification of therapy. Neurolupus manifestations are common and immunosuppressive treatment often avoids severe complications. Characteristic findings on MR spectroscopy may be useful for clinicians to determine poor prognosis and resistance to therapy.

Influence in the outcome of neuropathic pediatric patients after early treatment.

BACKGROUND: Clean intermittent catheterization (CIC) and anticholinergic drugs are the mainstay treatment for neuropathic bladder (NB). However, there is not consensus about the time therapy should be started in pediatric patients. AIM: To analyze the impact of early start (first year of life) of CIC and anticholinergic treatment on long-term renal and bladder function. Our hypothesis is that those children who start conservative treatment in the first year of life have better outcome in terms of bladder and renal function and less need of surgical procedures, compared to those who started treatment later in life. PATIENTS AND METHOD: Retrospective study of pediatric patients with NB treated in our hospital (1995-2005) dividing them for comparison in two groups: group 1 started treatment in the first year of life and group 2 between 1 and 5 years old. Collected data included: date of CIC and anticholinergic initiation, presence of VUR or UHN, renal function, UTIs, renal scars, bladder behavior, surgery and urinary continence. RESULTS: Sixty-one patients were included, 25 in group 1 and 36 in group 2. Initially vesico-ureteral reflux (VUR) and overactive bladders were more frequent in group 2. In group 1 one overactive bladder changed to low compliant and in group 2, one normal bladder and 4 overactive bladders changed. At the end of follow-up there were 11 low compliant bladders in group 1 and 17 in group 2. However, in group 1, only 2 patients required bladder augmentation (BA) while in group 2, 12 patients needed it. At the end of the study only 2 patients in group 2 had slight renal insufficiency. CONCLUSIONS: Patients who started conservative treatment in the first year of life have better long-term outcome in terms of UTI, renal scars and surgical procedures. Even if they initially had low compliant bladders, these patients require less BA.

Influence in the outcome of neuropathic pediatric patients after early treatment. / Influencia del inicio precoz del tratamiento conservador en la evolución del paciente pediátrico con vejiga neuropática.

BACKGROUND: Clean intermittent catheterization (CIC) and anticholinergic drugs are the mainstay treatment for neuropathic bladder (NB). However, there is not consensus about the time therapy should be started in pediatric patients. AIM: To analyze the impact of early start (first year of life) of CIC and anticholinergic treatment on long-term renal and bladder function. Our hypothesis is that those children who start conservative treatment in the first year of life have better outcome in terms of bladder and renal function and less need of surgical procedures, compared to those who started treatment later in life. PATIENTS AND METHOD: Retrospective study of pediatric patients with NB treated in our hospital (1995-2005) dividing them for comparison in two groups: group 1 started treatment in the first year of life and group 2 between 1 and 5 years old. Collected data included: date of CIC and anticholinergic initiation, presence of VUR or UHN, renal function, UTIs, renal scars, bladder behavior, surgery and urinary continence. RESULTS: Sixty-one patients were included, 25 in group 1 and 36 in group 2. Initially vesico-ureteral reflux (VUR) and overactive bladders were more frequent in group 2. In group 1 one overactive bladder changed to low compliant and in group 2, one normal bladder and 4 overactive bladders changed. At the end of follow-up there were 11 low compliant bladders in group 1 and 17 in group 2. However, in group 1, only 2 patients required bladder augmentation (BA) while in group 2, 12 patients needed it. At the end of the study only 2 patients in group 2 had slight renal insufficiency. CONCLUSIONS: Patients who started conservative treatment in the first year of life have better long-term outcome in terms of UTI, renal scars and surgical procedures. Even if they initially had low compliant bladders, these patients require less BA.

Female reproductive biology of the bluemouth Helicolenus dactylopterus dactylopterus: spawning and fecundity.

