ABSTRACT
OBJECTIVES: Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to analyse the incidence of severe infection in the first two years of follow-up and how predictors of infection change during the course of systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The study included 282 patients. Markers of lupus activity, prednisone doses and immunosuppressive therapy were compared between patients with and without infections in the first and second year of the disease. Drug therapy administered during the first month of follow-up has been considered as a potential predictor of infections during the first year and medications administered during the first year have been considered potential predictors of infections during the second. RESULTS: Nineteen patients (6.4%) had a documented episode of major infection during the first year of follow-up and 16 patients (5.67%) during the second. The following variables were associated with infections during the first year: hypocomplementaemia at diagnosis ( p < 0.01), nephritis at diagnosis ( p = 0.03), SLEDAI score ( p < 0.01), prednisone >30 mg/day ( p = 0.01), methylprednisolone pulses ( p = 0.05) and mycophenolate use ( p = 0.02). The independent variables in the final model were hypocomplementaemia (odds ratio (OR) 4.41, 95% confidence interval (CI) 0.96-20.20, p = 0.05) and a dose of prednisone >30 mg/day (OR 6.60, 95% CI 1.34-32.42, p = 0.02). The following variables were associated with infections during the second year: dose of prednisone > 7.5 mg/day ( p = 0.05), methylprednisolone pulses ( p = 0.07), duration of therapy with antimalarials ( p = 0.09), therapy with mycophenolate ( p = 0.01), therapy with cyclophosphamide ( p = 0.05). The independent variables in the final model were a dose of prednisone >7.5 mg/day (OR 4.52, 95% CI 0.99-21, p = 0.054) and duration of therapy with antimalarials as a protective factor (OR 0.99, 95% CI 0.99-1.00, p = 0.053). CONCLUSIONS: The low incidence of early infections in the RELES cohort is partially explained by the extended use of antimalarials and by the general avoidance of prolonged high doses of prednisone. Patients with high baseline activity are at a higher risk of infection during the first months but therapy with medium-high doses of prednisone is the main predictor of infectious events. Thus, every effort should be made to limit oral glucocorticoid use from the very beginning of the SLE course.
Subject(s)
Antimalarials/therapeutic use , Immunosuppressive Agents/therapeutic use , Infections/epidemiology , Lupus Erythematosus, Systemic/drug therapy , Prednisone/administration & dosage , Adolescent , Adult , Aged , Aged, 80 and over , Cohort Studies , Cyclophosphamide/therapeutic use , Drug Therapy, Combination , Female , Humans , Incidence , Infections/classification , Logistic Models , Lupus Erythematosus, Systemic/complications , Male , Methylprednisolone/therapeutic use , Middle Aged , Multivariate Analysis , Severity of Illness Index , Spain/epidemiology , Young AdultABSTRACT
Both acute pancreatitis and diffuse alveolar haemorrhage are rare conditions associated with systemic lupus erythematosus (SLE). In this case report, a 23-year-old female with SLE was diagnosed with lupus-associated pancreatitis and, within a few days and despite initial therapy with pulse methyl-prednisolone, subsequently suffered an acute respiratory failure due to a diffuse alveolar haemorrhage. The patient was admitted to the intensive care unit and treatment was intensified with cyclophosphamide and rituximab, which shortly induced the complete remission of SLE with resolution of both clinical conditions. She completed treatment with six pulses of cyclophosphamide followed by azathioprine, hydroxychloroquine and prednisone at initial doses of 20 mg/d with rapid tapering to 5 mg/d, without relapse of the disease during the following year. This case can illustrate that, even in severe, life-threatening SLE flares, it is possible to avoid high-dose prednisone, which has been associated with severe side effects, including infections. Acute pancreatitis and diffuse alveolar haemorrhage are rare conditions caused by SLE. DAH can be a life-threatening complication, with an early mortality of at least 50%. When facing such severe SLE activity, there is a general tendency to use high doses of prednisone as the initial therapy, maintaining such high doses for long periods of time, even after the clinical situation has subsided. We report a case of a young woman with SLE, suffering from acute pancreatitis and diffuse alveolar haemorrhage, who was successfully treated with pulse methyl-prednisolone, hydroxychloroquine, cyclophosphamide and rituximab, combined with medium doses of prednisone.
