Teaching cardiopulmonary resuscitation using virtual reality: A randomized study.

BACKGROUND: The main functions of healthcare professionals include training and health education. In this sense, we must be able to incorporate new technologies and serious game to the teaching cardiopulmonary resuscitation. METHODS: a multicenter, comparative and cross-sectional study was carried out to assess the learning of resuscitation of a group that was trained with the use of serious gaming with virtual reality, as compared to a control group trained with conventional classroom teaching. RESULTS: the mean quality obtained in chest compressions for the virtual reality group was 86.1 % (SD 9.3), and 74.8 % (SD 9.5) for the control group [mean difference 11.3 % (95 % CI 6.6-16.0), p < 0.001]. Salivary Alpha-Amylase was 218.882 (SD 177.621) IU/L for the virtual reality group and 155.190 (SD 116.746) IU/L for the control group [mean difference 63.691 (95 % CI 122.998-4.385), p = 0.037]. CONCLUSION: using virtual reality and serious games can improve the quality parameters of chest compressions as compared to traditional training.

Prevalence, risk factors and evolution of diabetes mellitus after treatment in primary aldosteronism. Results from the SPAIN-ALDO registry.

PURPOSE: To evaluate the prevalence, risk factors and evolution of diabetes mellitus (DM) after targeted treatment in patients with primary aldosteronism (PA). METHODS: A retrospective multicenter study of PA patients in follow-up at 27 Spanish tertiary hospitals (SPAIN-ALDO Register). RESULTS: Overall, 646 patients with PA were included. At diagnosis, 21.2% (n = 137) had DM and 67% of them had HbA1c levels < 7%. In multivariate analysis, family history of DM (OR 4.00 [1.68-9.53]), the coexistence of dyslipidemia (OR 3.57 [1.51-8.43]) and advanced age (OR 1.04 per year of increase [1.00-1.09]) were identified as independent predictive factors of DM. Diabetic patients were on beta blockers (46.7% (n = 64) vs. 27.5% (n = 140), P < 0.001) and diuretics (51.1% (n = 70) vs. 33.2% (n = 169), p < 0.001) more frequently than non-diabetics. After a median follow-up of 22 months [IQR 7.5-63.0], 6.9% of patients developed DM, with no difference between those undergoing adrenalectomy and those treated medically (HR 1.07 [0.49-2.36], p = 0.866). There was also no significant difference in the evolution of glycemic control between DM patients who underwent surgery and those medically treated (p > 0.05). CONCLUSION: DM affects about one quarter of patients with PA and the risk factors for its development are common to those of the general population. Medical and surgical treatment provides similar benefit in glycemic control in patients with PA and DM.

Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert / Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease

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Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease. / Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert.

BACKGROUND AND OBJECTIVES: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. MATERIAL AND METHODS: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. RECOMMENDATIONS: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. CONCLUSION: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up.

A specific CBP/p300-dependent gene expression programme drives the metabolic remodelling in late stages of spermatogenesis.

Histone hyperacetylation is thought to drive the replacement of histones by transition proteins that occur in elongating spermatids (ElS) after a general shut down of transcription. The molecular machineries underlying this histone hyperacetylation remain still undefined. Here, we focused our attention on the role of Cbp and p300 in histone hyperacetylation and in the preceding late-gene transcriptional activity in ElS. A strategy was designed to partially deplete Cbp and p300 in ElS. These cells progressed normally through spermiogenesis and showed normal histone hyperacetylation and removal. However, a genome-wide transcriptomic analysis, performed in the round spermatids (RS) and ElS, revealed the existence of a gene regulatory circuit encompassing genes presenting high expression levels in pre-meiotic cells, undergoing a repressed state in spermatocytes and early post-meiotic cells, but becoming reactivated in ElS, just prior to the global shutdown of transcription. Interestingly, this group of genes was over-represented within the genes affected by Cbp/p300 knock down and were all involved in metabolic remodelling. This study revealed the occurrence of a tightly regulated Cbp/p300-dependent gene expression programme that drives a specific metabolic state both in progenitor spermatogenic cells and in late transcriptionally active spermatids and confirmed a special link between Cpb/p300 and cell metabolism programming previously shown in somatic cells.