The bluemouth Helicolenus dactylopterus dactylopterus is a zygoparous species that spawns multiple batches of embryos enclosed within a gelatinous matrix. Oocyte development is asynchronous, and the recruitment of secondary growth oocytes occurs continuously during the developing phase, but stops before the start of the first spawning (i.e. fecundity is determinate). The number of developing oocytes can be estimated as a function of the total length of the fish, its ovary mass and its gonado-somatic index. Only at the onset of spawning, when potential fecundity is determined, does condition also have a significant effect. The low levels of atresia detected during most of the spawning season show that this mechanism does not substantially affect the process. There is variability both in the spawning interval (with a mean of 2 days) and in the number of embryos comprising every single batch (up to 37,000). Expected effect of fisheries on the reproductive traits of this deep-sea species is also discussed.

Efficacy of moxifloxacin in the treatment of bronchial colonisation in COPD.

This study was designed to investigate the efficacy of moxifloxacin for the eradication of bacterial colonisation of the airways in patients with moderate-to-severe chronic obstructive pulmonary disease (COPD). Out of 119 stable patients with COPD screened, 40 (mean age 69 yrs, mean forced expiratory volume in 1 s 50% predicted) were colonised with potentially pathogenic microorganisms (PPMs) and were included in a randomised, double-blind, placebo-controlled trial with moxifloxacin 400 mg daily for 5 days. Eradication rates were 75% with moxifloxacin and 30% with placebo at 2 weeks (p = 0.01). Bacterial persistence at 8 weeks was still higher (not significantly) in the placebo arm (five (25%) out of 20 versus one (5%) out of 20; p = 0.18). The frequencies of acquisition of a new PPM were high and similar in both treatment groups; consequently, the prevalence of colonisation at 8 weeks was also similar between treatment arms. No difference was found in the number of patients with exacerbations during the 5-month follow-up. Only the acquisition of a new PPM during follow-up showed a statistically significant relationship with occurrence of an exacerbation. Moxifloxacin was effective in eradicating PPMs in patients with positive sputum cultures. However, most patients were recolonised after 8 weeks of follow-up. Acquisition of a new strain of bacteria was associated with an increased risk of developing an exacerbation.

[Telemedicine and dementia: a need for the 21st century]. / Telemedicina y demencia: una necesidad para el siglo XXI.

INTRODUCTION: The ongoing process of information and communication technologies in health services implies a change in the conception, organization and management of these services. Telemedicine is a working method that allows health professionals to explore and/or treat a patient from an off-site location. In this review we provide a historical background on telemedicine, the evolution of its bibliometric impact, and its application for people with dementia. DEVELOPMENT: Telemedicine's applications have been developed in order to provide greater availability and easier access to healthcare to underserved people. The bibliometric study of telemedicine literature shows an increase in the number of bibliographic references related to telemedicine since 1995. During the last few years in Spain the research and development of telemedicine programs have increased significantly and at present there are telemedicine programs in all regions of the country. Although some of the needs of the patients with dementia may be unsuitable for communication and information technologies, their application could offer an added value to health services. This technology does not attempt to replace face-to-face medical consultations but rather to prevent some difficulties this kind of patients can present and improve their quality of life. CONCLUSIONS: Dementia could be an appropriate field in order to implement some telemedicine programs that may improve patient medical care, and reduce medical and management expenses for social and healthcare services.

[The feasibility of a registry of dementias: clinical features and diagnostic coverage]. / Viabilidad de un registro de demencias: características clínicas y cobertura diagnóstica.

INTRODUCTION AND AIM: Classic epidemiological studies do not allow to know the dementia patterns of derivation and diagnosis in a defined territory. This information is fundamental for the planning and distribution of the sanitary and social resources to a medium-to-long term. The results of a pilot-registry program for dementia cases based on the population surveillance principles is presented. MATERIALS AND METHODS: Consecutive and standardized registry of the incident dementia diagnoses of a memory unit during biennium 2004-2005. RESULTS: 670 new cases of dementia, of which 74% corresponded to Alzheimer's disease, were registered. The presenile dementias were 10.3% of the cases. The mean time between the beginning of the symptoms and the clinical diagnosis was of 2.4 years and the severity of the dementia was mild in 60.0%. 90.1% of the cases lived in their homes or in a relative's home. The hypertension, the diabetes mellitus and the antecedents of depressive disease were the more frequent pathological antecedents (> 20%). The diagnostic coverage based on the estimated dementia cases was 75% for the memory unit's reference territorial area. The diagnostic coverage for the health region of Girona was 38%. CONCLUSIONS: The registered data show the viability and validity of the proposed registry program for dementia cases. Nowadays the registry program is able to extend the diagnostic coverage all over the health region of Girona.

