Satisfaction in the Intensive Care Unit (ICU). Patient opinion as a cornerstone. / Satisfacción en la Unidad de Cuidados Intensivos (UCI): la opinión del paciente como piedra angular.

OBJECTIVE: To study the agreement between the level of satisfaction of patients and their families referred to the care and attention received during admission to the ICU. DESIGN: A prospective, 5-month observational and descriptive study was carried out. SETTING: ICU of Marqués de Valdecilla University Hospital, Santander (Spain). SUBJECTS: Adult patients with an ICU stay longer than 24h, who were discharged to the ward during the period of the study, and their relatives. INTERVENTION: Instrument: FS-ICU 34 for assessing family satisfaction, and an adaptation of the FS-ICU 34 for patients. The Cohen kappa index was calculated to assess agreement between answers. RESULTS: An analysis was made of the questionnaires from one same family unit, obtaining 148 pairs of surveys (296 questionnaires). The kappa index ranged between 0.278-0.558, which is indicative of mild to moderate agreement. CONCLUSIONS: The families of patients admitted to the ICU cannot be regarded as good proxies, at least for competent patients. In such cases, we must refer to these patients in order to obtain first hand information on their feelings, perceptions and experiences during admission to the ICU. Only when patients are unable to actively participate in the care process should their relatives be consulted.

Angioimmunoblastic T-cell lymphoma with a clonal plasma cell proliferation that underwent immunoglobulin isotype switch in the skin, coinciding with cutaneous disease progression.

Plasma cell proliferations in specific cutaneous lesions of angioimmunoblastic T-cell lymphoma(AITL) are very uncommon. Here, we report a case of clonal plasma cell proliferation in skin with heavy-chain-immunoglobulin-isotype-switch after cutaneous disease progression. Histopathologically, initial plaque lesions were suggestive of marginal-zone B-cell-lymphoma. Nevertheless, this 77-year-old lady was diagnosed with AITL after the progression of skin lesions from plaques to nodular tumors. A lymph node biopsy confirmed the diagnosis. Both cutaneous specimens showed a polymorphic cellular infiltrate with atypical T-cell-lymphocytes arranged in a pseudonodular pattern that expressed CD3, PD1 and BCL6, with patchy expression of CD30. Interestingly, a slight IgG-Lambda plasma cell component was seen at the periphery of the infiltrate in the first specimen which increased in number in the later nodular lesion, showing not only Lambda light chain restriction and IgG but also IgG4. PCR studies for IgH and TCR genes showed an IgH clonal peak on both skin lesions but not on lymph node biopsy. On the contrary, the same clonal TCR peak was found in the three specimens. Neoplastic follicular helper T-cells within cutaneous-specific microenvironment could be responsible for the modulation of the immunoglobulin isotype class switch change. Further studies are needed to support this hypothesis.

[Measuring the satisfaction of patients admitted to the intensive care unit and of their families]. / Medición de la satisfacción de los pacientes ingresados en unidad de cuidados intensivos y sus familiares.

OBJECTIVE: To determine the level of satisfaction of family members with the care and decision making process, and to know the level of satisfaction of patients discharged from ICU. DESIGN: A prospective, observational and descriptive study with a duration of 5 months was carried out. SETTING: The ICU of Marqués de Valdecilla University Hospital, Santander (Spain). SUBJECTS: Family members of adult patients admitted to the ICU and patients discharged to the ward. INSTRUMENT: Family Satisfaction Intensive Care Survey (FS-ICU 34) of family members of patients discharged to the ward. We adapted the FS-ICU 34 in relation to care for application to the patients. RESULTS: A total of 385 questionnaires were obtained: 192 from families of survivors and 162 from patients, and 31 from relatives of non-survivors. The majority of relatives were satisfied with overall care and overall decision making (survivors: 83.46 ± 11.83 and 79.42 ± 13.58, respectively; non-survivors: 80.41 ± 17.27 and 79.61 ± 16.93, respectively). Patients were very satisfied with the care received (84.71 ± 12.85). CONCLUSIONS: The level of satisfaction of the relatives of patients admitted to the ICU is high, in the same way as the degree of patient satisfaction. Still, there are several points that should be improved, such as the waiting room environment and the atmosphere of the ICU in terms of noise, privacy and lighting. In relation to the decision making process, there are also some aspects that may be improved, such as the provision of hope regarding recovery of the critically ill relative.

A polymorphism in the regulatory region of APOE associated with risk for Alzheimer's dementia.