Homocistinuria; curso clínico y tratamiento dietético; a propósito de dos casos / Clinical management of homocystinuria; case report and review of the literature

La homocistinuria es un error congénito del metabolismo de la metionina que conduce al acúmulo de metionina y de su principal metabolito, homocisteína, en plasma, orina y tejidos. El acúmulo de homocisteína posee toxicidad sobre los sistemas óseo (osteoporosis), ocular (luxación del cristalino), nervioso (convulsiones, alteraciones psiquiátricas) y vascular (accidentes cerebrovasculares, enfermedad cardiovascular). Presentamos 2 casos de homocistinuria en 2 pacientes hermanos y, a continuación, revisamos las estrategias terapéuticas disponibles (AU)

Homocystinuria is a congenital disorder of methyonine metabolism that leads to increased plasmatic, urinary and tissue deposits of methyonine and its main metabolite: homocysteine. Homocysteine deposits are toxic for the skeletal system (osteoporosis), the eyes (lens dislocation), central nervous system (seizures, psychiatric disorders) and also induce vascular damage (stroke and other cardiovascular events). This article reports two patients with homocystinuria in two siblings, followed by a concise review on the therapeutic strategies available for this disorder (AU)

[Molecular characterization of corona radiata cells from patients with diminished ovarian reserve]. / Profil d'expression des cellules de la corona radiata chez les patientes présentant une réserve ovarienne diminuée.

OBJECTIVES: Diminished ovarian reserve (DOR) is one of the causes of infertility. In this prospective study, gene expression profiling (GEP) of corona radiata cells (CRC) was performed to identify genes deregulated in DOR patients. PATIENTS AND METHODS: Microarray-based GEP of CRC isolated from eight women undergoing IVF was performed to identify genes differentially expressed between patients with normal ovarian reserve and DOR patients. Microfluidic-based quantitative RT-PCR assay were used to validate selected transcripts on 40 independent patients. RESULTS: Forty-eight transcripts were differentially expressed, including CXXC5 and FOXC1 down regulated in DOR, as well as CTGF, FSTL3, PTGS2 and SOCS2 up regulated in DOR. According to these transcripts, two DOR patients'subgroups (DOR Gr1 and Gr2) were identified. In DOR Gr2 patients, CITED2, CTGF, GAS-1, IRS2, PTGS2, SOCS2, VCAN were expressed at significantly higher levels, and CXXC5, FOXC1, GBP2 and ZMIZ1 at significantly lower level. Eleven of those genes are transcriptional targets of Estrogens and higher baseline oestradiol levels were observed in DOR Gr2 patients (P<0.006). DISCUSSION AND CONCLUSION: Twelve genes deregulated in CRC of DOR patients were identified, which could be involved in DOR pathogenesis. The distinction of a particular subgroup of DOR patients suggests the possibility of deregulation of estrogen response genes.

[Clinical management of homocystinuria: case report and review of the literature]. / Homocistinuria; curso clínico y tratamiento dietético: a propósito de dos casos.

Homocystinuria is a congenital disorder of methyonine metabolism that leads to increased plasmatic, urinary and tissue deposits of methyonine and its main metabolite: homocysteine. Homocysteine deposits are toxic for the skeletal system (osteoporosis), the eyes (lens dislocation), central nervous system (seizures, psychiatric disorders) and also induce vascular damage (stroke and other cardiovascular events). This article reports two patients with homocystinuria in two siblings, followed by a concise review on the therapeutic strategies available for this disorder.

Pituitary apoplexy in non-functioning pituitary adenomas: long term follow up is important because of significant numbers of tumour recurrences.