Subject(s)
Antibodies, Monoclonal, Murine-Derived/administration & dosage , Cyclophosphamide/administration & dosage , Immunosuppressive Agents/administration & dosage , Lupus Erythematosus, Systemic/drug therapy , Pancreatitis/drug therapy , Prednisone/administration & dosage , Respiratory Insufficiency/drug therapy , Acute Disease , Adult , Antibodies, Monoclonal, Murine-Derived/adverse effects , Cyclophosphamide/adverse effects , Disease Progression , Drug Therapy, Combination , Female , Hemorrhage/immunology , Humans , Immunosuppressive Agents/adverse effects , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/immunology , Pancreatitis/immunology , Prednisone/adverse effects , Remission Induction , Respiratory Insufficiency/immunology , Rituximab , Severity of Illness Index , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
The present study compares four different structural magnetic resonance imaging techniques used to measure gray matter (GM) atrophy in Alzheimer's disease (AD): manual and automated volumetry, cortical thickness (CT) and voxel-based morphometry (VBM). These techniques are used interchangeably in AD research and thus far it is unclear which technique is superior in detecting abnormalities early in the disease process. 18 healthy participants without any memory impairment, 18 patients with MCI, and 17 patients with mild AD were included and between-group differences were investigated in AD signature regions (areas in the prefrontal cortex (PFC), medial temporal lobe (MTL) and posterior parietal cortex (PPC)). Both manual volumetric measurements and VBM were able to detect GM atrophy in the early stages (differentiation controls and MCI), mainly in the MTL. In the early phase, automated volumetric measurements showed GM differences in the PPC but not in the MTL. In our sample, CT measurements were not sensitive for group differences in the early stages. PFC regions showed abnormalities in the later stages (controls vs AD) when manual volumetric measurements or VBM are employed. Manual volumetric measurements together with VBM are preferred techniques for assessing GM differences showing abnormalities in most of the investigated regions, with a predominance of the MTL in the early phase. Automated FreeSurfer volumetric measurements show similar performances in the early phase, displaying group differences in the PPC but not in MTL regions. Measurements of CT are less sensitive in the MCI stage and its sensitivity is restricted to the MTL and PPC regions in later stages of the disease (AD).
Subject(s)
Alzheimer Disease/pathology , Brain/pathology , Magnetic Resonance Imaging/methods , Nerve Fibers, Unmyelinated/pathology , Aged , Aged, 80 and over , Atrophy/pathology , Humans , Image Processing, Computer-Assisted , Male , Middle Aged , Neuroimaging/methods , Neuropsychological Tests , Sensitivity and SpecificityABSTRACT
The prevalence of neurodegenerative disorders increases dramatically with advancing age. Although in recent decades the study of many neurodegenerative disorders has evolved greatly, the concept of neurodegeneration still remains elusive. Although neurodegenerative disorders are classified according to the major components of protein deposits, coexpression of several abnormal proteins in the brain tissue is more common than that was previously thought. The aim of this report is to describe the type of protein deposits found in brains with neuropathological diagnosis of neurodegenerative disease. The report shows the experience obtained in the Brain Bank of Navarra (Spain). The target population for this retrospective descriptive study comprised 178 brains autopsied in the "Hospital of Navarra" in Pamplona between 1994 and 2004 and 201 brains donated to the Brain Bank of Pamplona between 2004 and 2009. The diagnosis of the 201 brains from the Brain Bank was 62 (30.8%) Alzheimer's disease (AD), 43 (21.3%) multiprotein deposit, 31 (15.4%) α-synucleinopathies, 31 (15.4%) frontotemporal lobar degeneration (FTLD), 17 (8.4%) tauopathies, 9 (4.4%) prion disease, 6 (2.9%) vascular dementia (VD), and 2 (0.9%) Huntington's disease. Among the 43 cases with multiprotein deposits, we found 35 brains with deposits of 3 proteins (tau, ß-amyloid, and α-synuclein). In these two series of brains, the high incidence of deposition of multiple proteins in neurodegenerative disorders is shown. Our results are in agreement with previous findings showing that tau, ß-amyloid, and α-synuclein are the proteins most frequently deposited together.