The epsilon4 allele of the apolipoprotein E gene (APOE) has been associated with an increased risk of developing Alzheimer's disease (AD; refs 1,2). However, it is apparent that the APOEepsilon4 allele alone is neither necessary nor sufficient to cause the disease. We have recently found three new polymorphisms within the APOE transcriptional regulatory region (M.J.A. et al., manuscript submitted) and now establish an association between one of these polymorphisms (-491A/T) and dementia as observed in Alzheimer's disease, in two independent clinical populations. The results suggest that homozygosity of a common variant (-491A) is associated with increased risk for AD, and that this association is independent of APOEepsilon4 status. In vitro studies suggest that the -491A/T polymorphism may increase risk for AD by altering the level of ApoE protein expression.

[EICS (Extended Intensive Care Service): looking outside the ICU]. / SECI (Servicio Extendido de Cuidados Intensivos): mirando fuera de la UCI.

Early warning systems (EWS) identify patients at risk with a view to improving morbidity and mortality rates using early therapeutic and transfer actions. We have recently implemented an EWS that focuses on two main aspects: the guidance of care after discharge from the ICU, and recognition of the onset of deteriorating health among adult patients in general wards through physiologically based early warning scores.

Allelic polymorphisms in the transcriptional regulatory region of apolipoprotein E gene.

In this work, we explored the existence of genetic variants within the apolipoprotein E gene transcriptional regulatory region, using a denaturing gradient gel electrophoresis screening of a region comprising nucleotides -1017 to +406. Upon a population study, three new polymorphic sites (-491, -427 and -219) and two mutations were found. Functional effects of the polymorphisms, assayed by transient transfection and electrophoretic mobility shift assays in a human hepatoma cell line, showed that polymorphisms at sites -491 and -219 of the APOE promoter produce variations in the transcriptional activity of the gene, most probably through differential binding of nuclear proteins.

Contribution of APOE promoter polymorphisms to Alzheimer's disease risk.

OBJECTIVE: To determine whether the effects of APOE promoter polymorphisms on AD are independent of the APOE-epsilon4 allele. BACKGROUND: Recently, the -491 A-->T and -219 G-->T polymorphisms located in the APOE promoter have been suggested to be risk factors for AD. However, the effects of these polymorphisms have not always been reproduced in case-control studies, possibly because of the strong linkage disequilibrium existing at this locus or the characteristics of the populations studied. METHODS: Data collection was performed from six independent samples (1,732 patients with AD and 1,926 control subjects) genotyped for APOE exon 4 and the two APOE promoter polymorphisms. The risks associated with the APOE polymorphisms for developing AD were estimated using logistic regression procedures and calculation of odds ratios with 95% CI adjusted by age, sex, and collection center. Independence of the APOE promoter polymorphisms was tested by stratification for APOE-epsilon4 and tertile design was used for age stratification. RESULTS: The independence of the -491 AA genotype was observed in the whole sample whereas the independence of the -219 TT genotype was observed only in the oldest population. CONCLUSION: The -491 and -219 APOE promoter polymorphisms incur risk for AD in addition to risk associated with the APOE-epsilon4 allele, with age accentuating the effect of the -219 TT genotype. Because these polymorphisms appear to influence apoE levels, these results suggest that APOE expression is an important determinant of AD pathogenesis.

The -491 A/T polymorphism in the regulatory region of the apolipoprotein E gene and early-onset Alzheimer's disease.

The -491 polymorphism in the promoter region of the apolipoprotein E gene (APOE) has been suggested to be associated with increased risk for Alzheimer's disease (AD) independent of APOE status. We studied the association between the -491 polymorphism and risk for early-onset Alzheimer's disease in 99 Dutch and 78 Spanish patients. In patients with early-onset AD, we found no consistent relationship with a single allele of the -491 polymorphism. Linkage disequilibrium between the polymorphism and the APOE gene was found which most likely might explain the inconsistent findings.

Alzheimer's risk associated with human apolipoprotein E, alpha-2 macroglobulin and lipoprotein receptor related protein polymorphisms: absence of genetic interactions, and modulation by gender.

Apolipoprotein E (apoE), the lipoprotein receptor related protein (LRP) and alpha-2 macroglobulin (alpha2M) have been proposed as a functional complex involved in amyloid clearance, a crucial event for Alzheimer's disease development. In this work, we present an epidemiological approach aimed to study the interactions among these genes, age and gender. This approach did not reveal significant associations between the genes; however, the present study indicated that the risk associated with APOE promoter and LRP gene polymorphisms is modulated by gender.

Apolipoprotein E promoter and alpha2-macroglobulin polymorphisms are not genetically associated with Chinese late onset Alzheimer's disease.