OBJECTIVES: The frequency of pituitary tumour regrowth after an episode of classical pituitary apoplexy is unknown. It is thus unclear whether regrowth, if it occurs, does so less frequently than with non-apoplectic non-functioning pituitary macroadenomas that have undergone surgery without postoperative irradiation. This has important repercussions on follow up protocols for these patients. DESIGN: Retrospective cohort study of patients diagnosed with classical pituitary apoplexy in Oxford in the last 24 years. MEASUREMENTS: MRI/CT scans of the pituitary were performed post-operatively and in those patients who did not receive pituitary irradiation, this was repeated yearly for 5 years and 2 yearly thereafter. RESULTS: Thirty-two patients with non-functioning pituitary adenomas who presented with classical pituitary apoplexy were studied. There were 23 men and the mean age was 56·6 years (range 29-85). The mean follow up period was 81 months (range 6-248). Five patients received adjuvant radiotherapy within 6 months of surgery and were excluded from further analysis. In this group, there were no recurrences during a mean follow up of 83 months (range 20-150). In the remaining 27 cases there were 3 recurrences, with a mean of 79 months follow up (range 6-248) occurring 12, 51 and 86 months after surgery. This gives a recurrence rate of 11·1% at a mean follow up of 6·6 years post surgery. All recurrences had residual tumour on the post operative scan. CONCLUSIONS: Patients with classical pituitary apoplexy may show recurrent pituitary tumour growth and therefore these patients need continued post-operative surveillance if they have not had post-operative radiotherapy.

A 10-year median follow-up study after allogeneic stem cell transplantation for chronic myeloid leukemia in chronic phase from HLA-identical sibling donors.

We report long-term outcome in 102 patients with cCML transplanted from an HLA-identical sibling donor from 1982 to 1998. The conditioning regimen was based on cyclophosphamide associated with either total body irradiation (TBI) (37 patients) or with busulfan (63 patients). Graft-versus-host disease (GvHD) prophylaxis consisted of cyclosporin and methotrexate in the majority of the patients. Fifteen year overall survival was estimated at 53% (95% confidence interval (CI), 44-65) with a plateau after 2.5 years. Long-term survival was adversely affected by: longer time from chronic myeloid leukemia (CML) diagnosis to transplantation, older age at time of transplantation and GvHD (acute grade III-IV or chronic extensive). The main cause of death was infection, related to GvHD in 69% of patients. Splenectomy also significantly increased the risk of bacterial infection. 15-year relapse was estimated at 8% (95% CI, 0.1-14). Late malignancies occurred in seven patients, four of whom had an invasive cancer. Other frequent late complications included cataracts, psychological depression, osteonecrosis and hypothyroidism. These complications were more frequent following splenectomy, TBI and in patients with chronic extensive GvHD. We conclude that allogeneic transplantation with a related donor can cure more than half of CML patients in chronic phase, although physicians should be alert to long-term complications.

[Health care workers' expectations: what features of health centers do they value most? A qualitative and quantitative study]. / Las expectativas de los profesionales: 'qué aspectos valoran en un centro de salud? Un estudio cuali-cuantitativo.

OBJECTIVES: To identify features of health care centers valued by health care workers as positive, to group features into dimensions, and to determine their relative importance. DESIGN: Qualitative phase: focus groups and content analysis. Quantitative phase: survey with a questionnaire developed from the features identified in the qualitative phase. SETTING: Primary care services in Reus and Tarragona (Catalonia, northeastern Spain). PARTICIPANTS: Managers, medical care providers and admissions staff. A total of 33 workers took part in focus groups, and 136 questionnaires were distributed for the survey, with a 78.6% response rate. MAIN MEASURES: Identification by focus groups of the features to be evaluated. Features were grouped into dimensions at different levels by content analysis. Survey to determine the relative importance of different features. RESULTS: We identified 133 features to be evaluated by workers: 36 related with structural features of the center (architecture, staffing and equipment), 33 with organization (accessibility, team functioning), 23 with workers (knowledge and attitudes) 20 with the services provided (needs and information management, care services provided) and 21 with management. The most highly valued dimensions were workers´ attitudes and management. CONCLUSIONS: Relations with patients and colleagues, and management issues, were valued most highly by workers. Some problematic features such as shared decision-making, team work and minority cultures revealed different levels of awareness and sensitivity within the health care system.

Allogeneic bone marrow transplantation for acute myeloblastic leukaemia in remission: risk factors for long-term morbidity and mortality.