Sluggish Cognitive Tempo: Sociodemographic, Behavioral, and Clinical Characteristics in a Population of Catalan School Children.

OBJECTIVE: The aims of the present study were to examine the presence of sluggish cognitive tempo (SCT) symptoms in children; associations of the symptoms with sociodemographic characteristics of the children; and relationships between SCT symptoms and symptoms of ADHD, dyslexia, academic performance, and behavioral problems. METHOD: We evaluated Catalan schoolchildren aged 7 to 10 years in Barcelona, 2012-2013. Parents filled out the SCT-Child Behavior Checklist (SCT-CBCL), the Strengths and Difficulties Questionnaire (SDQ), and a questionnaire concerning sociodemographic characteristics. Teachers completed the ADHD criteria of Diagnostic and Statistical Manual of Mental Disorders (4th ed.; DSM-IV) (ADHD- DSM-IV), a list of dyslexia symptoms, and evaluated the children's academic performance. SCT symptoms were studied as a continuous and dichotomous variable. RESULTS: In all, 11% of the children in our sample scored above the clinical cut-off on the SCT-CBCL scale. We observed a higher rate of SCT symptoms in boys, children whose father was unemployed, those whose maternal educational level was lower, children with a high socioeconomic vulnerability index at home, those who reported maternal smoking during pregnancy and current second-hand smoke exposure at home, and children with an ADHD diagnosis. More SCT symptoms were associated with inattention symptoms, symptoms of dyslexia, academic problems, and emotional and peer relationship problems. CONCLUSION: We observed a higher prevalence of SCT symptoms in our sample than expected in the general population. While girls are less prone to SCT symptoms, some socioeconomic indicators, dyslexia, and inattention symptoms as well as exposure to smoking at home increase the risk of SCT and must be taken into account during assessments.

[A proposal for a clinical registry of dementias]. / Propuesta para un registro clinico de demencias.

AIMS: We outline a proposal for the structural and functional features needed to develop a registry of dementias which can be used to collect standardised information that is both reliable and valid concerning cases of dementia in the specialised health care centres within a particular geographical area. DEVELOPMENT: Due to the shortage of information about aspects concerning the impact of dementias on the health care system (in terms of the usage of resources and patterns of detection, referral, diagnosis and treatment in usual clinical practice in primary and secondary care), a sequential implementation of the registry is proposed so that it can be adapted to each health district or region. The first step is to identify the cases and sources of information; second, a system for collecting data must be developed that allows information to be gathered in a standardised manner while at the same time making it possible to work in close collaboration with the specialists who diagnose dementia; and, third, it must be set up with the logistics and staff needed to centralise all the functions and activities of the registry. CONCLUSIONS: Epidemiological surveillance is an essential instrument for planning, managing and distributing community health resources, for following up the natural history of chronic diseases and for assessing the impact of programmes of prevention. In this respect, and from a functional point of view, the proposed registry of dementias meets all the basic requirements of epidemiological surveillance.

Diet therapy for hypercholesterolemia in children and adolescents. A follow-up.