Subject(s)
Amyloid beta-Protein Precursor/metabolism , Brain/metabolism , Neurodegenerative Diseases/diagnosis , Neurodegenerative Diseases/metabolism , alpha-Synuclein/metabolism , tau Proteins/metabolism , Aged, 80 and over , Autopsy , Humans , Immunoenzyme Techniques , Retrospective Studies , Spain , Tissue BanksABSTRACT
The main aim of the present study was to compare volume differences in the hippocampus and parahippocampal gyrus as biomarkers of Alzheimer's disease (AD). Based on the previous findings, we hypothesized that there would be significant volume differences between cases of healthy aging, amnestic mild cognitive impairment (aMCI), and mild AD. Furthermore, we hypothesized that there would be larger volume differences in the parahippocampal gyrus than in the hippocampus. In addition, we investigated differences between the anterior, middle, and posterior parts of both structures. We studied three groups of participants: 18 healthy participants without memory decline, 18 patients with aMCI, and 18 patients with mild AD. 3 T T1-weighted MRI scans were acquired and gray matter volumes of the anterior, middle, and posterior parts of both the hippocampus and parahippocampal gyrus were measured using a manual tracing approach. Volumes of both the hippocampus and parahippocampal gyrus were significantly different between the groups in the following order: healthy>aMCI>AD. Volume differences between the groups were relatively larger in the parahippocampal gyrus than in the hippocampus, in particular, when we compared healthy with aMCI. No substantial differences were found between the anterior, middle, and posterior parts of both structures. Our results suggest that parahippocampal volume discriminates better than hippocampal volume between cases of healthy aging, aMCI, and mild AD, in particular, in the early phase of the disease. The present results stress the importance of parahippocampal atrophy as an early biomarker of AD.
Subject(s)
Alzheimer Disease/pathology , Hippocampus/pathology , Memory Disorders/pathology , Parahippocampal Gyrus/pathology , Aged , Atrophy/pathology , Biomarkers , Brain Mapping , Humans , Magnetic Resonance Imaging , Male , Middle AgedSubject(s)
Insomnia, Fatal Familial/physiopathology , Polysomnography , Video Recording , Circadian Rhythm/genetics , Codon/genetics , Fatal Outcome , Humans , Insomnia, Fatal Familial/genetics , Male , Memory Disorders/etiology , Middle Aged , Mutation, Missense/genetics , Neuropsychological Tests , Personality Disorders/etiology , Point Mutation/genetics , Prion Proteins , Prions/genetics , Terminology as TopicABSTRACT
Sleep disorders are very frequent in the elderly. Bearing in mind the growth of this population group in western societies, it is of great importance to understand the sleep diseases that affect them and what their treatment should be. On the other hand, it is in this age group where we find the majority of patients with dementias. The treatment of sleep disorders in these patients is complex. An adequate control of insomnia and of the excessive nocturnal activity that usually occurs in the advanced phases of dementias has an important social repercussion. This article summarises the peculiar characteristics of sleep disorders in the elderly as well as the diagnostic and therapeutic handling of sleep disorders in patients with dementias.