In this study, we investigated two newly reported polymorphisms in association with late onset Alzheimer's disease (AD) in Chinese. They were a -491 A/T polymorphism in the Apolipoprotein E (APOE) promoter region and a five base pair deletion at exon 18 of alpha2-Macroglobin (A2M). There were 196 AD and 180 normal controls (N), which were age- and sex-matched. APOE epsilon4 alleles were significantly increased in AD vs. N (chi2 = 33.3, P < 0.000001). However, neither the -491 A/T (chi2 = 1.13, P = 0.29) nor A2M (chi2 = 0.18, P = 0.67) polymorphism was associated with AD risk, suggesting that these polymorphisms do not represent risk factors for AD in the Chinese population.

[Varicella pneumonia in adults: 30 cases]. / Neumonía varicelosa en adultos: 30 casos.

OBJECTIVES: During the past 10 years, 30 adults (age > 15 years) were treated for varicella pneumonia in our centre. METHODS: There were 16 males and 14 females. Ages ranged from 15 to 58 years (mean, 32.73+/-7.67 years). Twenty-seven patients (90%) were non-pregnant adult smokers and three patients (10%) were pregnant women. The hospital stay ranged from 4 to 57 days (mean, 14.96+/-12.02 days). RESULTS: Seven patients (23.3%) were managed in the intensive care unit and two patients (6,6%) required mechanical ventilation. The most common radiographic findings were interstitial infiltrates in twenty-one patients (70%) and interstitial-alveolar infiltrates in seven patients (23.3%). Physical examination of the chest did not reveal abnormalities in twenty patients (66,6%). Fifteen patients (50%) were severely hipoxic with pO2/FiO2 ratio less than 300. Twelve patients (40%) presented thrombocytopenia and fifteen (50%) presented hyponatremia. The most frequent clinical features included: fever (100%), dry cough (86.6%), dysnea (66.6%) and chest pain (50%). One patient (3.3%) died. Three patients (10%) developed asthma and one other patient developed pulmonary fibrosis. CONCLUSIONS: Smoking is associated with an increase incidence of varicella pneumonia in adults. A chest x-ray should be practised in all cases of varicella in adults and they all should also be admitted to hospital. Intravenous aciclovir is recommended for treatment of varicella pneumonia in adults and in seriously ill patients the association of corticosteroids should be considered.

Risk for Alzheimer's disease correlates with transcriptional activity of the APOE gene.

While the straightepsilon4 allele of apolipoprotein E ( APOE, gene; ApoE, protein) is widely accepted as a major genetic risk factor for the late onset form of Alzheimer's disease (AD), recent evidence points to variations in ApoE levels as another important factor. We have previously reported that a common variant in the regulatory region of APOE (-491A) is associated with risk for late onset AD. In this report we analyze the association of another APOE promoter polymorphism (-427T/C) with AD in two case-control clinical samples and demonstrate a correlation between APOE promoter transcriptional activity and risk for AD. The association studies show that the allelic variant (-427C) and the haplotype [-491A-427C] of the APOE promoter are associated with increased risk for AD. Study of the transcriptional activity of the common haplotypes defined by combination of the -491 and -427 alleles indicated that the risk for late onset AD positively correlates with transcriptional activity of the APOE gene, suggesting that increases in the local expression of ApoE could be responsible for the association of APOE promoter polymorphism with AD.

Pathologic gambling in Parkinson's disease: a behavioral manifestation of pharmacologic treatment?

We describe 12 patients with Parkinson's disease and pathologic gambling. This association has apparently never been reported. The patients were selected from a Parkinson's disease unit of 250 patients. They met Diagnostic and Statistical Manual of Mental Disorders, 4th edition, criteria for pathologic gambling. All patients underwent a neurologic, psychiatric, and psychologic examination, specifically noting the presence or absence of psychopathology in the spectrum of impulse control disorder and the nature of the gambling. Ten patients started gambling after the onset of Parkinson's disease and treatment with levodopa. The pathologic behavior was exclusively present or was markedly increased in "on" periods in 11 patients. All patients had motor fluctuations at the time of the study. Slot machines were the preferred source of gambling for 10 patients, similar to the Spanish gambling population. That the gambling behavior appears more often in the "on" periods of motor fluctuations and that it begins after the onset of Parkinson's disease in most patients and worsens with levodopa therapy suggest that it could be related to the dopaminergic tone in patients with Parkinson's disease and motor fluctuations (that is, it could represent a behavioral manifestation of pharmacologic treatment).