OBJECTIVE: To determine the effectiveness and compliance of dietary restriction in a group of children and adolescents with hypercholesterolemia. RESEARCH DESIGN: Prospective clinical trial. SETTING: Pediatric hospital in Madrid, Spain. PARTICIPANTS: Four hundred fifty-one children and adolescents of both sexes aged 2 to 18 years diagnosed as having hypercholesterolemia and treated with dietary restriction (American Heart Association Step-One and Step-Two Diets). The follow-up period ranged from 6 months to 2 years and was performed by one pediatrician. RESULTS: Total cholesterol, low-density lipoprotein cholesterol, and apolipoprotein B-100 levels as well as the low-density lipoprotein cholesterol/high-density lipoprotein cholesterol ratio decreased significantly (P < .01) from the first month of dietary restriction, whereas the high-density lipoprotein cholesterol level increased (P < .01). The apolipoprotein A-I level increased significantly (P < .01) only after 6 months of diet therapy. After 12 months of follow-up, 30.7% (58/189) of patients did not follow the diet strictly. In this group, significantly higher levels of total cholesterol, low-density lipoprotein cholesterol, and apolipoprotein B-100 were found. CONCLUSIONS: Diet therapy is effective in the treatment of hypercholesterolemia in children and adolescents. Dietary restriction has a beneficial effect not only on total cholesterol and low-density lipoprotein cholesterol levels but also on apolipoprotein A-I and B-100 levels.

The kallikrein-kinin system in the colon and lung fluid from a cribriform degeneration (cri) mutant mouse.

The cribriform degeneration (cri) mutant mouse was widely studied in regard to the electrolyte and kallikrein metabolism because of its potentiality as a cystic fibrosis (CF) genetic animal model. In this paper the activity of the kallikrein-kinin system, and the kininase activity and glycoproteins concentration in colon and pulmonary lavage fluid (PLF) in homozygous mutant (cri/cri) and control sibling mice are described. The mutant mice showed a diminished kininogenase and kininase activity and glycoproteins concentrations in both studied organs. It is concluded that a kallikrein-kinin system alteration could be responsible of the cri/cri electrolyte defect.

Kallikrein-like esteroprotease activity and kininase activity in submandibular gland from a mutant mouse with human cystic fibrosis like alterations.

The submandibular gland of cri/cri and control mice were compared for their activity of glandular kallikrein like esteroprotease and kininase. Esteroprotease activity is significantly reduced in cri/cri mice with respect to control, with an increased kininase activity in cri/cri mice. Since previous work showed an electrolyte abnormality in the salivary glands of this mutant mouse (1) a possible relationship between this alteration with the low activity of cellular esteroprotease and the high kininase activity is suggested.

Kallikrein and amylase contents in tissues from a mutant mouse model for human cystic fibrosis.

Kallikrein and amylase activities are decreased in the pancreas and salivary glands from cri/cri homozygote mutant mice. Kallikrein is decreased in the cri/cri kidney too. With reference to nucleic acid concentrations there is no difference between control and mutant mice. The previously described electrolyte abnormalities of the cribriform degeneration (cri) mutant mouse, could be due to the abnormal activity of the kallikrein-kinin system on the transport mechanism of tubular cells in the organs mentioned. These findings represent a new step on our efforts to develop a useful animal model for human cystic fibrosis research.

Influence of deficient alpha1-anti-trypsin phenotypes on clinical characteristics and severity of asthma in adults.

Severe alpha1-anti-trypsin (AAT) deficiency implies a high risk of pulmonary emphysema development. The possible relationship between partial deficiencies of this enzyme and bronchial asthma remains controversial. The objective of this study was to ascertain the distribution of AAT phenotypes in a non-selected asthmatic patient population. Across-sectional study on a sample of 111 patients with asthma was carried out. Demographic and clinical variables were collected with serum IgE concentrations, plasma eosinophil number and serum AAT concentrations determined, together with the Pi phenotype. Asthma was mild in 36 (32.4%) patients, moderate in 45 (40.5%) and severe in 30 (27%). No differences were observed in eosinophil count or serum IgE or AAT concentrations among patients with different degrees of severity. Twenty-two (19.8%) asthmatics with deficient phenotypes for AAT were identified, distributed equally in all severity stages of the disease. No significant differences were found in clinical and functional characteristics, or in asthma morbidity between PiMM and PiMS patients or the heterozygote group (PiMS and PiMZ). Eosinophil count and IgE concentrations did not differ significantly between asthmatics with normal phenotype and heterozygotes. In conclusion, the distribution of AAT phenotypes in asthmatic patients did not differ from that found in the general population. Heterozygote phenotypes for the deficiency do not appear to confer greater severity or different clinical expression of asthma in adults.