Subject(s)
Dementia/complications , Sleep Wake Disorders/etiology , Aged , Humans , Sleep Wake Disorders/therapyABSTRACT
Los trastornos del sueño son muy frecuentes en los ancianos. Teniendo en cuenta el crecimiento de este grupo de población en las sociedades occidentales, tiene gran importancia el conocimiento de las enfermedades del sueño que les afectan y cuál debe ser su tratamiento. Por otro lado, es en este grupo de edad donde nos encontramos la mayor parte de los pacientes con demencias. El manejo de los trastornos del sueño en estos pacientes es complejo. El adecuado control del insomnio y de la excesiva actividad nocturna que suelen producirse en las fases avanzadas de las demencias tiene una importante repercusión social. En este capítulo se resumen las características peculiares de los trastornos del sueño en los ancianos así como el abordaje diagnóstico y terapéutico de los trastornos del sueño en los pacientes con demencia
Sleep disorders are very frequent in the elderly. Bearing in mind the growth of this population group in western societies, it is of great importance to understand the sleep diseases that affect them and what their treatment should be. On the other hand, it is in this age group where we find the majority of patients with dementias. The treatment of sleep disorders in these patients is complex. An adequate control of insomnia and of the excessive nocturnal activity that usually occurs in the advanced phases of dementias has an important social repercussion. This article summarises the peculiar characteristics of sleep disorders in the elderly as well as the diagnostic and therapeutic handling of sleep disorders in patients with dementias
Subject(s)
Male , Female , Aged , Humans , Sleep Wake Disorders/physiopathology , Dementia/physiopathology , Hallucinations , Caregivers/psychology , REM Sleep Behavior Disorder , Alzheimer DiseaseABSTRACT
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Subject(s)
Aged, 80 and over , Female , Humans , Head/pathology , Myopathies, Nemaline/complications , Myopathies, Nemaline/pathology , Myopathies, Nemaline/physiopathology , Diagnosis, Differential , Muscle, Skeletal/pathology , Muscle, Skeletal/ultrastructure , SyndromeABSTRACT
Noninvasive mechanical ventilation through a nasal mask is a recently introduced therapeutic tool that represents a noteworthy advance in home treatment for patients with respiratory insufficiency secondary to ventilatory pump failure. We present the preliminary results of a program for early detection of respiratory insufficiency in patients with Duchenne's disease. Sixteen patients (mean age 15.8 years) with this disease were evaluated between January 1994 and January 1995. Mean lung function parameters were FVC 1,440 ml (46.7%), PO2 87.3 mmHg, PCO2 40.8 mmHg, PIM 40.1 cmH2O (30.6%), and PEM 41 cmH2O (25%). Two patients had abnormal pulse oximetry readings at night and abnormal gasometric readings during the day and were started on mechanical ventilation through nasal masks. These 2 patients were older, more hypoxemic and hypercapnic, had lower FVC values and showed greater deterioration of inspiratory and expiratory muscle pressures.
Subject(s)
Muscular Dystrophies/complications , Respiratory Insufficiency/diagnosis , Adolescent , Child , Home Care Services , Humans , Male , Masks , Muscular Dystrophies/therapy , Respiration, Artificial , Respiratory Function Tests/statistics & numerical data , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy , Time FactorsABSTRACT
OBJECTIVE: To determine the characteristics, family surrounding and place of death of terminal oncologic patients from 1991. DESIGN: Descriptive and retrospective study. We used Fisher's test. SITE. San Gregorio, Telde (Las Palmas province). PATIENTS: We worked with 27 patients in terminal disease (17 were men and 10 were women). MEASUREMENTS AND MAIN RESULTS: Average age was 66.7 +/- 17 years. The most frequent type was digestive (10), breast (5) and lung (3). Average time of survival was 17.5 +/- 26.5 months. Principal symptoms were: pain (23), anorexia (17), constipation (13) and depression (11). 23 received analgesia (11 with morphine), 17 laxatives and 13 benzodiazepines. The numbers of visit they received was 13.6 +/- 9.3 patients knew their diagnostic. 22 of the families were considered collaborators. 70% of patients in control by Primary Care Center died at home; whereas no one did so in control by hospital (p = 0.029). CONCLUSIONS: Half of the patients in terminal phase in our zone die in their home. Patients under control by Primary Care Center are significantly more likely to die in their own homes. We give special attention to the high percentage of patients who use opiates. Finally we consider that workers in Primary Care Centers are a vital element in handle these patients and their families.