In this single-centre retrospective study, we analysed risk factors for nonrelapse long-term morbidity and mortality in patients with acute myeloblastic leukaemia (AML) who had undergone allogeneic transplantation. A total of 112 patients with de novo AML in first complete remission (CR1), n=90 or second complete remission (CR2, n=22) who received un-manipulated bone marrow grafts from human leukocyte antigen identical siblings between January 1985 and August 2000 were included. Of these, 97 patients alive and disease-free for at least 100 days after transplant were selected for the purpose of this long-term analysis. The use of an intensified conditioning regimen, Gram-negative bacteriaemia before transplantation, year of transplantation and number of pretransplant chemotherapy courses for patients in CR1 significantly affected the 7-year event-free survival which was 57%. 7-year transplant-related mortality TRM was 22%. Significant predictors for TRM were: bacterial infections before transplantation, major ABO blood group incompatibility, late severe bacterial infections, and chronic (graft-versus-host disease) GvHD. Predictive factors for late severe bacterial infections were infections before transplant, total body irradiation and GvHD. Incidence and risk factors for other late events including, chronic GvHD, late infections, osteonecrosis, cataract, endocrine- cardiac- and lung-complications, cancer and performance status at last follow-up were also studied. The analysis strongly suggests that the combination of pretransplant factors such as chemotherapy and conditioning, and posttransplant factors such as chronic GvHD had a major impact on late nonrelapse morbidity and mortality.

[Expectations of patients: what aspects of a health centre do they value? A qualitative-quantitative study]. / Las expectativas de los pacientes: ¿qué aspectos valoran en un centro de salud? Un estudio cualicuantitativo.

OBJECTIVES: To identify the factors valued by users of health centres; to weigh the relative importance of each factor. DESIGN: Qualitative stage (4 focus groups) to identify the factors valued. Quantitative stage (questionnaire to 225 people) to weigh their relative importance. SETTING: Primary care. PARTICIPANTS: Citizens from middle-high and middle-low social classes, urban, rural and over 65, were chosen through key informants for their interest in the health services. They were recruited with the assistance of various residents' associations and town councils. METHOD: The factors valued were identified through focus groups and classified in categories. Their relative importance was weighed through a questionnaire and a factorial analysis to identify the main components was run. RESULTS: 60 factors that could be valued by patients were identified. Eight of these referred to the centre and concrete assets, nine to organisation and acessibility, 18 to relationship with the health professionals, and 25 to the services available. The most highly valued factor was: "The centre has sufficient material available for cures, minor surgery, bandages, etc." The factorial analysis confirmed the categories established. Organisation and accessibility, and relationship with professionals were the most highly valued dimensions. CONCLUSIONS: The combination of qualitative and quantitative methods seems very fitting for this kind of study. Although many of the factors were to be expected, other little-expected ones emerged. In addition, users seem to value certain factors in a different way from how the professionals do.

Las expectativas de los pacientes: ¿qué aspectos valoran en un centro de salud? Un estudio cualicuantitativo / Expectations of patients: what aspects of a health centre do they value? A qualitative-quantitative study

Objetivos. Identificar los aspectos que valoran los pacientes en los centros de salud. Ponderar la importancia relativa de cada uno de ellos. Diseño. Fase cualitativa (4 grupos focales) para identificar los aspectos que se valoran. Fase cuantitativa (encuesta a 225 ciudadanos) para ponderar su importancia relativa. Emplazamiento. Atención primaria. Participantes. Ciudadanos de clase social media-alta, media-baja, urbanos, rurales y mayores de 65 años, seleccionados a través de informantes clave por su interés en los servicios sanitarios. Se reclutaron con la colaboración de diferentes asociaciones de vecinos y ayuntamientos. Método. Mediante grupos focales se identificaron los aspectos que se valoran, y se clasificaron en categorías. Mediante encuesta se ponderó la importancia relativa de los mismos y se realizó un análisis factorial para identificar los componentes principales. Resultados. Se identificaron 60 aspectos valorables por los pacientes. De ellos, 8 se referían al centro y eran tangibles, 9 a organización y accesibilidad, 18 a relación con los profesionales y 25 a servicios disponibles. El aspecto más valorado fue: "el centro dispone de suficiente material para curas, pequeña cirugía, vendajes, etc.". El análisis factorial confirmó las categorías que se habían establecido. La organización y accesibilidad y las relaciones con los profesionales parecen ser las dimensiones más valoradas. Conclusiones. La combinación de métodos cualitativos y cuantitativos parece muy adecuada para este tipo de estudios. Aunque muchos de los aspectos eran de esperar, aparecen otros poco previsibles. Además, los clientes parecen valorar ciertos aspectos de manera distinta a como lo harían los profesionales (AU)

Second malignancies after allogeneic hematopoietic stem cell transplantation: new insight and current problems.