Smoking and apolipoproteins in adolescents. The Niño Jesus Group.

The relation between smoking and blood lipids and apolipoproteins (A1,B100) were studied in a group of 1024 12- to 18-year-old school children in the Comunidad de Madrid. The percentage of smokers was 19% (17% for girls and 21% for boys). The average consumption of cigarettes per day was 7.83 +/- 5.06 in boys and 6.04 +/- 3.49 in girls (p less than 0.05). As compared with male nonsmokers, male smokers showed a higher mean level of low-density lipoprotein (LDL) cholesterol (112 versus 100 mg/dL, p less than 0.05), a higher LDL cholesterol to HDL-cholesterol ratio (2.27 versus 1.94, p less than 0.001), a higher mean level of apolipoprotein B100 (59 versus 53 mg/dL, p less than 0.05), and a higher apolipoprotein B100 to apolipoprotein A1 ratio (0.45 versus 0.40, p less than 0.01). Female smokers tended to show the same results, although significant differences were only found for LDL cholesterol to HDL cholesterol ratio and apolipoprotein B100 to apolipoprotein A1 ratio (1.8 versus 1.59 and 0.41 versus 0.38 respectively, both p less than 0.05). This work provides new data about the effects of smoking on apolipoproteins in adolescents and emphasizes on the need for preventive programs.

Possible relationship between pervasive developmental disorders and platelet monoamine oxidase activity.

1. The present study was undertaken to determine if the platelet monoamine oxidase (MAO) activity of children with childhood-onset Pervasive Developmental Disorders (PDD), atypical PDD and autistic children differs from MAO of normal children of the same age. 2. The kinetic parameters of MAO activity (Km and Vmax for kynuramine as substrate in 100 mM sodium phosphate buffer, pH 7.4 at 37 degrees C) were determined for platelets from autistic (N = 6), childhood onset PDD (N = 6) and atypical PDD (N = 6) children and 14 controls aged 6-10 years. 3. PDD children had significantly lower Km (4.41 +/- 0.26 vs 5.30 +/- 0.23 microM) and Vmax (16.77 +/- 1.56 vs 22.15 +/- 2.16 nmol h-1 mg protein-1) than control children. The reduction in Vmax was demonstrable in MAO activity measured with 100 microM substrate (14.93 +/- 1.13 vs 20.96 +/- 2.10 nmol h-1 mg-1). 4. These data show that childhood-onset PDD patients, in which the syndrome was complete, presented the lowest levels of platelet MAO activity.

Effect of furosemide on the rat submandibular gland kallikrein secretion.

The effects of furosemide and Captopril were studied in normals and nephrectomized rats. Different doses of furosemide (5 to 50 mg/kg) increased the saliva kallikrein activity of submaxillary gland perfused with pilocarpine. Rats injected with captopril (10 mg) increased the blood flow of the gland, but did not modify the blood pressure. After furosemide (50 mg/kg) and captopril (10 mg), a decrease in arterial blood pressure was observed. The results suggest a release of glandular kallikrein which is secreted from the gland directly into the vascular compartment. On the other hand, rats sialodectomized showed no alterations in blood pressure in response to both drugs. These data suggest that submaxillary gland kallikrein play a role in regulating blood flow of the gland and blood pressure, at least in our experimental conditions.

Mechanism of urinary kallikrein excretion and its action on water excretion.

In male Wistar rats urinary kallikrein excretion was positively correlated with urinary flow and glomerular filtration rate and negatively with urinary osmolality and with TcH2O. The mechanism of urinary kallikrein excretion is interpreted as a wash-out effect of renal kallikrein. The renal kallikrein regulates the water excretion decreasing the reabsorption of water in the distal nephron.