With increased number of patients surviving on the long term, late effect after allogeneic hematopoietic stem cell transplantation have become of major clinical importance. Among these late effect, second malignancies have increasingly been recognized in the recent years. It has been usual to divide the problem of secondary malignancies following hematopoietic stem cell transplantation into three groups, i.e. leukemia, lymphoma and solid tumors. Recent clinical and biological data on these three types of malignancies, occurring after allogeneic stem cell transplantation, are summarized in this review. We will focus here only on second malignancies after allogeneic stem cell transplantation with particular emphasis on recent development on the pathogenesis, and early diagnosis, and treatment of these transplant-related complications.

Prolonged immune deficiency following allogeneic stem cell transplantation: risk factors and complications in adult patients.

To evaluate the long-term immune reconstitution after allogeneic haematopoietic stem cell transplantation (SCT), we prospectively screened standard immune parameters in a series of 105 patients, at a median time of 15 months after SCT. Analysing lymphoid phenotypes, in vitro immune functions and immunoglobulin levels, we found that, more than 1 year post SCT, cellular and humoral immunity was still altered in a significant number of patients. CD4+ T cells were < 200/microl in one third of patients, and the CD4/CD8 ratio was still reversed in 78% of patients. Almost all patients showed positive T-cell responses against mitogens, but antigen-specific proliferation assays identified 20% to 80% of non-responders. B-cell counts were reconstituted in 61% of the patients, but levels of total immunoglobulins were still low in 59%. In multivariate analyses, human leucocyte antigen (HLA) disparity between donor and recipient and chronic graft-versus-host disease were the leading causes affecting immune reconstitution. Interestingly, cytomegalovirus (CMV) infections were strongly associated with normal CD8+ T-cell counts. Studying the impact of impaired immune reconstitution on the rate of infections occurring in the 6 years following screening, we identified three parameters (low B-cell count, inverted CD4/CD8 ratio, and negative response to tetanus toxin) as significant risk factors for developing such late infections.

Myeloablation and autologous peripheral blood stem cell rescue results in hematologic and clinical responses in patients with myeloid metaplasia with myelofibrosis.

Current therapeutic options for myeloid metaplasia with myelofibrosis (MMM) are limited. A pilot study was conducted of autologous peripheral blood stem cell (PBSC) collection in 27, followed by transplantation in 21 patients with MMM. The median age was 59 (range 45-75) years. PBSCs were mobilized at steady state (n = 2), after granulocyte colony-stimulating factor (G-CSF) alone (n = 17), or after anthracycline-cytarabine induction plus G-CSF (n = 8). A median of 11.6 x 10(6) (range 0 to 410 x 10(6)) CD34(+) cells per kilogram were collected. Twenty-one patients then underwent myeloablation with oral busulfan (16 mg/kg) and PBSC transplantation. The median times to neutrophil and platelet recovery after transplantation were 21 (range 10-96) and 21 (range, 13 to > or = 246) days, respectively. Five patients received back-up PBSC infusion because of delayed neutrophil or platelet recovery. The median follow-up is 390 (range 70-1623) days after transplantation, and the 2-year actuarial survival is 61%. After transplantion, 6 patients died: 3 of nonrelapse causes (1 within 100 days of PBSC infusion) and 3 of disease progression. Erythroid response (hemoglobin > or = 100 g/L [10 gm/dL] without transfusion for > or = 8 weeks) occurred in 10 of 17 anemic patients. Four of 8 patients with a platelet count less than 100 x 10(9)/L (100 000/microL) responded with a durable platelet count more than 100 x 10(9)/L (100 000/microL). Symptomatic splenomegaly improved in 7 of 10 patients. It is concluded that (1) PBSC collection was feasible and stable engraftment occurred after transplantation in most patients with MMM, (2) myeloablation with busulfan was associated with acceptable toxicity, (3) a significant proportion of patients derived clinical benefit after treatment, and (4) further investigation of this novel approach is warranted. (Blood. 2001;98:586-593)